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

• Primary angiosarcoma accounts for 0.04% of all malignant breast tumors.
• Exclusion of Kaposi sarcoma according to immunochemistry stains was necessary in this case of a transplant recipient because of the gross similarities in clinical presentation.
• Large lesions have been reported to lead to platelet sequestration and the manifestations of Kasabach-Merrit syndrome.
• Association of this primary angiosarcoma of the breast with Kasabach-Merritt syndrome has only been reported in 3 cases. To our knowledge, this is the first report of a case of a solid-organ recipient.
• Angiosarcoma of the breast has a poor prognosis and association with Kasabach-Merritt syndrome, usually seen at advanced stage, which makes the patient’s clinical course worse.

BackgroundPrimary 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.MethodsA 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.ResultsThe 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.ConclusionsA 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.