Pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma, fibroma-like variant of epithelioid sarcoma) of the thoracic spine

Abstract

Pseudomyogenic hemangioendothelioma is a soft tissue tumor found in young adults, predominantly males. The tumor has been reported in various locations in the body, including the head, neck, chest wall, abdominal wall, genital region, and extremities. Until now, there has been no indication of occurrence in the spine. A 25-year-old male presented with spinal cord compression, due to an extradural tumor involving the third and fourth thoracic vertebrae with extension into the right pleural cavity. Histopathologic examination revealed a pseudomyogenic hemangioendothelioma, also described as epithelioid sarcoma-like hemangioendothelioma, or fibroma-like variant of epithelioid sarcoma. We describe the first occurrence of pseudomyogenic hemangioendothelioma in the thoracic spine. According to previous reports based on other locations, the tumor has an indolent clinical course with a small risk of metastasis, therefore complete macroscopic excision is the treatment of choice. Local recurrence may occur even with complete surgical resection, requiring close follow-up; adjuvant therapy is warranted.