Radiogenic angiosarcoma of the breast: case report and systematic review of the literature

Abstract

BackgroundRadiogenic angiosarcoma of the breast (RASB) is a rare late sequela of local irradiation of the breast or chest wall after breast cancer. The prognosis of women with RASB is poor and there is no standardized therapy for this type of malignancy.Case presentationWe present the case of a 54 year old woman with RASB (poorly differentiated angiosarcoma of the left breast; pT1, pNX, M0, L0, V0) and a history of invasive-ductal cancer of the left breast (pT1b, G2, pN0, ER positive, PR positive, HER-2/neu negative) treated in July 2012 with breast-conserving surgery, adjuvant chemotherapy with 6 cycles of epirubicin and cyclophosphamide, adjuvant irradiation of the left breast with 50 Gray, and adjuvant endocrine therapy with an aromatase inhibitor. In August 2016, a bilateral salpingo-oophorectomy was performed to remove a tumor of the left ovary, which was diagnosed as breast cancer recurrence. At the same time, a small, purple skin lesion of 1.2 cm in diameter was noted in the inner upper quadrant of the right breast. RASB was diagnosed by punch biopsy and the tumor was excised with clear margins. Imaging studies showed no evidence of further metastases. A systemic chemotherapy with 6 cycles of liposomal doxorubicin was initiated. Five months later, a local recurrence of RASB was diagnosed and mastectomy was performed. Six months later, the patient is alive with no evidence of disease.Three hundred seven cases of RASB were identified. The pooled incidence rate of RASB was 1/3754 women. The most common treatment of RASB was mastectomy in 83% of cases. Adjuvant radiotherapy or chemotherapy were rarely used with 6 and 4%, respectively, whereas in case of recurrence, chemotherapy was the mainstay of treatment, used in 58% of cases. Radiotherapy and repeated surgery were also common with 30 and 33% of cases, respectively. Overall, the prognosis of women with RASB was poor and the recurrence-free survival was short with a mean of 15.9 months. Mean overall survival was 27.4 months.ConclusionRASB is a rare late complication of breast irradiation. The prognosis of women with RASB is poor. Surgery is the mainstay of treatment for localized disease while systemic chemotherapy and re-irradiation are appropriate for women with disseminated or recurrent RASB.

Mammography and MRI manifestations of breast angiosarcoma

“…PBA usually occurs to young women without any inducing factor, while SBA is commonly seen in older women who have a history of breast surgery and radiotherapy. PBA is rarely seen in clinical practice and it is predicted that PBA will have a higher morbidity and be more aggressive in the future [3–5]. Increasing use of breast-conserving surgery leads to increase in the incidence of SBA and among all secondary tumors resulted from radiotherapy, SBA is believed to be the most common one.…”

Secondary angiosarcoma in a hormone receptor negative breast cancer patient

“…Secondary angiosarcoma are more likely to be of high-grade (82%) compared to primary angiosarcoma [6]. Literature review shows many reports of secondary post-irradiation angiosarcoma in females to be hormone-receptor positive breast cancers under hormonal therapy [6, 7]. Our case is unique in that the angiosarcoma was of intermediate grade and occurred in hormone-receptor negative patient without any hormonal therapy.…”

Radiation-induced angiosarcoma: case report

“…As opposed to primary angiosarcoma of the breast that affects young women, mean patient age at diagnosis of RASB was 70 years and the mean latency period between radiation therapy and diagnosis was 6 years. (3) The pathogenesis of RASB is still unclear. (4) The two largest published case series of RASB, one with 79 cases and another with 95 cases reported that the 5-year disease-free survival (DFS) was 47% and 62.6%, respectively.…”

“…Nevertheless, nodules, plaques or patches may also appear. (3) Imaging of angiosarcoma is nonspecific. Mammography and ultrasonography have no pathognomonic features.…”