Adrenal gland and paraganglia
Adrenocortical carcinoma
Myxoid carcinoma

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

Revised: 18 January 2016, last major update September 2013

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

PubMed Search: myxoid[title] carcinoma adrenal

Cite this page: Adrenocortical Carcinoma - Myxoid. website. Accessed April 27th, 2017.
Definition / General
Clinical Features
  • Patients can present with Cushing syndrome, primary hyperaldosteronism, gynecomastia, breast pain, virilization and hypertension (most cases show increased serum hormone levels, such as cortisol, aldosterone, adrenocorticotropic hormone, follicle-stimulating hormone (FSH), luteinizing hormone (LH) (Am J Clin Pathol 2011;136:783)
Case Reports
Gross Description
  • 7-24 cm, 45 g to 2.5 kg
  • Partially encapsulated with gelatinous myxoid areas, hemorrhage and necrosis
Gross Images
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Figure 1A: myxoid adrenocortical adenoma; 1B: myxoid carcinoma

Micro Description
  • Cells usually are arranged in delicate arborizing cords or trabeculae, 1 to 2 cells thick surrounded by relatively large, acellular, clear myxoid spaces resembling the background of a myxoma
  • Also diffuse and alveolar patterns
  • Small uniform cells are polygonal or elongated with moderate eosinophilic cytoplasm
Micro Images
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Various images

Figures 1G / 1H

Positive Stains
  • Cytochemistry: prominent Alcian blue+ extracellular mucosubstances
  • IHC: vimentin, MelanA, synaptophysin, Neurofilament(NF) and α-inhibin overexpression
Negative Stains
  • Cytochemistry: negative or focal weak staining with PAS and mucicarmine.
  • IHC: cytokeratin, chromogranin A
Electron Microscopy Description
  • Same as adrenocortical carcinoma
Molecular / Cytogenetics Description
Differential Diagnosis
  • Adrenocortical carcinoma (ACC) with focal myxoid features: focal myxoid areas always merging with conventional ACC areas, solid/diffuse growth pattern, large, heterogenous cell size, moderate to high nuclear atypia and abundant, granular eosinophilic cell cytoplasm
  • Other myxoid neoplasm in retroperitoneum:
    • Chordoma (S100+)
    • Carcinoma with myxoid areas (CK+, synaptophysin-)
    • Benign or malignant nerve sheath tumor (S100+)
    • Myxoid leiomyoma and leiomyosarcoma (SMA+)
    • Gastrointestinal stromal tumor (CD117+)
    • Myxoid malignant fibrous histiocytoma