Adrenal gland and paraganglia
Adrenal medulla
Malignant melanotic tumors

Author: Pallav Gupta, M.D. (see Authors page)

Revised: 19 January 2016, last major update December 2013

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Malignant melanotic tumors adrenal

Cite this page: Malignant melanotic tumors. PathologyOutlines.com website. http://pathologyoutlines.com/topic/adrenalmalignantmelanotic.html. Accessed June 24th, 2017.
Definition / general
  • Rare tumors with high fatality rate
  • Primary tumors may arise from sympathetic ganglia or pheochromocytoma
  • May represent metastasis
Epidemiology
  • Mean age 50 17 years
  • Almost 50% male
Pathophysiology / etiology
  • Adrenal medullary blasts and melanoblasts both have neuroectodermal origin
  • As a result, ectopic melanocytes can exist in adrenal gland, causing metaplasia and malignant transformation, leading to melanoma
Clinical features
  • Most commonly pain; also GI symptoms secondary to compression of adjacent structures
  • Survival usually <19 months
Radiology description
  • Voluminous non-functional tumor showing heterogenous contrast enhancement on CT scan
Radiology images
Images hosted on other servers:

Tumor in left adrenal gland

Mass in retroperitoneum

Lesion behind tail of pancreas

Case reports
Treatment
  • Surgery is preferred; complete removal of tumor is usually accompanied by removal of adrenal and kidney
Gross description
  • Mean size 11 4 cm
  • Brown or black
Microscopic (histologic) description
  • Pigmented cytoplasm, fusiform to spindle shaped nuclei, marked anisomorphism, prominent nucleoli
Microscopic (histologic) images
Images hosted on other servers:

H&E

Positive stains
  • MelanA, HMB45, S100
Negative stains
  • Synaptophysin, chromogranin
Electron microscopy description
  • Premelanosomes or melanosomes
Differential diagnosis
  • Hematoma
  • Metastases from occult primary in skin, mucous membranes, eyes
  • Pheochromocytoma: synaptophysin+, chromogranin+, MelanA-, EM shows neurosecretory granules
  • Pigmented adrenal adenoma: usually < 5cm, low molecular weight cytokeratin+, negative for HMB45, S100, MelanA
Additional references