Clinicopathological Characteristics, Treatment, and Survival in Patients Diagnosed With Proximal-Type Epithelioid Sarcoma: A Case Report and Systematic Review

Epithelioid sarcoma is a rare entity that shows a predilection for the young and middle-aged population. There are two subtypes, i.e., the distal or conventional type, which is located in distal extremities, and the proximal type, located in proximal areas of the extremities, pelvis, perineal, and genital region. The latter is characterized by more aggressive behavior, a higher recurrence rate, and poor prognosis. Histopathological and immunohistochemical diagnoses are key to correct and timely treatment and a higher survival rate. We report a case of a 41-year-old man who presented a palpable progressive growth mass in the hypogastrium. The disease time was of nine months, and the tumor was resected, but it recurred a few months later in the same location. Computed tomography (CT) scans showed images suggestive of lung metastasis and the patient had to undergo a second surgery. He received eight cycles of chemotherapy and a subsequent CT scan control showed the progression of the disease, so a new chemotherapy regimen was established. The patient received three cycles of chemotherapy without improvement, so he decided to discontinue treatment. His last outpatient medical consultation was in January 2022. A systematic review of the studies published in PubMed and Google Scholar was performed. We identified 291 articles, but only 41 reports and case series were included, with a total of 55 patients. It is important to include this type of tumor in the differential diagnosis of epithelial tumors due to its aggressive behavior. Correct and timely diagnosis is crucial to obtain lower recurrence rates, lower mortality, and higher survival rates in these patients.