Primary Epithelioid Sarcoma Manifesting as a Fungating Scalp Mass - Imaging Features and Treatment Options. A Case Report and Literature Review.

Abstract

Primary epithelioid sarcoma is an extremely rare malignancy of the scalp. To date, less than a dozen such cases have been reported in the literature. The diagnosis often is a challenge to both radiologists and clinicians. This is largely attributed to the lack of literature on the imaging features of scalp epithelioid sarcoma. In this report, we highlight the role of multimodality imaging in the diagnosis of primary scalp epithelioid sarcoma and review the epidemiology, imaging, treatment options, and prognosis of these malignant scalp tumors. Displaying a multilobulated morphology, heterogeneous enhancement, and restricted diffusion on MRI, these tumors typically show central degeneration with hemorrhage, necrosis, and calcification. Wide surgical resection and adjuvant radiotherapy are the mainstays of treatment for localized scalp tumors. Nevertheless, the prognosis of patients with distant metastases at diagnosis is extremely poor compared to those with local or regional diseases.