Primary Non-metastatic Epithelioid Sarcoma of Lumbar Vertebra L3. Pediatric Case Report and Brief Review of the Literature.

Abstract

Epithelioid Sarcoma (ES) is a rare non-rhabdomyosarcoma soft tissue sarcoma. It is a high-grade malignancy that most commonly occurs in young adult males. These tumors are characterized for slow growing nodules with predilection for the extremities. A proximal form arising in the deep part of the pelvis, perineum, and genital tract and proximal extremities has been described. Only three cases with localization to the spine have been reported. We report the case of an 11-year old female with a primary ES of the lumbar spine, who presented with antalgic gait and evidence of L3 vertebral collapse on x-rays. She had upfront surgery for spine stabilization and biopsy. Treatment consisted of neoadjuvant chemotherapy with ifosfamide and doxorubicin, local control with radiation therapy, and second-look surgery with complete reconstruction of L3. She tolerated treatment well, without significant toxicities or complications. When this paper was submitted for publication, she had completed 6 months of follow up in first clinical remission without evidence of local or distant disease. This is the first case of ES diagnosed at our center, likely the first pediatric case of ES diagnosed in the Dominican Republic; although a national cancer registry does not exist. Establishing the diagnosis would not have been possible without access to international experts in pediatric oncologic pathology thought existing institutional collaboration. This is not a novel case but a diagnostic challenge, issue exacerbated in developing countries. We are proud to have offered appropriate, timely treatment, with a similar integral quality as in a developed country.