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Abstract

Angiosarcoma is a rare malignant neoplasm of vascular endothelial cells. A majority are primarily in the skin. Angiosarcoma of the head and neck is the most common form, but only constitutes 0.1% of all head and neck malignancies. We present an extraordinary case of a 71 year-old male six months status post orthotopic liver transplant secondary to end stage liver disease from cryptogenic cirrhosis, who presented to the liver clinic with a rapidly growing scalp tumor and hypodense lesions within the liver. Further work-up and biopsies confirmed the first documented case of primary scalp angiosarcoma with metastasis to the liver in an orthotopic liver transplant patient. Case ReportA 71-year-old man with an orthotopic liver transplant 6 months earlier, secondary to end-stage liver disease from cryptogenic cirrhosis, presented to the liver clinic with abdominal distension and worsening ascites. The patient was otherwise asymptomatic and denied having fever, chills, abdominal pain, nausea, vomiting, and diarrhea. For immunosuppression, he had been receiving tacrolimus 2 mg twice daily, with tacrolimus levels between 5 and 8. An ultrasound, followed by a computed tomography scan and magnetic resonance imaging of the abdomen, showed numerous small cystic lesions with peripheral enhancement throughout the liver. These findings led the team to be concerned about liver abscesses (Figure 1). The patient was admitted to the hospital for a further work-up.Piperacillin and tazobactam IV were given as antibiotic therapy, and the patient underwent computed tomography-guided paracentesis, aspiration of cystic fluid, and a core needle liver biopsy. The dermatology department was consulted to evaluate a rapidly enlarging and bleeding scalp lesion, present for 5 weeks. A physical examination revealed a bleeding, firm, indurated nodule on the right temporal scalp, with peripheral purpura and ecchymosis extending down the forehead to the right temple (Figure 2). A satellite skin-colored nodule also was noted posterior to the primary lesion. Biopsy of the primary nodule showed dense, diffuse hemorrhagic proliferation of enlarged pleomorphic, mitotic epithelioid, and spindled CD31+ cells, consistent with the diagnosis of epithelioid angiosarcoma (Figure 3)