Journal of Clinical Oncology, Vol 19, Issue 9
(May), 2001: 2570-2571
© 2001 American Society for Clinical Oncology
Unusual Aspects of Breast Cancer
Case 1. Angiosarcoma of the Breast
Hamid Mansouri,
Abdeloahed Jalil,
Leila Choho,
Abdelilah Souadka,
Brahim El Gueddari
Rabat University School of Medicine, Rabat, MoroccoCopyright © 2001 American Society of Clinical Oncology
A 23-year-old unmarried, nulliparous woman presented with a chief complaint of left breast enlargement of 10 months’ duration. On physical examination, a hard mass was located in the left breast, which measured 12 x 12 cm and was accompanied by edema and hyperemia in the overlying skin, without regional node involvement ( Fig 1). Tumor markers, including carcinoembryonic antigen, CA 15-3, and CA 19-9, were all within normal limits. A mammary echogram showed a low echoic heterogenous mass that measured 8 x 8 cm in the left breast with involvement of the skin on mammography. Thoracic computed tomography ( Fig 2) and breast magnetic resonance imaging revealed a large mass in the left breast with cystic components, partly invading the underlying pectoralis major muscle, but without invasion into the thorax. An incisional biopsy of the mass was performed. Many dilated capillaries were found, including an irregular network of vasculature with proliferation of cells, suggestive of an angiogenic tumor, the cells of which were immunohistochemically weakly positive for factor VIII with presence of necrosis ( Fig 3). The patient had a left mastectomy with biopsy of axillary lymph nodes, which were negative, accompanied by breast reconstruction using a latissimus dorsi flap. There was a complete resection with negative margins. No adjuvant therapy was given. Multiple lung, liver, and bone metastases were detected approximately 9 months later and she succumbed from respiratory failure caused by progressive pulmonary metastases.
Angiosarcoma of the breast occurs mainly during the third and fourth decades of life, in contrast to breast carcinoma, which generally arises later.1 This malignant tumor occurs primarily in young women, with 6% to 12% of the cases found during pregnancy, implying a hormonal effect. In most cases, the tumor size is more than 4 cm in diameter, which does not correlate with survival.2 Preoperative diagnosis of angiosarcoma of the breast by aspiration cytology and biopsy is often difficult, with a false-negative biopsy rate of 37% in one large review.3 Ultrastructural examination can reveal the vascular nature of angiosarcoma and demonstrate the existence of diagnostic Weibel-Palade bodies and pinocytic vesicles.4 Immunostaining for factor VIII–related antigen is helpful for the diagnosis of angiosarcoma of the breast.5 The histologic features are classified into three groups and the features in our patient are compatible with those of group III.4 Other attempts to correlate histologic features with clinical behavior were made in a series of 10 cases, where an unfavorable prognosis was related to a high mitotic count.6 Angiosarcoma has a high mortality rate, with only 10% to 21% of patients remaining disease-free after 5 years.2 Although some individuals seem to benefit from adjuvant chemotherapy, available treatment for patients with disseminated disease is of minimal benefit.4
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