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

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
Micro Description
  • Pigmented cytoplasm, fusiform to spindle shaped nuclei, marked anisomorphism, prominent nucleoli
Micro Images
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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