oa Journal of Endocrinology, Metabolism and Diabetes in South Africa - Disseminated large cell neuroendocrine carcinoma associated with ectopic adrenocorticotropic hormone secretion : case study

Volume 19, Issue 1
  • ISSN : 1608-9677
  • E-ISSN: 2220-1009



Ectopic 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.

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