Radiation-associated angiosarcoma: diagnostic and therapeutic implications--two case reports and a review of the literature.

Abstract BACKGROUND: Angiosarcoma (AS) accounts for 1 to 2% of all soft tissue sarcoma. Both primary and secondary AS may occur, the latter being reported in the upper extremity with lymphedema after extended radical mastectomy for breast cancer (postmastectomy AS) or following radiotherapy of the breast, the thoracic wall, or other sites (radiation-associated AS). The authors report two cases of cutaneous radiation-associated AS and review literature regarding treatment planning and follow-up data to define the most appropriate therapy for cutaneous and noncutaneous radiation-associated AS. METHODS: The clinical records of two patients with radiation-associated AS were analyzed and previously reported cases were reviewed. RESULTS: Case 1: a female age 67 years developed cutaneous AS in the residual breast 27 months after breast-conserving therapy and conventional external beam radiotherapy (EBR). She underwent chemotherapy followed by simple mastectomy and chemotherapy with the same regimen but developed early recurrence that was treated with hyperthermia and EBR, wide excision, and second-line chemotherapy. She died 30 months after primary diagnosis of AS with multiple metastases. Case 2: a male age 59 years developed cutaneous AS in the left groin, 10 years after conservative surgery and EBR for a penile carcinoma. Early recurrence following wide excision was treated with chemotherapy, re-excision, and immunochemotherapy but the patient died 24 months after the primary diagnosis of cutaneous AS with local progression and distant metastases. CONCLUSIONS: The prognosis of radiation-associated AS is dismal, due mostly to its poor differentiation and frequent diagnostic delay. Simple mastectomy is advised for patients with cutaneous AS after breast-conserving surgery with wide tumor-free margins. If primary surgery fails, survival is seriously compromised because adjuvant or palliative treatments are not effective.