Disseminated large cell neuroendocrine carcinoma associated with ectopic adrenocorticotropic hormone secretion

  • Andrew van der Walt University of Witwatersrand
  • Kenneth Huddle University of Witwatersrand
  • Sugeshnee Pather National Health Laboratory Services University of Witwatersrand
  • Anneli Korb University of Witwatersrand
Keywords: Neuroendocrine tumour, large cell neuroendocrine carcinoma, ectopic ACTH, Cushing’s syndrome, superior mediastinum, superior vena cava syndrome

Abstract

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.

Author Biographies

Andrew van der Walt, University of Witwatersrand
MBChB; FCP(SA) Registrar Chris Hani Baragwanath Academic Hospital Department of Internal Medicine Division of Endocrinology Chris Hani Baragwanath Academic Hospital; University of Witwatersrand
Kenneth Huddle, University of Witwatersrand
MBBCh; FCP(SA); FRCP(London) Head of Department Department of Internal Medicine Division of Endocrinology Chris Hani Baragwanath Academic Hospital; University of Witwatersrand
Sugeshnee Pather, National Health Laboratory Services University of Witwatersrand
MBBCh; FCPath(SA)Anat Consultant Department of Anatomical Pathology Chris Hani Baragwanath Academic Hospital University of Witwatersrand; National Health Laboratory Services
Anneli Korb, University of Witwatersrand
MBChB; Dip PEC; FCP(SA) Registrar Department of Internal Medicine Division of Endocrinology Chris Hani Baragwanath Academic Hospital University; University of Witwatersrand
Published
2014-03-17
Section
Case Study