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A7343 - Multiple Tumorlets in the Setting of Pulmonary Carcinoid, What Is the Appropriate Management?
Author Block: F. G. Wilder1, J. Lin1, L. Li2, D. Upadhyay3; 1Surgery, UCSF - Fresno, Fresno, CA, United States, 2UCSF - Fresno, Fresno, CA, United States, 3UCSF, Fresno, CA, United States.
Background: Diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disease that is believed to a precursor of pulmonary tumorlets. Tumorlets are nodular proliferations of neuroendocrine cells that are no greater than 5mm. The clinical significance of DIPNECH has been long debated, but in 2011 was classified by the World Health Organization (WHO) as a preinvasive precursor to carcinoid. Patients are often asymptomatic with mild airflow limitations. However, care for patients with this disease process remains unclear. Specifically, care for patients with multiple tumorlets in the setting of a carcinoid tumor is unclear as there is no established algorithm for diagnosis or management. Reported management approaches have included steroids, chemotherapy and surgical resection. The aim of this review was to evaluate the presentation and outcomes of patients with multiple tumorlets in the setting of carcinoid tumors to help determine which management strategy may be associated with better outcomes. Methods: A review of a single-institution experience was performed to identify patients with diagnosis of carcinoid tumor in the setting of multiple pulmonary tumors. Patients either underwent medical or surgical management for their disease. Data collected includes age at diagnosis, gender, smoking status, pulmonary symptoms, disease pathology, location of disease, size of tumors, stage, intervention (medical vs surgical), pathology and mortality. Results: Twelve patients had a diagnosis of atypical or typical carcinoid in the setting of one or more additional nodules between 2012 and 2017. Eight of the patients (67%) were male. The median age was 65. Nine patients (75%) were symptomatic. The most common symptoms were cough and shortness of breath/dyspnea on exertion. Four patients (33%) had biopsy proven carcinoid in one or more of the additional nodules identified. Of the twelve patients, four underwent surgical resection of the primary tumor, 3 patients underwent medical management alone, one patient had resection followed by adjuvant chemo-radiation, one tumor was treated with gamma knife, and three patients are being followed with serial imaging and serum studies. There was no mortality. Conclusions: The presence of multiple pulmonary nodules in patients with a diagnosis of typical or atypical carcinoid may suggest the need for close surveillance. This study suggests that in patients with typical and atypical carcinoid with multiple tumorlets, there is no difference in outcome between medical intervention, surgery and surveillance imaging.