Adrenal gland and paraganglia
Adrenocortical carcinoma
Myxoid variant

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

Revised: 2 October 2017, last major update September 2013

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

PubMed Search: Myxoid[title] carcinoma adrenal

Cite this page: Gupta, P. Adrenocortical carcinoma - myxoid variant. website. Accessed September 22nd, 2018.
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 PathOut server:

Myxoid variant -
contributed by
Debra Zynger, M.D.

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