Programmed death ligand 1 and CD8+ immune cell infiltrates in resected primary tracheal malignant neoplasms.
Tapias Luis F,Shih Angela,Mino-Kenudson Mari,Muniappan Ashok,Gaissert Henning A,Lanuti Michael,Mathisen Douglas J,Ott Harald C
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
OBJECTIVES:Many patients with primary malignant tracheal neoplasms are not surgical candidates nor do they experience residual or recurrent disease after surgery and may benefit from alternative therapies. This study explores the expression of programmed death ligand 1 (PD-L1) in patients with primary tracheal malignancy as a biomarker for candidacy for treatment with immune checkpoint inhibitors. METHODS:We conducted a retrospective review of the medical records of 23 patients with resected primary tracheal malignant tumours from 2010 to 2016. Paraffin-embedded blocks of tumour tissue were evaluated immunohistochemically to determine the expression of PD-L1 and infiltration by CD8+ immune cells. RESULTS:We identified 14 (61%) adenoid cystic carcinomas, 4 (17%) squamous cell carcinomas (SCC), 4 (17%) mucoepidermoid carcinomas and 1 adenosquamous carcinoma. PD-L1 expression was observed in 3 (75%) cases of SCC and 1 (100%) case of adenosquamous carcinoma, but it was absent in cases of adenoid cystic carcinomas and mucoepidermoid carcinomas. PD-L1 expression was significantly higher in tumours with a SCC component than in salivary-type tumours (P = 0.001). The presence of CD8+ immune cells in the tumour or peritumoural stroma was significantly higher in cases of tracheal tumours with a SCC component than in salivary-type tumours. CONCLUSIONS:Salivary-type primary malignant tracheal tumours do not significantly express PD-L1. In contrast, most primary tracheal tumours with a SCC component show membranous expression of PD-L1 and larger numbers of infiltrating CD8+ immune cells. PD-L1 expression may serve as a biomarker in patients with primary tracheal squamous cell malignant neoplasms when the patients are being considered for alternative treatments and inclusion in clinical trials. IRB APPROVAL:Protocol No. 2017P000415 (22 March 2017).
10.1093/ejcts/ezy370
[Clinicopathological features and prognostic factors of primary pulmonary adenoid cystic carcinoma: a study of 59 cases].
Han J,Gao X Z,Wei J G,Xie Y L,Liu Y Q,Li W C,Li S L
Zhonghua bing li xue za zhi = Chinese journal of pathology
To investigate the clinicopathological features and prognostic indicators of primary pulmonary adenoid cystic carcinoma. Fifty-nine cases of primary pulmonary adenoid cystic carcinoma were collected from August 2011 to December 2017 at the First Affiliated Hospital of Zhengzhou University. All cases were retrospectively studied by hematoxylin-eosin staining and immunohistochemistry. The clinicopathological features were reviewed and patient survival analysis was performed using Kaplan-Meier method and Cox regression model. Status of epidermal growth factor receptor (EGFR), KRAS, BRAF genes was analyzed in 15 of the 59 study cases. Among 59 cases, there were 25 males and 34 females with male to female ratio of 1.0 to 1.4. The patient age ranged from 29 to 81 years with a mean age of 55 years. The tumor max diameters ranged from 1.0 to 9.6 cm with an average diameter of 2.8 cm. Fifteen (25.4%) patients were smokers while 44 patients (74.6%) were non-smokers. Tumors predominantly occurred in the trachea (28/59,47.5%), the left main bronchus (7/59,11.9%) and the right bronchus (5/59,8.5%). Grossly, the tumors were well circumscribed, greyish-white nodules. Microscopically the tumor cells were small and uniform, and arranged in tubular, cribriform, and solid patterns. Immunohistochemistry showed that the tumor cells were positive for CK7, S-100 protein, Sox-10, CD117 and p63. TTF1 was only positive in 2 cases and Ki-67 index ranged from 3% to 40%. Eighteen cases (30.5%) were gradeⅠ, 26 cases (40.1%) grade Ⅱ, and 15 cases (25.4%) grade Ⅲ. Overall, 39 cases (66.1%), 7 cases (11.9%), 10 cases (16.9%), and 3 cases (5.1%) were at stages Ⅰ, Ⅱ, Ⅲ, and Ⅳ, respectively. Twenty-three patients (39.0%) received surgical therapy, 3 patients (5.1%) surgery combined with radiotherapy, 9 patients (15.2%) surgery combined with chemotherapy, and 24 cases (40.7%) chemotherapy only. No mutation of EGFR, KRAS and BRAF was detected in all 15 tested cases. The overall survival rate at the first, third and fifth years was 94.9%, 86.4% and 84.7%, respectively. Prognostic analysis showed that patient's age and tumor size were statistically associated with the survival (0.05). Majority of the patients with primary pulmonary adenoid cystic carcinoma are at an early clinical stage with a favorable prognosis. The size of the tumor and the age of the patients are independent prognostic indicators.
10.3760/cma.j.issn.0529-5807.2019.03.007
Combined surgery and radiotherapy as curative treatment for tracheal adenoid cystic carcinoma: a case report.
Spinelli Gian Paolo,Miele Evelina,Prete Alessandra Anna,Lo Russo Giuseppe,Di Marzo Alessandro,Di Cristofano Claudio,Tomao Silverio
Journal of medical case reports
BACKGROUND:Adenoid cystic carcinoma of the trachea is a rare tumor, characterized by slow growth and low rate of local and distant metastasis. When achievable, complete surgical resection represents the optimal treatment approach, with the highest results in terms of overall survival. Radiation therapy is a reasonable alternative in cases of inoperable disease. CASE PRESENTATION:We report a case of an 82-year-old white man affected by primary adenoid cystic carcinoma of the trachea, treated with debulking surgery and radiotherapy on the residual disease. A three-dimensional conformal radiation therapy was conducted. The total dose amounted to 70 Gy, administered in 35 fractions of 2 Gy. The medium doses given to the esophagus and lungs were 23 Gy and 4.2 Gy respectively. The maximum dose delivered to the spinal cord was 31 Gy with satisfactory results in terms of local control of the disease. CONCLUSION:A combined approach of surgical resection followed by radiotherapy on the residual disease provided an excellent result in terms of disease control, quality of life, and overall survival in a patient with locally advanced tracheal adenoid cystic carcinoma.
10.1186/s13256-019-1996-9
Demography, patterns of care and survival outcomes in patients with malignant tumors of trachea: A systematic review and individual patient data analysis of 733 patients.
Mallick Supriya,Benson Rony,Giridhar Prashanth,Rajan Singh Angel,Rath Goura K
Lung cancer (Amsterdam, Netherlands)
BACKGROUND:Malignant tumors of the trachea (MTT) account for 0.01-0.4% of all cancer cases. The rarity of the tumor along with different histologies makes it is a great challenge on how to optimally treat tracheal tumors and most of the available data is from small retrospective data series. We performed a systematic review and individual patient data analysis to evaluate the patterns of care and survival outcomes in patient with MTT. METHODOLOGY:A comprehensive search in Pub Med and Google scholar was done to find all possible publications related to malignant tumors of the trachea. The data on patient demograpphy, treatment, survival and recurrence pattern of individual patient was collected from the published data and was entered in a predesigned proforma. Progression free survival [PFS] and overall survival [OS] was calculated from the date of diagnosis to the date of documented progression and death respectively. Kaplan- Meier method was used for survival analysis and uni-variate analysis was performed using log rank test. SPSS v16 was used for all statistical analysis. RESULTS:733 patients were included in this analysis. The most common histology was adenoid cystic carcinoma (ACC) followed by squamous cell carcinoma (SCC). The gender ratio was 4.43: 1[male: female] in patients with SCC while it was 0.85:1[male: female] in ACC. Smoking and age >50 years were associated with worse OS. The estimated median overall survival for entire cohort was 96 months. Survival was significantly better in patients with ACC than in patients with SCC [165 vs. 14 months, p < 0.001]. The use of definitive surgery was associated with a significantly better survival of 180 months when compared to 48 months with radiation as local therapy, [p < 0.001]. The radiation dose used also affected survival in patients with SCC with a better median OS of 24 months in patients who recieved more than 60 Gy vs 6 months in whom the dose was less than 60 Gy although not statistically significant (p = 0.011). CONCLUSION:ACC and SCC are the most common MTT. ACC has better prognosis compared to SCC. Surgery seems to provide better outcomes than radiation for ACC and sarcoma. Role of definitive radiotherapy versus surgery in SCC needs to be further studied.
10.1016/j.lungcan.2019.04.017
Tracheo-bronchial adenoid cystic carcinoma: A retrospective study.
Ning Ye,He Wenxin,Bian Dongliang,Xie Dong,Jiang Gening
Asia-Pacific journal of clinical oncology
OBJECTIVES:Adenoid cystic carcinoma (ACC) of the trachea and bronchus is rare, representing 1% of all respiratory tract cancers. We presented our experiences in treating tracheal-bronchial ACC and the results of long-term surveillance. METHODS:We conducted a retrospective study of treating tracheo-bronchial ACC. From 2009 to 2014, 42 patients presented to our department. All of them received surgical resection and adjunctive therapy. RESULTS:R0 resections were achieved in 33 patients, whereas 15 patients whose lesions spread outside the tracheo-bronchial lumens. Nine patients had R1 resections followed by radiation and chemotherapy. The 5 year survival rate of R1 resection group showed no difference compared to the R0 resection group, but the 5 year disease-free survival rate showed difference in extra-lumenal invasion (ELI) and non-ELI group (P = 0.0357 < 0.05), although no difference was seen in the overall survival rate in these two groups. CONCLUSIONS:ACC of the trachea and bronchus is a rare and low-to-moderate grade malignant tumor. When the R0 resection is over risky or may cause mortal complication, the R1 resection with adjunctive therapy is acceptable for patients to obtain a promising prognosis, whereas pathological ELI is an adverse prognostic indicator.
10.1111/ajco.13162
Primary adenoid cystic carcinoma of the trachea: clinical outcome of 38 patients after interdisciplinary treatment in a single institution.
Radiation oncology (London, England)
BACKGROUND:Primary adenoid cystic carcinomas (ACCs) of the trachea are rare tumors of the central bronchial system. In patients presenting with unresectable tumors, severe comorbidities, or incomplete surgical resection, definitive radiotherapy is currently the recommended treatment. Irradiation with carbon ions (C) has shown promising local control (LC) and survival rates in cases of ACCs of the head and neck. No data on the therapeutic efficacy of C12 radiotherapy in treating tracheal ACC has been published. METHODS:All patients with histologically confirmed ACC of the trachea treated with surgery and/or radiation treatment at Heidelberg University Hospital between 1991 and 2017 were included in this analysis. Patient and treatment characteristics, short- and long-term toxicity after radiotherapy, overall survival (OS), freedom from local progression (FFLP), and freedom from distant progression (FFDP) were prospectively acquired and retrospectively analyzed. RESULTS:Thirty-eight patients (23 women and 15 men) with a median age of 51 were treated by surgery (n = 20) and/or radiotherapy with either C (n = 7) or photons (n = 24). Of these patients, 61% presented with locally advanced (stage 4) ACC. The median follow-up for all patients was 74.5 months. The 5-year OS for all patients was 95% (10-year: 81%). The 5-year FFLP and FFDP were 96% (10-year: 83%) and 69% (10-year: 53%), respectively. In patients who underwent surgery alone, the 5-year OS was 100% (10-year: 80%). The 5-year FFLP and FFDP were 100% (10-year: 100%) and 80% (10-year: 60%), respectively. In patients who underwent radiotherapy alone, the 5-year OS was 100% (10-year: 83%). The 5-year FFLP and FFDP were 88% (10-year: 44%) and 67% (10-year: 34%), respectively. In patients who received multi-modal treatment including surgery and adjuvant radiotherapy, the 5-year OS was 84% (10-year: 84%). The 5-year FFLP was 100% (10-year: 100%) and the 5-year FFDP was 65% (10-year, 65%). CONCLUSIONS:The long-term prognosis is favorable if surgery is performed. In cases of an incomplete resection, good OS can still be achieved following adjuvant radiotherapy. For radiotherapy, irradiation with C shows promising first results. However, more data is needed to prove the long-term advantage of C over photons. TRIAL REGISTRATION:The ethics committee of the Heidelberg University Hospital approved the retrospective data analysis (S-174/2019).
10.1186/s13014-019-1323-z
How thoracic surgeon manage tracheal tumors in African country? (Case series).
Annals of medicine and surgery (2012)
INTRODUCTION:Tracheal tumors are a rare pathological entity whose diagnosis is usually delayed by clinical latency. Surgery, which consists of a tracheal resection-anastomosis with or without reconstructive reconstruction, remains the treatment that ensures the best long-term survival. METHODS:This is a retrospective study about 8 patients admitted in the department of thoracic surgery of Hassan II's university hospital of Fes for tracheal tumors management during 7 years time (December 2010 to December 2017). RESULTS:There were 6 men and 2 women with an average age of 44.4 years ranged from 17 to 65 years, 4 were smokers. Dyspnea was the main trigger sign. Seven (7) have undergone bronchial fibroscopy diagnostic with a finding of budding process in 5 patients, the middle of the trachea is often concerned in 3 patients, obstructing the lumen of the trachea in almost all patients. The treatment in all patients was surgical with an intubation via the operative field, 4 trachea resection-anastomosis, 4 plasty (Lateral resection with V plasty, Kergin's plasty, Mattey's tracheobronchial anastomosis and widened V-resection to the carina). The most common histological type in our series was Adenoid Cystic Carcinoma in 2 of our patients. For the other patients we have found squamous cell carcinoma (1 case), adenocarcinoma (1 case), atypical carcinoid tumor (1 case), low grade mucoepidermoid carcinoma (1 case), an adenoma pleomorph (1 case) and endotracheal goiter (1 case). The operative follow-up was simple in 7 of our patients, all of whom underwent post-operative fibroscopy within an average of 9 days. Two cases of post-operative recurrent palsy had been observed, all of which had progressed well under treatment. We have noted 2 deaths, including one at day 4 post-operative, and the other died from complications of post-radiation tracheal stenosis. Back to 32 months' average follow-up, we have enregistered a case of a distant relapse by cervical lymph node metastasis in one patient, 5 years after surgery. CONCLUSION:Primary tumors of the trachea remain of reserved prognosis with 5-year survival of 57% of all histological types combined. Computed tomodensitometry and tracheobronchial fibroscopy remain the means of reference exploration in the diagnosis and assessment of surgical resectability.
10.1016/j.amsu.2019.06.007
Adenoid Cystic Carcinoma of Trachea: Findings on F FDG Positron Emission Tomography-Computed Tomography.
Tripathy Sarthak,Shamim Shamim Ahmed,Behera Abhishek,Bal Chandrasekhar,Kumar Rakesh
Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
Adenoid cystic carcinoma (ACC) of the trachea is the second most common tumor of trachea after squamous cell carcinomas. It arises from the submucosal layer and predominantly has a rapid locoregional spread. We describe the F FDG positron emission tomography-computed tomography findings of a 51-year-old woman, a biopsy-proven case of ACC of trachea who underwent the scan for initial staging.
10.4103/ijnm.IJNM_172_18
Syncope due to tracheal adenoid cystic carcinoma.
Respirology case reports
We present a case of a 34-year-old male who presented with syncope secondary to a large adenoid cystic carcinoma (ACC) of the distal trachea. A computed tomography and flexible bronchoscopy showed almost complete occlusion of the distal trachea. Resection with curative intent was performed, but resection margins were unfortunately not clear. The patient was subsequently offered adjuvant radiotherapy. Tracheal tumours comprise a small proportion of respiratory tract neoplasm, accounting for only about 2% of airway malignancies. Squamous cell carcinoma is the most common tracheal tumour, followed by ACC. Symptoms are usually attributable to the intraluminal component of the tumour causing an obstruction of the airway, resulting in stridor, dyspnoea, wheezing, haemoptysis, and cough. Syncope as a presenting symptom is exceedingly rare.
10.1002/rcr2.452
Adenoid Cystic Carcinoma of the Trachea: A Case Report.
Nicolini Eveline Montessi,Montessi Jorge,Vieira João Paulo,Rodrigues Guilherme de Abreu,Costa Víctor de Oliveira,Teixeira Fabrício Machado,Kassis Mariana de Oliveira
The American journal of case reports
BACKGROUND Adenoid cystic carcinoma of trachea (ACCT) is an unusual low-grade tumor from the tracheal and bronchial wall glands. The histological structure and biological behavior of ACCT are similar to that of tumors found in the salivary glands. ACCT occurs most commonly in the upper trachea, being found in the articular cartilage in the posterior aspect of the trachea. CASE REPORT We describe the case of a 62-year-old male ex-smoker 25 years/pack (abstinence 20 years ago), who began with mild dyspnea 2 years ago, with intense evolution and cough. He was evaluated as an outpatient and was treated for sinusitis and later bronchitis after pulmonary function tests. With the worsening of the aforementioned symptoms, he sought prompt care, with hospitalization and computed tomography (CT) of the chest being indicated, showing an expansive lesion in the anterior wall of the trachea with an extension of approximately 3.1 cm, ending at the level of the carina, measuring 3.4×2.8 cm, with moderate stenosis of the tracheal lumen, with an exophytic component to the mediastinum. Bronchoscopy of the lesion was done, and later immunohistochemistry showed a probable pleomorphic adenoma. However, in a new analysis, after the removal of the surgical specimen, it was observed that it was a ACCT. CONCLUSIONS ACCT is a rare tumor that should be diagnosed as soon as possible in order to ensure its best prognosis. Moreover, it is evident that the analysis of the surgical specimen is sovereign to immunohistochemistry with regard to histological typing.
10.12659/AJCR.917136
Impact of Positive Margins and Radiation After Tracheal Adenoid Cystic Carcinoma Resection on Survival.
Yang Chi-Fu Jeffrey,Shah Shivani A,Ramakrishnan Divya,Raman Vignesh,Diao Kevin,Wang Hanghang,Commander Sarah J,D'Amico Thomas A,Berry Mark F
The Annals of thoracic surgery
BACKGROUND:Achieving negative margins for adenoid cystic carcinoma (ACC) of the trachea can be technically difficult. This study evaluated the impact of positive margins on prognosis and tested the hypothesis that radiation improves survival in the setting of incomplete resection. METHODS:The impact of margin status and adjuvant therapy on overall survival of patients with tracheal ACC in the National Cancer Database (1998 to 2014) who underwent resection with known margin status and with no documented nodal or distant disease was evaluated using Kaplan-Meier and Cox proportional hazard analysis. RESULTS:Of 132 patients who met study criteria, 79 (59.8%) had positive margins after resection. Adjuvant radiation was given to 95 patients overall (72.0%) and to 62 of the 79 patients with positive margins (78.5%). The survival of patients with positive margins was not significantly different from that of patients with negative margins (5-year survival, 82.2% [95% confidence interval (CI), 71.3-89.3] compared with 82.0% [95% CI, 67.0-90.6], P = .97), even after multivariable adjustment (hazard ratio, 1.73; 95% CI, 0.62-4.84; P = .30). In the subset of patients with positive margins, there was no significant difference in survival between patients who did or did not receive postoperative radiation therapy (5-year survival, 82.0% [95% CI, 68.8-89.9] compared with 82.4% [95% CI, 54.7-93.9]; P = .80), even after multivariable adjustment (hazard ratio, 1.04; 95% CI, 0.21-5.25; P = .96). CONCLUSIONS:The majority of tracheal ACC resections performed in this national cohort had positive margins. Adjuvant radiation was commonly used for positive margins but was not associated with an overall survival benefit.
10.1016/j.athoracsur.2019.08.094
Adenoid cystic carcinoma of trachea: long-term disease control after endoscopic surgery and radiotherapy.
Spatola Corrado,Tocco Alessandra,Marletta Dario,Milazzotto Roberto,Marletta Francesco,Pergolizzi Stefano,Migliore Marcello,Basile Antonio,Privitera Giuseppe,Acquaviva Grazia
Future oncology (London, England)
Adenoid cystic carcinoma is a rare tumor of head and neck region and its development in the thoracic region is even less frequent. This implies the absence of guidelines for therapeutic management and a consequent case-by-case approach. The role of radiotherapy is not yet clearly defined, but intensity-modulated radiotherapy allows for improved organ-at-risk sparing. We have collected the cases of four patients treated at our institutions by the means of intensity-modulated radiotherapy, after endoscopic resection. Patients treated achieved long-term disease control of about 5 years, with a minimal acute toxicity. Longer follow-up is needed to drain conclusion on the impact of this treatment on overall survival.
10.2217/fon-2018-0685
Clinical features and prognosis analysis of 57 patients with primary tracheal tumors.
Translational cancer research
BACKGROUND:Primary tracheal tumor (PTT) is a rare disease with poor prognosis. Its clinical features are variable. Treatment strategies for patients with PTTs are not standardized. The purpose of this study was to explore the clinical features, management and prognosis of PTT. METHODS:From 2009 to 2019, 57 patients were diagnosed with PTTs at Xiangya Hospital, China. A retrospective review of their medical records was performed. RESULTS:Among the 57 patients, 8 (14.0%) had benign tumors and 49 (86.0%) had malignant. Squamous cell carcinoma (SCC) (n=20, 40.8%) and adenoid cystic carcinoma (ACC) (n=10, 20.4%) were the most common histologic types. Seven (87.5%) of the benign PTT patients underwent bronchoscopic intervention and had no further complications. The 5-year survival rate for malignant PTTs was 13.8%. Patients who had ACC tended to have better survival rates than those with SCC, although the difference was not statistically significant (P=0.104). Compared with tumors located in upper third of the trachea, tumors located in the lower portion showed worse survival (P=0.0003). Patients who underwent complete surgical resection had significantly better survival compared to non-surgical therapies (P=0.016). The combination of chemoradiotherapy and bronchoscopic intervention was better than chemoradiotherapy alone (P=0.028). CONCLUSIONS:Histologic type, tumor location and treatment approaches likely influence clinical outcomes. Within the cohort described here, complete surgical resection was the optimal therapy for PTTs. For unresectable malignant PTTS, chemoradiotherapy with bronchoscopic intervention was a superior modality. Given the poor 5-year survival of PTT, larger-scale, multi-center studies are warranted to validate our findings and identify optimal therapeutic interventions.
10.21037/tcr.2019.11.55
Treatment outcomes of patients with tracheobronchial mucoepidermoid carcinoma compared with those with adenoid cystic carcinoma.
Wang Yalong,Cai Songhua,Xue Qi,Mu Juwei,Gao Yushun,Tan Fengwei,Mao Yousheng,Wang Dali,Zhao Jun,Gao Shugeng,He Jie
European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
OBJECTIVE:Tracheobronchial mucoepidermoid carcinoma (TMEC) is an extremely rare salivary gland-type neoplasm. We aimed to explore the clinical characteristics and prognosis of TMEC and to compare them with those of another rare salivary gland-type neoplasm, tracheobronchial adenoid cystic carcinoma (TACC). METHOD:We performed a retrospective review of all patients pathologically diagnosed with TMEC between 1965 and 2017 at our institution. We reviewed the patients' clinical characteristics, treatment methods and outcomes and compared the results of TMEC and TACC patients. RESULTS:A total of 115 consecutive patients, including 107 who underwent surgery and 8 who received nonoperative therapy, were included in our study. The 1-, 2-, and 5-year survival rates were 97.89%, 94.17%, and 90.50%, respectively, in the surgical group and 83.33%, 41.67% and 0.00%, respectively, in the nonoperative group. The multivariate analysis showed that N stage was an independent prognostic factor for overall survival (OS). TMEC patients were younger, had a shorter complaint duration, had fewer symptoms, had more bronchial tumors, and were more likely to undergo surgical treatment and achieve an R0 resection (surgically treated patients) than TACC patients; furthermore, TMEC patients had a significantly better OS than TACC patients (P < 0.050). CONCLUSIONS:TMEC has different characteristics and a better prognosis than TACC, which may reflect the different biological behaviors of these two salivary gland neoplasms. Radical treatment and close follow-up are critical for surgically treated TMEC patients with lymph node metastasis.
10.1016/j.ejso.2020.04.020
ADENOID CYSTIC CARCINOMA OF DISTAL TRACHEA: A CASE REPORT.
Djaković Željko,Janevski Zoran,Cesarec Vedran,Slobodnjak Zoran,Stančić-Rokotov Dinko
Acta clinica Croatica
Primary malignant tumors of the trachea are very rare with the incidence of less than two million people year, and only ten percent of them are adenoid cystic carcinomas. Eighty percent of all tracheal tumors are malignant. Diagnosis is usually late because the symptoms mimic other conditions such as asthma. Clinical picture may sometimes be dramatic when airway is almost closed and emergency recanalization is necessary. Diagnosis is made by chest computed tomography scan or magnetic resonance imaging. Definitive treatment is surgical resection alone or followed by radiation therapy or radiation therapy alone. Radical resection is only accomplished in about half of all cases because of the submucosal tumor growth and limited length of tracheal resection. The role of adjuvant radiation therapy in negative resection margin cases is not clear but all patients with positive resection margin benefit from radiation therapy. We present a case of a 43-year-old patient with primary adenoid cystic carcinoma of distal trachea treated by emergency bronchoscopic recanalization and resection of the tracheal tumor with end-to-end anastomosis.
10.20471/acc.2019.58.04.27
Adenoid cystic carcinoma cytology: Salivary gland and nonsalivary gland.
Chowsilpa Sayanan,An Daniel,Maleki Zahra
Diagnostic cytopathology
OBJECTIVES:Adenoid cystic carcinoma (ACCA) is an uncommon primary malignancy of salivary glands and rarely nonsalivary tissue. This study aims to evaluate the diagnostic accuracy of ACCA on fine needle aspiration (FNA) material and the associated challenges. METHODS:A search on electronic pathology database from 2006 to 2016 at The Johns Hopkins Hospital found 83 cytology specimens diagnosed as ACCA, 49 with histology follow-up. RESULTS:Fifty-two females and 31 males were found ranging from 37 to 95 years old (mean 62.5). The tumor size was 1 to 11.5 cm (mean 3.4). FNAs were performed on 46 salivary glands (54.88%), 12 head and neck masses (14.45%), 9 lymph nodes (10.84%), 9 tracheas/lungs (10.84%), 4 vaginal/perineum/gluteal masses (4.82%), and one for each kidney, liver and abdominal/pelvic mass (1.21%). 83 FNA diagnoses revealed 3 nondiagnostics (3.61%), 20 neoplasms with unspecified features (24.10%), 30 basaloid neoplasms (36.14%), 18 ACCA (21.69%), and 12 other malignancies (14.46%). The accuracy of FNA in diagnosis of ACCA comparing to histologic follow-up in 49 cases was 87.5% sensitivity, 66.67% specificity, with 92.11% positive predictive value and 54.55% negative predictive value. The most common mimicker was pleomorphic adenoma. CONCLUSION:ACCA can be diagnosed not only in the salivary gland FNAs, but also respiratory tract, intra-abdominal, kidney, and gynecologic regions. FNA is a preferred technique to assess mass lesions. However, a diagnosis of ACCA on FNA material should be rendered with caution since there are benign and malignant neoplasms with overlapping features. Awareness of prior medical history and ancillary studies can improve the diagnosis.
10.1002/dc.24573
The incidence of mTOR marker in tracheal adenoid cystic carcinoma by immunohistochemical staining.
Pourabdollah Toutkaboni Mihan,Farahani Mehrdad,Sadegh Abdolreza,Kiani Arda,Sadr Makan,Taghavi Kimia,Abedini Atefeh
Advances in respiratory medicine
INTRODUCTION:There is an association between the activation of mammalian target of rapamycin (mTOR) signaling and aggressive tumor growth in multiple forms of cancer,including adenoid cystic carcinoma (ACC). ACCs are uncommon yet a malignant form of neoplasms that arises within the secretory glands. Therefore, the aim of this study was to investigate the increase of mTOR in the ACC tumors in order to survey the possibility of treating these tumors with mTOR inhibitors. MATERIAL AND METHODS:Samples from known cases of the lung and tracheal ACC were retrievedfrom the archives of the pa-thology department of Masih Daneshvari hospital, and immunohistochemical (IHC) staining for mTOR was performed on them. After preparation of the blocks with specific antibodies, tumor cells with cytoplasmic and/or nuclear expression of mTOR were considered as positive cells by applying a specific scoring method introduced in this study. RESULTS:The paraffin blocks of 26 patients were surveyed and the IHC marker of mTOR was positive in the tumors of 10 patients (38.5%). Out of 10 mTOR positive cases, 5 were females and 5 were males. The primary site of the surveyed tumors was the trachea and bronchus in 12 cases (46%), salivary glands in 7 individuals (27%), and lung tissue in 7 cases (27%), and there was no significant correlation between the primary site of the ACC tumors and the existence of the mTOR markers in them (P = 0.67). From all cases, 13 patients (50%) had cribriform and tubular cells without solid components, 9 cases (34.6%) had cribriform and tubular with less than 30% of solid components, and 4 cases (15.4%) had cribriform and tubular cells with more than 30% of solid com-ponents. There was no significant difference between the morphologies and the existence of mTOR markers in them (P = 0.741). CONCLUSIONS:As the incidence of mTOR markers is seen in patients with tracheal ACC, evaluation and scoring of mTOR in these persons can be helpful as further studies can distinguish the use of it in the treatment of the disease. .
10.5603/ARM.a2020.0120
Effective Radiotherapy in Tracheobronchial Adenoid Cystic Carcinoma With Positive Surgical Margin.
Zhao Lei,Zhao Yang,Guo Jin-Dong,Zeng Ya,Yao Feng,Liu Mi-Na,Wang Jia-Ming,Lv Chang-Xing,Liu Jun,Fu Xiao-Long,Zhao Heng,Cai Xu-Wei
The Annals of thoracic surgery
BACKGROUND:The study aimed to evaluate the role of postoperative radiotherapy (PORT) in the treatment of trachea and main bronchus adenoid cystic carcinoma (ACC) with a positive surgical margin. METHODS:Patients with pathologically confirmed trachea or main bronchus ACC operated on at Shanghai Chest Hospital were enrolled. Survival, univariate, and multivariate analyses were performed. The χ test was applied to analyze the failure patterns among different groups (R0/0: negative margin resection without PORT; R1/0: positive margin resection without PORT; R1/1: positive margin resection with PORT). RESULTS:From January 2001 to December 2014, 77 patients were deemed eligible for the study. Pairwise comparisons showed that the overall survival rate of group R1/1 was comparable to that of group R0/0 (P = .438), and significantly longer than the rate of group R1/0 (P = .032). Additionally, the local disease-free survival rate of group R1/1 was much higher than that of group R0/0 (P = .023) and R1/0 (P = .001). Cox multivariate analysis identified the radiologic feature (P = .012) and PORT (P = .006) as significantly favorable prognostic factors for locoregional disease-free survival. By contrast, for overall survival, PORT (P = .032) was the only corresponding variable identified by univariate analysis. Furthermore, PORT significantly decreased the locoregional recurrence rate (P = .002) but not distant metastases (P > .999). CONCLUSIONS:PORT helped patients with tracheobronchial ACC and microscopic positive surgical margins to achieve a similar outcome as patients with complete resection. R0 resection may not be necessary for tracheobronchial ACC if it is difficult to be completely resected.
10.1016/j.athoracsur.2020.11.033
A Case of Solid Variant of Adenoid Cystic Carcinoma from Trachea: A Case Report and Review of Literature.
Gao Feng,Zang Lijuan,He Jin,Xu Weiqing
OncoTargets and therapy
Introduction:Primary tracheal adenoid cystic carcinoma (ACC) is a rare and heterogeneous group of neoplasms arising from the respiratory tract. The solid variant of ACC is a histologically distinct subtype with an unfavorable clinical course. We report on a case of tracheal ACC with immunohistochemical and molecular analysis together with a review of the literature. Case Report:We observed a case in which a 31-year-old male presented with a neoplasm bulging into the lumen and caused symptoms of tracheal obstruction and even hemoptysis. Cytological smears of an endobronchial fine needle aspiration revealed aggregates of basaloid cells with small to medium size, scant cytoplasm, and evenly hyperchromatic nuclei. Histologically, the tumor is characterized by a predominant compact sheet-like and nested pattern of rounded basaloid cells. Immunohistochemically, the tumor was diffusely positive for CK and CD117. CK7 and CK5/6 were focally positive in the genuine glandular structures. P63 was completely negative in the majority of neoplastic cells. Fluorescence in situ hybridization analysis revealed MYB gene rearrangement. Conclusion:The solid variant of ACC from trachea is rare and hence poses diagnostic difficulty. Computed tomography (CT) scan and bronchoscopy help assess the extent of the disease. Histological features combined with immunophenotypic and molecular analysis aid in distinguishing this uncommon type from other round cell neoplasms. The accurate diagnosis can help expedite treatment of this highly aggressive tumor.
10.2147/OTT.S296400
Patterns of Care and Outcomes of Primary Adenoid Cystic Carcinoma of the Trachea.
The Annals of otology, rhinology, and laryngology
OBJECTIVE:Primary tracheal malignancies are relatively rare cancers, representing 0.1% to 0.4% of all malignancies. Adenoid cystic carcinoma (ACC) is the second most common histology of primary tracheal malignancy, after squamous cell carcinoma. This study aims to analyze demographic characteristics and potential influencing factors on survival of tracheal ACC (TACC). METHODS:This was a retrospective cohort study utilizing the National Cancer Database (NCDB). The NCDB was queried for all cases of TACC diagnosed from 2004 to 2016 (n = 394). Kaplan-Meier (KM) and Cox proportional-hazards models were used to determine clinicopathological and treatment factors associated with survival outcomes. RESULTS:Median age of diagnosis was 56 (IQR: 44.75-66.00). Females were affected slightly more than males (53.8% vs 46.2%). The most prevalent tumor diameter range was 20 to 39 mm (34.8%) followed by greater than 40 mm in diameter (17.8%). Median overall survival (OS) was 9.72 years with a 5- and 10-year OS of 70% and 47.5%, respectively. Localized disease was not associated with a survival benefit over invasive disease ( = .388). The most common intervention was surgery combined with radiation therapy (RT) at 46.2%, followed by surgery alone (16.8%), and standalone RT (8.9%). When adjusting for confounders, surgical resection was independently associated with improved OS (HR 0.461, 95% CI 0.225-0.946). Tumor size greater than 40 mm was independently associated with worse OS (HR 2.808; 95% CI 1.096-7.194). CONCLUSION:Our data suggests that surgical resection, possibly in conjunction with radiation therapy, is associated with improved survival, and tumor larger than 40 mm are associated with worse survival.
10.1177/00034894211008101
CT diagnosis and prognosis prediction of tracheal adenoid cystic carcinoma.
Wang Shu Chao,Yin Le Kang,Zhang Yu,Xue Li Min,Ye Jian Ding,Tao Guang Yu,Yu Hong,Qiang Jin Wei
European journal of radiology
PURPOSE:To evaluate computed tomography (CT) features and establish a predictive model for the clinical diagnosis and prognosis of tracheal adenoid cystic carcinoma (ACC). METHOD:From January 2010 to December 2018, 82 patients with tracheal tumors, including 46 patients with ACC confirmed by surgery and histopathology, were enrolled in this study. These patients' clinicopathologic information, CT features and survival outcomes were recorded and analyzed. Independent predictors of diagnosis and prognosis of tracheal ACC were determined by both univariate and multivariate analyses. RESULTS:Compared with tracheal non-ACC patients, univariate analysis showed that ACC patients were more likely to have extensive longitudinal length (p < 0.001) and to appear as annular wall thickening (p = 0.001), transmural growth (p = 0.036), poorly defined border (p = 0.003) and mild enhancement (p = 0.001). Multivariate logistic analysis showed that longitudinal length and enhancement degree were independent predictors of tracheal ACC. The 3-year and 5-year disease-free survival (DFS) were 75.7 % and 64.5 %, respectively. Longitudinal length (≥ 34 mm), transverse length (≥ 20 mm) and transmural growth were associated with poor DFS in univariate analysis. After multivariate adjustment, only transverse length (≥ 20 mm) was an adverse prognostic factor for DFS (hazard ratio = 4.594, 95 % confidence interval = 1.240-17.017; p = 0.022). CONCLUSIONS:CT longitudinal length and enhancement degree of tumors showed satisfactory discrimination for tracheal ACC. Excessive CT transverse length might be an unfavorable indicator for ACC recurrence and could be helpful for predicting the survival outcomes of ACC at the initial diagnosis.
10.1016/j.ejrad.2021.109746
A case of adenoid cystic carcinoma of trachea: treatment complications and radiotherapy role.
Journal of contemporary brachytherapy
PURPOSE:Adenoid cystic carcinoma (ACC) of trachea is a relatively rare malignant neoplasm, for which there is a lack of prospective clinical trials investigating treatment effectiveness. Most of the authors prefer surgical resection followed by post-operative radiation therapy in case of incomplete excision. There are no available prospective data on post-relapse treatment. CASE PRESENTATION:The current paper presents a case of tracheal ACC in a young woman, treated solely with surgical resection without radiotherapy due to postoperative neurological complications requiring additional diagnostics, management, and treatment. As a complication itself, spinal cord dysfunction after tracheal surgery is extremely rare, in which radical radiotherapy and brachytherapy were successfully administered after disease recurrence. CONCLUSIONS:Lack of post-operative radiotherapy resulting from neurological complications could be a reason for ACC recurrence in our patient. Administration of radiotherapy after incomplete resection of recurrent disease may lead to long-term locoregional control.
10.5114/jcb.2021.109853
Left sleeve pneumonectomy for a rare lung tumor: A case report.
Bouacida Imen,Essid Rime,Bousnina Mouna,Zribi Hazem,Ouerghi Sonia,Merghli Adel
International journal of surgery case reports
INTRODUCTION AND IMPORTANCE:Primary adenoid cystic carcinoma (ACC) of the lung is extremely rare. This tumor can be asymptomatic or have non characteristics symptoms, and the diagnosis is often late. The treatment of choice is surgery when it's possible. CASE PRESENTATION:We herein report the case of a young patient with ACC of the left main bronchus. He had dyspnea and chest pain for 6 months. Complete atelectasis of the left lung was found on the chest x-ray. Bronchoscopy showed a tumor obstructing the LMB and invading the carina. The CT scan revealed a 5 cm tumor obstructing the left main bronchus (LMB) with extension to the carina and thoracic trachea. The extension assessment was without abnormalities. The treatment was surgical. A left carinal pneumonectomy by double lateral thoracotomy was performed. The postoperative results were satisfactory. There was no recurrence with a follow-up of 2 years. CLINICAL DISCUSSION:The therapeutic management of ACC is essentially based on surgical resection, which should be as radical as possible. However, complete resection is often difficult given the infiltrating nature of the tumor. CONCLUSION:Sleeve pneumonectomy with carinal resection is a curative option for patients with ACC of the main bronchi and carina that require expertise of the surgeons.
10.1016/j.ijscr.2021.106528
Rapid response to radiotherapy in unresectable tracheal adenoid cystic carcinoma: A case report.
Wu Qiang,Xu Feng
World journal of clinical cases
BACKGROUND:Adenoid cystic carcinoma (ACC) occurs mainly in the head and neck. Tracheal ACC (TACC) is uncommon. Primary resection is recommended as the main treatment of choice, and radiotherapy is considered for residual disease in the postoperative setting. Definitive radiotherapy is an alternative approach to cure unresectable TACC. As the status of radiosensitivity in TACC is uncertain, the evidence for radiotherapy in unresectable TACC is not well established, especially in terms of the optimal dosage and its response evaluation. Herein, we report a case of locally advanced TACC. CASE SUMMARY:A 49-year-old woman was diagnosed with TACC, which included a range of lesions arising in the upper trachea extending caudally 2 cm to 7 cm of the glottis. She was treated with definitive radiotherapy, given the low likelihood of complete resection of the disease. Due to the indolent growth and the propensity for infiltration along the airways, the scheduled radiation dose of 76 Gy in 38 fractions with 6-MV X-ray delivered by intensity-modulated radiotherapy was conducted to the primary tumor volume. After irradiation of 40 Gy, the patient's dyspnea on exertion was dramatically relieved and bronchoscopy revealed that the previous large polypoid intra-luminal mass was significantly eliminated, with near-complete response. The patient completed two phases of scheduled radiotherapy, and acute reactions to treatment included subjective chest tightness and grade 2 esophagitis, managed medically. After 5 years of treatment, the patient is alive without recurrent disease, and there were no serious late radiation esophagus and lung damage, with only slight dysphagia without perforation and fistula. CONCLUSION:Taken together, TACC is uncommon and the treatment of unresectable TACC is challenging. This case indicated that patients with unresectable TACC who rapidly respond to radiation may benefit from primary radical radiotherapy. Radiotherapy may be considered an effective alternative treatment modality.
10.12998/wjcc.v9.i31.9535
Do We Need TNM for Tracheal Cancers? Analysis of a Large Retrospective Series of Tracheal Tumors.
Cancers
Due to the low incidence of primary tracheal neoplasms, there is no uniform system for staging of this disease. Our retrospective analysis based on registry data included 89 patients diagnosed with primary tracheal cancer at the National Research Institute of Oncology in Warsaw, Poland, between January 2000 and December 2016. We analyzed demographic, clinical, pathological, therapeutic, and survival data. The staging-for the purpose of our analysis-was performed retrospectively on the basis of imaging results. Tumor (T) category was defined as a disease confined to the trachea or lesion derived from the trachea and spreading to adjacent structures and organs. Node (N) and metastases (M) categories were divided into absence/presence of metastasis in regional lymph nodes and the absence/presence of distant metastasis. Survival analysis was performed depending on the clinical presentation of these features. There was a significant difference in overall survival depending on the T, N, M categories in the entire group. In the group of patients undergoing radical treatment, the T and N categories had a statistically significant impact on overall survival. In the group of patients treated with palliative aim, only the T category had an impact on overall survival. Multivariate analysis showed statistical significance for the T category in patients undergoing radical and those receiving palliative treatment. The assessment of the anatomical extent of lesions may help decide about treatment options and prognosis.
10.3390/cancers14071665
Clinical characteristics, surgical treatments, prognosis, and prognostic factors of primary tracheal cancer patients: 20-year data of the National Cancer Center, China.
Translational lung cancer research
Background:Tracheal cancer is a rare malignancy of which previous reports are mostly case reports or small series. Herein, we sought to evaluate the clinical characteristics, surgical treatments, and prognosis of surgically treated primary tracheal cancer patients. Methods:Patients with primary tracheal cancer who had received surgery in our center between January 2000 and December 2020 were enrolled. Clinical and surgical features were collected by retrospective review of medical records and follow-up was done by telephone interview. The statistical tests were two-sided. Results:A total of 128 patients were included in the study, 49.2% of whom were male, and the average age was 49.4±13.6 years. The most common histological subtype was adenoid cystic carcinoma (ACC; 78/128, 60.9%) followed by squamous cell carcinoma (SCC; 24/128, 18.8%). The percentage of tumors located in the cervical trachea, thoracic trachea, and carina were 50%, 41.4%, and 8.6%, respectively. Among those analyzed, 32.0% of the primary tumors had invaded adjacent organs (E2 disease) and 7.8% of patients had lymph node involvement. Tracheal resection plus reconstruction (with or without thyroidectomy) was the predominant surgical procedure, followed by carinal resection with neocarina. Radical resection (R0) was performed on 61.7% of patients and 63 (49.2%) patients received adjuvant therapy. Compared to ACC, SCC patients had significantly higher risk of tumor of the carina, nodal metastasis, and complications. The 5-year overall survival (OS) for the entire cohort was 84.5% and factors associated with poor prognosis included carinal tumor [hazard ratio (HR) =10.206; P<0.001], E2 disease (HR =8.870; P=0.001), lymph node metastasis (HR =15.197; P<0.001), and postoperative complications (HR =12.497; P=0.001). Conclusions:The two major subtypes of tracheal cancer are ACC and SCC. Tumor location, extension, lymph node metastasis and complication are survival related factors for surgically treated patients.
10.21037/tlcr-22-258
Therapeutic bronchoscopy followed by sequential radiochemotherapy in the management of life-threatening tracheal adenoid cystic carcinoma: a case report.
Journal of medical case reports
BACKGROUND:Adenoid cystic carcinoma of the lung is a distinctive salivary-gland-type malignant epithelial neoplasm that rarely presents as a primary tumor of the respiratory tract. Complete surgical resection remains the treatment of choice for adenoid cystic carcinoma. We present a case of large ACC tumors that caused severe central airway obstruction and were effectively treated with therapeutic bronchoscopy followed by radiotherapy and chemotherapy. CASE PRESENTATION:A 31-year-old Malay Indonesian female patient who was a nonsmoker and had no family history of cancer was admitted to the emergency ward because of worsening breathlessness accompanied by stridor since 1 week prior. Chest computed tomography revealed segmental atelectasis of the left lung; a mass on the left main bronchus, with infiltrates in segments 1, 2, and 3 of the left lung; and consolidation in the left inferior lobe, with narrowing of the main left bronchus. Lobulated masses obstructing almost the entire distal trachea up to the carina and the entire left main bronchus were found on bronchoscopy. Owing to the large tumors causing severe central airway obstruction, the medical team decided to perform central airway mass removal through rigid bronchoscopy. A neodymium-doped yttrium-aluminum-garnet laser was used first to facilitate mass shrinkage. After the laser treatment, mechanical mass removal using a rigid scope was performed. The tracheal and carinal lumens were opened to > 50% of their diameter, with the left main bronchus lumen opened only slightly. After the treatment, the patient was stable, and no stridor was found. Adjuvant intensity-modulated radiotherapy and chemotherapy were performed after the therapeutic bronchoscopy. At the end of the entire treatment, reevaluation by thoracic computed tomography scan and bronchoscopy revealed no remaining mass. CONCLUSIONS:In cases of nonresectable large adenoid cystic carcinoma tumors with life-threatening central airway obstruction, therapeutic bronchoscopy followed by sequential radiochemotherapy might achieve a complete response outcome.
10.1186/s13256-022-03452-1
Surgical Treatment of Primary Tracheal Adenoid Cystic Carcinoma.
Ear, nose, & throat journal
Primary tracheal adenoid cystic carcinoma (TACC) occurring in the cervical trachea and invading the thyroid is very rare. Surgical resection and airway reconstruction are the main treatment methods, and other treatments include radiotherapy and endoscopic intervention. Herein, we present the case of a 74-year-old female patient with TACC. The patient underwent surgery and postoperative pathology showed that the tumor invaded the adventitia of the trachea and bilateral thyroid, where nerve involvement was observed. The patient recovered well after the operation without adjuvant therapy. Eight months after the operation, Computed tomography showed that the trachea was unobstructed and there was no recurrence.
10.1177/01455613221111497
A voluminous tumour of the tracheal bifurcation.
Respirology case reports
The tracheal bifurcation is the point at which the trachea splits into the left and right main stem bronchus conducting to the lungs. Primary tumours of the tracheal bifurcation are rare. We report images of an adenoid cystic carcinoma of the tracheal bifurcation diagnosed accidentally following recurrent episodes of haemoptysis.
10.1002/rcr2.1003
Primary adenoid cystic carcinoma of the trachea: an elusive diagnosis of chronic dyspnoea.
BMJ case reports
Primary adenoid cystic carcinoma (ACC) of the trachea is a rare entity, with a 5-year survival between 50% and 80% for resectable cases and 30% in case of unresectable disease. We report a case of a primary ACC on a woman in her 70s that presented with a drawn-out history of dyspnoea. She was diagnosed with an unresectable obstructive tumour of the trachea, which required the placement of a Y-shaped stent. The patient underwent concomitant chemoradiotherapy, with partial response, and is still in follow-up, without evidence of disease progression.
10.1136/bcr-2022-250227
Retrospective study of outcomes after extended resection for tracheobronchial adenoid cystic carcinoma.
The Journal of thoracic and cardiovascular surgery
OBJECTIVE:Tracheobronchial adenoid cystic carcinoma is a rare, slow-growing malignancy with a considerable propensity for local extension that may require complex airway resection to achieve tumor-free margins. The objective of this study was to assess whether our experience supports complex airway resection for tracheobronchial adenoid cystic carcinoma. METHODS:Consecutive patients who underwent curative resection for tracheobronchial adenoid cystic carcinoma at our institution between 1970 and 2019 were included retrospectively and classified as having had complex or standard resection. Complex surgery included total tracheal replacement, associated esophageal resection, pneumonectomy, total laryngectomy with tracheal resection, and carinal resection. Standard surgery included tracheal resection, bronchoplastic resection, lobectomy, and bilobectomy. We obtained data from medical records, referring physicians, patients, relatives, and public death records. RESULTS:Of 59 included patients, 38 had complex and 21 had standard surgery. All 4 (6.8%) patients who died postoperatively had undergone complex surgery. Postoperative morbidity was 32.2% overall and was significantly higher after complex surgery (P = .043). Overall 5- and 10-year survival rates were 81.5% and 60.2%, with no significant differences between groups (P = .31). By univariate analysis, T4 tumor and microscopically detectable tumor in the operative specimen margins and gross tumor in the operative specimen margins were associated with poorer survival (P < .05). In the subgroup with microscopically detectable tumor resection, survival was significantly better with adjuvant radiotherapy (P < .05). CONCLUSIONS:Complex resection for extended tracheobronchial adenoid cystic carcinoma may achieve local control and satisfying long-term survival. However, this demanding procedure is associated with high postoperative morbidity and mortality rates. Because adjuvant radiotherapy improved outcomes after resection resulting in microscopically detectable tumor in the operative specimen margins, expected outcomes after resection with no detectable tumor in the margins must be compared to those after resection resulting in microscopically detectable tumor in the margins plus radiotherapy, according to the operative risk.
10.1016/j.jtcvs.2022.10.048
Proton Beam Therapy as a Curative Treatment for a Young Case of Unresectable Tracheal Adenoid Cystic Carcinoma.
Internal medicine (Tokyo, Japan)
Primary tracheal adenoid cystic carcinoma (TACC) is a rare malignancy without an established treatment. Central airway obstruction due to TACC often decreases the quality of life and has life-threatening consequences. A 19-year-old man with unresectable TACC and central airway obstruction suffered from progressive cough and dyspnea after exercise. Proton beam therapy (PBT) was selected as the preferred treatment over systemic anti-cancer chemotherapy for TACC. PBT led to complete remission of TACC and the almost complete disappearance of the respiratory symptoms without adverse events. PBT is a useful and safe treatment for unresectable primary TACC.
10.2169/internalmedicine.0574-22
Stent loaded with radioactive Iodine-125 seeds for adenoid cystic carcinoma of central airway: A case report of innovative brachytherapy.
Frontiers in oncology
Adenoid cystic carcinoma (ACC) of central airway is very rare. More than half of ACCs are unresectable for tumor extension. There's rare report on local ACCs only in central airway. We present a case of ACC in central airway who underwent an innovative brachytherapy. A 44-year-old woman was diagnosed with primary ACC in central airway without regional lymphadenopathy or metastatic disease. Stenosis was observed in lower trachea and both left and right main bronchi (stenosis in lumen ≥50%) with bronchoscopy. The tumor was unresectable due to local extension. A Y-shaped and stainless-steel stent loaded with radioactive I seeds was placed in the central airway using bronchoscope. The number and distribution of I seeds were planed using treatment planning system. The stent was removed three months later. The patient tolerated the procedure well. She was alive without relapse three years after removing the stent with I seeds. This case demonstrates the successful use of stent with radioactive I seeds for unresectable ACCs in central airway. In the procedure, the stent was placed with bronchoscope and under the vision from bronchoscope. This innovative brachytherapy is well-tolerated, safe, precise and individualized designed. The patient with unresectable ACCs could get a long-term relapse-free survival. Clinical trials could be taken to validate its effectiveness and tolerability in patients with ACCs of central airway.
10.3389/fonc.2023.837394
Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome.
Lung India : official organ of Indian Chest Society
Introduction:Primary pulmonary adenoid cystic carcinoma is a rare disease. Its clinico-pathological features, course of disease, therapeutic strategy, and survival data have not been fully elucidated. We aimed to study the clinicopathological characteristics of primary pulmonary adenoid cystic carcinomas in north India. Methods:This study was a retrospective, single-centre, cohort study. The database of the hospital was searched for a period of seven years to identify all patients with primary pulmonary adenoid cystic carcinoma. Results:Out of 6050 lung tumors, 10 were primary adenoid cystic carcinomas. The mean age of diagnosis was 42 (±12) years. Six patients had the lesion in the trachea, main bronchus or truncus intermedius and four had parenchymal lesions. Seven patients had resectable tumours. Three patients achieved an R0 resection, two achieved R1 resection and two had R2 resection. On histopathological examination, almost all of the patients had cribriform pattern. Only four patients (57.1%) showed positivity for TTF-1 staining. The five-year survival of patients with resectable tumour and un-resectable tumour was 85.7% and 33.3%, respectively (P = 0.01). The predictors of poor outcome included non-operability of the tumour, presence of metastasis at the time of diagnosis, and macroscopically positive tumour margin during surgery. Conclusion:Primary pulmonary adenoid cystic carcinoma is a unique and rare tumour that affects relatively younger individuals, males and females, as well as smokers and non-smokers equally. The features of bronchial obstruction are the most common. Surgery is the primary modality of treatment and completely resectable lesions have the best prognosis.
10.4103/lungindia.lungindia_204_22
TACC diagnosed by transoesophageal endoscopic ultrasonography: A case report.
World journal of clinical cases
BACKGROUND:Primary adenoid cystic carcinoma in the trachea (TACC) is a rare tumour. Tracheal bronchoscopy is always chosen as a routine approach to obtain a pathological diagnosis, but it can be associated with an increased risk of asphyxia. CASE SUMMARY:We describe a case of TACC in a patient evaluated by chest computed tomography (CT) with three-dimensional reconstruction imaging and diagnosed by transoesophageal endoscopic ultrasonography. The pathological diagnosis confirmed tracheal adenoid cystic carcinoma. CONCLUSION:We highlight the importance of CT and provide a successful exploration of transoesophageal biopsy as a safe alternative approach.
10.12998/wjcc.v11.i16.3847
Treatment and outcomes of minor salivary gland cancers of the larynx and trachea: a systematic review.
Acta otorhinolaryngologica Italica : organo ufficiale della Societa italiana di otorinolaringologia e chirurgia cervico-facciale
Objectives:Malignant minor salivary glands carcinomas (MiSGC) of the larynx and trachea are rare tumours and published evidence is sparse. We conducted a systematic review to describe shareable treatment strategies and oncological outcomes of these neoplastic entities. Methods:Full text English manuscripts published from January 1 2000 to December 14 2022 were included. Data on demographics, treatments and outcomes were collected. A pooled analysis of 5-year overall survival (OS) was performed. Results:Seventeen articles and 365 patients met the inclusion criteria. The most common subsites involved were subglottic and distal trachea. Adenoid cystic carcinoma was, by far, the most frequent histotype. The first-choice treatment strategy was surgery (86.8%), while adjuvant treatments were delivered in 57.4% of patients. Only 12.9% were treated with definitive radiotherapy with/without chemotherapy. The mean follow-up was 68.3 months. One hundred nine (34.9%) deaths were recorded and 62.4% were cancer-related. Five-year OS ranged from 20% to 100% and, at pooled analysis, it was 83% (range, 78-87%). Conclusions:In case of MiSGC of the larynx and trachea, surgery remains the mainstay of treatment. Adjuvant treatments are frequently delivered. Survival estimates are good overall, but highly heterogeneous.
10.14639/0392-100X-N2635
Tracheal adenoid cystic carcinoma with microscopic positive margin-how we approached with a systematic analysis review of its management.
Indian journal of thoracic and cardiovascular surgery
Purpose:Adenoid cystic carcinoma (ACC) of the trachea is a rare malignancy. We report a patient with ACC who underwent multimodal management including tracheal resection. A systematic review was also conducted on tracheal resection for ACC. Methods:A literature search was conducted on MEDLINE, Embase, and PubMed using the search terms "trachea AND adenoid cystic carcinoma AND (surgery OR resection)" and articles from 2000 to August 2021 were identified. A total of 29 journal articles were included in the review. Results:A total of 403 patients underwent surgery for tracheal ACCs. The mean age was 48.1 years and 54.7% were female. The commonest anatomical location was the lower trachea (46.9%). The mean time from symptom onset to diagnosis was 16.6 months with the commonest symptom being dyspnoea (52%). Fifty-eight percent of the patients had intraluminal growth. Tracheal resection (46.2%) and access via thoracotomy (41.4%) were the commonest procedures described. The mean length of trachea resected was 39.2 mm and the mean tumour size was 31.5 mm. 16.8% of lymph nodes were involved and 73.8% of cases had positive resection margins. The overall complication rate was 1.4-5.4% and the in-hospital mortality rate was 9.8%. The overall survival reported was 61.7% at 5 years and 54.6% at 10 years. Conclusion:Surgical resection followed by adjuvant radiotherapy is the mainstay in the treatment of tracheal ACC, notwithstanding the high rates of involved margins. Achieving tension-free anastomosis should be the first priority given the favourable response of adjuvant therapies in reducing recurrence rate and improving overall survival.
10.1007/s12055-023-01600-w
Radiation Therapy for Primary Adenoid Cystic Carcinoma of the Trachea: Photons, Protons, or Carbon.
International journal of particle therapy
Primary adenoid cystic carcinoma of the trachea (ACC-T) is an extremely rare cancer of the central bronchial system. It is usually associated with an excellent prognosis. Surgery is the standard treatment for resectable tumors, while radiation therapy is used for unresectable tumors or medically inoperable patients. Radiation therapy can be delivered with photons, protons, or carbon ion therapy. In this report, we review a case of unresectable ACC-T in a middle-aged female patient who was treated with radiation therapy and review the potential benefits of the different types of radiation therapy.
10.14338/IJPT-22-00036.1
The Novel Application of Robotic-Assisted Bronchoscopy Combined with Photodynamic Therapy for Adenoid Cystic Carcinoma of the Trachea.
Respiration; international review of thoracic diseases
Robotic platforms have been widely used in the various fields of clinical diagnosis and therapy of diseases in the past decade. Robotic-assisted bronchoscopy (RAB) demonstrates its advantages of visibility, flexibility, and stability in comparison to conventional bronchoscopic techniques. Improving diagnostic yield and navigation yield for peripheral pulmonary lesions has been defined; however, RAB platform of treatment was not reported. In this article, we report a case of a 52-year-old woman who was diagnosed with the tracheal adenoid cystic carcinoma and recurred in the second postoperative year, leading to the involvement of the entire tracheal wall and lumen obstruction. Since the lesion was inoperable, we combined RAB and photodynamic therapy (PDT) for the patient. The potential advantages of using RAB for PDT delivery include precise light irradiation of target lesions and stable intra-operative control over the long term. This is a novel application of RAB combined with PDT for airway diseases. The case report may provide a new insight into the diagnosis and treatment of pulmonary diseases. In addition to improving the diagnostic rates, the RAB platform may also play an important role in the treatment of airway and lung disease in the future.
10.1159/000534352