Paragangliomas are neuroendocrine tumours that present uncommonly in the thoracic cavity. Due to their rarity, they are usually not appropriately managed. This study aims to look at the outcomes of thoracic paragangliomas reported in literature.
Retrospective cohort analysis of cases published on thoracic paragangliomas during the study period from 2010 to 2023. Clinical, biochemical, radiological and treatment parameters were collected.
Of the 35 reported cases during the study period, 11 (31.4%) were functional and 28 were non-functional (68.6%). 26 cases were in the mediastinum and the rest in the aortopulmonary window. 11 cases (39.2%) presented with hypertension and palpitations and the rest were asymptomatic. Symptomatic cases were treated with alpha blockers. CT was the most commonly used modality of imaging - 24 cases (80%) and 6 (20%) used scintigraphy. Pre-operative biopsy was performed in 13 patients, of whom 7 had excision biopsies.
21 of 35 (60%) underwent surgery, of whom 2 cases (10%) underwent minimally invasive resection while 19 cases (90%) underwent open resection. R0 resection was achieved in 21 cases (56%) underwent R0 resection, R1 resection in 3 cases (8%), 12 cases (32%) underwent chemotherapy or radiotherapy and 1 case (4%) on active surveillance. Follow up data was available in only 10% of cases with no available data on mortality or recurrence. Only 20% of patients underwent genetic testing to rule out any syndromic conditions.
Management of thoracic paragangliomas appeared to be sub optimal, often not adhering to the guidelines set by ENET.