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Monday 23 August 2004


Definition : Angiosarcoma (AS) is a rare sarcoma subtype showing considerable clinicopathologic and genetic heterogeneity. Most radiation-induced AS show MYC gene amplifications, with a subset of cases harboring KDR, PTPRB, and PLCG1 mutations.

Angiosarcoma is a rare tumor accounting for less than one percent of all sarcomas. It occurs typically in the skin and soft tissues of the head and neck.

It is a very malignant neoplastic proliferation arising from vascular endothelial cells. Systemic metastasis at the time of diagnosis is reported to be as high as 80%.

The typical primary site for metastatic cerebral angiosarcoma is the heart, accounting for 57% of cases.

Other primary sites have been described, such as Dacron aortic vascular grafts, aorta, pleura, skin, liver, penis, bone, placenta and the orbit. Cerebral metastases are multiple in 57.9% of the cases. There is a male predominance and the mean age is 42.2 years.

Digital case

- JRC:11005 : Cutaneous angiosarcoma.


- vesical angiosarcoma

- angiosarcoma on the lower extremity of a 48 year old man

- Angiosarcoma in 47 year old with a large periprostatic mas

- Agiosarcoma with papillary appearance

The described risk factors for the development of angiosarcomas are radiation therapy, foreign object, chronic lymphedema associated with radical mastectomy, A-V fistulas and certain environmental carcinogens like vinyl chloride, arsenic and Thorotrast.

In a series of 53 post-radiation sarcomas, Laskin and al. reported that the most frequent radiation-induced sarcoma was malignant fibrous histiocytoma (68%) and that an angiosarcoma represented only one case (2%), on the thoracic wall. Another series of 2500 patients treated for ENT cancer reported only 5 sarcomas in the irradiated zone. None were angiosarcoma.

Vascular prostheses, especially Dacron prostheses, also have the potential to induce malignant mesenchymatous changes (2,4,11,16). We, however, did not find any cases that occurred after insertion of a vascular endoprosthesis.

Angiosarcoma classically presents with cutaneous involvement of the head and neck region in elderly patients or in the limbs of patients with lymphedema.

Angiosarcoma most frequently occurs in the skin of the head and neck region of elderly persons, lymphedematous limbs, or in deep soft tissue

A smaller, but increasingly recognized subset of angiosarcoma has been described in the deep soft tissues and in areas of prior irradiation, trauma or in association with foreign bodies such as ateriovenous shunts.

Deep-seated angiosarcoma

Deep-seated angiosarcoma frequently has an epithelioid cytomorphology. The epithelioid phenotype of angiosarcoma was first described in cases of cutaneous angiosarcoma by Rosai et al in 1976 and later documented in deep soft tissue by Weiss et al and Fletcher et al.

Intra-abdominal angiosarcoma has been described previously, usually arising in the liver, spleen and rarely adrenal glands, ovaries or as diffuse angiosarcomatosis.


- littoral cell angiosarcoma
- pediatric angiosarcomas


- cutaneous angiosarcoma
- digestive angiosarcoma
- pulmonary angiosarcoma
- ovarian angiosarcoma (9591733)
- angiosarcoma of the scalp and face


- complex cytogenetic aberrations without consistent recurring chromosome aberration
- some recurrent aberrations among angiosarcomas arising in the same location


- xeroderma pigmentosum (11044816)

Molecular biology

- Concurrent CIC mutations and CIC rearrangements with possible CIC-LEUTX fusion

  • CIC abnormalities occurred (9%), affecting younger patients with primary AS.

- PLCG1 mutations (9.5%)
- KDR mutations (7%)
- MYC amplification present in most secondary AS related to breast cancer (91%) compared with other causes (25%) or primary AS (7%).

- FLT4-amplified angiosarcoma

  • lacked PLCG1/KDR mutations,
  • occurring predominantly in MYC-amplified population,
  • showed poor prognosis.

Case report

- UPMC #597

See also

- Tumors

Paywall References

- Recurrent CIC Gene Abnormalities in Angiosarcomas: A Molecular Study of 120 Cases With Concurrent Investigation of PLCG1, KDR, MYC, and FLT4 Gene Alterations. Huang SC, Zhang L, Sung YS, Chen CL, Kao YC, Agaram NP, Singer S, Tap WD, D’Angelo S, Antonescu CR. Am J Surg Pathol. 2016 May;40(5):645-55. doi : 10.1097/PAS.0000000000000582 PMID: 26735859

- Autocrine and paracrine roles of VEGF/VEGFR-2 and VEGF-C/VEGFR-3 signaling in angiosarcomas of the scalp and face. Tokuyama W, Mikami T, Masuzawa M, Okayasu I. Hum Pathol. 2009 Nov 12. PMID: 19913279

- Billings SD, McKenney JK, Folpe AL, Hardacre MC, Weiss SW. Cutaneous angiosarcoma following breast-conserving surgery and radiation: an analysis of 27 cases. Am J Surg Pathol. 2004 Jun;28(6):781-8. PMID: 15166670

- Allison KH, Yoder BJ, Bronner MP, Goldblum JR, Rubin BP. Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases. Am J Surg Pathol. 2004 Mar;28(3):298-307. PMID: 15104292

- Nucci MR, Krausz T, Lifschitz-Mercer B, Chan JK, Fletcher CD. Angiosarcoma of the ovary: clinicopathologic and immunohistochemical analysis of four cases with a broad morphologic spectrum. Am J Surg Pathol. 1998 May;22(5):620-30. PMID: 9591733


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