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Bronchoscopic management of a primary endobronchial salivary epithelial-myoepithelial carcinoma: A case report.

ABSTRACT: Here, we discussed a 55 y/o African man who recently immigrated from Nigeria to the United States and who presented to Parkland Memorial Hospital with a productive, intermittent cough of one year duration. The cough was associated with shortness of breath and chest pain. Cough was not associated with voice hoarseness, hemoptysis, melanoptysis, and wheezing. He had a computed tomography (CT) scan of the chest that showed a 1.9 cm mass in the right main stem bronchus with ipsilateral right lower lobe consolidation and bronchiectasis. The patient was seen by pulmonology who recommended bronchoscopy for diagnosis and possible intervention. Bronchoscopy showed a 90% obstructing mass in the proximal right mainstem bronchus and bronchus intermedius. The mass was large and endobronchial, circumferential, exophytic, and polypoid. The decision was made to undergo bronchoscopic tumor ablation using electrocautery snare, argon plasma coagulation (APC), suction, and forceps. The tumor was successful ablated. Microscopic examination revealed eosinophilic ducts tightly coupled with a surrounding layer of clear cell myoepithelial cells and the diagnosis of epithelial-myoepithelial carcinoma (EMC) of the lung was made. The patient was discharged from the hospital with scheduled outpatient visits for monitoring of the carcinoma by pulmonology and thoracic surgery. Unfortunately, he was lost to follow up.

SUBMITTER: Patterson DT

PROVIDER: S-EPMC7232110 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Bronchoscopic management of a primary endobronchial salivary epithelial-myoepithelial carcinoma: A case report.

Patterson Dalton T DT Halverson Quinn Q Williams Sarah S Bishop Justin A JA Ochoa Cristhiaan D CD Styrvoky Kim K

Respiratory medicine case reports 20200507

Here, we discussed a 55 y/o African man who recently immigrated from Nigeria to the United States and who presented to Parkland Memorial Hospital with a productive, intermittent cough of one year duration. The cough was associated with shortness of breath and chest pain. Cough was not associated with voice hoarseness, hemoptysis, melanoptysis, and wheezing. He had a computed tomography (CT) scan of the chest that showed a 1.9 cm mass in the right main stem bronchus with ipsilateral right lower l ...[more]

PMID: 32435581

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Project description:BackgroundSecretory myoepithelial carcinomas (SMCA) are rare, mucinous, signet ring predominant tumors with primitive myoepithelial features. While many mucinous salivary gland tumors have now been molecularly characterized, key drivers in SMCA have yet to be elucidated. Recently, NKX3.1, a homeodomain transcription factor implicated in salivary mucous acinar development was also shown in a subset of salivary mucinous neoplasms, salivary intraductal papillary mucinous neoplasms (SG-IPMN). To date, NKX3.1 expression has not been characterized in other mucinous salivary lesions. Here, we report molecular and extended immunophenotypic findings in SMCA and NKX3.1 expression in the context of other head and neck lesions.MethodsWe retrieved 4 previously reported SMCA, performed additional immunohistochemical and targeted next-generation sequencing (NGS). We also investigated the use of NKX3.1 as a marker for SMCA in the context of its prevalence and extent (using H-score) in a mixed cohort of retrospectively and prospectively tested head and neck lesions (n = 223) and non-neoplastic tissues (n = 66).ResultsNKX3.1 positivity was confirmed in normal mucous acini as well as in mucous acinar class of lesions (5/6, mean H-score: 136.7), including mucinous adenocarcinomas (3/4), SG-IPMN (1/1), and microsecretory adenocarcinoma (MSA) (1/1). All SMCA were positive. Fluorescence in situ hybridization for SS18 rearrangements were negative in all successfully tested cases (0/3). NGS was successful in two cases (cases 3 and 4). Case 3 demonstrated a PTEN c.655C>T p.Q219* mutation and a SEC16A::NOTCH1 fusion while case 4 (clinically aggressive) showed a PTEN c.1026+1G>A p.K342 splice site variant, aTP53 c.524G>A p.R175H mutation and a higher tumor mutation burden (29 per Mb). PTEN immunohistochemical loss was confirmed in both cases and a subset of tumor cells showed strong (extreme) staining for P53 in Case 4.ConclusionDespite a partial myoepithelial phenotype, SMCA, along with mucinous adenocarcinomas/SG-IPMN and MSA, provisionally constitute a mucous acinar class of tumors based on morphology and NKX3.1 expression. Like salivary mucinous adenocarcinomas/SG-IPMN, SMCA also show alterations of the PTEN/PI3K/AKT pathway and may show progressive molecular alterations. We document the first extramammary tumor with a SEC16A::NOTCH1 fusion.

Dataset Information

Bronchoscopic management of a primary endobronchial salivary epithelial-myoepithelial carcinoma: A case report.

Publications

Bronchoscopic management of a primary endobronchial salivary epithelial-myoepithelial carcinoma: A case report.

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