Disseminated large cell neuroendocrine carcinoma associated with ectopic adrenocorticotropic hormone secretion
Keywords: Neuroendocrine tumour, large cell neuroendocrine carcinoma, ectopic ACTH, Cushing’s syndrome, superior mediastinum, superior vena cava syndrome
AbstractEctopic adrenocorticotropic hormone (ACTH) secretion is associated with a heterogeneous spectrum of neuroendocrine tumours (NETs) as well as non-NET tumours. Both ectopic ACTH secretion (EAS) and NETs are relatively uncommon. Large cell neuroendocrine carcinoma (LCNEC) is considered to be a poorly differentiated NET, and is very rarely associated with EAS. Only case reports of EAS associated with LCNEC have been documented. We present a case of disseminated LCNEC associated with EAS. The LCNEC was diagnosed on bone marrow trephine biopsy, and the likely but unproven primary was a NET arising from the superior mediastinum, which also caused superior vena cava (SVC) syndrome at presentation.
Material submitted for publication in the Journal of Endocrinology, Metabolism and Diabetes of South Africa (JEMDSA) is accepted provided it has not been published elsewhere. JEMDSA reserves copyright of the material published. Neither JEMDSA nor the Publisher may be held responsible for statements made by the authors.