Neurological and neuropsychological outcome after resection of craniopharyngiomas.
Giese Henrik,Haenig Benjamin,Haenig Anna,Unterberg Andreas,Zweckberger Klaus
Journal of neurosurgery
OBJECTIVE:Craniopharyngiomas are rare and benign tumors of the sellar and/or parasellar region. Primary treatment involves resection followed by adjuvant radiotherapy. While the grade of resection was frequently analyzed following surgery, the neurological outcome and especially neuropsychological deficits and quality of life have been neglected for many decades. Therefore, the authors retrospectively analyzed their patient series and prospectively assessed neuropsychological outcome and quality of life following resection of craniopharyngiomas in adults. METHODS:In total, 71 patients (39 men and 32 women) with a mean age of 49 years were enrolled in the retrospective analysis. In addition, 36 of the 71 patients were included in the prospective arm of the study and underwent neurological and neuropsychological testing as well as quality of life (36-Item Short-Form Health Survey; SF-36) assessment. Factors influencing outcome were identified and correlations calculated. RESULTS:Resection was performed mostly using a pterional (41.6%, 47/113 surgical procedures) or bifrontal translamina terminalis (30.1%, 34/113 surgical procedures) approach. Following surgery, visual acuity was significantly improved (> 0.2 diopters) in 32.4% (23/71) of patients, or remained stable in 45.1% (32/71) of patients. During long-term follow up, 80.3% (57/71) of patients developed pituitary insufficiency, particularly involving the corticotropic and thyrotrophic axes. In total, 75% (27/36) of patients showed neuropsychological deviations in at least 1 test item. In particular, attentiveness, cognitive speed, and short-term memory were affected. Referring to the SF-36 score, quality of life was affected in both the mental and physical score in 19.4% (7/36) and 33.3% (12/36), respectively. The risk factors that were identified were a tumor volume larger than 9 cm3, tumor extension toward/into the third ventricle or the brainstem, and resection using a bifrontal translamina terminalis or left-sided approach. CONCLUSIONS:This study demonstrated that resection of craniopharyngiomas is frequently associated with postoperative neuropsychological deficits and hence an impaired quality of life. In addition to tumor size and extension toward/into the third ventricle or the brainstem, selection of the surgical approach may play a crucial role in the patient's neuropsychological outcome and quality of life.
The endoscopic endonasal approach for the management of craniopharyngiomas: a series of 103 patients.
Cavallo Luigi Maria,Frank Giorgio,Cappabianca Paolo,Solari Domenico,Mazzatenta Diego,Villa Alessandro,Zoli Matteo,D'Enza Alfonso Iodice,Esposito Felice,Pasquini Ernesto
Journal of neurosurgery
OBJECT:Despite their benign histological appearance, craniopharyngiomas can be considered a challenge for the neurosurgeon and a possible source of poor prognosis for the patient. With the widespread use of the endoscope in endonasal surgery, this route has been proposed over the past decade as an alternative technique for the removal of craniopharyngiomas. METHODS:The authors retrospectively analyzed data from a series of 103 patients who underwent the endoscopic endonasal approach at two institutions (Division of Neurosurgery of the Università degli Studi di Napoli Federico II, Naples, Italy, and Division of Neurosurgery of the Bellaria Hospital, Bologna, Italy), between January 1997 and December 2012, for the removal of infra- and/or supradiaphragmatic craniopharyngiomas. Twenty-nine patients (28.2%) had previously been surgically treated. RESULTS:The authors achieved overall gross-total removal in 68.9% of the cases: 78.9% in purely infradiaphragmatic lesions and 66.3% in lesions involving the supradiaphragmatic space. Among lesions previously treated surgically, the gross-total removal rate was 62.1%. The overall improvement rate in visual disturbances was 74.7%, whereas worsening occurred in 2.5%. No new postoperative defect was noted. Worsening of the anterior pituitary function was reported in 46.2% of patients overall, and there were 38 new cases (48.1% of 79) of postoperative diabetes insipidus. The most common complication was postoperative CSF leakage; the overall rate was 14.6%, and it diminished to 4% in the last 25 procedures, thanks to improvement in reconstruction techniques. The mortality rate was 1.9%, with a mean follow-up duration of 48 months (range 3-246 months). CONCLUSIONS:The endoscopic endonasal approach has become a valid surgical technique for the management of craniopharyngiomas. It provides an excellent corridor to infra- and supradiaphragmatic midline craniopharyngiomas, including the management of lesions extending into the third ventricle chamber. Even though indications for this approach are rigorously lesion based, the data in this study confirm its effectiveness in a large patient series.
Intraventricular craniopharyngiomas: surgical management and outcome analyses in 24 cases.
Yu Tao,Sun Xingwen,Ren Xiaohui,Cui Xiangli,Wang Junmei,Lin Song
OBJECTIVE:Pure intraventricular craniopharyngioma is a rare subtype of craniopharyngioma that attaches frequently to the hypothalamus. The main challenge in tumor removal is protection of hypothalamic structure. The aim of our study was to set up a feasible risk-evaluation approach to help the surgeons make an individual treatment plan. METHODS:We reviewed retrospectively 24 patients with pure intraventricular craniopharyngioma who underwent surgical therapy. Third ventricular deformation and thalamic attachment of the tumor were assessed by preoperative magnetic resonance imaging and intraoperative inspection. Correlations between the outcome, extent of removal, and different attachment grades were analyzed. RESULTS:Preoperative magnetic resonance imaging grade had a significant correlation with functional outcome. Attachment grade was significantly correlated with endocrine, functional, and radiologic outcome. In the gross total removal (GTR) group, significantly more newly developed endocrinologic deficits were observed compared with patients who underwent subtotal removal (STR). All 6 GTR cases with the greatest attachment grade (grade 2) acquired newly developed endocrine insufficiency. CONCLUSIONS:Attachment and deformation grade evaluated based on our criteria are significantly correlated with postoperative outcome. GTR of tightly attached tumor is associated with worse endocrinologic, functional, and radiologic outcomes compared with STR. Hence, we suggest that individual surgical plans should be made according to the grade of tumor attachment and hypothalamic deformation. STR should used in tumors with a high grade to achieve a good long-term outcome and avoid severe postoperative sequelae.
Extended Endoscopic Endonasal Approach for Suprasellar Craniopharyngioma.
Locatelli D,Pozzi F,Agresta G,Padovan S,Karligkiotis A,Castelnuovo P
Journal of neurological surgery. Part B, Skull base
We illustrate a suprasellar craniopharyngiomas treated with an extended endoscopic endonasal approach (EEEA). Case report of a 43-year-old male affected by cerebral lesion located in suprasellar region involving the third ventricle and compressing the neurovascular structures, causing an anterosuperior dislocation of the chiasma. There is a complete disruption of the pituitary stalk that can explain the clinical finding of partial anterior hypopituitarism and hyperprolactinemia. The lesion is characterized by a solid and cystic component. Considering the absence of lateral extension and the suprasellar location of the lesion, an EEEA is preferred. University Hospital "Ospedale di Circolo," Department of Neurosurgery, Varese, Italy. Neurosurgical and ENT Skull Base Team. A bilateral parasagittal approach is performed using a four-hand technique. The first step of the surgery is the preparation of the Hadad's flap. The approach is extended to the planum sphenoidalis to expose the suprasellar region. The lesion is completely removed employing also an ultrasound aspirator. Skull base reconstruction is performed with three-layer technique: graft of fat tissue, fascia lata, and nasoseptal flap. No postoperative complications occurred. In the post-op, the patient presents a panhypopituitarism and an improvement in neurological status. The visual deficit remains stable. Post-op magnetic resonance imaging at 1 year documents the complete absence of pathological contrast enhancement. EEEA is a feasible approach in treating craniopharyngioma with suprasellar extension. The advantages include optimal visualization, good resection rate, and absence of brain retraction. The link to the video can be found at: https://youtu.be/IYm-8P1jbBo .
Is the chiasm-pituitary corridor size important for achieving gross-total resection during endonasal endoscopic resection of craniopharyngiomas?
Omay Sacit Bulent,Almeida João Paulo,Chen Yu-Ning,Shetty Sathwik R,Liang Buqing,Ni Shilei,Anand Vijay K,Schwartz Theodore H
Journal of neurosurgery
OBJECTIVE Craniopharyngiomas arise from the pituitary stalk, and in adults they are generally located posterior to the chiasm extending up into the third ventricle. The extended endonasal approach (EEA) can provide an ideal corridor between the bottom of the optic chiasm and the top of the pituitary gland (chiasm-pituitary corridor [CPC]) for their removal. A narrow CPC in patients with a prefixed chiasm and a large tumor extending up and behind the chiasm has been considered a contraindication to EEA, with a high risk of visual deterioration and subtotal resection. METHODS A database of all patients treated in the authors' center (Weill Cornell Medical College, NewYork-Presbyterian Hospital) between July 2004 and August 2016 was reviewed. Patients with craniopharyngiomas who underwent EEA with the goal of gross-total resection (GTR) were included in the study. Patients with postfixed chiasm or limited available preoperative imaging were excluded. Using preoperative contrast-enhanced T1-weighted sagittal midline MR images, the authors calculated the CPC as well as the distance from the chiasm to the top of the tumor (CTOT). From these numbers, they calculated a ratio of the CPC to the CTOT as a measure of difficulty in removing the tumors through the EEA and called this ratio the corridor index (CI). The relationship between the CI and the ability to achieve GTR and visual outcome were measured. RESULTS Thirty-four patients were included in the study. The mean CPC was 10.1 mm (range 5.2-19.1 mm). The mean CTOT was 12.8 mm (range 0-28.3 mm). The median CI was 0.8; the CI ranged from 0.4 to infinity (for tumors with a CTOT of 0). Thirty-two patients had GTR (94.1%) and 2 had subtotal resection. The CPC value had no relationship with our ability to achieve GTR and no effect on visual or endocrine outcome. CONCLUSIONS EEA for craniopharyngioma is generally considered the first-line surgical approach. Although a narrow corridor between the top of the pituitary gland and the bottom of the chiasm may seem to be a relative contraindication to surgery for larger tumors, the authors' data do not bear this out. EEA appears to be a successful technique for the majority of midline craniopharyngiomas.
CRANIOPHARYNGIOMA - CLINICAL AND THERAPEUTIC OUTCOME DATA IN A MIXED COHORT OF ADULT AND PAEDIATRIC CASES.
Capatina C,Vintila M,Gherlan I,Dumitraşcu A,Caragheorgheopol A,Procopiuc C,Ciubotaru V,Poiana C
Acta endocrinologica (Bucharest, Romania : 2005)
Background:Craniopharyngiomas are benign but locally invasive tumours of the sellar region that arise from ectopic embryonic remnants of Rathke's pouch, affecting both children (adamantinomatous type -aCP) and adults (papillary type -pCP) and associated with significant morbidity. Objective:To study the clinical presentation of CRF as well as the posttreatment evolution of craniopharyngioma in children adults in a large mixed cohort. Material and methods:We performed a retrospective review of CRF patients evaluated in the National Institute of Endocrinology in Bucharest between 1990 and 2016. Results:A total of 107 patients (72 adults, 35 children) with a mean follow-up of 6.2 years were included. The presenting symptoms were mostly headache, visual impairment, symptoms of hypopituitarism, diabetes insipidus. Some symptoms or hormonal abnormalities were significantly more prevalent in the children group (p<0.05): nausea/ vomiting (47.8% vs 16.7%), photophobia (21.7% vs 5.6%), diabetes insipidus(28.5% vs 8.3%), GH deficiency (68.8% vs 17.1%). Impaired visual acuity (67.6%of cases) or visual fields (71.4%) were more frequent in adults compared to children (44.1%; 51.6%). The tumor dimensions were similar in both groups (3.05± 1.05 cm in children; 2.7± 1.07 cm in adults). Massive suprasellar extension reaching the third ventricle was frequently present in all cases. All cases underwent surgery but only a minority of those not cured received postoperative adjuvant radiotherapy. Frequent postoperative complications were: aggravation of the endocrine deficit (>80% of cases in both groups needed chronic replacement therapy), central diabetes insipidus (68.2% children, 34.3% of adults). Conclusions:Despite similar tumor dimensions and extension compared to adults, craniopharyngioma in children is more frequently associated with signs of intracranial pressure. The results and complications of treatment are similar in adults and children.
Growth patterns of craniopharyngiomas: clinical analysis of 226 patients.
Pan Jun,Qi Songtao,Liu Yi,Lu Yuntao,Peng Junxiang,Zhang XiAn,Xu YiKai,Huang Guang-Long,Fan Jun
Journal of neurosurgery. Pediatrics
OBJECT Craniopharyngiomas (CPs) are rare epithelial tumors that are often associated with an enigmatic and unpredictable growth pattern. Understanding the growth patterns of these tumors has a direct impact on surgical planning and may enhance the safety of radical tumor removal. The aim of this study was to analyze the growth patterns and surgical treatment of CPs with a focus on the involvement of the hypothalamopituitary axis and the relationship of the tumor to the arachnoid membrane and surrounding structures. METHODS Clinical data from 226 consecutive patients with primary CP were retrospectively reviewed. Tumor location and the relationship of the tumor to the third ventricle floor and the pituitary stalk were evaluated using preoperative MRI and intraoperative findings. A topographic classification scheme was proposed based on the site of tumor origin and tumor development. The clinical relevance of this classification on patient presentation and outcomes was also analyzed. RESULTS The growth of CPs can be broadly divided into 3 groups based on the site of tumor origin and on tumor-meningeal relationships: Group I, infrasellar/infradiaphragmatic CPs (Id-CPs), which mainly occurred in children; Group II, suprasellar subarachnoid extraventricular CPs (Sa-CPs), which were mainly observed in adults and rarely occurred in children; and Group III, suprasellar subpial ventricular CPs (Sp-CPs), which commonly occurred in both adults and children. Tumors in each group may develop complex growth patterns during vertical expansion along the pituitary stalk. Tumor growth patterns were closely related to both clinical presentation and outcomes. Patients with Sp-CPs had more prevalent weight gain than patients with Id-CPs or Sa-CPs; the rates of significant weight gain were 41.7% for children and 16.7% for adults with Sp-CPs, 2.2% and 7.1% for those with Id-CPs, and 12.5% and 2.6% for those with Sa-CPs (p < 0.001). Moreover, patients with Sp-CPs had increased hypothalamic dysfunction after radical removal; 39% of patients with Sp-CPs, 14.5% with Id-CPs, and 17.4% with Sa-CPs had high-grade hypothalamic dysfunction in the first 2 postoperative years (p < 0.001). CONCLUSIONS The classification of CPs based on growth pattern may elucidate the best course of treatment for this formidable tumor. More tailored, individualized surgical strategies based on tumor growth patterns are mandatory to provide long-term tumor control and to minimize damage to hypothalamic structures. Differences in the distribution of growth patterns between children and adults imply that hierarchical comparison is necessary when investigating outcomes and survival across treatment paradigms in patients with CP.
Endoscopic endonasal transsphenoidal approach for pediatric craniopharyngiomas: A case series.
Schelini Juliana Carolina,Cavalheiro Sergio,Dastoli Patrícia Alessandra,Hirai Élcio Roldan,Atallah Camila,Costa Marcos,Nicacio Jardel,Capellano Andrea Maria,Silva Nasjla,Zymberg Samuel,de Paula Santos Rodrigo
International journal of pediatric otorhinolaryngology
OBJECTIVES:This study aims to analyze our series of pediatric patients who underwent craniopharyngioma resection using the endoscopic endonasal transsphenoidal approach (EETA). METHODS:We collected clinical and surgical data from the charts of 20 children who underwent craniopharyngioma removal surgery using the EETA from 2007 to 2017. From the charts, we collected demographic information, results of imaging tests (size and extension of the tumor), and information regarding the surgical procedure and postoperative complications. RESULTS:From the 20 patients included in this series (12 women and eight men), 17 underwent EETA as a primary procedure, and the remaining three underwent EETA as a secondary procedure due to a relapsing tumor following previous transcranial surgery. The mean age of the patients at the time of the surgical procedure was 7.5 years (range 3-18 years). Regarding their location, 12 tumors were in the sellar and suprasellar regions, three extended into the third ventricle, and five were exclusively intrasellar. We achieved a gross total resection (GTR) of the tumor in 14 patients (70%), subtotal in five (25%), and partial in one (5%). One patient (5%) developed a cerebrospinal fluid fistula after the surgical procedure. In the postoperative follow-up period (mean time = 5.3 years; range = 2-9 years), 11 (55%) patients developed panhypopituitarism, and a relapsing tumor was later found in three (15%) patients. Regarding visual impairment, four patients had visual abnormalities preoperatively (amaurosis, n = 2; bilateral visual acuity decrease, n = 1; bilateral visual field defect, n = 1), and those did not improve or worsened postoperatively. None of the patients who did not have vision problems before the surgery developed those postoperatively. CONCLUSION:Our results showed that the EETA is a safe and effective approach for removing craniopharyngiomas in children, as it associated with low operative morbidity and complication rates. Also, our data demonstrated that the EETA may be performed regardless of the size of the nasal cavity, pneumatization of the sphenoid sinuses, and location or extension of the tumors.
Craniopharyngiomas Primarily Involving the Hypothalamus: A Model of Neurosurgical Lesions to Elucidate the Neurobiological Basis of Psychiatric Disorders.
Pascual Jose María,Prieto Ruth,Castro-Dufourny Inés,Mongardi Lorenzo,Rosdolsky Maria,Strauss Sewan,Carrasco Rodrigo,Barrios Laura
OBJECTIVE:This study provides a systematic review and meta-analysis of psychiatric disorders caused by craniopharyngiomas and the hypothalamic alterations underlying these symptoms. METHODS:We investigated a collection of 210 craniopharyngiomas reported from 1823 to 2017 providing detailed clinical and pathologic information about psychiatric disturbances, including 10 of our own series, and compared the hypothalamic damage in this cohort with the present in a control cohort of 105 cases without psychiatric symptoms. RESULTS:Psychiatric disorders occurred predominantly in patients with craniopharyngiomas developing primarily at the infundibulotuberal region (45%) or entirely within the third ventricle (30%), mostly affecting adult patients (61%; P < 0.001). Most tumors without psychic symptoms developed beneath the third ventricle floor (53.5%; P < 0.001), in young patients (57%; P < 0.001). Psychiatric disturbances were classified in 6 major categories: 1) Korsakoff-like memory deficits, 66%; 2) behavior/personality changes, 48.5%; 3) impaired emotional expression/control, 42%; 4) cognitive impairments, 40%; 5) mood alterations, 32%; and 6) psychotic symptoms, 22%. None of these categories was associated with hydrocephalus. Severe memory deficits occurred with damage of the mammillary bodies (P < 0.001). Mood disorders occurred with compression/invasion of the third ventricle floor and/or walls (P < 0.012). Coexistence of other hypothalamic symptoms such as temperature/metabolic dysregulation or sleepiness favored the emergence of psychotic disorders (P < 0.008). Postoperative psychiatric outcome was better in strictly intraventricular craniopharyngiomas than in other topographies (P < 0.001). A multivariate model including the hypothalamic structures involved, age, hydrocephalus, and hypothalamic symptoms predicts the appearance of psychiatric disorders in 81% of patients. CONCLUSIONS:Craniopharyngiomas primarily involving the hypothalamus represent a neurobiological model of psychiatric and behavioral disorders.
Approach selection and outcomes of craniopharyngioma resection: a single-institute study.
Lei Cao,Chuzhong Li,Chunhui Liu,Peng Zhao,Jiwei Bai,Xinsheng Wang,Yazhuo Zhang,Songbai Gui
Since there are many approaches for successful craniopharyngioma resection, how to choose a suitable approach remains problematic. The aim of this study was to summarize experience of approach selection and outcomes of craniopharyngioma resection in our institute. The data of 182 primary craniopharyngiomas between January 2013 and June 2019 were retrospectively reviewed. Craniopharyngiomas were classified into intrasellar, intra-suprasellar, suprasellar, and intra-third ventricle types based on the location. The surgical approaches, extent of resection, endocrine and ophthalmological outcomes, and complications were evaluated. Gross total resection (GTR) was achieved in 158 (86.8%) patients, near-total resection (NTR) in 20 (11%), and partial resection (PR) in 4 (2.2%). New-onset hypopituitarism occurred in 90 (49.5%) and new-onset diabetes insipidus in 48 (26.4%). Visual function was improved in 110 of the 182 patients, unchanged in 52, and deteriorated in 20. For intra-suprasellar and suprasellar tumors, patients in the endoscopic endonasal approach (EEA) group had higher GTR rate, lower incidence of new-onset hypopituitarism, and better visual outcome than patients in transcranial approach group, but no significant difference in the incidence of new-onset diabetes insipidus was found. There were no surgery-related deaths, and the common complications included permanent oculomotor nerve palsy, hemorrhage, and cerebrospinal fluid leaks. During the follow-up period, tumor recurrence or regrowth occurred in 6.6% of the cases. Tumor location is key for choosing an optimal surgical approach for craniopharyngioma resection. The EEA should be considered as the first choice for intra-suprasellar and suprasellar craniopharyngiomas to achieve better visual outcomes and fewer pituitary hormonal disorders.
Craniopharyngioma treatment: an updated summary of important clinicopathological concepts.
Prieto Ruth,Rosdolsky Maria,Hofecker Verena,Barrios Laura,Pascual José M
Expert review of endocrinology & metabolism
INTRODUCTION:Craniopharyngiomas (CPs) are benign histological tumors that may develop at different positions along the hypothalamic-pituitary axis. Their close, heterogenous relationship to the hypothalamus makes surgical removal challenging even though this remains the primary treatment strategy. AREAS COVERED:This article presents a critical overview of the pathological and clinical concepts regarding CPs that should be considered when planning treatment. Thus, we have performed a comprehensive review of detailed CP reports published between 1839 and 2020. EXPERT OPINION:CP surgery should pursue maximal tumor resection while minimizing the risk of injuring the hypothalamus. Therefore, surgical strategies should be individualized for each patient. Accurate assessment of presenting symptoms and preoperative MRI has proven useful to predict the type of CP-hypothalamus relationship that will be found during surgery. CPs with dense and extensive adhesions to the hypothalamus should be highly suspected when MRI shows the hypothalamus positioned around the mid-third of the tumor and an amputated upper portion of the pituitary stalk. Symptoms related to functional impairment of the infundibulo-tuberal area of the third ventricle floor, such as obesity/hyperphagia, Fröhlich's syndrome, diabetes insipidus, and/or somnolence, also indicate risky CP-hypothalamic adhesions. In these cases, limited tumor removal is strongly advocated followed by radiation therapy.
The endoscopic endonasal approach for the management of craniopharyngiomas.
Solari Domenico,Morace Roberta,Cavallo Luigi M,Amoroso Francesca,Cennamo Gilda,Del Basso DE Caro Marialaura,Cappabianca Paolo
Journal of neurosurgical sciences
Craniopharyngiomas are disembryogenetic, benign, tumors that origin from squamous epithelial remnants of Rathke's pouch, developing from any segment of its course, virtually from rhino-pharynx to the hypothalamus. Historically, different microscopic transcranial routes, have been advocated as possible surgical options for the treatment of craniopharyngiomas. The endonasal technique offers a direct approach that permits access to the suprasellar, retrosellar and retroclival space, obviating brain retraction; it provides the advantage of appraoching cranioopharyngiomas without optic nerve manipulation and/or retraction. We herein present the surgical nuances of the endoscopic endonasal approach for the treatment of craniopharyngiomas, highlighting hints, advantages and drawbacks, also in regards of the anatomy dealt with. The endoscopic endonasal technique has been emerging as a viable approach/alternative for the treatment of this disease as the endoscope itself increased its safety and effectiveness. It allows the removal of both infra and supradiaphragmatic lesions - eventually involving the third ventricle chamber but not extending laterally off the ICA out of the visibility and maneuverability of the instruments - avoiding brain and optic nerve manipulation and retraction, with good visualization of the pituitary gland and stalk and the main neurovascular structures.
[The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part II: Anatomical and neuroradiological evidence to define triple-axis topography and its usefulness in predicting individual surgical risk].
Pascual José María,Prieto Ruth,Carrasco Rodrigo,Castro-Dufourny Inés,Strauss Sewan,Gil-Simoes Ricardo,Barrios Laura
Neurocirugia (Asturias, Spain)
INTRODUCTION AND OBJECTIVES:This study evaluates the pathological and magnetic resonance imaging evidence to define the precise topographical relationships of craniopharyngiomas and to classify these lesions according to the risks of hypothalamic injury associated with their removal. MATERIAL AND METHODS:An extensive, systematic analysis of the topographical classification models used in the surgical series of craniopharyngiomas reported in the literature (n=145 series, 4,588 craniopharyngiomas) was performed. Topographical relationships of well-described operated craniopharyngiomas (n=224 cases) and of non-operated cases reported in autopsies (n=201 cases) were also analysed. Finally, preoperative and postoperative magnetic resonance imaging studies displayed in craniopharyngiomas reports (n=130) were compared to develop a triple-axis model for the topographical classification of these lesions with qualitative information regarding the associated risk of hypothalamic injury. RESULTS:The 2 major variables with prognostic value to define the topography of a craniopharyngioma are its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. A multivariate diagnostic model including 5 variables -patient age, presence of hydrocephalus and/or psychiatric symptoms, the relative position of the hypothalamus and the mammillary body angle- makes it possible to differentiate suprasellar craniopharyngiomas displacing the third ventricle upwards (pseudointraventricular craniopharyngiomas) from either strictly intraventricular craniopharyngiomas or lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas). CONCLUSIONS:A triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning the surgical approach and degree of resection. Infundibulo-tuberal craniopharyngiomas represent 42% of all cases. These lesions typically show tight, circumferential adhesion to the third ventricle floor, with their removal being associated with a 50% risk of hypothalamic injury. The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the degree and extension of craniopharyngioma adherence to the hypothalamus.
Hypothalamic involvement predicts cognitive performance and psychosocial health in long-term survivors of childhood craniopharyngioma.
Fjalldal Sigridur,Holmer Helene,Rylander Lars,Elfving Maria,Ekman Bertil,Osterberg Kai,Erfurth Eva Marie
The Journal of clinical endocrinology and metabolism
CONTEXT:Hypothalamic damage caused by craniopharyngioma (CP) is associated with poor functional outcome. OBJECTIVE:To assess cognitive function and quality of life in childhood-onset CP on hormonal replacement, including GH treatment. DESIGN:A cross-sectional study with a median follow-up time of 20 years (1-40). SETTING:Patients were recruited from the South Medical Region of Sweden. PARTICIPANTS:The study included 42 patients (20 women) surgically treated for a childhood-onset CP between 1958 and 2000. Patients were aged ≥17 years. Equally many controls, matched for age, sex, residence, and smoking habits, were included. Tumor growth into the third ventricle was found in 25 patients. MAIN OUTCOME MEASURES:All subjects were examined with a battery of cognitive tests and the following questionnaires: Symptom Checklist-90, the Interview Schedule for Social Interaction, and the Social Network concept. RESULTS:The CP patients had lower cognitive performance, reaching statistical significance in 12 of 20 test variables, including executive function and memory. Comparison of patients with tumor growth into the third ventricle to controls revealed a significant lower mean total score (P = .006). A significant negative correlation was recorded between mean z-score of cognitive performance and years since operation (r = -0.407; P = .014). No statistically significant group differences were observed across any of the 9 Symptom Checklist-90 subscales. CONCLUSIONS:Adults with childhood-onset CP, on hormone replacement, including GH treatment, have memory defects, disturbed attention, and impaired processing speed. Patients with hypothalamic involvement are more affected. Patients rated their quality of life as good as their matched controls.
Extended Endoscopic Endonasal Resection of a Suprasellar and Third Ventricular Retrochiasmatic Craniopharyngioma with a Narrow Pituitary Gland-Optic Chiasm Interval: Techniques to Optimize Resection.
Kenning Tyler J,Pinheiro-Neto Carlos D
Journal of neurological surgery. Part B, Skull base
The extended endoscopic endonasal approach can be utilized to surgically treat pathology within the suprasellar space. This relies on a sufficient corridor and interval between the superior aspect of the pituitary gland and the optic chiasm. Tumors located in the retrochiasmatic space and within the third ventricle, however, may not have a widened interval through which to work. With mass effect on the superior and posterior aspect of the optic chiasm, the corridor between the chiasm and the pituitary gland might even be further narrowed. This may negate the possibility of utilizing the endoscopic endonasal approach for the management of pathology in this location. We present a case of a retrochiasmatic craniopharyngioma with a narrow resection corridor that was treated with the extended endoscopic approach and we review techniques to potentially overcome this limitation. The link to the video can be found at: https://youtu.be/ogRZj-aBqeQ .
Neuroendoscopic treatment of cystic craniopharyngioma in the third ventricle.
Nakamizo A,Inamura T,Nishio S,Inoha S,Ishibashi H,Fukui M
Minimally invasive neurosurgery : MIN
The third ventricle is a relatively uncommon location for craniopharyngiomas. Generally, craniotomy has been considered the procedure of choice in such cases. We describe a girl in whom a cystic third ventricular craniopharyngioma was successfully treated by evacuation of the cyst contents via a flexible neuroendoscope and precise placement of an Ommaya reservoir catheter within the tumor.
Endonasal endoscopic transsphenoidal resection of intrinsic third ventricular craniopharyngioma: surgical results.
Forbes Jonathan A,Ordóñez-Rubiano Edgar G,Tomasiewicz Hilarie C,Banu Matei A,Younus Iyan,Dobri Georgiana A,Phillips C Douglas,Kacker Ashutosh,Cisse Babacar,Anand Vijay K,Schwartz Theodore H
Journal of neurosurgery
OBJECTIVEIntrinsic third ventricular craniopharyngiomas (IVCs) have been reported by some authors to "pose the greatest surgical challenge" of all craniopharyngiomas (CPAs). A variety of open microsurgical approaches have historically been used for resection of these tumors. Despite increased utilization of the endoscopic endonasal approach (EEA) for resection of CPAs in recent years, many authors continue to recommend against use of the EEA for resection of IVCs. In this paper, the authors present the largest series to date utilizing the EEA to remove IVCs.METHODSThe authors reviewed a prospectively acquired database of the EEA for resection of IVCs over 14 years at Weill Cornell Medical College, NewYork-Presbyterian Hospital. Preoperative MR images were examined independently by two neurosurgeons and a neuroradiologist to identify IVCs. Pre- and postoperative endocrinological, ophthalmological, radiographic, and other morbidities were determined from retrospective chart review and volumetric radiographic analysis.RESULTSBetween January 2006 and August 2017, 10 patients (4 men, 6 women) ranging in age from 26 to 67 years old, underwent resection of an IVC utilizing the EEA. Preoperative endocrinopathy was present in 70% and visual deterioration in 60%. Gross-total resection (GTR) was achieved in 9 (90%) of 10 patients, with achievement of near-total (98%) resection in the remaining patient. Pathology was papillary in 30%. Closure incorporated a "gasket-seal" technique with nasoseptal flap coverage and either lumbar drainage (9 patients) or a ventricular drain (1 patient). Postoperatively, complete anterior and posterior pituitary insufficiency was present in 90% and 70% of patients, respectively. In 4 patients with normal vision prior to surgery, 3 had stable vision following tumor resection. One patient noted a new, incongruous, left inferior homonymous quadrantanopsia postoperatively. In the 6 patients who presented with compromised vision, 2 reported stable vision following surgery. Each of the remaining 4 patients noted significant improvement in vision after tumor resection, with complete restoration of normal vision in 1 patient. Aside from the single case (10%) of visual deterioration referenced above, there were no instances of postoperative neurological decline. Postoperative CSF leakage occurred in 1 morbidly obese patient who required reoperation for revision of closure. After a mean follow-up of 46.8 months (range 4-131 months), tumor recurrence was observed in 2 patients (20%), one of whom was treated with radiation and the other with chemotherapy. Both of these patients had previously undergone GTR of the IVC.CONCLUSIONSThe 10 patients described in this report represent the largest number of patients with IVC treated using EEA for resection to date. EEA for resection of IVC is a safe and efficacious operative strategy that should be considered a surgical option in the treatment of this challenging subset of tumors.
Multimodality, Multidirectional Resection of Craniopharyngioma: Versatility in Alternating the Principal and Auxiliary Surgical Corridors and Visualization Modalities.
Jean Walter C
BACKGROUND:Large tumors of the skull base may require multiple approaches for safe removal, as unidirectional approaches may require excessive brain retraction. METHODS:Two patients underwent simultaneous, endoscopic and microscopic resection of tumors using 2 anatomic corridors. The corridor used for most of the tumor dissection was designated as "principal," whereas the secondary corridor used for assisting the main operation was designated "auxiliary." The endoscope and microscope were used interchangeably in the 2 corridors. RESULTS:For the first patient, the principal corridor was transventricular, and the auxiliary corridor was orbitofrontal. The endoscope was used exclusively in the latter and yielded visual information of the undersurface of the tumor, used for protection of the optic chiasm. For the second case, the corridors were reversed. Tumor resection was performed using the microscope and endoscope in alternating fashion. The endoscope, when used in the auxiliary ventricular corridor, was useful in delivering tumor components into the principal operative field. CONCLUSIONS:Multidirectional approaches to large tumors can be considered less invasive if the surgical corridors are combined in a way to minimize traction forces on both brain and tumor and maximize visualization and protection of critical structures. These combination approaches can be made simpler with the seamless integration of the endoscope and microscope. The choice between the principal and auxiliary corridors should alternate just as smoothly as the visual modality and must be dictated by the anatomy and minute-to-minute tactical situation during the operation.
[Growth of craniopharyngioma involving the third ventricular floor in relation to the hypothalamus].
Liu Bao-guo,Qi Song-tao,Pan Jun,Peng Yu-ping,Fang Lu-xiong
Nan fang yi ke da xue xue bao = Journal of Southern Medical University
OBJECTIVE:To investigate the growth of craniopharyngioma involving the third ventricular floor with regard to the hypothalamus by detecting expressions of leukocyte common antigen (CD45) and intercellular adhesion molecule (ICAM-1) in the tumor tissue. METHODS:The expressions of CD45 and ICAM-1 proteins in 30 craniopharyngioma samples with third ventricular floor involvement were detected by SP immunohistochemistry. RESULTS:The inflammations labeled by CD45 were identified commonly in the craniopharyngioma tissues involving the third ventricular floor. The expression of ICAM-1 was mainly in the inner tumor cells and interstitial cells, but not detected in the basilar tumor cells growing toward the third ventricular floor. Adamantinomatous craniopharyngiomas showed markedly higher CD45 and ICAM-1 expressions than squamous papillary tumors (P<0.05). CONCLUSION:Inflammatory adhesion largely characterizes the growth of the craniopharyngioma tissues involving the third ventricular floor toward the hypothalamus without the tendency of invasion. The difference in the inflammation between the two types of craniopharyngioma may affect the prognosis of the patients.
Clinical features and operative technique of transinfundibular craniopharyngioma.
Tang Bin,Xie ShenHao,Huang GuanLin,Wang ZhiGang,Yang Le,Yang XuanYong,Xu Shan,Zeng ErMing,Hong Tao
Journal of neurosurgery
OBJECTIVE:Transinfundibular craniopharyngioma (TC) is one of the 4 subtypes of suprasellar craniopharyngioma. In this study, the authors analyzed the clinical features of and operative technique for TC. METHODS:A total of 95 consecutive cases of suprasellar craniopharyngioma that had been resected via the endoscopic expanded endonasal approach were retrospectively reviewed. Patients were divided into 2 groups: 34 in the TC group and 61 in the nontransinfundibular craniopharyngioma (NC) group. Clinical and radiographic features, intraoperative findings, histopathological and genetic findings, and surgical outcomes were analyzed and compared between groups. RESULTS:Compared with NC, TC was mostly seen in adult patients (97.1%); it was rare in children (2.9%). Clinical presentations tended toward headache, hydrocephalus, and diabetes insipidus. The relatively smaller volume, midline location (consistent with the stalk position), unidentifiable stalk, no shift of the third ventricle, and greater likelihood to involve the third ventricle and cause hydrocephalus were the characteristic features of TC in the preoperative MRI study. According to the degree of vertical extension of the tumor, the 34 TCs could be classified into 3 subtypes: type 1, entity was limited to stalk (n = 2, 5.9%); type 2, tumor extended up to the third ventricle (type 2a) or down to the subdiaphragmatic cavity (type 2b) (n = 23, 67.6%); and type 3, tumor extended in both directions (n = 9, 26.5%). For TC resection, the chiasm-pituitary corridor, lamina terminalis corridor, and pituitary corridor could be used separately or jointly. Most of the TCs originated from the infundibulum-tuber cinereum, grew within and along the long axis of the infundibulum, and the pituitary stalk was not usually preserved in TCs (20.6%), whereas the rate of preservation was higher (80.3%) in NCs. Bilateral hypothalamic injury was found in nearly all TCs if radical resection was performed, whereas the relationship between NCs and hypothalamus was either compression (32.8%) or unilateral invasion (67.2%). Meanwhile, the postoperative endocrine and neuropsychological function outcomes in patients with TC were worse than in patients with NC. The genetic analysis with whole-exome sequencing studies showed no differential mutations of CTNNB1 (β-catenin) and BRAF (V600E) between TC and NC subtypes, but there was a difference between adamantinomatous craniopharyngioma and papillary craniopharyngioma. CONCLUSIONS:TC is a special subtype of suprasellar craniopharyngioma, which is remarkably different from NC. Identification of this type of tumor preoperatively is essential for the planning of appropriate surgical approach and degree of excision.
Intrinsic Third Ventricular Papillary Craniopharyngioma: A Report of Five Cases and Literature Review.
Hung Nguyen Duy,Ngan Vuong Kim,Duc Nguyen Minh
International medical case reports journal
Background:Craniopharyngiomas are common lesions that occur in the suprasellar region; however, strictly intrinsic third ventricular craniopharyngiomas are rare. Case Series:We aimed to describe the magnetic resonance imaging features observed in five cases of strictly intrinsic third ventricular papillary craniopharyngiomas, including two cases of mixed cystic and solid tumors and three cases of pure solid masses. Conclusion:Among the adult population, intrinsic third ventricular papillary craniopharyngiomas should be considered when either solid or mixed cystic and solid masses are observed, in which the solid component shows heterogeneous intensity, heterogeneous and strong enhancement, and is strictly located in the third ventricle.
Infundibulo-tuberal or not strictly intraventricular craniopharyngioma: evidence for a major topographical category.
Pascual José M,Prieto Ruth,Carrasco Rodrigo
PURPOSE:This study investigates retrospectively the clinical, neuroradiological, pathological and surgical evidence verifying the infundibulo-tuberal topography for craniopharyngiomas (CPs). Infundibulo-tuberal CPs represent a surgical challenge due to their close anatomical relationships with the hypothalamus. An accurate definition of this topographical category is essential in order to prevent any undue injury to vital diencephalic centres. METHODS:A systematic review of all scientific reports involving pathological, neuroradiological or surgical descriptions of either well-described individual cases or large series of CPs published in official journals and text books from 1892 to 2011 was carried out. A total of 1,232 documents providing pathological, surgical and/or neuroradiological evidence for the infundibulo-tuberal or hypothalamic location of CPs were finally analysed in this study. FINDINGS:For a total of 3,571 CPs included in 67 pathological, surgical or neuroradiological series, 1,494 CPs (42%) were classified as infundibulo-tuberal lesions. This topography was proved in the autopsy of 122 non-operated cases. The crucial morphological finding characterizing the tubero-infundibular topography was the replacement of the third ventricle floor by a lesion with a predominant intraventricular growth. This type of CP usually presents a circumferential band of tight adherence to the third ventricle floor remnants, formed by a functionless layer of rective gliosis of a variable thickness. After complete surgical removal of an infundibulo-tuberal CP, a wide defect or breach at the floor of the third ventricle is regularly observed both in the surgical field and on postoperative magnetic resonance imaging studies. CONCLUSIONS:Infundibulo-tuberal CPs represent a major topographical category of lesions with a primary subpial development at the floor of the third ventricle. These lesions expand within the hypothalamus itself and subsequently occupy the third ventricle; consequently, they can be classified as not strictly intraventricular CPs. A tight attachment to the hypothalamus and remnants of the third ventricle floor is the pathological landmark of infundibulo-tuberal CPs.
Topographic Diagnosis of Craniopharyngiomas: The Accuracy of MRI Findings Observed on Conventional T1 and T2 Images.
Prieto R,Pascual J M,Barrios L
AJNR. American journal of neuroradiology
BACKGROUND AND PURPOSE:The topography of craniopharyngiomas has proved fundamental in predicting the involvement of vital brain structures and the possibility of achieving a safe radical resection. Beyond the imprecise term "suprasellar," indiscriminately used for craniopharyngiomas, an accurate definition of craniopharyngioma topography should be assessed by preoperative MR imaging. The objective of this study was to investigate the MRI findings that help define craniopharyngioma topography. MATERIALS AND METHODS:This study retrospectively investigated a cohort of 200 surgically treated craniopharyngiomas with their corresponding preoperative midsagittal and coronal conventional T1- and T2-weighted MR images, along with detailed descriptions of the surgical findings. Radiologic variables related to the occupation of the tumor of intracranial compartments and the distortions of anatomic structures along the sella turcica-third ventricle axis were analyzed and correlated with the definitive craniopharyngioma topography observed during the surgical procedures. A predictive model for craniopharyngioma topography was generated by multivariate analysis. RESULTS:Five major craniopharyngioma topographies can be defined according to the degree of hypothalamic distortion caused by the tumor: sellar-suprasellar, pseudointraventricular, secondary intraventricular, not strictly intraventricular, and strictly intraventricular. Seven key radiologic variables identified on preoperative MRI allowed a correct overall prediction of craniopharyngioma topography in 86% of cases: 1) third ventricle occupation, 2) pituitary stalk distortion, 3) relative level of the hypothalamus in relation to the tumor, 4) chiasmatic cistern occupation, 5) mammillary body angle, 6) type of chiasm distortion, and 7) tumor shape. CONCLUSIONS:Systematic assessment of these 7 variables on conventional preoperative T1 and T2 MRI is a useful and reliable method to ascertain individual craniopharyngioma topography.
Intraventricular craniopharyngioma: morphological analysis and outcome evaluation of 17 cases.
Pan Jun,Qi Songtao,Lu Yuntao,Fan Jun,Zhang Xi'an,Zhou Jie,Peng Junxiang
PURPOSE:There is still some confusion with regard to the tumor-third ventricle floor (3rd VF) relationship of craniopharyngiomas located exclusively within the third ventricle. This study aims to provide some evidence to clarify the growth pattern of intraventricular craniopharyngiomas (IVC), and to summarize the surgical strategy and outcome. METHODS:Seventeen cases of IVC were reviewed retrospectively in relation to preoperative imaging, clinical presentation, intraoperative findings, tumor pathology, and surgical outcome. The tumor-3rd VF relationship and the tumor's stratification were analyzed based on intraoperative inspection and histology. FINDINGS:Variable adherence patterns of IVC to the 3rd VF were found, which were classified as (a) purely IVC with pedicle attachment to 3rd VF (two cases), (b) intra-3rd VF tumors with wide-based attachment but a dissectible tumor boundary (seven cases), and (c) intra-3rd VF tumors with an undissectible wide, tight attachment (eight cases). Histological analysis revealed that both of the two cases with growth pattern "a" intruded into the third ventricular cavity without a covering layer of neural tissue (which only exists in the squamous-papillary subtype). Tumors with growth pattern "b" and "c," in contrast, were noted to have a thin layer of neural tissue. This occurred in both subtypes (11 adamantinomatous, 4 papillary). Total removal was accomplished in all tumors demonstrating growth pattern "a" and "b." There was also better preservation of the 3rd VF and consequently a better outcome. On the other hand, total removal was only achieved in 50% of tumors showing growth pattern "c" including one mortality. No recurrence has been encountered in patients whose tumors were totally removed. CONCLUSION:Variable adherence patterns and tumor subtypes were observed in IVCs, which were correlated to the tumor pathology, resectability, and subsequent prognosis.
Limits of endoscopic endonasal surgery for III ventricle craniopharyngiomas.
Hardesty Douglas A,Montaser Alaa S,Beer-Furlan André,Carrau Ricardo L,Prevedello Daniel M
Journal of neurosurgical sciences
Craniopharyngiomas represent one of the most challenging brain tumors for the neurosurgeon. For most of the 20th century, these parasellar lesions have been approached via the classic open approaches of neurosurgery such as pterional, frontobasal, interhemispheric, and transpetrosal craniotomies. The endoscopic endonasal approach to these tumors, rather than craniotomy, has risen in popularity over the last two decades. Regardless of approach, a detailed knowledge of surgical anatomy and careful preoperative surgical planning are essential to achieve good clinical results; iatrogenic morbidity can potentially be severe due to hypothalamus, optic apparatus, and/or vascular injuries. Especially challenging, and highlighting the limitations of endoscopic endonasal surgery, are the tumors that arise primarily from within the third ventricle and do not expand the pituitary stalk and suprasellar region or tumors that have projected to areas far from the parasellar region as such as the sylvian and ambient cisterns. Herein we review the published literature regarding endoscopic endonasal surgery for craniopharyngioma, and the anatomical and functional limitations therein. The benefits and drawbacks of each surgical approach to this deep-seated area are discussed, and a strategy for surgical decision-making proposed.
Preoperative Assessment of Craniopharyngioma Adherence: Magnetic Resonance Imaging Findings Correlated with the Severity of Tumor Attachment to the Hypothalamus.
Prieto Ruth,Pascual José M,Rosdolsky Maria,Barrios Laura
BACKGROUND AND OBJECTIVE:Craniopharyngioma (CP) adherence represents a heterogeneous pathologic feature that critically influences the potentially safe and radical resection. The aim of this study was to define the magnetic resonance imaging (MRI) predictors of CP adherence severity. METHODS:This study retrospectively investigated a cohort of 200 surgically treated CPs with their corresponding preoperative conventional MRI scans. MRI findings related to the distortions of anatomic structures along the sella turcica-third ventricle axis caused by CPs, in addition to the tumor's shape and calcifications, were analyzed and correlated with the definitive type of CP adherence observed during the surgical procedures. RESULTS:CP adherence is defined by 3 components, as follows: 1) the specific structures attached to the tumor, 2) the adhesion's extent, and 3) its strength. Combination of these 3 components determines 5 hierarchical levels of adherence severity with gradually increasing surgical risk of hypothalamic injury. Multivariate analysis identified 4 radiologic variables that allowed a correct overall prediction of the levels of CP adherence severity in 81.5% of cases: 1) the position of the hypothalamus in relation to the tumor-the most discriminant factor; 2) the type of pituitary stalk distortion; 3) the tumor shape; and 4) the presence of calcifications. A binary logistic regression model including the first 3 radiologic variables correctly identified the CPs showing the highest level of adherence severity (severe/critical) in almost 90% of cases. CONCLUSIONS:A position of the hypothalamus around the middle portion of the tumor, an amputated or infiltrated appearance of the pituitary stalk, and the elliptical shape of the tumor are reliable predictors of strong and extensive CP adhesions to the hypothalamus.
Harvey Cushing's craniopharyngioma treatment: Part 2. Surgical strategies and results of his pioneering series.
Prieto Ruth,Pascual José María,Barrios Laura
Journal of neurosurgery
OBJECTIVE:Harvey Cushing (1869-1939) developed pioneering surgical techniques for craniopharyngioma (CP) removal. This study exhaustively analyzes the pathological variables and surgical strategies that influenced Cushing's results in his entire series of CP patients. METHODS:The CP records from Cushing's Brain Tumor Registry were carefully reviewed, as were his CP cases published in medical monographs and scientific reports. RESULTS:One hundred twenty-four tumors with characteristics typical of CP comprise Cushing's entire series (CP124). Cushing performed 198 surgical procedures in the patients in whom these tumors were treated, with a 23% mortality rate within the first 2 months after surgery. Three periods in Cushing's CP surgical career can be differentiated: an early period (1901-1917, 39 patients) characterized by his use of the transsphenoidal approach and limited cyst drainage procedures, an intermediate period (1919-1925, 42 patients) in which the subfrontal approach was the standard procedure and maximal removal was attempted, and a late period (1926-1932, 43 patients) characterized by the use of air ventriculography for topographical diagnosis and limited resections via a transventricular approach. Among Cushing's CP series were 92 cases that were pathologically verified (CP92). In this subcohort, the unilateral subfrontal approach was predominantly used (72% of cases), followed by the transsphenoidal (15%) and frontal transcortical-transventricular (8%) approaches. Drainage of the CP cystic component or partial excision of the solid component was achieved in 61% of the cases, subtotal removal in 23%, and macroscopic total removal in 10%. Satisfactory outcomes were obtained in 55% of the patients in CP92, whereas poor outcomes and/or death related to hypothalamic injury was observed in 28%. Postoperative symptoms related to hypothalamic dysfunction occurred 53% of the time. The subfrontal approach yielded the highest rates of radical removal (p < 0.001) and good outcomes (p = 0.01). Partial removals were associated with the highest rates of poor outcomes, including death (p = 0.009). Cushing's removal of CPs with a primary infundibulo-tuberal topography or showing third ventricle invasion was associated with the highest rates of hypothalamic injury (p < 0.001) and the worst outcomes (p = 0.009). CONCLUSIONS:Harvey Cushing's techniques and surgical philosophy varied substantially throughout his career. The experience he gained with this large CP series made him aware of the importance of limiting the extent of tumor removal and leaving untouched the tumor portion strongly adhered to the hypothalamus.
Pathological Relationship Between Adamantinomatous Craniopharyngioma and Adjacent Structures Based on QST Classification.
Liu Yi,Qi Song-Tao,Wang Chao-Hu,Pan Jun,Fan Jun,Peng Jun-Xiang,Zhang Xi'an,Bao Yun,Liu Ya-Wei
Journal of neuropathology and experimental neurology
The aim of this study was to clarify pathological and anatomical relationships between adamantinomatous craniopharyngiomas (ACP) and their surrounding structures. We previously established a QST classification scheme based on the apparent anatomic origin of the tumors. According to this classification, 13 type Q tumors, 6 type S tumors, and 42 type T ACPs were analyzed. Type Q tumors, which are most likely to involve the pituitary gland, did not invade the area of contact with the adenohypophysis. Instead, tumor invasion was observed in areas where the tumor contacted the neurohypophysis. Type S tumors primarily involved the pituitary stalk; the arachnoid remained present between these tumors and normal structures. Type T tumors were located beneath the basal arachnoid membrane and outside the pia mater. The pia mater was disrupted and finger-like invasions were found in the neural layer of the third ventricle floor along the invasive front. Tumors were never observed to break through the ependymal layer of the third ventricle. The QST classification has important implications for understanding the growth pattern of tumors and can be used to guide surgical procedures.
Harvey Cushing's craniopharyngioma treatment: Part 1. Identification and clinicopathological characterization of this challenging pituitary tumor.
Pascual José María,Prieto Ruth,Barrios Laura
Journal of neurosurgery
OBJECTIVE:Harvey Cushing (1869-1939) coined the term "craniopharyngioma" (CP) in 1929 to describe a kaleidoscopic group of epithelial tumors involving the hypothalamic-pituitary axis. Throughout his career, he endured a long struggle to accurately diagnose and safely remove these complex lesions, and his resulting surgical series has never before been analyzed in depth. The authors here conduct such an analysis. METHODS:In this study, the authors retrospectively examined the CP patient records available in the Cushing Brain Tumor Registry, as well as those CP cases reported by Cushing in medical monographs and scientific reports. RESULTS:Cushing's CP series comprises a total of 124 tumors (CP124) compatible with a CP diagnosis. Among this series are 92 cases that could be pathologically verified (CP92). This subcohort showed a bimodal age distribution (41% aged ≤ 19 years old) and a balanced sex distribution. Clinical evolution up to diagnosis was longer than 3 years in half of the patients. Typical symptoms found at diagnosis were severe headache (94%), visual deficits (97%), panhypopituitarism (76%), psychiatric disturbances (47%), and abnormal somnolence (47%). The highest rate of endocrine deficits occurred in patients younger than 19 years of age (p < 0.001), whereas hypothalamic disturbances were observed mainly in adults between 30 and 49 years (p = 0.02). Hydrocephalus was present in 63% of the patients, predominantly involving the younger subgroup (p < 0.001). Preoperative diagnosis was based on clinical signs, funduscopic exams, and skull radiographs, the latter study showing suprasellar calcifications in 64% of cases. The majority of tumors (61%) had developed within the third ventricle (3V) or had invaded it. The adamantinomatous histological variant was the predominant one (73%). Squamous-papillary CPs occurred only in adults older than 40 years of age (p < 0.001). Strong CP adherences to the hypothalamus were demonstrated in 63% of cases. The infundibulo-tuberal and sellar/suprasellar-3V CP topographies were associated with the highest rates of hypothalamic dysfunction before surgery (p < 0.001), surgical hypothalamic injury (p < 0.001), and severe postoperative morbidity and/or mortality (p = 0.009). Both topographies showed the strongest adherences to the hypothalamus and 3V (p < 0.001). CONCLUSIONS:Cushing's CP series comprises severely ill patients with tumors in the late stages of progression, with a high rate of tumors developing primarily within the hypothalamus (infundibulo-tuberal CPs) or invading this structure from the sellar/suprasellar regions. Craniopharyngioma topography was the fundamental variable influencing the clinical manifestations, tumor features, and patient outcomes in this series.
Craniopharyngioma adherence: a comprehensive topographical categorization and outcome-related risk stratification model based on the methodical examination of 500 tumors.
Prieto Ruth,Pascual José María,Rosdolsky Maria,Castro-Dufourny Inés,Carrasco Rodrigo,Strauss Sewan,Barrios Laura
OBJECTIVE Craniopharyngioma (CP) adherence strongly influences the potential for achieving a radical and safe surgical treatment. However, this factor remains poorly addressed in the scientific literature. This study provides a rational, comprehensive description of CP adherence that can be used for the prediction of surgical risks associated with the removal of these challenging lesions. METHODS This study retrospectively analyzes the evidence provided in pathological, neuroradiological, and surgical CP reports concerning 3 components of the CP attachment: 1) the intracranial structures attached to the tumor; 2) the morphology of the adhesion; and 3) the adhesion strength. From a total of 1781 CP reports published between 1857 and 2016, a collection of 500 CPs providing the best information about the type of CP attachment were investigated. This cohort includes autopsy studies (n = 254); surgical studies with a detailed description or pictorial evidence of CP adherence (n = 298); and surgical CP videos (n = 61) showing the technical steps for releasing the attachment. A predictive model of CP adherence in hierarchical severity levels correlated with surgical outcomes was generated by multivariate analysis. RESULTS The anatomical location of the CP attachment occurred predominantly at the third ventricle floor (TVF) (54%, n = 268), third ventricle walls (23%, n = 114), and pituitary stalk (19%, n = 94). The optic chiasm was involved in 56% (n = 281). Six morphological patterns of CP attachment were identified: 1) fibrovascular pedicle (5.4%); 2) sessile or patch-like (21%); 3) cap-like (over the CP top, 14%); 4) bowl-like (around the CP bottom, 13.5%); 5) ring-like (encircling central band, 19%); and 6) circumferential (enveloping the entire CP, 27%). Adhesion strength was classified in 4 grades: 1) loose (easily dissectible, 8%); 2) tight (requires sharp dissection, 32%); 3) fusion (no clear cleavage plane, 40%); and 4) replacement (loss of brain tissue integrity, 20%). The types of CP attachment associated with the worst surgical outcomes are the ring-like, bowl-like, and circumferential ones with fusion to the TVF or replacement of this structure (p < 0.001). The CP topography is the variable that best predicts the type of CP attachment (p < 0.001). Ring-like and circumferential attachments were observed for CPs invading the TVF (secondary intraventricular CPs) and CPs developing within the TVF itself (infundibulo-tuberal CPs). Brain invasion and peritumoral gliosis occurred predominantly in the ring-like and circumferential adherence patterns (p < 0.001). A multivariate model including the variables CP topography, tumor consistency, and the presence of hydrocephalus, infundibulo-tuberal syndrome, and/or hypothalamic dysfunction accurately predicts the severity of CP attachment in 87% of cases. CONCLUSIONS A comprehensive descriptive model of CP adherence in 5 hierarchical levels of increased severity-mild, moderate, serious, severe, and critical-was generated. This model, based on the location, morphology, and strength of the attachment can be used to anticipate the surgical risk of hypothalamic injury and to plan the degree of removal accordingly.
Suprachiasmatic translamina terminalis corridor used in endoscopic endonasal approach for resecting third ventricular craniopharyngioma.
Gu Ye,Zhang Xiaobiao,Hu Fan,Yu Yong,Xie Tao,Sun Chongjing,Li Wensheng
Journal of neurosurgery
OBJECT:The translamina terminalis corridor was used in the transcranial anterior route to treat third ventricular craniopharyngioma (TVC), which presents a challenge to neurosurgeons. The endoscopic endonasal approach (EEA) has recently been used to treat craniopharyngiomas. However, there are few reports of the EEA being used to treat TVC. The authors' novel surgical approach of treating selected TVC by the endoscopic endonasal route via the suprachiasmatic translamina terminalis (STLT) corridor is described. METHODS:In this single-center study, the EEA via the STLT corridor was used to resect TVC with great upper and anterior extension causing bulged lamina terminalis, and TVC with a residual upper compartment, after routine infrachiasmatic transmetastalk corridor resection. RESULTS:The STLT corridor was used in 3 patients. Gross-total resection was achieved in all cases. One patient achieved visual improvement, and the other 2 patients showed partial visual improvement. Leakage of CSF occurred in 1 patient. Postoperative hormone replacement therapy was required in all patients. CONCLUSIONS:The STLT corridor is a complementary minimally invasive corridor used in the EEA for treating selected TVC. The STLT alone or combined with infrachiasmatic transmetastalk corridors should be selected depending on the size of suprachiasmatic and infrachiasmatic space.
Trans-eyebrow supraorbital keyhole approach in suprasellar and third ventricular craniopharyngioma surgery: the experience of 27 cases and a literature review.
Cai Meiqin,Ye Zhuopeng,Ling Cong,Zhang Baoyu,Hou Bo
Journal of neuro-oncology
BACKGROUND:The trans-eyebrow supraorbital keyhole approach, a minimal transcranial approach, has been widely used in different types of surgery for sellar and parasellar lesions. In this study, we investigated the outcome of this approach in the surgical treatment of suprasellar and third ventricular craniopharyngioma. METHODS:Twenty-seven patients with suprasellar and third ventricular craniopharyngioma underwent surgery via a supraorbital approach between June 2007 and June 2018. The medical data and follow-up results were retrospectively analyzed. RESULTS:All tumors were located in the suprasellar region and the third ventricle. The mean tumor size was 29.1 mm. The mean follow-up period was 49.6 months. Gross total resection (GTR) was achieved in 23 patients (85.2%). Of 17 patients with preoperative visual impairment, 12 patients (70.6%) showed improvement. Following surgery, 11 patients exhibited new-onset anterior hypopituitarism, ten developed diabetes insipidus, and two became overweight. One residual tumor relapsed 1 year after surgery. No perioperative death, cerebrospinal fluid (CSF) rhinorrhea, or meningitis occurred. All patients exhibited satisfactory cosmetic results. At the last follow-up, the Extended Glasgow Outcome Scale Score was 8 in 25 patients (92.6%). CONCLUSION:The supraorbital trans-eyebrow keyhole approach is characterized by minimal invasion and a satisfactory cosmetic outcome. According to our experience, craniopharyngiomas located in the suprasellar region and the third ventricle can be safely resected via a trans-eyebrow supraorbital keyhole approach.
Displacement of mammillary bodies by craniopharyngiomas involving the third ventricle: surgical-MRI correlation and use in topographical diagnosis.
Pascual José María,Prieto Ruth,Carrasco Rodrigo,Barrios Laura
Journal of neurosurgery
OBJECT:Accurate diagnosis of the topographical relationships of craniopharyngiomas (CPs) involving the third ventricle and/or hypothalamus remains a challenging issue that critically influences the prediction of risks associated with their radical surgical removal. This study evaluates the diagnostic accuracy of MRI to define the precise topographical relationships between intraventricular CPs, the third ventricle, and the hypothalamus. METHODS:An extensive retrospective review of well-described CPs reported in the MRI era between 1990 and 2009 yielded 875 lesions largely or wholly involving the third ventricle. Craniopharyngiomas with midsagittal and coronal preoperative and postoperative MRI studies, in addition to detailed descriptions of clinical and surgical findings, were selected from this database (n = 130). The position of the CP and the morphological distortions caused by the tumor on the sella turcica, suprasellar cistern, optic chiasm, pituitary stalk, and third ventricle floor, including the infundibulum, tuber cinereum, and mammillary bodies (MBs), were analyzed on both preoperative and postoperative MRI studies. These changes were correlated with the definitive CP topography and type of third ventricle involvement by the lesion, as confirmed surgically. RESULTS:The mammillary body angle (MBA) is the angle formed by the intersection of a plane tangential to the base of the MBs and a plane parallel to the floor of the fourth ventricle in midsagittal MRI studies. Measurement of the MBA represented a reliable neuroradiological sign that could be used to discriminate the type of intraventricular involvement by the CP in 83% of cases in this series (n = 109). An acute MBA (< 60°) was indicative of a primary tuberal-intraventricular topography, whereas an obtuse MBA (> 90°) denoted a primary suprasellar CP position, causing either an invagination of the third ventricle (pseudointraventricular lesion) or its invasion (secondarily intraventricular lesion; p < 0.01). A multivariate model including a combination of 5 variables (the MBA, position of the hypothalamus, presence of hydrocephalus, psychiatric symptoms, and patient age) allowed an accurate definition of the CP topography preoperatively in 74%-90% of lesions, depending on the specific type of relationship between the tumor and third ventricle. CONCLUSIONS:The type of mammillary body displacement caused by CPs represents a valuable clue for ascertaining the topographical relationships between these lesions and the third ventricle on preoperative MRI studies. The MBA provides a useful sign to preoperatively differentiate a primary intraventricular CP originating at the infundibulotuberal area from a primary suprasellar CP, which either invaginated or secondarily invaded the third ventricle.
The endoscopic endonasal approach for the management of craniopharyngiomas involving the third ventricle.
Cavallo Luigi Maria,Solari Domenico,Esposito Felice,Cappabianca Paolo
The third ventricle has historically represented one of the most challenging areas to access surgically, so that lesions directly harboring into the ventricular chamber or secondarily extending into it from adjacent areas have been approached by means of different transcranial routes. The aim of this work is to report our experience with the endoscopic endonasal approach in the management of a series of patients affected by craniopharyngiomas, extending into or arising from the third ventricle, evaluating pros and cons of this technique, also in regards of the anatomy and the pathology dealt with. During the period between January 2001 and February 2011, 12 patients, 9 male and 3 female (mean age 50.4 years; range 12-68) underwent an endoscopic endonasal approach for the treatment of a craniopharyngioma involving or arising from the third ventricle. According to the grade of involvement of the third ventricle, we identified three main ventricular growth patterns: (1) stalk-infundibulum; (2) infundibulum-ventricular chamber; (3) stalk-infundibulum-ventricular chamber. Though gross total removal was achieved in eight patients (66.7%), in three patients (25%) was possible a near total removal (>95%) and only in one case (8.3%) tumor removal has been partial (<50%). The overall analysis revealed a rate of 77.8% improvement of post-operative visual defects. Concerning the complications, we reported an overall CSF rate of 16.7%; two patients developed a subdural hematoma that has been treated with a surgical drainage. One patient died after the occurrence of a brainstem hemorrhage. The endoscopic endonasal route provides a good exposure, especially of the sub- and retro-chiasmatic areas, as well as of the stalk-infundibulum axis, which represents, when directly involved by a lesion, a gate to access the third ventricle chamber. Despite this study reporting only a preliminary experience, it seems that in properly selected cases--namely tumors growing mostly along the pituitary stem-infundibulum-third ventricle axis--this approach could be advocated as a valid route among the wide kaleidoscope of surgical approaches to the third ventricle.
Postoperative outcome of body core temperature rhythm and sleep-wake cycle in third ventricle craniopharyngiomas.
Zoli Matteo,Sambati Luisa,Milanese Laura,Foschi Matteo,Faustini-Fustini Marco,Marucci Gianluca,de Biase Dario,Tallini Giovanni,Cecere Annagrazia,Mignani Francesco,Sturiale Carmelo,Frank Giorgio,Pasquini Ernesto,Cortelli Pietro,Mazzatenta Diego,Provini Federica
OBJECTIVE One of the more serious risks in the treatment of third ventricle craniopharyngiomas is represented by hypothalamic damage. Recently, many papers have reported the expansion of the indications for the endoscopic endonasal approach (EEA) to be used for these tumors as well. The aim of this study was to assess the outcome of sleep-wake cycle and body core temperature (BCT), both depending on hypothalamic control, in patients affected by craniopharyngiomas involving the third ventricle that were surgically treated via an EEA. METHODS All consecutive adult patients with craniopharyngiomas that were treated at one center via an EEA between 2014 and 2016 were prospectively included. Each patient underwent neuroradiological, endocrinological, and ophthalmological evaluation; 24-hour monitoring of the BCT rhythm; and the sleep-wake cycle before surgery and at follow-up of at least 6 months. RESULTS Ten patients were included in the study (male/female ratio 4:6, mean age 48.6 years, SD 15.9 years). Gross-total resection was achieved in 8 cases. Preoperative BCT rhythm was pathological in 6 patients. After surgery, these disturbances resolved in 2 cases, improved in another 3, and remained the same in 1 patient; also, 1 case of de novo onset was observed. Before surgery the sleep-wake cycle was pathological in 8 cases, and it was restored in 4 patients at follow-up. After surgery the number of patients reporting diurnal naps increased from 7 to 9. CONCLUSIONS The outcome of the sleep-wake cycle and BCT analyzed after EEA in this study is promising. Despite the short duration of the authors' experience, they consider these results encouraging; additional series are needed to confirm the preliminary findings.
Operative outcomes and adjuvant treatment of purely third ventricle craniopharyngioma after a transcallosal approach.
Jung Tae-Young,Jung Shin,Jang Woo-Youl,Moon Kyung-Sub,Kim In-Young,Kang Sam-Suk
British journal of neurosurgery
We reviewed four surgical cases of purely third ventricle craniopharyngioma, focusing on surgical outcomes and adjuvant treatments. From 2002 to 2008, we performed surgical treatments, via a transcallosal transforaminal approach, on four patients. All were males, with a median age of 42 (36-45) years. Most patients complained of headaches, while two (50%) patients presented with visual disturbances, and one (25%) presented with an endocrinological disturbance. Patients' follow-up periods ranged from 1.6 to 8.6 years. We totally removed the tumor in each of the four patients. The tumors originated in the infundibulum of the third ventricular floor. The pituitary stalk was anatomically preserved. The histopathological findings showed the adamantinomatous type of craniopharyngioma in all patients. Postoperatively, two patients who had experienced visual disturbances showed improvement, and there was no aggravation. Two patients had intact pituitary functioning, while two needed complete hormone replacement. The patients experienced no surgery-related complications. Two patients experienced recurrences 4.5 and 1.6 years later. One patient received gamma knife surgery for the recurred lesion, which controlled the lesion well. Purely third ventricle craniopharyngioma showed good visual and endocrinological outcomes after surgery. Gamma knife surgery could be of help in the event of a recurred lesion.
Endoscopic Endonasal Surgery for Purely Intrathird Ventricle Craniopharyngioma.
Nishioka Hiroshi,Fukuhara Noriaki,Yamaguchi-Okada Mitsuo,Yamada Shozo
BACKGROUND:Extended endoscopic transsphenoidal surgery (EETS) is a safe and effective treatment for many suprasellar craniopharyngiomas, including those with third-ventricle involvement. Craniopharyngioma entirely within the third ventricle (purely intraventricular type), however, is generally regarded unsuitable for treatment with EETS. CASE DESCRIPTION:Three patients underwent total removal of a purely intraventricular craniopharyngioma with inferior extension via EETS by direct incision of the bulging, stretched ventricular floor and fine dissection from the ventricular wall. In 2 patients with an anteriorly displaced chiasm, the space between the chiasm and pituitary stalk created a wide corridor to the ventricle, whereas in the third case, in which the infrachiasmal space was somewhat narrowed, partial sacrifice of the pituitary gland was necessary to obtain sufficient space. Despite preservation of the stalk in 2 patients, hypopituitarism and diabetes insipidus developed after surgery. There was no other complication including obesity. CONCLUSIONS:Selected patients with purely intraventricular craniopharyngioma can be treated effectively and safely with EETS. Those with inferior extension in the interpeduncular fossa and anterior displacement of the chiasm may be suitable candidates.
Membrane Structures Between Craniopharyngioma and the Third Ventricle Floor Based on the QST Classification and Its Significance: A Pathological Study.
Qi Songtao,Liu Yi,Wang Chaohu,Fan Jun,Pan Jun,Zhang Xi'an,Lu Yuntao
Journal of neuropathology and experimental neurology
The aim of this study was to clarify the relationship between craniopharyngiomas (CP) and the third ventricle floor by analyzing the membranes between them. Eight fetal specimens were first examined by hematoxylin and eosin and immunofluorescence staining to determine optimal markers for identifying membrane structures in the sellar region. Then, 17 CP with third ventricle floor involvement that had been removed by total en bloc resection through a transsphenoidal approach were examined. We found that the dura mater, arachnoid membrane, and pia mater could be seen to separate type Q tumors from the third ventricle floor. The arachnoid membrane and pia mater could be seen between type S tumors and the third ventricle floor. Pia mater could be seen between type T tumors and the third ventricle floor; however, at the origin point of the tumor, pia mater could be loosened or replaced by the tumor. Although some type T tumors compressed the third ventricle, the ependymal layer remained intact. Based on these embryonic and pathological data, we suggest that CP are nonneuroepithelial, epi-pia mater, and epi-third ventricle tumors.
Surgical management of craniopharyngioma with third ventricle involvement.
de Lara Danielle,Ditzel Filho Leo F S,Muto Jun,Otto Bradley A,Carrau Ricardo L,Prevedello Daniel M
Craniopharyngiomas are notorious for their ability to invade the hypothalamus and third ventricle. Although several transcranial approaches have been proposed for their treatment, the endonasal route provides direct access to the tumor with no need for cerebral retraction or manipulation of the optic apparatus. After the lesion is debulked, the unique angle of approach achieved with this technique enables the surgeon to perform an extra-capsular dissection and visualize the walls of the third ventricle, the foramina of Monro, and the anterior comissure. Moreover, the enhanced magnification and lighting afforded by the endoscope facilitate safe tumor removal, particularly in areas where there is loss of clear lesion delimitation and greater infiltration of the surrounding structures. Herein we present the case of a 68-year-old female patient with a 3-month history of visual deterioration accompanied by worsening headaches. Investigation with magnetic resonance imaging revealed a heterogeneous mass in the suprasellar region, extending into the third ventricle and displacing the pituitary gland and stalk inferiorly. Hormonal profile was within expected range for her age. An endonasal, fully endoscopic, transplanum transtuberculum approach was performed. Gross-total removal was achieved and pathology confirmed the diagnosis of craniopharyngioma. Postoperative recovery was marked by transient diabetes insipidus. Closure was achieved with a pedicled nasoseptal flap; despite exploration of the third ventricle, there was no cerebrospinal fluid leakage. Pituitary function was preserved. Visual function has fully recovered and the patient has been uneventfully followed since surgery. The video can be found here: http://youtu.be/it5mpofZl0Q. (http://thejns.org/doi/abs/10.3171/2013.V1.FOCUS12330)