SESSION TITLE: Systemic Diseases with Deceptive Pulmonary ManifestationsSESSION TYPE: Rapid Fire Case ReportsPRESENTED ON: 10/18/2022 12:25 pm - 01:25 pmINTRODUCTION: Multiple endocrine neoplasia type 1 (MEN1) is a rare heritable disorder with an occurrence of 2 in 100,000 and is characterized by a predisposition to tumors of the parathyroid, anterior pituitary, and pancreatic islet cells. Other tumors associated with MEN 1 are thymic or bronchial carcinoids, gastric tumors, adrenocortical adenomas, and lipomas. Bronchopulmonary carcinoid is an uncommon phenotypic manifestation of MEN1 with a minimum histologically confirmed prevalence of 5 %.CASE PRESENTATION: A 37-year-old female with MEN 1 and a history of recurrent pituitary and parathyroid tumors status post resection in 2010 and 2011 respectively, was admitted for evaluation of shortness of breath. Her symptoms were insidious in onset and gradually progressive with associated wheezing and weight loss of 15 pounds over one month. She has a family history of MEN 1 with pancreatic and pituitary tumors in her mother and maternal uncle. Physical examination and was pertinent for tachypnea, wheezing, decreased breath sounds in the right lung field. Computed tomography (CT) of chest showed tracheal and bilateral mainstem bronchial mass with exophytic extension compressing adjacent structures. Flexible fiberoptic bronchoscopy with endobronchial ultrasound revealed endotracheal papillary lesion, bilateral mainstem endobronchial lesions, and mediastinal lymphadenopathy. She underwent debulking using cauterization and cryotherapy. Tracheal mass biopsy was positive for atypical carcinoid with a high mitotic count.DISCUSSION: Lung neuroendocrine tumors (NETs) are groups of pulmonary neoplasms and account for 1-2 % of all lung malignancies and roughly 20-30% of all NETs. They are mostly sporadic, however, they can rarely occur in the setting of MEN 1 (5%). NETs are classified as low/ intermediate grade (typical and atypical carcinoid) and high grade ( small cell lung carcinoma and large cell carcinoma). Majority of them arise in proximal airways and cause symptoms from obstruction or bleeding. 25% of cases are found incidentally as a peripheral solitary pulmonary nodule. Approximately 75% of lung NETs are centrally located and are amenable to bronchoscopic biopsy. Lung NETs are staged using TNM classification which is used to stage other bronchogenic lung cancers. Surgical resection and mediastinal lymph node dissection are recommended for patients with low or intermediate grade NET whose medical condition and pulmonary reserve will tolerate it. Endobronchial laser resection is reserved for patients with central airway occlusion who are poor surgical candidates. Radiation, platinum-based chemotherapy, and everolimus are reserved for patients with aggressive and unresectable NETs.CONCLUSIONS: Bronchopulmonary NETs can be seen in MEN 1, but do not decrease overall survival. However, poorly differentiated and aggressive NETs can lead to death with 10-year survival rates of 59% in atypical carcinoids.Reference #1: Kamilaris, C. D., & Stratakis, C. A. (2019). Multiple endocrine neoplasia type 1 (MEN1): An update and the significance of early genetic and clinical diagnosis. Frontiers in Endocrinology, 10. https://doi.org/10.3389/fendo.2019.00339Reference #2: Ramirez RA, Beyer DT, Diebold AE, et al. Prognostic Factors in Typical and Atypical Pulmonary Carcinoids. Ochsner J. 2017;17(4):335-340.Reference #3: Yao, J. C., Fazio, N., Singh, S., Buzzoni, R., Carnaghi, C., Wolin, E., Tomasek, J., Raderer, M., Lahner, H., Voi, M., Pacaud, L. B., Rouyrre, N., Sachs, C., Valle, J. W., Fave, G. D., Van Cutsem, E., Tesselaar, M., Shimada, Y., Oh, D.-Y., … Pavel, M. E. (2016). Everolimus for the treatment of advanced, non-functional neuroendocrine tumours of the lung or gastrointestinal tract (radiant-4): A randomised, placebo-controlled, phase 3 study. The Lancet, 387(10022), 968–977. https://doi.org/10.1016/s0140-6736(15)00817-xDISCLOSURES: No relevant relationships by Navdeep DehalNo relevant relationships by Suchitra PilliNo relevant relationships by Stanley Thomas SESSION TITLE: Systemic Diseases with Deceptive Pulmonary Manifestations SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 12:25 pm - 01:25 pm INTRODUCTION: Multiple endocrine neoplasia type 1 (MEN1) is a rare heritable disorder with an occurrence of 2 in 100,000 and is characterized by a predisposition to tumors of the parathyroid, anterior pituitary, and pancreatic islet cells. Other tumors associated with MEN 1 are thymic or bronchial carcinoids, gastric tumors, adrenocortical adenomas, and lipomas. Bronchopulmonary carcinoid is an uncommon phenotypic manifestation of MEN1 with a minimum histologically confirmed prevalence of 5 %. CASE PRESENTATION: A 37-year-old female with MEN 1 and a history of recurrent pituitary and parathyroid tumors status post resection in 2010 and 2011 respectively, was admitted for evaluation of shortness of breath. Her symptoms were insidious in onset and gradually progressive with associated wheezing and weight loss of 15 pounds over one month. She has a family history of MEN 1 with pancreatic and pituitary tumors in her mother and maternal uncle. Physical examination and was pertinent for tachypnea, wheezing, decreased breath sounds in the right lung field. Computed tomography (CT) of chest showed tracheal and bilateral mainstem bronchial mass with exophytic extension compressing adjacent structures. Flexible fiberoptic bronchoscopy with endobronchial ultrasound revealed endotracheal papillary lesion, bilateral mainstem endobronchial lesions, and mediastinal lymphadenopathy. She underwent debulking using cauterization and cryotherapy. Tracheal mass biopsy was positive for atypical carcinoid with a high mitotic count. DISCUSSION: Lung neuroendocrine tumors (NETs) are groups of pulmonary neoplasms and account for 1-2 % of all lung malignancies and roughly 20-30% of all NETs. They are mostly sporadic, however, they can rarely occur in the setting of MEN 1 (5%). NETs are classified as low/ intermediate grade (typical and atypical carcinoid) and high grade ( small cell lung carcinoma and large cell carcinoma). Majority of them arise in proximal airways and cause symptoms from obstruction or bleeding. 25% of cases are found incidentally as a peripheral solitary pulmonary nodule. Approximately 75% of lung NETs are centrally located and are amenable to bronchoscopic biopsy. Lung NETs are staged using TNM classification which is used to stage other bronchogenic lung cancers. Surgical resection and mediastinal lymph node dissection are recommended for patients with low or intermediate grade NET whose medical condition and pulmonary reserve will tolerate it. Endobronchial laser resection is reserved for patients with central airway occlusion who are poor surgical candidates. Radiation, platinum-based chemotherapy, and everolimus are reserved for patients with aggressive and unresectable NETs. CONCLUSIONS: Bronchopulmonary NETs can be seen in MEN 1, but do not decrease overall survival. However, poorly differentiated and aggressive NETs can lead to death with 10-year survival rates of 59% in atypical carcinoids. Reference #1: Kamilaris, C. D., & Stratakis, C. A. (2019). Multiple endocrine neoplasia type 1 (MEN1): An update and the significance of early genetic and clinical diagnosis. Frontiers in Endocrinology, 10. https://doi.org/10.3389/fendo.2019.00339 Reference #2: Ramirez RA, Beyer DT, Diebold AE, et al. Prognostic Factors in Typical and Atypical Pulmonary Carcinoids. Ochsner J. 2017;17(4):335-340. Reference #3: Yao, J. C., Fazio, N., Singh, S., Buzzoni, R., Carnaghi, C., Wolin, E., Tomasek, J., Raderer, M., Lahner, H., Voi, M., Pacaud, L. B., Rouyrre, N., Sachs, C., Valle, J. W., Fave, G. D., Van Cutsem, E., Tesselaar, M., Shimada, Y., Oh, D.-Y., … Pavel, M. E. (2016). Everolimus for the treatment of advanced, non-functional neuroendocrine tumours of the lung or gastrointestinal tract (radiant-4): A randomised, placebo-controlled, phase 3 study. The Lancet, 387(10022), 968–977. https://doi.org/10.1016/s0140-6736(15)00817-x DISCLOSURES: No relevant relationships by Navdeep Dehal No relevant relationships by Suchitra Pilli No relevant relationships by Stanley Thomas