Abstract

A 61-year-old Caucasian female with history of autosomal dominant polycystic kidney disease presented to the dermatology clinic for a progressively growing mass on the right chest of two-months duration. She had mild tenderness to palpation, and she denied any other associated symptoms. She denied any personal or family history of breast cancer nor prior history of radiation treatment. On physical examination, a well-circumscribed, 2 cm by 3 cm, firm, mobile, skin-colored, subcutaneous nodule was present on the right superior anteriolateral chest. She was without lymphadenopathy. Punch biopsy revealed a large proliferation of atypical spindled and epithelioid cells with diffuse extravasated red blood cells. A solidly cellular sheet-like growth pattern of cells with numerous mitoses and several irregular vasoformative areas with hobnailing was present. The tumor cells had eosinophilic cytoplasm and plump vesicular nuclei with variably prominent nucleoli. Immunohistochemistry staining was positive for CD31 and ERG and negative for CD34, and a diagnosis of epithelioid angiosarcoma of soft tissue was rendered. A PET-CT scan was obtained, and the patient was urgently referred to general surgery for excision and sentinel lymph node biopsy. Epithelioid angiosarcoma is a rare subtype of the vascular neoplasm, angiosarcoma. It is characterized by an abundance of epithelioid cells often arranged in a sheet-like architecture. It can arise in a variety of primary sites and age groups with deep soft tissues of the extremities and in elderly patents most commonly seen, respectively. It is usually aggressive with early metastasis and poor prognosis with greater than 50% mortality rate in the first 2-3 years after diagnosis. We present a rare case of epithelioid angiosarcoma and discuss its interdisciplinary work-up and management. Excellent clinical and histological images, along with clinical follow-up, will be presented.

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