Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma.
Ayala A R,Basaria S,Udelsman R,Westra W H,Wand G S
The Journal of clinical endocrinology and metabolism
Although nonsecreting suprarenal embryonic remnants are frequently found in the urogenital tract, adenomatous transformation resulting in glucocorticoid excess is a rare phenomenon. We report a case of a 63-yr-old woman that presented with new-onset hirsutism, facial plethora, hypertension, centripetal obesity, and a proximal myopathy. The 24-h urinary free cortisol excretion rate was elevated, and the serum ACTH level was suppressed. The patient failed an overnight and low dose dexamethasone suppression test and did not respond to CRH stimulation. In light of the undetectable baseline morning ACTH levels and the blunt response to CRH, the diagnosis of corticotropin-independent Cushing's syndrome was made. Imaging studies revealed normal adrenal glands and enlargement of a left pararenal nodule incidentally observed 4 yr before the onset of symptoms. Dramatic resolution of symptoms was observed after surgical removal of the 3.5-cm mass. Pathological exam confirmed adrenocortical adenoma in ectopic adrenal tissue. The case reported here represents the unusual circumstance in which the development of adenomatous transformation of ectopic adrenal tissue has been prospectively observed with imaging studies. It illustrates the importance of considering ectopic corticosteroid-secreting tumors in the context of corticotropin-independent Cushing's syndrome.
Recurrent Cushing's syndrome due to recurrent adrenocortical tumor--fragmentation or tumor in ectopic adrenal tissue?
Leibowitz J,Pertsemlidis D,Gabrilove J L
The Journal of clinical endocrinology and metabolism
A 33-yr-old woman was found to have Cushing's syndrome due to a left adrenal cortical tumor. The tumor and the surrounding adrenal gland were removed intact and in toto. Four years later, she noticed recurrent symptoms of Cushing's syndrome, and 6 yr postoperatively, an adrenal tumor was demonstrable on computed tomography. Fourteen years after the initial procedure, a left adrenal tumor, presumably arising in ectopic adrenal tissue, was removed with relief of her symptoms of Cushing's syndrome. The site and functional capacity of ectopic adrenal tissues are reviewed.
Spinal adrenal cortical adenoma with oncocytic features: report of the first intramedullary case and review of the literature.
Cassarino David S,Santi Mariarita,Arruda Arnaldo,Patrocinio Regia,Tsokos Maria,Ghatak Nitya,Quezado Martha
International journal of surgical pathology
Ectopic adrenal cortical neoplasms are extremely rare, and only a few have involved the CNS. We report the first case of an intramedullary oncocytic adrenal cortical neoplasm of the spinal cord with immunohistochemical (IMHC) confirmation. A 27-year-old man presented with progressive lower extremity weakness, spastic paraparesis, decreased reflexes, and hypoesthesia below T10. A spinal myelogram showed cauda equina blockade and obliteration of sacral nerve roots. This prompted emergent surgical intervention. A well-circumscribed, approximately 3 x 2 cm, light brown to tan, intramedullary tumor was identified at the level of the conus medullaris. Histologically, the tumor showed sheets and nests of plump, cytologically bland polygonal cells with abundant eosinophilic cytoplasm. A single mitosis, but no necrosis, was identified. By IMHC, the cells were positive for inhibin, melan-A, and synaptophysin, and negative for GFAP, EMA, cytokeratins, S-100, HMB-45, and chromogranin. Electron microscopy study performed from paraffin-embedded tissues demonstrated abundant mitochondria, and lipid vacuoles. This case confirms the occurrence of adrenal cortical neoplasms in the CNS and is the first report of an intradural, intramedullary adrenal cortical adenoma of the spinal cord, and the first to occur in a male. This tumor should be considered in the differential diagnosis of tumors of the CNS.
Hyperandrogenism in a postmenopausal woman: a rare case of ectopic adrenal cortical gland.
Guarino Antonella,Di Benedetto Luisa,Giovanale Valentina,Rampioni Vinciguerra Gian Luca,Stoppacciaro Antonella,Bellati Filippo,Caserta Donatella
Gynecological endocrinology : the official journal of the International Society of Gynecological Endocrinology
Most frequent causes of androgenic manifestation are Cushing's syndrome, PCO, benign and malignant androgen-secreting non adrenal tumors and iatrogenic hirsutism. Hyperplasia or neoplasms of ectopic adrenocortical gland are rare. We report a case of a 63-year old female with hirsutism and alopecia. Laboratory data highlighted increased levels of androgens. Diagnostic imaging revealed normal morphology of adrenocortical gland and ovaries. In view of the clinical picture and suspected diagnosis of extra-adrenal cause, she underwent bilateral salpingo-oophorectomy. Histologic examination showed an ectopic adrenal gland with adenoma in the ovarian and peri-ovarian tissue. At six months of follow up, the patients has no sign of hyperandrogenism. In case of hyperandrogenism in postmenopausal women and in the absence of the adrenocortical gland abnormality, ovarian origin should be considered in the differential diagnosis.
Intrarenal ectopic adrenal tissue and renal-adrenal fusion: a report of nine cases.
Ye Huihui,Yoon Ghil Suk,Epstein Jonathan I
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
Intrarenal ectopic adrenal tissue and renal-adrenal fusion are rare findings in the adult population. We reviewed seven cases of intrarenal adrenal tissue and two cases of renal-adrenal fusion. Patients ranged in age from 35 to 75 years (mean 55). Ectopic adrenal tissue was identified at the superior pole of the kidney in all but one case, which was located in the mid-portion of the kidney. Ectopic adrenal tissue varied in its growth from subcapsular lesions that were plaque-like (n=3), wedge-shaped (n=2), or spherical (n=1) to irregular nests deep in the renal parenchyma (n=1). In all nine cases, the adherent and intrarenal adrenal tissue was composed of adrenal cortical tissue, with no adrenal medullary tissue present. In six cases, adrenal tissue focally extended into renal parenchyma in an infiltrative manner. Of the nine cases, two were diagnostic problems for the contributing pathologists. In one case, intrarenal adrenal tissue mimicked low-grade clear cell renal cell carcinoma. In another case, an adrenocortical adenoma adherent to the kidney resembled renal invasion by adrenocortical carcinoma. This study summarizes key morphological features of intrarenal ectopic adrenal tissue and renal-adrenal fusion along with histological pitfalls and its differential diagnoses.
Oncocytic adrenocortical neoplasm arising from adrenal rest in the broad ligament of the uterus.
Kasajima Atsuko,Nakamura Yasuhiro,Adachi Yuzu,Takahashi Yayoi,Fujishima Fumiyoshi,Chiba Yutaka,Uehara Shigeki,Watanabe Mika,Sasano Hironobu
Oncocytic adrenocortical neoplasm is characterized by abundant eosinophilic cytoplasm containing mitochondria, occasional nuclear atypia and diffuse growth pattern. Oncocytic adrenocortical neoplasm arising in adrenal rest is, however, extremely rare. We report a case of oncocytic adrenocortical neoplasm arising in adrenal rest of the broad ligament with associated marked lipomatous metaplasia. A well circumscribed tumor was accidentally detected in the pelvic cavity of a 29 year old Japanese woman, adjacent to the broad ligament of the uterus. The tumor was composed of large eosinophilic cells associated with diffuse growth pattern and abundant mature adipose tissue admixed with foci of clear cells. Both steroidgenic factor 1 (SF-1) and alpha-inhibin were immunohistochemically positive in tumor cells. Abundant mitochondria detected by immunohistochemical and electron microscopic examination confirmed the diagnosis of oncocytic adrenocortical neoplasm. The absence of necrosis, capsular and vascular invasion as well as the low mitotic index indicated the benign potential of this tumor. The tumor cells were also positive for dehydroepiandrosteron-sulfotransferase (DHEA-ST), 17β-hydroxysteroid dehydrogenase type 5 (17β-HSD5), 3β-hydroxysteroid dehydrogenase (3β-HSD) and steroid 17α-hydroxylase (P450-c17), suggesting a possible production of testosterone of this tumor. This is the first reported case of oncocytic adrenocortical adenoma arising in adrenal rest of the broad ligament.
Laparoscope resection of ectopic corticosteroid-secreting adrenal adenoma.
Wang Xian-Ling,Dou Jing-Tao,Gao Jiang-Ping,Zhong Wen-Wen,Jin Du,Hui Lüzhao,Lu Ju-Ming,Mu Yi-Ming
Neuro endocrinology letters
Tumors originating from ectopic adrenal tissue are relatively rare. In this article, we describe a case with Cushing's syndrome caused by an ectopic adrenal adenoma. A 38 year-old male patient presenting with cushingoid appearance for 2 years was diagnosed to have ACTH-independent Cushing's syndrome based on endocrinological evaluation. Mutiple radiological examinations detected bilateral adrenal atrophy. When the images were investigated in a more expanded scope, a 3.0×3.5×5.3 cm mass was detected in the anterior of left renal hilum and left renal vein. The mass was successfully resected with intraoperative endoscopy and pathological evaluation revealed an ectopic adrenal tumor. It is suggested that when the endocrinlogically confirmed adrenal neoplasm could not be well and definitely localized, the possibility of ectopic adrenal should be presumed and further radiography examinations should extend to the field where ectopic adrenal usually presents.
Adrenal Cortical Adenoma in the Spinal Canal: A Case Report and Review of the Literature.
Nemir Jakob,Štenger Martina,Jakovčević Antonia,Domazet Ivan,Njirić Niko,Paladino Josip
Journal of neurological surgery. Part A, Central European neurosurgery
Ectopic adrenal cortical neoplasms of the spinal cord are extremely rare. To date only 10 such cases have been described. We present a case of a 46-year-old woman with lower back pain radiating to the right gluteal and posterior femoral regions, without a history of traumatic injury. Magnetic resonance imaging (MRI) of the thoracic and lumbar spine showed an intradural, extramedullary, well-circumscribed, contrast-enhancing lesion located in the T12-L1 region, hypo- to isointense on T2-weighted imaging, and isointense on T1. Complete surgical removal of the lesion, measuring 3 × 2.5 × 1 cm, was performed. The histopathologic findings revealed the lesion was an ectopic adrenal cortical adenoma, with sheets and nests of round and polygonal cells, mostly round regular nuclei, abundant eosinophilic cytoplasm, 1 mitosis per 10 high-power fields, and without necrosis. These tumors have nonspecific MRI features and therefore can be easily confused with other common spinal tumor types such as ependymoma, schwannoma, meningioma, and metastasis. Although rare, ectopic adrenal spinal cord adenomas should be taken into account in the differential diagnosis of spinal canal intradural neoplasms.
Secreting ectopic adrenal adenoma: A rare condition to be aware of.
Zhao Yang,Guo Hui,Zhao Ying,Shi Bingyin
Ectopic adrenal adenoma causing chronic Cushing's syndrome (CS) is a rare phenomenon. Diagnosis is usually made years after disease onset because of the insidious nature of the ectopic adrenal gland and because it overlaps with common symptoms, such as overweight and hypertension, in the general population (Kreitschmann-Andermahr et al., 2015). Here, we report the case of a 46-year-old male with a 15-year history of severe hypertension, facial plethora, and centripetal obesity. During treatment for herpes zoster, the patient presented with severe hypokalemia and flaccid paralysis, characteristic changes associated with CS. The serum cortisol level was elevated and baseline adrenocorticotrophic hormone (ACTH) was suppressed. After administration of the low-dose overnight dexamethasone suppression test, plasma cortisol was 38.9μg/dL. A contrast computed tomography (CT) scan revealed normal adrenal glands and significant tumor adjacent to the right renal hilum measuring ∼3.6×2.3cm. The tumor was removed through retroperitoneoscopy, and pathological examination confirmed adrenocortical adenoma and myelolipoma metaplasia. The serum potassium level gradually became normal after surgical removal of the mass. In the current report, we have reviewed the pertinent literature and highlighted the importance of considering ectopic adrenal adenoma in the differential diagnosis of chronic CS with nonspecific symptoms.
Ectopic adrenal cortical adenoma in the gastric wall: case report.
Ren Pei-Tu,Fu Hong,He Xiao-Wen
World journal of gastroenterology
Ectopic adrenal cortical neoplasms are extremely rare. Ectopic adrenocortical tissue can be found in locations such as the celiac axis, the broad ligament, the adnexa of the testes, and the spermatic cord; however, they rarely involve the stomach. We report an unusual case of a patient with an ectopic adrenal cortical adenoma in the gastric wall. The patient was a 72-year old female admitted to our hospital with upper abdominal discomfort. Physical examination revealed tenderness below the xiphoid process. Both computed tomography and fibergastroscopy revealed a mass on the lesser curvature side of the gastric antrum; it was initially diagnosed as a gastric stromal tumor. After adequate preparation, the patient underwent surgery. During the procedure, we found a 30 mm × 30 mm mass with medium density in the lesser curvature near the gastric antrum within the serosa. Following immunohistochemistry examination, we corrected the diagnosis to an ectopic adrenal cortical adenoma; the tumor was nonfunctional.
An ectopic adreocortical adenoma of the renal sinus: a case report and literature review.
Zhang Jiexiu,Liu Bianjiang,Song Ninghong,Lv Qiang,Wang Zenjun,Gu Ming
BACKGROUND:Ectopic adrenal tumors are very rare, especially in the renal sinus in adults. An unusual case of ectopic adrenal cortical adenoma in the right renal sinus is reported here. CASE PRESENTATION:This patient was a 37-year-old woman. She was admitted to our hospital for hypertension and bilateral limb weakness. Computed tomography (CT) revealed a mass in right renal sinus. It was initially considered a tumor of the renal pelvis. Further computed tomographic angiography (CTA) showed the mass to be located outside the renal pelvis. After adequate preoperative preparation (blood pressure control and serum potassium supplement), the patient underwent laparoscopic resection of retroperitoneal tumor. During the procedure, a soft tissue tumor 3.4*3.0 cm(2) in size with a golden color was found in the right renal sinus. The final immunohistochemistry examination showed an ectopic adreocortical adenoma. CONCLUSION:Ectopic adrenal tumors are rare in the renal sinus and difficult to diagnose and treat. Large and functional tumors should be treated with complete resection. The procedure is sometimes difficult for tumors located deep in the renal sinus. The decision to perform an open or minimally invasive surgery should be made according to the surgeon's experience.
Intrarenal Adrenocortical Adenoma Treated by Robotic Partial Nephrectomy with Adrenalectomy.
Sappal Samay,Sulek Jay,Smith Steven C,Hampton Lance J
Journal of endourology case reports
BACKGROUND:We present an intrarenal adrenocortical adenoma discovered incidentally after robot-assisted partial nephrectomy and total adrenalectomy for a suspicious renal mass. Current literature describes the rare occurrence of an adrenocortical adenoma arising from a renal-adrenal fusion. This case represents an uncommon, benign pathology that should be considered in the differential diagnosis of an enhancing renal mass. CASE PRESENTATION:The patient is a 62-year-old female found to have an enhancing mass at the anterolateral aspect of the upper pole of the right kidney concerning for renal-cell carcinoma. CT imaging was performed to work up a cause for hyperparathyroidism. During robot-assisted partial nephrectomy, the lesion was found to be partially adherent to the lateral limb of the right adrenal gland. Microscopic evaluation with Melan-A staining showed the mass to be of adrenal origin with benign features and lack of capsulation, indicating an adrenal adenoma arising from intrarenal ectopic adrenal rests. CONCLUSION:An intrarenal adrenal adenoma arising from ectopic adrenal tissue is a unique pathology that represents a benign differential diagnosis in the evaluation of an enhancing renal mass. However, it cannot be differentiated from renal-cell carcinoma based on cross-sectional imaging alone and requires postoperative pathologic assessment to confirm the diagnosis.
A unique case of synchronous functional adrenocortical adenoma and myelolipoma within the ectopic adrenal cortex in a child with Beckwith-Wiedemann syndrome.
Cardinalli Izilda A,de Oliveira-Filho Antonio G,Mastellaro Maria José,Ribeiro Raul C,Aguiar Simone S
Pathology, research and practice
We report a unique case of synchronous functional adrenocortical adenoma and an incidental myelolipoma within ectopic cortical adrenal tissue located in the renal hilum in a child with Beckwith-Wiedemann syndrome and review the association between adrenal gland disorders and myelolipomas. To the best of our knowledge, this is the first documented case of a simultaneous occurrence of these three conditions. A 17-month-old child with Beckwith-Wiedemann syndrome was diagnosed with a left adrenal tumor during complementary radiologic studies. Biochemical investigation before surgery showed elevated blood levels of cortisol and dehydroepiandrosterone hormones. The patient underwent a left adrenalectomy with ipsilateral renal hilar and intercaval-aortic lymph node dissection. Pathology findings revealed a left adrenocortical adenoma and an incidental myelolipoma growing within ectopic cortical adrenal tissue in the renal hilum. The patient is doing well and does not have any current health issues. Patients with adrenal cortex disorders, such as hyperplasias and neoplasms, particularly when associated with hormonal imbalances, may have an increased risk of developing myelolipomas. Whether Beckwith-Wiedemann syndrome may, by itself, contribute to simultaneous occurrence of adrenocortical adenomas and myelolipomas remains to be clarified.
ACTH-independent Cushing's syndrome with bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma.
Louiset Estelle,Gobet Françoise,Libé Rossella,Horvath Anelia,Renouf Sylvie,Cariou Juliette,Rothenbuhler Anya,Bertherat Jérôme,Clauser Eric,Grise Philippe,Stratakis Constantine A,Kuhn Jean-Marc,Lefebvre Hervé
The Journal of clinical endocrinology and metabolism
CONTEXT:Bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma are two rare causes of ACTH-independent Cushing's syndrome. OBJECTIVE:The aim of the study was to evaluate a 35-yr-old woman with ACTH-independent hypercortisolism associated with both micronodular adrenal hyperplasia and ectopic pararenal adrenocortical adenoma. DESIGN AND SETTING:In vivo and in vitro studies were performed in a University Hospital Department and academic research laboratories. INTERVENTION:Mutations of the PRKAR1A, PDE8B, and PDE11A genes were searched for in leukocytes and adrenocortical tissues. The ability of adrenal and adenoma tissues to synthesize cortisol was investigated by immunohistochemistry, quantitative PCR, and/or cell culture studies. MAIN OUTCOME MEASURE:Detection of 17alpha-hydroxylase and 21-hydroxylase immunoreactivities, quantification of CYP11B1 mRNA in adrenal and adenoma tissues, and measurement of cortisol levels in supernatants by radioimmunological assays were the main outcomes. RESULTS:Histological examination of the adrenals revealed nonpigmented micronodular cortical hyperplasia associated with relative atrophy of internodular cortex. No genomic and/or somatic adrenal mutations of the PRKAR1A, PDE8B, and PDE11A genes were detected. 17alpha-Hydroxylase and 21-hydroxylase immunoreactivities as well as CYP11B1 mRNA were detected in adrenal and adenoma tissues. ACTH and dexamethasone activated cortisol secretion from adenoma cells. The stimulatory action of dexamethasone was mediated by a nongenomic effect involving the protein kinase A pathway. CONCLUSION:This case suggests that unknown molecular defects can favor both micronodular adrenal hyperplasia and ectopic adrenocortical adenoma associated with Cushing's syndrome.
Ectopic adrenocortical adenoma in the renal hilum: a case report and literature review.
Liu Yang,Jiang Yue-Feng,Wang Ye-Lin,Cao Hong-Yi,Wang Liang,Xu Hong-Tao,Li Qing-Chang,Qiu Xue-Shan,Wang En-Hua
BACKGROUND:Ectopic (accessory) adrenocortical tissue, also known as adrenal rests, is a developmental abnormality of the adrenal gland. The most common ectopic site is in close proximity to the adrenal glands and along the path of descent or migration of the gonads because of the close spatial relationship between the adrenocortical primordium and gonadal blastema during embryogenesis. Ectopic rests may undergo marked hyperplasia, and occasionally induce ectopic adrenocortical adenomas or carcinomas. CASE PRESENTATION:A 27-year-old Chinese female patient who presented with amenorrhea of 3 months duration underwent computed tomography urography after ultrasound revealed a solitary mass in the left renal hilum. Histologically, the prominent eosinophilic tumor cells formed an alveolar- or acinar-like configuration. The immunohistochemical profile (alpha-inhibin+, Melan-A+, synaptophysin+) indicated the adrenocortical origin of the tumor, diagnosed as ectopic adrenocortical adenoma. The patient was alive with no tumor recurrence or metastasis at the 3-month follow-up examination. CONCLUSIONS:The unusual histological appearance of ectopic adrenocortical adenoma may result in its misdiagnosis as oncocytoma or clear cell renal cell carcinoma, especially if the specimen is limited. This case provides a reminder to pathologists to be aware of atypical cases of this benign tumor. Although uncommon, an ectopic adrenal lesion should be included in the differential diagnosis of tumors involving the renal hilum. A misdiagnosis of this benign condition as a malignant renal tumor may have severe consequences for the patient, including unnecessary radical nephrectomy. Preoperative biopsy and appropriate immunohistochemical staining will assist in determining the origin and nature of the tumor and in avoiding intraoperative uncertainty.
An ectopic adrenocortical adenoma in renal hilum presenting with Cushing's syndrome: A case report and literature review.
Lu Difei,Yu Nan,Ma Xiaowei,Zhang Junqing,Guo Xiaohui
RATIONALE:Ectopic adrenal tissue is the adrenal rests along the path from gonads to adrenal glands during embryogenesis. Ectopic adrenocortical adenoma is a rare disease represented with over-production of cortisol by the ectopic adrenocortical tissue. PATIENT CONCERNS:An 18-year-old Chinese female patient was presented with weight-gain for 6 months. She had elevated plasma cortisol and a solitary mass was revealed using computed tomography scan in the left renal hilum. DIAGNOSIS:The tumor was removed and the immunohistochemical profile indicated an ectopic adrenocortical adenoma. INTERVENTIONS:After the tumor was removed, the patient was under glucocorticoid replacement therapy in 6-month. OUTCOMES:During 6-month of follow-up, the patient showed no signs of tumor recurrence. LESSONS:Ectopic adrenocortical adenoma is difficult to diagnose due to its low incidence, and the ectopic rests in renal hilum could be misdiagnosed as renal cell carcinoma. This case reminds clinicians to be aware of ectopic site in the diagnosis of adrenocorticotropic hormone (ACTH) independent Cushing's syndrome. Immunohistochemical stain may assist in evaluating the origin of the ectopic rests. A certain rate of local recurrence indicated the need of long-term follow-up.
Incidental retroperitoneal oncocytoma (Ectopic oncocytic adrenocortical adenoma): Case report and review of the literature.
Saygin Ismail,Cakir Emel,Ercin Mustafa Emre,Eyüboğlu Ilker
Indian journal of pathology & microbiology
Ectopic adrenocortical tissue may be present in many anatomical localizations. Hyperplasia, adenoma or carcinoma can occasionally develop from the ectopic adrenal tissue. Therefore, it should be surgically excised when it is detected. Adrenocortical tumors are the most common type of adrenal neoplasms. Ectopic adrenocortical adenomas are rarely seen. A total of 34 cases of ectopic adrenocortical adenoma (14 of which are oncocytomas) have been reported at different localizations in English literature. Most of them are non-functional. Differential diagnosis is required with other benign or malign oncocytic neoplasms. We report a 56-year-old male patient, who presented with a retroperitoneal mass. Our case is the seventh case of ectopic retroperitoneal adrenal adenoma with oncocytic cells.
Scalp hair cortisol for diagnosis of Cushing's syndrome.
Wester Vincent L,Reincke Martin,Koper Jan W,van den Akker Erica L T,Manenschijn Laura,Berr Christina M,Fazel Julia,de Rijke Yolanda B,Feelders Richard A,van Rossum Elisabeth F C
European journal of endocrinology
OBJECTIVE:Current first-line screening tests for Cushing's syndrome (CS) only measure time-point or short-term cortisol. Hair cortisol content (HCC) offers a non-invasive way to measure long-term cortisol exposure over several months of time. We aimed to evaluate HCC as a screening tool for CS. DESIGN:Case-control study in two academic referral centers for CS. METHODS:Between 2009 and 2016, we collected scalp hair from patients suspected of CS and healthy controls. HCC was measured using ELISA. HCC was available in 43 confirmed CS patients, 35 patients in whom the diagnosis CS was rejected during diagnostic work-up and follow-up (patient controls), and 174 healthy controls. Additionally, we created HCC timelines in two patients with ectopic CS. RESULTS:CS patients had higher HCC than patient controls and healthy controls (geometric mean 106.9 vs 12.7 and 8.4 pg/mg respectively, < 0.001). At a cut-off of 31.1 pg/mg, HCC could differentiate between CS patients and healthy controls with a sensitivity of 93% and a specificity of 90%. With patient controls as a reference, specificity remained the same (91%). Within CS patients, HCC correlated significantly with urinary free cortisol ( = 0.691, < 0.001). In two ectopic CS patients, HCC timelines indicated that cortisol was increased 3 and 6 months before CS became clinically apparent. CONCLUSIONS:Analysis of cortisol in a single scalp hair sample offers diagnostic accuracy for CS similar to currently used first-line tests, and can be used to investigate cortisol exposure in CS patients months to years back in time, enabling the estimation of disease onset.
Ectopic cortisol-producing adrenocortical adenoma in the renal hilum: histopathological features and steroidogenic enzyme profile.
Tong Anli,Jia Aihua,Yan Shujie,Zhang Yan,Xie Yi,Liu Guanghua
International journal of clinical and experimental pathology
Ectopic cortisol-producing adrenocortical adenomas (CPA) are extremely rare, and only four cases have previously been reported so far but the tumors were not ultrastructurally studied. Presented in this paper is the fifth case with ectopic CPA which was extensively examined to gain deeper insights in terms of the histopathological features and steroidogenic enzyme profile of the tumor. A 53-year-old woman complained of accidental discovery of left renal mass. She had a 5-year history of hypertension, weight gain, moon face, thin skin and systemic edema. These symptoms completely relieved after the tumor removal. Two years later, the above symptoms recurred, and a recurrent tumor was revealed in left renal hilum. The tumor was removed completely with relief of her symptoms of Cushing's syndrome. Histologically and ultrastructurally, the tumor was composed of compact cells and clear cells, and the former was prominent, suggesting an active secretory function of the tumor. The adenoma tissue showed a strong immunostaining for Melan-A, 3beta-hydroxysteroid dehydrogenase (HSD3B2) and 17alpha-hydroxylase1 (CYP17A1). Expression pattern for 11beta-hydroxylase 1 (CYP11B1), 11beta-hydroxylase 2 (CYP11B2), CYP17 and HSD3B2 mRNA in ectopic CPA was similar to that in the adrenal CPA. In conclusion, in terms of histopathological characteristic and steroidogenic enzyme profile, ectopic CPA is similar to adrenal CPA, suggesting that they are of identical cell origin.
Ectopic Cortisol-producing Adrenocortical Adenoma Detected by 131I-6β-iodomethyl-norcholesterol Scintigraphy.
Sato Seisuke,Imachi Hitomi,Kobayashi Toshihiro,Fukunaga Kensaku,Lyu Jingya,Dong Tao,Yoshioka Yuuki,Saheki Takanobu,Fukata Youko,Ban Natsuki,Urushihara Kurumi,Kadota Kyuuichi,Murao Koji
Internal medicine (Tokyo, Japan)
A 50-year-old man was referred to our department for overt Cushing's syndrome (CS). His plasma cortisol concentrations were 314 μg/L, and his urinary cortisol concentrations were 431 μg/day. The plasma adrenocorticotropic hormone (ACTH) concentration was below the detectable limit. Computed tomography revealed atrophy of both adrenal glands and the presence of a left pararenal tumor. 131I-6β-iodomethyl-norcholesterol scintigraphy showed an intense uptake by the left pararenal tumor. These findings suggested that the left pararenal tumor was ectopic cortisol-producing adrenocortical adenoma. This case serves as a reminder that 131I-6β-iodomethyl-norcholesterol scintigraphy is an effective method for diagnosing ACTH-independent CS in which no adrenal tumor has been found.