Angiosarcoma

Testis and epididymis
Germ cell tumors
Angiosarcoma

Author: Jennifer Gordetsky, M.D.

Topic Completed: 1 July 2013

Revised: 28 March 2019

Copyright: 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: angiosarcoma testis

Page views in 2019: 335

Page views in 2020 to date: 52

Table of Contents

Cite this page: Gordetsky J. Angiosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testisangiosarcoma.html. Accessed February 20th, 2020.

Definition / general

Rare, highly aggressive tumor of vascular and lymphatic endothelial cell origin
Either a primary testicular vascular sarcoma or sarcomatous transformation of a testicular germ cell tumor

Terminology

Also called hemangiosarcoma

Epidemiology

Rare tumors of men of all ages
Two reported groups:
1. Associated with teratomas in young men (J Urol 2000;163:1872)
2. Occurring in elderly, may be associated with chronic hydrocele or prior radiation (Diagn Pathol 2007;2:23)

Sites

Testis, epididymis (Urology 2007;69:576.e5), scrotum

Clinical features

Testicular mass, pain, swelling, intermittent fevers
Patients tend to have metastatic disease at presentation, identified with imaging

Prognostic factors

Tend to present with advanced disease / metastasis
Deep soft tissue and visceral angiosarcomas have a high mortality, up to 53% succumb to disease within a year
Organ confined disease may have a better prognosis
Sarcomatous differentiation of a germ cell tumor is a poor prognostic indicator (Am J Surg Pathol 2007;31:1356, Urol Oncol 2008;26:595)

Case reports

23 year old man with mature testicular teratoma and retroperitoneal metastasis exhibiting components of mature teratoma intermingled with high grade angiosarcoma (Arch Pathol Lab Med 2003;127:360)
24 year old man presenting with testicular pain, a pelvic mass and innumerable liver nodules (Diagn Cytopathol 2010;38:154)
74 year old man with primary testicular angiosarcoma (Virchows Arch 1999;434:351)

Treatment

Radical orchiectomy
Chemotherapy (Lancet Oncol 2006;7:92)

Gross description

Expansive solid, hemorrhagic nodule compressing surrounding testicular parenchyma

Gross images

Images hosted on other servers:

Expansile hemorrhagic nodule

Microscopic (histologic) description

Classically shows a proliferation of anastomosing blood filled channels
Solid pattern shows a proliferation of sheets or nests without evidence of lumen formation
Primitive pattern shows a nearly solid proliferation with angulated small capillary sized lumina reminiscent of tubules but filled with blood channels lined by plump hyperchromatic cells
May have epithelioid cytology (appearing like glandular carcinoma) and solid architectural patterns, resembling poorly differentiated carcinoma
No intratubular germ cell neoplasia

Microscopic (histologic) images

Images hosted on other servers:

Angiosarcoma with
classic architectural
pattern

CD34, CD31,
Ulex Europaeus lectin,
Pancytokeratin

Site unspecified

Bladder angiosarcoma:
atypical endothelial
cells

Positive stains

CD31 and CD34 highlight malignant endothelial cells lining anastamosing vascular channels; factor VIII

Negative stains

Pancytokeratin (AE1 / AE3), PLAP, CAM5.2, CD68, CD45 / LCA

Molecular / cytogenetics description

Isochromosome 12p and chromosome 12p alterations reported in metastatic lesion containing angiosarcoma arising from germ cell tumor (Hum Pathol 2010;41:139)

Differential diagnosis

Anastomosing hemangioma (Am J Surg Pathol 2009;33:1364)
Capillary hemangioma
Hemangioendothelioma