Abstract
Proximal-type epithelioid sarcoma is a rare soft tissue neoplasm which arises from the more proximal part of body and occurs more often in young people; the definite diagnosis depends mainly on the pathological examination; early detection and complete excision remain the foundation of treatment. Due to its aggressive behavior, high capacity of recurrence and the great ability to metastasize, a careful clinical long-term monitoring is required. We report a new case of a 20 years old girl, presented with proximal-type epithelioid sarcoma in her right scapular region, confirmed by pathological examination and removed surgically without recurrence or metastasis at eighteen months of follow-up.
Highlights
Proximal-type epithelioid sarcoma (PES) is a rare high grade softtissue sarcoma, typically presenting as a subcutaneous or deep dermal mass, with slow growing and high potential for distant metastasis in adolescents and young adults [1]
Aggressive behavior, high capacity of recurrence and the difficulty of its clinical and histopathological diagnosis, we report a new case of a 20 years old girl, presented with proximal-type epithelioid sarcoma in her right scapular region removed and treated successfully
The rereading of the histopathological slides made the diagnosis of superficial subcutaneous epithelioid sarcoma and a chest and abdominal computed tomography ruled out metastasis
Summary
Proximal-type epithelioid sarcoma (PES) is a rare high grade softtissue sarcoma (less than 1 of all soft tissue sarcomas), typically presenting as a subcutaneous or deep dermal mass, with slow growing and high potential for distant metastasis in adolescents and young adults [1]. These tumors arise in proximal locations (trunk, axilla, genital area...), have a much worse prognosis if delayed diagnosis and treatment. There was no recurrence or metastasis at eighteen months of follow-up
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