Adrenal gland & paraganglia

Adrenal cortical carcinoma

Myxoid variant

Topic Completed: 1 September 2013

Minor changes: 18 November 2021

Copyright: 2002-2021,, Inc.

PubMed Search: Myxoid[title] carcinoma adrenal

Pallav Gupta, M.D.
Page views in 2020: 558
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Cite this page: Gupta P. Myxoid variant. website. Accessed December 8th, 2021.
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 B

Microscopic (histologic) 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
Microscopic (histologic) images

Contributed by Debra Zynger, M.D.

Myxoid variant

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Myxoid adrenocortical carcinoma

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
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