Adrenal gland and paraganglia
Other adrenal malignancies
Angiosarcoma

Author: Lan L. Gellert, M.D., Ph.D. (see Authors page)

Revised: 18 May 2016, last major update May 2016

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

PubMed Search: Angiosarcoma [title] adrenal

Related: Epithelioid angiosarcomas

Definition / General
  • Very rare
  • Spindled or epithelioid
  • Resembles angiosarcoma of soft tissue
Essential Features
  • Wide range of morphological appearance from well formed anastomosing vessels to solid sheets of epithelioid or spindled cells
  • Epithelioid morphology common in adrenal gland
Terminology
  • Also called hemangiosarcoma, lymphangiosarcoma, malignant angioendothelioma, malignant hemangioendothelioma
Epidemiology
  • Affects all ages but extremely rare in children
Etiology
  • Unknown in most cases
  • Associated with prior radiation in a subset of cases
Clinical Features
  • Presents as an enlarging adrenal mass
  • Some cases associated with coagulopathy, anemia or hematoma
Prognostic Factors
Case Reports
Treatment
  • Surgery; chemotherapy or radiation therapy may be administered before or after surgery
Gross Description
  • Reddish-brown color
  • Predominantly solid
Micro Description
  • Wide range of morphological appearance from well formed anastomosing vessels to solid sheets of epithelioid or spindled cells
  • Vasoformative areas exhibiting ramifying channels lined by atypical endothelial cells forming papillations or intraluminal buds
  • Solid area exhibiting high grade spindled or epithelioid cells with abundant amphophilic to eosinophilic cytoplasm and prominent nucleoli
  • Brisk mitotic activity, necrosis and significant nuclear atypia common
  • Tumors with predominant epithelioid cells are classified as “epithelioid angiosarcoma”
Micro Images

Images hosted on Flickr:

Contributed by Dr. Lan Gellert

Area with anatomizing vasoformation and
area with solid pattern coexist in the tumor

Anastomosing channels lined by
atypical endothelial cells


Spindled cell morphology

Epithelioid morphology

Tumor necrosis

Positive Stains
Negative Stains
Electron Microscopy Description
  • Electron microscopy not typically used in routine practice
  • Weibel-Palade bodies rarely identified
Differential Diagnosis