Angiosarcoma

Soft tissue
High grade vascular lesions
Angiosarcoma

Author: Vijay Shankar, M.D.

Topic Completed: 1 November 2012

Revised: 26 March 2019

Copyright: 2002-2018, PathologyOutlines.com, Inc.

PubMed search: Angiosarcoma [title] soft tissue

Page views in 2018: 14,907

Page views in 2019 to date: 8,430

Table of Contents

Cite this page: Shankar V. Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissueangiosarcoma.html. Accessed June 26th, 2019.

Definition / general

Well differentiated (hemangiosarcoma) to anaplastic tumor resembling melanoma or carcinoma
Rare; older adults in skin (scalp, face) and soft tissue; also bone, breast, heart, lung, liver, spleen, thyroid (World J Surg Oncol 2012;10:73)
Very rare in children and young adults, similar histology but poor prognosis (Am J Surg Pathol 2009;33:264)
Arises from endothelial cells of blood vessels; includes aorta, inferior vena cava, pulmonary artery (usually undifferentiated, solid, difficult to identify as endothelial)
Risk factors: chronic lymphedema, PVC, radiation, sun exposure, Thorotrast
Nodal metastases in 14% (a high rate for sarcomas); also metastases to lungs, liver, bone

Sites

Breast:
- 3 - 12 years after radiation therapy for carcinoma
- Incidence is 1 per 1,000 - 2,000
- Usually women age 60+ with low grade, low stage lesions
- Poor prognosis with 3 year survival of 41%
Extremities:
- Associated with lymphedema ~10 years after radical mastectomy for breast cancer, arising from dilated lymphatics (lymphangiosarcomas, also called Stewart-Treves syndrome), not associated with radiation therapy
Kidney:
- Unusual neoplasm
- < 15 well documented cases of primary renal angiosarcoma
Liver:
- Associated with arsenic, Thorotrast, PVC
- Latent period of years
- Also associated with radiation to other sites, introduction of foreign material
Lung:
- Rarely presents as diffuse pulmonary hemorrhage due to metastases in young adults (Arch Pathol Lab Med 2001;125:1562)
- Lung metastases often multiple peripheral nodules accompanied by infiltrates, primary tumor usually not identified at presentation; tumor cells may have minimal atypia, may be keratin+; primary site is often heart (Mod Pathol 2001;14:1216)
Skin:
- Cases related to chronic lymphedema are usually in extremities
- Lymphedema due to radical mastectomy, postfilarial, congenital
- Cases unrelated to lymphedema are often in head and face
Spleen:
- Rare neoplasm that often remains asymptomatic until massive intraabdominal bleeding
Other sites::
- Small intestine
- Ovary

Case reports

22 year old woman with angiosarcoma in recurrent malignant phyllodes tumor of breast (Indian J Pathol Microbiol 2012;55:264)
25 year old woman with small intestine tumor (Ann Afr Med 2011;10:246)
29 year old woman with bilateral breast lumps (Indian J Med Paediatr Oncol 2011;32:160)
35 year old woman with cutaneous angiosarcoma complicating morbid obesity (Arch Pathol Lab Med 2001;125:531)
50 year old man with kidney mass (Arch Pathol Lab Med 2002;126:478)
60 year old man with splenomegaly and fever (Indian J Med Paediatr Oncol 2011;32:230)
69 year old man with raised lesion on nose (Indian J Dermatol Venereol Leprol 2012;78:496)
80 year old woman with epithelioid angiosarcoma (Indian J Dermatol Venereol Leprol 2012;78:494)
81 year old woman with ovarian tumor (Diagn Pathol 2011;6:65)

Treatment

Early surgery but survival > 5 years is rare

Clinical images

Images hosted on PathOut server:

Contributed by Mark R. Wick, M.D.

Nodular, scalp

Hemangioma-like

Cutaneous

Stewart-Treves syndrome

Images hosted on other servers:

Foot

Glistening erythematous ulcerated plaque

Nose

Gross description

Early: small, sharply demarcated, asymptomatic, multiple red nodules
Late: fleshy, grayish white with hemorrhage, necrosis, deeply invasive

Gross images

Images hosted on PathOut server:

Contributed by Mark R. Wick, M.D.

Deep gluteal muscles

Images hosted on other servers:

Ovary

Spleen

Thyroid

Microscopic (histologic) description

Atypical vascular spaces lined by endothelial cells with cytologic atypia, multilayering
In more solid areas are intracytoplasmic lumina containing red blood cells
Involves subcutaneous tissue
Variable grade (Stanford University)
Multinucleated cells may have prominent hyaline globules containing alpha-1-antitrypsin and alpha-1-antichymotrypsin
Brisk mitotic activity and necrosis are common
Postradiation lesions usually high grade

Microscopic (histologic) images

Scroll to see all images:

Images hosted on PathOut server:

Contributed by Mark R. Wick, M.D.

Spindle cell type, scalp

Spindle cell type, FLI1 stain

Scalp

Granular cell type

Face

Epithelioid type, skin

Epithelioid type

Epithelioid type, face

Epithelioid, deep soft tissue

Images hosted on other servers:

Breast

Ovary

Nose

Skin (image on right is epithelioid)

Spleen

Thyroid gland

Contributed by Dr. Deepa Santhosh, Banaras Hindu University (India):

Thigh mass

Cytology images

Images hosted on other servers:

Kidney

Positive stains

Factor VIII related protein, CD31, Ki67, FLI1 (nuclear stain, Am J Surg Pathol 2001;25:1061), thrombomodulin, CD34 (not specific), c-kit (50%)
VEGFR3 (variable, Mod Pathol 2000;13:180)
Keratin for epithelioid tumors

Molecular / cytogenetics description

Associated with high level amplifications of MYC, particularly in secondary tumors (Am J Pathol 2010;176:34, Genes Chromosomes Cancer 2011;50:25), but also in subset of primary tumors (Genes Chromosomes Cancer 2012;51:569)

Differential diagnosis

Atypical vascular lesions: circumscribed, no atypia, no mitotic figures
Florid vascular proliferation of colon due to intussusception (Am J Surg Pathol 2001;25:1167)
Hemangioma: usually < 2 cm, well circumscribed, contain fibrous septa and thick walled vessels, not invasive
MFH: intratumoral macrophages from non vascular tumors may be CD31+, (Mod Pathol 2001;14:1114)