Cafiero F, Gipponi M, Peressini A, Queirolo P, Bertoglio S, Comandini D, Percivale P, Sertoli M R, Badellino F
Dipartimento di Oncologia Clinica e Sperimentale, School of Medicine, Genoa, Italy.
Cancer. 1996 Jun 15;77(12):2496-502. doi: 10.1002/(SICI)1097-0142(19960615)77:12<2496::AID-CNCR12>3.0.CO;2-N.
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.
The clinical records of two patients with radiation-associated AS were analyzed and previously reported cases were reviewed.
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.
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.
血管肉瘤（AS）占所有软组织肉瘤的1%至2%。原发性和继发性AS均可发生，后者报道于乳腺癌扩大根治术后上肢出现淋巴水肿的患者（乳房切除术后AS），或乳房、胸壁或其他部位放疗后（放疗相关AS）。作者报告了2例皮肤放疗相关AS病例，并回顾了有关治疗计划和随访数据的文献，以确定皮肤和非皮肤放疗相关AS的最合适治疗方法。
分析了2例放疗相关AS患者的临床记录，并回顾了既往报道的病例。
病例1：一名67岁女性在保乳治疗和常规外照射放疗（EBR）27个月后，在残留乳房出现皮肤AS。她接受了化疗，随后行单纯乳房切除术，并采用相同方案化疗，但出现早期复发，接受了热疗和EBR、广泛切除及二线化疗。她在AS初诊30个月后因多发转移死亡。病例2：一名59岁男性在阴茎癌保守手术和EBR 10年后，左侧腹股沟出现皮肤AS。广泛切除术后早期复发接受了化疗、再次切除及免疫化疗，但患者在皮肤AS初诊24个月后因局部进展和远处转移死亡。
放疗相关AS的预后不佳，主要原因是其分化差且诊断常延迟。对于保乳手术后切缘阴性的皮肤AS患者，建议行单纯乳房切除术。如果初次手术失败，由于辅助或姑息治疗无效，生存将受到严重影响。
