Surgical Dead End in a Renal Transplant Recipient Associated With a Rare Thrombohemorrhagic Syndrome

Primary breast angiosarcoma is an extremely rare malignancy. Association of this type of tumor with Kasabach-Merritt syndrome has only been reported in 3 cases in the past. To our knowledge, this is the first reported case of a solid-organ recipient.

A 53-year-old woman who underwent a deceased-donor renal transplantation 5 years previously presented with a 12-month history of a giant ulcerated lesion on her left breast. Biopsy of the overlying skin suggested primary angiosarcoma. Concurrently, the patient's bleeding from the site of the biopsy and hematology investigations indicated the presence of Kasabach-Merritt syndrome.

The case was discussed in a multidisciplinary setting. The decision was to use anthracycline-based chemotherapy as up-front treatment to assess tumor response and gain a local benefit for a subsequent resection. After the completion of 1 cycle of chemotherapy, the patient died of cardiovascular insufficiency. Primary angiosarcoma of the breast occurs in the third to fourth decade and has been reported only in women.

A high clinical suspicion and referral to a specialized center are necessary. Total mastectomy appears to be the only treatment conferring benefit; chemotherapy and radiation therapy are of little value.