Cutaneous Angiosarcoma Arising in an Irradiated Breast

Abstract

Angiosarcoma (AS) is a rare, aggressive tumour of endothelial origin occurring in various clinical settings, including idiopathic AS on the head and neck in elderly people, lymphoedema-associated AS, post-irradiation AS, soft-tissue AS, and various others. Despite the widespread use of radiation therapy in the treatment of breast carcinoma, AS developing in the wake of a radiation therapy is extremely infrequent. Although there is little doubt that radiation in therapeutic doses can induce sarcomas, quantification of that risk is complicated by many variables, among them chronic lymphoedema. We describe a 70-year-old woman in generally good health who presented with a 2-year history of a maculo-papular eruption on the skin of her right breast. There was no lymphoedema of the thoracic area. The lesions developed 3 years after she had undergone ipsilateral quadrantectomy for an invasive ductal carcinoma followed by 25 tangent field radiotherapy sessions on the breast. The oncological follow-up did not disclose local recurrence of the tumour or metastases of breast carcinoma. Histopathologic examination of a papule was diagnostic for AS. In addition, signs of chronic radiation dermatitis were found in the biopsy specimens. The patient underwent monthly cycles of chemotherapy with intravenous doxorubicin with partial remission of the affected area after 24 months, followed by the occurrence of liver metastases and exitus 30 months after diagnosis. From the review of the literature, it appears that post-irradiation mammary AS mainly affects women over 60 who have undergone breast-sparing surgery and that it is usually associated with axillary lymphadenectomy. Whereas the role of lymphoedema does not seem relevant to the pathogenesis of this malignancy, the association with chronic radiation dermatitis in our case reinforces the supposed role of radiation in the development of this tumour. Onset of AS should be taken into consideration when treating patients who develop multiple lesions on the skin above the irradiated area, even many years after the therapy.

Post-radiotherapy vascular proliferations in mammary skin: A clinicopathologic study of 11 cases

“…The development of cutaneous AS in the setting of radiation therapy has been well described in the literature. [5][6][7][8]20,22,23,27,28,31,32,35,39,42 However, the natural history, potential precursor lesions, and definitive histologic and clinical features to differentiate AVL from well-differentiated AS have yet to be fully elucidated.…”

Potential risks and benefits of radiation therapy as adjuvant treatment in patients with low-risk carcinoma of the mammary gland: taking cutaneous postradiation angiosarcoma as an opportunity for a critical appraisal of postoperative radiotherapy

“…As an aggressive tumor of endothelial origin, angiosarcoma of the breast (AS) is occurring as idiopathic, lymphedemaassociated, postirradiation, soft tissue, and various other clinical settings [1,2]. Among these settings, iatrogenic AS, also known as ÔStewart-Treves syndromeÕ is usually attributed to chronic lymphedema, which can occur in a number of patients treated with BCS.…”

“…CPRASB is more often found in breasts developing postoperative and postirradiation edema and fibrosis [2]. It mainly affects women >60 and prevails in those who underwent axillary lymphadenectomy.…”

Radiation-Induced Angiosarcomas

“…Second in frequency are AS that originate in areas of chronic lymphoedema and which exhibit histopathological features similar to those of AS of the scalp and face. In this issue of Dermatology, Tomasini and Grassi [4] report a case of cutaneous AS following radiotherapy for breast cancer. Interestingly, this patient, as most women developing AS in the field of an irradiated breast, had no evidence of concurrent lymphoedema, strengthening the role of ionising radiation in the development of this neoplasm [4].…”

“…In this issue of Dermatology, Tomasini and Grassi [4] report a case of cutaneous AS following radiotherapy for breast cancer. Interestingly, this patient, as most women developing AS in the field of an irradiated breast, had no evidence of concurrent lymphoedema, strengthening the role of ionising radiation in the development of this neoplasm [4]. Although the appearance of malignant tumours caused by ionising radiation is a well-known phenomenon that may occur in the setting of radiotherapy [5,6], the incidence of radiotherapy-induced AS is low.…”