Tracheobronchial Adenoid Cystic Carcinoma Research Articles

Dose-Escalated Radiotherapy for Primary Tracheobronchial Adenoid Cystic Carcinoma.

Primary tracheobronchial adenoid cystic carcinoma (ACC) is a rare malignancy, so the optimal radiotherapy (RT) dose remains unestablished. We aimed to evaluate the effectiveness of dose-escalated RT for primary tracheobronchial ACC. We retrospectively reviewed 48 patients who had undergone definitive or postoperative RT. Patients classified into the low- and high-dose groups received RT doses <70.0 and ≥70.0 Gy in EQD2, respectively. The primary endpoint was freedom from local progression (FFLP) and overall survival (OS). Throughout the follow-up period, seven patients (14.6%) experienced local progression, while 31 (64.6%) exhibited distant metastasis, most commonly in the lungs. In total, the 5-year FFLP and OS rates were 85.7 and 84.7%, respectively. Multivariate analysis revealed that regional lymph node metastasis at diagnosis and receipt of definitive RT were associated with poorer OS. In the subgroup analysis, the definitive RT group had a 5-year FFLP rate of 33.3 and 78.2% in the low- and high-dose groups (p = 0.065), whereas 5-year OS rates were 66.7 and 79.0%, respectively (p = 0.022). Four patients (8.3%) experienced Grade 3 toxicity with tracheal or main bronchus stenosis. Dose-escalated RT with conventional fractionation may be effective in patients with tracheobronchial ACC, especially for a definitive aim.

原发性气管支气管腺样囊性癌CT影像表现

原发性气管支气管腺样囊性癌CT影像表现

Retrospective study of outcomes after extended resection for tracheobronchial adenoid cystic carcinoma

ObjectiveTracheobronchial 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. MethodsConsecutive 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. ResultsOf 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). ConclusionsComplex 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.

EP07.01-007 Is Extended Resection for Tracheo Bronchial Adenoid Cystic Carcinoma Warranted ?

Tracheobronchial adenoid cystic carcinoma (TBACC) is a rare, slow-growing, malignancy whose considerable propensity for local extension may require complex airway resection to achieve tumor-free (R0) margins. The objective of this study was to assess whether our experience supports complex airway resection for TBACC.

Photodynamic therapy for primary tracheobronchial malignancy in Northwestern China

BackgroundPhotodynamic therapy (PDT) has been routinely performed to treat tracheobronchial malignancy. However, the experience in tracheobronchial adenoid cystic carcinoma (ACC) and peripheral lung cancer is still insufficient. This study aimed to share the experience of PDT for patients with primary tracheobronchial malignancy, especially the adenoid cystic carcinoma and peripheral lung cancer, and evaluated the efficacy and safety of PDT in Northwestern Chinese patients. MethodsThis study retrospectively analyzed the clinical data of 23 patients with primary tracheobronchial malignancy receiving PDT in our center. The short-term effect was evaluated by the objective tumor response and the clinical response. The long-term effect was estimated by recurrence-free survival (RFS). ResultsOf 23 patients, SR was achieved in 18 patients and MR in 3 patients. The clinical symptoms and the quality of life were significantly improved after PDT (P<0.05). And the mean RFS was 8.9 ± 1.9 months. SR for 6 cases of ACC were achieved with significant improvement of clinical symptoms and quality of life. No procedure-related complications appeared. And PDT was successfully performed for the peripheral lung cancer with the guidance of electromagnetic navigation bronchoscopy (ENB). ConclusionsThis study demonstrated that PDT achieved satisfactory efficacy and safety for Northwestern Chinese patients with primary tracheobronchial malignancy. Patients with ACC can benefit from PDT. And ENB-guided PDT is a novel and available option for the peripheral lung cancer. In short, this study accumulated valuable experience for the application of PDT in Chinese patients with primary tracheobronchial malignancy.

Effective Radiotherapy in Tracheobronchial Adenoid Cystic Carcinoma With Positive Surgical Margin

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. 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 χ2 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). 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). 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.

Intensity-Modulated Radiation Therapy May Improve Survival and Local Tumor Control in Resected Tracheo-Bronchial Adenoid Cystic Carcinoma: A Retrospective Study of 65 Cases

The evidence about survival benefit of radiation therapy (RT) in resected tracheo-bronchial adenoid cystic carcinoma (TACC) is limited. Advanced intensity-modulated radiation therapy (IMRT) technique could improve tumor dose conformality and reduce normal tissue exposure, which would result in a higher rate of local control and a longer survival time compared with conventional RT. This study aimed to evaluate the impact of postoperative IMRT on survival of TACC patients. Patients with TACC treated with surgery and adjuvant radiotherapy in our institution between 1976 and 2016 were retrospectively reviewed. The adjuvant RT was given when the margin was positive or the tumor was close to the resection margin. Patients were dichotomized into IMRT or non-IMRT group. The overall survival (OS), loco-regional recurrence free survival (LRFS), and distant metastasis free survival (DMFS) were calculated from the date of diagnosis. Cox regression was used to identify factors associated with survival and adjust for covariates (P value <0.1 in the univariate analysis). A total of 65 patients were eligible, including 30 with IMRT and 35 with non-IMRT. Patients in the IMRT group had a higher proportion of pathologic N1 stage (30.0% vs. 8.6%, P = 0.058). Other baseline characters (age, sex, tumor location, T stage (T1-2 vs. T3-4), and Charlson/Deyo comorbidity score) were well balanced. The 5- and 10-year OS of the IMRT group were 89.4% (95%CI: 70.6%-96.5%) and 83.9% (95%CI: 61.4%-93.8%), which were significantly superior to those of the non-IMRT group (78.2 % [95%CI: 59.4%-89.0%] and 50.3 % [95%CI: 30.2%-67.4%]; P = 0.031). IMRT was associated with significantly improved 5- and 10-year LRFS in comparison with non-IMRT (5-year: 96.3% [95%CI: 76.5%-99.5%] vs. 68.7% [95%CI: 49.4%-81.9%]; 10-year: 89.9% [95%CI: 63.6%-97.5%] vs. 44.4% [95%CI: 22.4%-64.3%]; P = 0.002). Only 2 of 30 patients (6.6%) developed loco-regional recurrence after IMRT, whereas 14 of 35 (40.0%) failed locally in the non-IMRT group. No difference in DMFS was observed between IMRT and non-IMRT groups (P = 0.680). There were no statistical differences in OS as for RT dose or adjuvant chemotherapy. Multivariable analysis identified IMRT as an independent favorable factor of OS (HR = 0.31, 95% CI: 0.11-0.85, P = 0.022) and LRFS (HR = 0.18, 95% CI: 0.04-0.81, P = 0.025) after adjusting for T stage (P = 0.100 on univariable analysis) and tumor size (P = 0.033). IMRT was an effective treatment which might confer superior local control and survival benefit in the setting of postoperative TACC.

Treatment outcomes of patients with tracheobronchial mucoepidermoid carcinoma compared with those with adenoid cystic carcinoma

ObjectiveTracheobronchial 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). MethodWe 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. ResultsA 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). ConclusionsTMEC 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.

Detecting MYB and MYBL1 fusion genes in tracheobronchial adenoid cystic carcinoma by targeted RNA-sequencing

Primary tracheobronchial adenoid cystic carcinoma is rare, accounting for less than 1% of all lung tumors. Many adenoid cystic carcinomas have been reported to have a specific chromosome translocation t(6;9)/MYB-NFIB. More recently, t(8;9)/MYBL1-NFIB gene fusion was reported in salivary gland adenoid cystic carcinomas which lacked a t(6;9)/MYB-NFIB. Two prior studies showed t(6;9)/MYB-NFIB in tracheobronchial adenoid cystic carcinoma; however, only rare cases of MYBL1 rearrangement have been reported in this carcinoma. In this study, we used targeted RNA sequencing to investigate fusion genes in tracheobronchial adenoid cystic carcinoma at our institution. Fusions of either MYB or MYBL1 genes were detected in 7 of 7 carcinomas. Three cases had MYB-NFIB, and 3 had MYBL1-NFIB. The remaining case showed a rare MYBL1-RAD51B fusion. These findings suggest that rearrangement involving MYB or MYBL1 is a hallmark of tracheobronchial adenoid cystic carcinoma.

Tracheo-bronchial adenoid cystic carcinoma: A retrospective study.

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. 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 andadjunctive therapy. 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. ACC of the trachea and bronchus is a rare and low-to-moderate grademalignant 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.

Tracheobronchial Adenoid Cystic Carcinoma: 50-Year Experience at the National Cancer Center, China

Only a small series of studies has investigated primary tracheobronchial adenoid cystic carcinoma (TACC), and its prognosis and prognostic factors have not been well defined. Patients with TACC diagnosed histologically between January 1967 and December 2017 at our institution were retrospectively analyzed. A total of 191 consecutive patients were included in our study. One hundred sixty-three patients underwent surgery, while the remaining 28 patients received nonoperative therapy. The 5-year, 10-year, and 20-year survival rates of the surgical group were 85.00%, 63.40%, and 47.00%, while the 5-year and 10-year survival rates of the nonoperative group were 63.70% and 46.40%, respectively. The multivariate analysis showed that the complaint duration (<7 months or ≥7 months) and treatment methods (R0 resection, R1 resection with radiotherapy, R1 resection without radiotherapy) were independent prognostic factors for disease-free survival of the R0/1 (resection with no residual tumor or microscopic residual tumor) resected patients, while the tumor size (≤3 cm or >3 cm) and treatment methods were independent prognostic factors for the overall survival. Sixty-four (45.1%) patients in the R0/1 group experienced recurrence/progression, and compared with chemotherapy or supportive treatment, local treatment significantly improved the prognosis of these patients (P<.050). The complaint duration and tumor size are independent prognostic factors for disease-free survival and overall survival in TACC, respectively. Complete resection of the primary tumor, postoperative adjuvant radiotherapy for patients with positive margins, and local treatment after postoperative recurrence or progression may contribute to better survival.

Predictors of nodal metastasis and prognostic significance of lymph node ratio and total lymph node count in tracheobronchial adenoid cystic carcinoma.

PurposeTo identify potential risk factors of lymph node metastasis and to verify the prognostic significance of the lymph node ratio (LNR) and the total number of lymph nodes examined (NNE) in tracheobronchial adenoid cystic carcinoma (ACC) patients, using a large population-based database.Patients and methodsFrom Surveillance, Epidemiology, and End Results database, we identified 263 patients with tracheobronchial ACC in whom complete lymph node data could be obtained. Logistic regression analysis was performed to determine predictive factors of nodal metastasis. X-tile software determined the optimal cut-off points for LNR and NNE. Kaplan– Meier analyses and Cox regression models were adopted for survival analysis.ResultsOf 263 patients, 75 (28.5%) had lymph node involvement. Tumors of bronchial origin (P<0.001) and tumors larger than 30 mm (P<0.001) were associated with a higher likelihood of nodal involvement. Examination of more than ten lymph nodes could avoid understaging and resulted in improved survival; meanwhile, patients with a LNR of 0.07 or less had favorable prognosis.ConclusionPatients with tracheobronchial ACC have significant risk of lymph node metastasis. Bronchial ACC and larger tumor size are both risk factors of lymph node metastasis. LNR and NNE may provide a more precise prediction of survival and could be taken into account in future clinical work.

A new approach to left sleeve pneumonectomy: complete VATS left pneumonectomy followed by right thoracotomy for carinal resection and reconstruction

BackgroundLeft sleeve pneumonectomy is a challenging operation that requires individualized approaches. Here, we present a new minimally invasive combined thoracoscopic approach.Case presentationA 61-year-old woman was diagnosed with tracheobronchial adenoid cystic carcinoma. The tumor originated from the left main stem bronchus, and tumor with carinal involvement was observed. We judged that complete resection would be possible via left sleeve pneumonectomy. However, because tumor involvement with the esophagus and descending aorta was suspected, evaluation of resectability in advance was necessary. After confirmation via examination thoracoscopy of no involvement with the surrounding organs, complete VATS left pneumonectomy was performed and followed by right thoracotomy for carinal resection and reconstruction.ConclusionsWhen thoracoscopic surgery becomes mainstream, this minimally invasive combined thoracoscopic approach might be an optimal option for patients who require left sleeve pneumonectomy.

The Prognostic Significance of Notch1 and Fatty Acid Binding Protein 7 (FABP7) Expression in Resected Tracheobronchial Adenoid Cystic Carcinoma: A Multicenter Retrospective Study.

PurposeAdenoid cystic carcinoma (ACC) of the trachea and bronchus is a rare tumor. Although MYB-NFIB oncogene fusion and Notch1 mutation have been identified in ACC, little is known about the expression and clinical significance of Notch1 and its target gene fatty acid binding protein 7 (FABP7) in tracheobronchial ACC.Materials and MethodsPrimary tracheobronchial ACC that were resected between 1998 and 2014 were identified through the pathology and oncology database from five thoracic oncology centers in China. A tissue array was constructed from the patients’ samples and the expressions of Notch1 and FABP7 were evaluated by immunohistochemistry. The association between the expression of both markers and survival was determined.ResultsOverexpression of Notch1 and FABP7, detected in 37.8% and 38.3% of 368 patients with tracheobronchial ACC, respectively, was an independent prognostic indicator for recurrencefree survival (RFS) by multivariable Cox proportional hazard model (p=0.032 and p=0.048, respectively). Overexpression of Notch1, but not of FABP7, predicted overall survival (OS) (p=0.018). When categorized into four groups according to coexpression of Notch1 and FABP7, patients with overexpression of both Notch1 and FABP7 belonged to the group with the shortest RFS and OS (p=0.01 and p=0.048, respectively).ConclusionExpression of Notch1 and FABP7, and coexpression of Notch1 and FABP7, is strongly associated with poor survival in resected tracheobronchial ACC. These data are consistent with the hypothesis that poor differentiation of tracheobronchial ACC correlates with the activation of Notch signaling.

Tracheobronchial adenoid cystic carcinoma treated with radical radiochemotherapy: Case report and review of literature

Tracheobronchial adenoid cystic carcinoma treated with radical radiochemotherapy: Case report and review of literature

Bronchoscopic intervention as a main treatment for tracheobronchial adenoid cystic carcinoma

Background: Bronchial adenoid cystic carcinoma (ACC) is a rare disease with low malignancy and indolent progression. Airway obstruction caused by ACC can be resolved by endoscopic procedures. The efficacy of different techniques of bronchoscopic interventions for ACC has not been determined. Material and Methods: From November 2004 to March 2012, ACC patients, mainly treated with different techniques of bronchoscopic interventions in our hospital, were reviewed. Results: The study included 37 ACC patients. Five patients (13.5%) with intra-luminal type underwent bronchoscopic therapies for a median of three times (range 1–6 times). Thirty-two patients (86.5%) with mixed type underwent bronchoscopic interventions for a median of 14 times (range 4–20 times). The dyspnea index was significantly improved after the first endoscopic procedure. The overall five- and ten-year survival rate was 85.9% and 45.9%, respectively, similar to surgery-dominant treatments. Conclusions: The present study demonstrates that different procedures of bronchoscopic interventions, as main treatments for ACC, are as effective as surgery-dominant treatment. More prospective and multicentric studies are required to confirm these favorable results, which may influence the therapeutic strategy for ACC in the future.

Adenoid cystic carcinoma of trachea: A rare tumor managed by diode laser and tracheal resection

Introduction: Adenoid cystic carcinoma (ACC) is a rare type of cancer, which mostly occurs in the salivary glands of the head and neck, and has also been reported in other parts including trachea as case reports or small case series. The patient may present as asthma or acute tracheobronchial obstruction and may require urgent management to relive obstruction. It has a propensity to spread along both submucosal and perineural planes which are responsible for late local recurrence even after complete resection. The best results can be obtained by resection with or without adjuvant radiotherapy. Case Report: We report a rare case of endoluminal adenoid cystic carcinoma of the distal trachea presenting with acute tracheal obstruction managed bronchoscopically with high power diode laser followed by segmental resection of trachea and radiotherapy with 18 months of follow-up with no evidence of recurrence. Conclusion: There are only very few reports available for the use of diode laser with flexible bronchoscope in tracheobronchial adenoid cystic carcinoma. However, rarity of such tumors makes it impossible to obtain such data. This technique, in such rare tumors, is safe with almost no or limited morbidity from our experience and available data.

Clinicopathological features of salivary and non-salivary adenoid cystic carcinomas

Adenoid cystic carcinoma (ACC), commonly from salivary glands, is known for its insidious local growth and usually protracted clinical course. ACC developing from non-salivary glands (i.e., non-salivary ACC) is heterogeneous, and its clinicopathological features remain poorly defined. Patients treated for ACC in a single institution between 1995 and 2007 were included in this study. Immunohistochemical evaluation of Ki-67, E-cadherin, p16, and cyclinD1 was performed. The prognostic significance of clinical and immunophenotypic markers was evaluated. 83 cases of salivary ACC and 24 cases of non-salivary ACC were included. The expression levels of Ki-67 (54.8%), E-cadherin (90.4%), p16 (32.9%), and cyclinD1 (19.2%) between ACCs present at various sites were not different. Sinonasal, lacrimal, and tracheobronchial ACCs had significantly worse outcomes than those of ACC of the major salivary glands. Postoperative radiotherapy reduced the recurrence rate of patients with a negative resection margin (P=0.028). Older age (age >60 years), advanced stage, positive resection margin, high histological grade, and high expression of Ki-67 were significantly correlated with poor prognosis. In conclusion, the site of origin plays a role in the prognosis of ACC, in which positive resection margin and advanced stage are possible factors underlying the differences in outcomes.

Clinicopathological study of surgically treated cases of tracheobronchial adenoid cystic carcinoma

Between 1980 and 2007, five patients were pathologically diagnosed as tracheobronchial adenoid cystic carcinoma (ACC). All five patients were women aged 37-67 years. Four tumors were located in the larger airways, and one tumor was located in the peripheral lung. The following operations were done: bronchoplastic procedures in three (carinal resection with doublebarreled carinoplasty in one, sleeve right pneumonectomy in one, sleeve middle lobectomy in one), left pneumonectomy in one, and left upper lobectomy in one. Three of the five patients have survived for 172, 144, and 10 months after surgery, respectively. The best local treatment for ACC of the major airway is considered to be sleeve resection of the trachea or bronchus in an area where airway reconstruction may not be disturbed and to add postoperative irradiation when there is residual carcinoma at the stump. However, it seems controversial to recommend adjuvant radiotherapy in all patients undergoing resection.

Palliation of a tracheobronchial adenoid cystic carcinoma using a Harrell Y Stent: The complex anesthetic considerations of a shared airway, a stenotic, necrotic tracheal lesion and bronchoscopic stent placement

Palliation of a tracheobronchial adenoid cystic carcinoma using a Harrell Y Stent: The complex anesthetic considerations of a shared airway, a stenotic, necrotic tracheal lesion and bronchoscopic stent placement