Metastatic epithelioid hemangioendothelioma of the penis managed with surgery and interferon-α

Hemangioendothelioma of the penis is a rare tumor, with approximately 11 cases reported in the literature and only 1 case with documented metastases. Epithelioid hemangioendothelioma (EHE) arising in the skin is extremely rare, with the majority of documented cases developing in association with an underlying bone tumor.1 We report a case of metastatic EHE of the penis managed by multimodal therapy with long-term followup. CASE REPORT A 46-year-old white male presented to his primary care physician with a nodule in the left groin that was managed with antibiotics for a presumed infection. When the lesion progressed an excisional biopsy showed EHE in a lymph node. Seven months later skin nodules developed in the subcutaneous lymphatics of the penis as well as the groin. The patient underwent resection of the nodules and inguinal nodes in the left groin, and subcutaneous stripping of penile lymphatic tissue, leaving the skin intact. The surgical margin in the penile specimen was approximately 1 mm from EHE within vascular spaces. He was subsequently referred to us for further treatment. At presentation there was no evidence of recurrence in the penis and the left groin had healed. There was a palpable mobile 1 to 2 cm node medial to the right femoral vessels, which was negative on fine needle aspiration. A staging brain computerized tomogram revealed a left frontal lobe periventricular lesion, which was stereotactically biopsied using magnetic resonance imaging guidance and demonstrated demyelination and reactive gliosis. We then performed an en bloc resection of the skin and subcutaneous tissue of the penis, upper scrotum and lower abdominal wall, with continuous bilateral superficial and deep inguinal lymph node dissection, and bilateral pelvic lymph node dissection (fig. 1, A). The abdominal defect was closed by advancement techniques and an unmeshed thick split-thickness skin graft was used to cover the entire penis up to the glans. The remaining scrotal sac was advanced to cover the testes (fig. 1, B). Pathological evaluation showed multifocal residual EHE in the deep dermis and subcutaneous tissue with metastasis to the right superficial inguinal lymph nodes and left pelvic lymph nodes (fig. 2). After an uneventful recovery the patient was placed on 10 million units interferon- 3 times weekly for 1 year. He tolerated therapy with usual flu-like symptoms. At last followup the patient was 65 months following surgery and had no evidence of recurrent disease on physical examination or whole body computerized tomography. He is able to have satisfactory erections with sildenafil and the only sequela of the surgery is minimal right ankle edema. DISCUSSION