Diagnosis and management of small pulmonary atypical carcinoid tumor associated with Cushing syndrome

Abstract

Ectopic adrenocorticotropic hormone (ACTH) syndrome is rare and identification of its source is often challenging. We report the case of an ectopic Cushing syndrome in a young adult male secondary to an occult ACTH producing atypical carcinoid tumor. Extensive biochemical and imaging workup was unrevealing. The diagnosis was aided by Ga-DOTA PET scan demonstrating a suspicious left upper lobe lung nodule. The patient underwent video-assisted thoracoscopic exploration with wedge resection and mediastinal lymphadenectomy of a T2aN2M0 atypical carcinoid, resulting in the normalization of ACTH levels and complete resolution of symptoms. The role of a Ga-DOTA PET scan in diagnosing pulmonary carcinoid tumors and their management are discussed.

Keywords: Ga-DOTA PET; atypical carcinoid; ectopic ACTH syndrome; ectopic Cushing syndrome; hypercortisolemia; neuroendocrine tumors; pulmonary carcinoid; video-assisted thoracoscopic surgery.