Testis & epididymis
Germ cell tumors
Angiosarcoma
Author: Jennifer Gordetsky, M.D.
Topic Completed: 1 July 2013
Minor changes: 24 December 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: angiosarcoma testis
Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Clinical features | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosisCite this page: Gordetsky J. Angiosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testisangiosarcoma.html. Accessed January 16th, 2021.
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:
- Associated with teratomas in young men (J Urol 2000;163:1872)
- 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
Positive stains
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
