Adrenal gland and paraganglia
Adrenocortical carcinoma
Myxoid variant

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

Revised: 8 August 2017, last major update September 2013

Copyright: (c) 2002-2017,, Inc.

PubMed Search: myxoid[title] carcinoma adrenal

Cite this page: Adrenocortical Carcinoma - Myxoid variant. website. Accessed August 20th, 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
Images hosted on other servers:

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
Images hosted on other servers:

Myxoid adrenocortical carcinoma

Myxoid adrenocortical carcinoma with
extensive lipomatous metaplasia

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