UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Case Authors: Joanne K. Simpson MD, MPH, Joseph C. English IIII MD, Muammar Arida, MD, Drazen M. Jukic, MD, PhD
FEBRUARY 2006 CASE OF THE MONTH
DISCUSSION & DIAGNOSIS
Primary angiosarcoma of the skin.
Angiosarcoma is a rare malignant tumor of vascular endothelial cells that most commonly arises on the head and neck of elderly patients. Of all the sarcomas occurring on the head and neck, angiosarcoma comprises approximately 10% of cases.1 Although its presentation is protean, it most often presents as an enlarging bruise-like macule or plaque, or nodule. Some can bleed or ulcerate, but the majority of patients (79.3%) are asymptomatic.2 Men are 3 times more likely to present with angiosarcoma than women, and the median age of diagnosis is 71 years. Angiosarcoma can be classified into 3 major types of angiosarcoma: 1) post-radiation angiosarcoma, 2) post-breast cancer, presenting within chronic lymphedema (Stewart-Treaves syndrome), and 3) classic cutaneous angiosarcoma.
All forms of angiosarcoma have a tendency for metastasis by lymphatic or hematogenous routes. For this reason, in addition to a skin biopsy, further diagnostic studies should include a PET or CT scan. Pawlik et al. recommend a multidisciplinary approach to managing angiosarcoma.2 Dermatology, pathology, medical oncology, radiation oncology, and otolaryngology specialists should all be included in the evaluation and treatment of these patients.
Due to the rarity of this tumor, there is no standardized treatment regimen. Treatments are often derived from review of small case reports and individualized on the basis of the extent of disease and anatomic location. The largest case series, reported from the University of Michigan, concluded that most patients are treated with wide local excision or Mohs of the tumor as the initial treatment. Unfortunately, achieving negative margins often is impossible, especially with larger lesions, and recurrences frequently develop. Wide local radiation is used as adjuvant therapy and has been shown to be associated with increased survival. Patients who received radiation therapy had a median survival that was almost 4 times longer than patients not receiving radiation therapy (36.1 vs 9.2 months).2 Unlike other sarcomas, chemotherapy has not been shown improve survival in cutaneous angiosarcoma. Paclitaxel was shown to have a high response rate in patients who were previously treated with chemotherapy or radiation.3 There is one reported case of gene therapy with intralesional cDNA for interferon a-2b resulting in the disappearance of injected and distant cutaneous angiosarcoma lesions.
Overall, the prognosis for angiosarcoma is dismal. Factors associated with reduced survival include: age >70 years, lesion size >5 cm, advanced clinical and pathological stage of the lesion, and no adjuvant therapy with radiation therapy.2,4 Tumor recurrence and metastasis are also associated with reduced survival. Metastatic spread often occurs to the lungs, followed by the liver, cervical lymph nodes , spleen, and rarely, the heart and brain. The median 5 year survival rate ranges between 12 and 33%.5 The mean survival after metastases in the University of Michigan study was reported as 4 months.
1. Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol 2003; 15:239-252.
2. Pawlik TM, Paulino AF, McGinn CJ, et al. Cutaneous Angiosarcoma of the Scalp: A Multidisciplinary Approach. Cancer 2003; 98(8):1716-26. 3. Fata F, O’Reilly E, Ilson D, et al. Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer 1999. 86:2034-37. 4. Morgan MB, Swann M, Somach S, et al. Cutaneous Angiosarcoma: A case series with prognostic correlation. J Amer Acad Derm 2004;50:867-74.
5. Bolognia JL, Jorizzo JL, Rapini RP. Vascular Neoplasms and Neoplastic-like Proliferations. Dermatology. Toronoto. 2003. p. 1833-1835.
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