Tracheobronchial Adenoid Cystic Carcinoma Treated Successfully With Chemoradiotherapy Followed by Durvalumab: A Case Report.

BACKGROUND/AIM: Tracheobronchial adenoid cystic carcinoma (ACC) is a rare type of malignancy. Although complete resection is standard treatment for localized ACC, treatment for unresectable ACC has not been established. It is unclear whether concurrent chemoradiotherapy (CCRT) followed by immune checkpoint inhibitor (ICI) therapy is effective for ACC. CASE REPORT: A 49-year-old man was admitted to our hospital for the treatment of dyspnea and thickening of the bronchial wall from the tracheal carina to the left main bronchus, as observed on a CT scan. Systemic examinations and transbronchial biopsy led to a diagnosis of locally advanced ACC. Although radiotherapy and chemotherapy are not regarded as very sensitive for ACC, a favorable response was obtained with CCRT. Following CCRT, he received ICI therapy with durvalumab for 1 year. The patient has remained in a stable condition 18 months after therapy, with no recurrence. CONCLUSION: ICI after CCRT might be a promising treatment option for unresectable tracheobronchial ACC.

Diagnostic and Therapeutic Role of Electrocautery Snare Use in Endobronchial Polypoid Lesions.

INTRODUCTION: Electrocautery with a snare probe offers a tool for the treatment of endobronchial polyps. The aim of this study was to demonstrate the efficacy of the snare probe in patients undergoing rigid bronchoscopy due to central airway obstruction. METHODS: This retrospective descriptive study included patients who underwent rigid bronchoscopy with an electrocautery snare probe for the diagnosis and/or treatment of endobronchial polyps in an interventional pulmonology unit. RESULTS: The mean age of the 47 patients (38 men) with endobronchial polypoid lesions was 61.5 years. Six lesions (12.8%) were located in the trachea, 6 (12.8%) in the right main bronchus, 11 (23.4%) in the left main bronchus, and 24 (51.1%) in the lobar bronchi. Twenty-eight lesions (59.5%) were malignant. Prior to the procedure, 6 (12.8%) patients had grade 1 obstruction, 6 (12.8%) had grade 2 obstruction, 15 (31.9%) had grade 3 obstruction, and 20 (42.6%) had grade 4 obstruction. In terms of airway obstruction after the procedure, grade 1 was present in 46 (97.9%) patients and grade 2 was present in 1 (2.1%) patient. No major complication developed in 93.6% of the patients. During a mean follow-up period of 48 months, 85.1% of the patients did not experience recurrence. CONCLUSION: Our results demonstrate that the snare probe can be used to effectively and reliably establish airway patency in patients with central airway obstruction due to endobronchial polypoid lesions.

Misdiagnosed Asthma Turned Out to Be Bronchial Fibroepithelial Polyp: Case Report and Review of Literature.

Bronchial fibroepithelial polyps are exceedingly rare with few cases have been reported. They can manifest with a wide array of symptoms; ranging from being totally asymptomatic, cough, refractory dyspnea, and hemoptysis. In our case, our patient's condition was diagnosed and was managed as asthma. It is one of the rare benign conditions to be encountered, shares similar morphology with other tumors such as angiomyofibroblastoma, aggressive angiomyxoma, and cellular angiofibroma. These lesions have a slow growth pattern which may end up with obstruction. According to the tumor size and symptoms caused by it, treatment varies from observation to complete resection. This case describes an incidental finding of fibroepithelial polyp in the main bronchus for a patient with long-term refractory cough for 5 years, was misdiagnosed to have asthma. Diagnosis typically involves imaging and bronchoscopy, followed by appropriate therapeutic measures and careful monitoring to assess the prognosis.

Treatment outcome, toxicity, and quality of life of patients with bronchus-associated lymphoid tissue lymphoma.

The disease failure patterns and optimal treatment of bronchus-associated lymphoid tissue (BALT) lymphoma are unknown. This retrospective study involved 71 patients with primary BALT lymphoma who had received radiotherapy (RT), surgery, immunochemotherapy (IC), or observation. The median follow-up time was 66 months. The 5-year overall survival and lymphoma-specific survival were 91.2% and 96.1%, respectively, and were not significantly different among treatments. The 5-year cumulative incidence of overall failure for RT, surgery, IC, and observation was 0%, 9.7% (p = .160), 30.8% (p = .017), and 31.3% (p = .039). There was no grade ≥3 toxicity in RT group according to the CTCAE 5.0 reporting system. Quality of life (QoL) was at similarly good levels among the treatment groups. BALT lymphoma had a favorable prognosis but persistent risk of relapse after IC or observation. Given the very low disease failure risk and good QoL, RT remains an effective initial treatment for BALT lymphoma.

BALT lymphoma has a favorable prognosis but a persistent progression and relapse risk.Radiotherapy is associated with lower failure of disease progression and relapse, low toxicity and good quality of life.

SALTT study: A retrospective analysis of 111 SAlivary gland tumors of Lung and Tracheobronchial Tree.

INTRODUCTION: Primary pulmonary salivary gland-type tumours (PPSGT) are rare lung neoplasms arising from submucosal seromucinous glands in the central airway. METHODS AND RESULTS: We retrospectively analysed the clinicopathological features of 111 PPSGTs diagnosed at our institute between 2003 and 2021. The mean age at diagnosis was 43.8 years(range 6-78 years) and a male-to-female ratio of 2:1. On imaging, 92 % of cases had centrally located tumours and 37.3 % were early stage. The histopathological types included 70 cases (63 %) of mucoepidermoid carcinoma (MEC), 31 cases (27.7 %) of adenoid cystic carcinoma (ADCC), two cases of myoepithelial carcinoma, one case each of acinic cell carcinoma (ACC), clear cell carcinoma (CCC), epithelial myoepithelial carcinoma (EMC) and 5 others [including adenocarcinoma of minor salivary gland origin(n = 3), carcinoma with sebaceous differentiation(n = 1) and poorly differentiated carcinoma of salivary gland type(n = 1)]. The size of the tumours found in the resection specimens ranged from 1 cm to 13 cm, with an average size of 4.9 cm. High-risk attributes such as lymphovascular invasion (LVI), perineural invasion (PNI), pleural involvement, positive resection margins, and nodal metastasis were identified in 15.3 %, 15.3 %, 13.6 %,15.2 % and 6.7 % of cases, respectively. These attributes were found to be more frequent in ADCC than in MEC. Surgery was the main treatment modality [68/84 (80 %) cases]. ADCC cases had more recurrence and distant metastasis than MEC cases. The 3- year overall-survival (OS) and recurrence-free survival(RFS) were better in patients with age lesser than 60 years(p-value <0.0001), low pT stage (p-value 0.00038) and lower grade of MEC(p-value-0.0067). CONCLUSION: It is crucial to have an acquaintance with the morphologic spectrum and immunophenotypic characteristics of PPSGT to recognize them in this unusual location. In tandem, it is crucial to differentiate them from conventional primary non-small cell lung carcinoma, as the management protocols and prognostic implications differ significantly.

Non-resolving pneumonia: primary pulmonary MALT lymphoma.

Mucosa-associated lymphoid tissue (MALT) lymphoma is an uncommon extranodal low-grade B-cell lymphoma. Pulmonary MALT lymphomas originate from bronchial MALT and are also referred to as bronchial-associated lymphoid tissue lymphomas. MALT lymphomas of the lung are slow-growing tumours and usually present as asymptomatic chronic alveolar opacities visible on chest radiographs or with non-specific pulmonary symptoms. Here we described a case of a male patient in his early 50s with cough and chest pain for 4 years. His CT chest scan showed consolidation in the lingula and left lower lobe. Histopathology of the specimen obtained from cryobiopsy of the lung lesion showed a dense monomorphic lymphoid infiltrate, and immunohistochemistry confirmed the diagnosis of MALT lymphoma. The prognosis of pulmonary MALT lymphomas is good with >80% 5-year survival rates. This case highlights that MALT lymphoma should be considered as a differential diagnosis while evaluating cases with non-resolving consolidation.

Multiple bronchial carcinoids associated with Cowden syndrome.

Cowden syndrome (CS) is a rare genetic condition due to the various germline mutations in the phosphatase and tensin homologue on chromosome ten (PTEN) tumour suppressor gene. As a result, CS is characterised by an increased risk of developing various benign and malignant tumours, such as thyroid, breast, endometrial and urogenital neoplasms, as well as gastrointestinal tract tumours. However, the neuroendocrine tumour association with CS is not elucidated yet. We present a case of a 46-year-old male patient diagnosed with testicular seminoma and follicular thyroid cancer in his medical history. Our patient met the clinical diagnostic criteria of Cowden syndrome. Genetic analysis established the clinical diagnosis; a known heterozygous PTEN mutation was detected [PTEN (LRG_311t1)c.388 C > T (p.Arg130Ter)]. Incidentally, he was also seen with multiple pulmonary lesions during his oncological follow-up. A video-assisted thoracoscopic left lingula wedge resection and later resections from the right lung were performed. Histological findings revealed typical pulmonary carcinoid tumours and smaller tumorlets. Somatostatin receptor SPECT-CT, 18F-FDG-PET-CT and 18F-FDOPA-PET-CT scans and endoscopy procedures could not identify any primary tumours in other locations. Our patient is the first published case of Cowden syndrome, associated with multifocal pulmonary carcinoids. Besides multiple endocrine neoplasia type 1, we propose Cowden syndrome as another hereditary condition predisposing to multiple pulmonary tumorlets and carcinoid tumours.

Prognostic factors and constructing a nomogram in tracheal cancer patients treated with surgical intervention: A study based on SEER database.

Although surgery is considered the first choice of treatment for patients diagnosed with tracheal cancer, the prediction of overall survival (OS) in patients undergoing surgical intervention is poor. To address this issue, we developed a nomogram that combined a risk classification system to estimate the OS of patients with tracheal cancer who underwent surgical intervention. A total of 525 qualified patients were selected from the surveillance, epidemiology, and end results database between 1975 and 2018 and were randomly divided into training and validation cohorts (7:3). The parameters of independent prognostic ability were determined using Cox regression analysis, and a nomogram was formed. The predictive ability of the nomogram was tested using the area under the curve of receiver operating characteristic curves and calibration curves. Survival curves were assessed between the different risk classification groups using the Kaplan-Meier method. The results indicated that Age, stage, histology, and tumor size were independent prognostic factors and were included in the predictive model. The calibration plots demonstrated good agreement between the nomogram prediction and actual observation for 24- and 36-month OS. The receiver operating characteristic curves suggested that the predictive model had good discrimination ability, with the area under the curves (training group 0.817, 0.785, and 0.801, respectively) and validation group (0.744, 0.794, and 0.822, respectively). Furthermore, the low-risk group had a better prognosis than the high-risk group in the total, training, and validation cohorts (all P < .001). This study established a novel nomogram system to predict OS and identify independent prognostic factors in patients with tracheal cancer who underwent surgical intervention. This model has the potential to assist doctors in making decisions regarding treatment options.

Interventional treatment of giant tracheal lymphoma under rigid bronchoscopy: A case report and literature review.

INTRODUCTION: Lymphoma can appear in all parts of the body and present with different symptoms. However, bronchial lymphoma is rare and can be misdiagnosed as airway malignancy or lung disease.Patient: An older adult woman with tracheal lymphoma experienced severe breathing difficulties, and chest computed tomography indicated severe narrowing of the airway. She did not respond to repeated antibiotic treatment, and she was eventually diagnosed with lymphoma based on pathology after surgical removal of the tumor. DIAGNOSIS: The patient received a diagnosis of thoracic tracheal stenosis due to intratracheal inflammatory granulomatous lesions or a tumor. INTERVENTIONS: Treatment involved the use of a high-frequency electrotome, freezing, and argon plasma coagulation. OUTCOMES: The patient reported improvements in dyspnea, cough, and other symptoms after the operation. The pathological results confirmed follicular lymphoma. Reexamination by fiberbronchoscopy indicated that the degree of stenosis in the middle and upper tracheal segments was significantly reduced following interventional therapy. CONCLUSION: Endoscopic interventional therapy can be an effective treatment for tracheal lymphoma.

What's new in benign lung tumours?

While lung cancer is one of the most common malignancies routinely encountered by pathologists, benign pulmonary neoplasms are quite rare. However, it is important for pathologists to be familiar with the typical diagnostic features of benign lung tumors to avoid confusing them with malignant morphological mimics. There have also been intriguing discoveries in the genetics of benign pulmonary neoplasms in the past decade. This review will cover several of the most common benign lung tumors, including the diagnostic categories of pulmonary adenomas, bronchial papillomas, and benign mesenchymal tumors, with discussion of the current classification, differential diagnosis, and current knowledge regarding genetic drivers.

[Features of surgical treatment of central tracheal and bronchial carcinoid]. / Osobennosti khirurgicheskogo lecheniya tsentral'nogo kartsinoida trakhei i bronkhov.

OBJECTIVE: To analyze features of surgical treatment of central tracheal and bronchial carcinoid. MATERIAL AND METHODS: A retrospective analysis included 115 patients with carcinoid tracheal and bronchial tumors who have been examined and treated from 1974 to the present. The majority of patients (97, 84.3%) had central form of carcinoid of the trachea, bronchi and lungs. Of these, 95 (97.9%) ones underwent surgical treatment. RESULTS: We used pre- and intraoperative diagnostics including bronchotomy. This approach provided organ-sparing surgery with resection and reconstruction of the bronchi and trachea in 71 (74.7%) patients including complete preservation of lung function in 20 (21.1%) cases and lobectomy/segmentectomy with resection and reconstruction of the bronchi in 51 (53.7%) cases. Two patients underwent pneumonectomy with wedge-shaped and marginal resection and reconstruction of tracheal bifurcation. Postoperative complications developed in 4 (4.2%) patients, and 2 (2.1%) ones died. Overall 5-year survival after radical surgeries was 89.2% (100% in typical carcinoid and 78.0% in atypical carcinoid).

Rare primary pulmonary mucosa-associated lymphoid tissue lymphoma misdiagnosed with tuberculosis: A case report.

RATIONALE: Primary pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) is a rare subtype of non-Hodgkin lymphoma with a relatively low incidence rate clinically. Atypical clinical symptoms and nonspecific chest computed tomography features of the disease make it difficult to determine and treatment is delayed. We discuss the diagnosis and treatment of a patient with primary pulmonary MALToma to raise clinicians' awareness of this condition. PATIENT CONCERNS: A 66-year-old male patient with a medical history of tuberculosis has been experiencing progressive exacerbation of respiratory symptoms and nonresponsive treatment without an unclear diagnosis for 5 years. He was transferred to our hospital because a nonspecific soft tissue mass in the right upper lobe of the lung was found on his chest computed tomography. Laboratory results with serum immunofixation electrophoresis showed polyclonal immunoglobulin (Ig) G, IgM, IgA, and λ-light chain on admission. DIAGNOSIS: Pathological examination and immunohistochemical staining of lung biopsy revealed a definitive diagnosis of pulmonary MALToma with stage IV. INTERVENTIONS AND OUTCOMES: The patient received immunotherapy with anti-CD20 monoclonal antibody (rituximab), and showed significant clinical improvement at the 6-month follow-up. CONCLUSIONS AND LESSONS: Diagnosis of primary pulmonary MALToma mainly relies on histopathological examination, and comprehensive laboratory examinations are also necessary. Clinicians should combine laboratory tests (such as immunofixation electrophoresis in our case) to assist in medical diagnosis in cases of atypical clinical manifestations and imaging characteristics. Immunotherapy appears to be the main treatment protocol for advanced patients.

An unexpected encounter and outcome between endobronchial lipoma and carcinoma: a case report and literature review.

Bronchial lipoma is a rare benign tumor of the lung, which is often misdiagnosed due to concomitant pulmonary diseases. In addition, the coexistence of endobronchial lipoma and lung cancer is extremely unusual. To date, no related computed tomography (CT) images have been reported. The patient was a 53-year-old man, who was admitted to our hospital with cough, yellow phlegm, and fever for 1 week. The CT image showed an irregular mass in the medial segment of the right middle lobe (B4a) with surrounding ground glass opacity, and another solid nodule in the right lower lobe (B6b). Unfortunately, after 2 weeks of anti-inflammatory treatment, the bronchial invasion of the B4a nodule did not decrease significantly, so further bronchoscopy was carried out and tumor resection was performed using endoscopic mucosal resection with a ligation device (EMR-L). During the follow-up 4 months, it was found that the B6b nodule was marked enlargement and then removed. The lesions of the B4a and B6b were confirmed as endobronchial lipoma and squamous cell carcinoma (T1aN0M0) by histopathology and immunohistochemical staining, respectively, and no postoperative radiotherapy or chemotherapy was performed. Regrettably, after 29 months of follow-up, we observed recurrence and slow enlargement of the lipoma in its original location, progressive emphysema in both lungs, and solitary chest wall metastasis from the B6b squamous cell carcinoma that had been resected. Therefore, endobronchial endoscopy resection should be carefully selected for larger endobronchial lipoma. If it is accompanied by early squamous cell carcinoma (T1aN0M0), we still recommend active postoperative chemoradiotherapy.

A rare case: Endobronchial solitary mixed papilloma.

Endobronchial solitary papillomas are extremely rare lung neoplasms originating from the bronchial surface epithelium. They often present with cough or recurrent hemoptysis. These tumors are benign, but they should be followed closely because they may even have a low probability of malignant transformation features. It should be kept in mind that malignancy may develop especially if the patient is a smoker. Although the etiology is not known for certain, it is thought to be caused by human papillomavirus in some cases. A 43-year-old male patient was admitted with a complaint of chronic cough. Rigid bronchoscopy was performed for diagnostic and therapeutic purposes after imaging techniques revealed a lesion obstructing the lumen of the right main bronchus. The pathology result was reported as mixed bronchial papilloma. We aimed to present our case because of its rarity and to indicate that chronic cough must be further evaluated.

Pulmonary mucosa-associated lymphoid tissue lymphoma with Sjögren's syndrome and literature review: A case report.

INTRODUCTION: A 54-year-old woman was admitted to hospital with chest tightness, shortness of breath, and chest pain on exertion. Her chest computed tomography showed a space-occupying lesion in the right lower lobe of the lung. CASE PRESENTATION: The ultrasound-guided right lung mass biopsy showed mucosa-associated lymphoid tissue (MALT), and the patient was diagnosed with Sjögren's syndrome (SS). The patient's symptoms were partially relieved with chemotherapy. CONCLUSIONS: Autoimmune diseases like SS and systemic lupus erythematosus are recognized risk factors for pulmonary MALT. It is necessary to identify predictors of malignant transformation of SS to pulmonary MALT lymphoma.

Identification of common genetic features and pathways involved in pulmonary lymphangioleiomyomatosis and ER-positive breast cancer.

Accumulating evidence suggests that patients with pulmonary lymphangioleiomyomatosis (PLAM) have a markedly higher prevalence of breast cancer (BC) than the general population. However, the underlying pathophysiological mechanisms remain unclear. Therefore, in this study, we employed a bioinformatics approach to understand the association between PLAM and estrogen receptor (ER)-positive BC. The PLAM (GSE12027) and ER-positive BC (GSE42568, GSE29044, and GSE29431) datasets were obtained from the Gene Expression Omnibus database, and GEO2R was used to identify common differentially expressed genes (DEGs) between them. Functional annotation was performed, and a protein-protein interaction (PPI) network was constructed. Hub genes were identified and verified using western blotting and immunohistochemistry. We conducted an immune infiltration analysis; based on the results, selected 102 common DEGs for follow-up analysis. Functional analyses revealed that the DEGs were mostly enriched in cell proliferation, gene expression regulation, and tumor-related pathways. Four hub genes-ESR1, IL6, PLA2G4A, and CAV1-were further analyzed, and CAV1 was revealed to be associated with clinical outcomes and immune infiltration in ER-positive BC. This study proposes a common, possible pathogenesis of PLAM and ER-positive BC. These common pathways and pivotal genes may provide new directions for further mechanistic studies.