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A Case of Intra-Abdominal Desmoplastic Small Round Cell Tumor in a Young Girl Patient

Objective: The main aim of this case report was to present the method of diagnosis, management, and prognosis of a young adolescent diagnosed with a desmoplastic small round cell tumor (DSRCT). Case Report: We report a new case of a 16-year-old female who proceeded complaining about non-specific abdominal pain. Physical examination revealed a palpable mass in the lower abdomen and a diffuse abdominal pain. Abdominal ultrasonography showed an irregular pelvic mass, with heterogeneous echo structure making 12 cm × 8 cm. She underwent an exploratory laparotomy and the excision of the mass revealed desmoplastic small round cell tumor. She died 6 months after diagnosis despite chemotherapy. Conclusion: Although a DSRCT can develop at various sites, the intra-abdominal site is the most common location. Due to our limited knowledge of the pathologic and clinical nature of this disease, there is no clear consensus regarding the optimal therapeutic procedures for treating this neoplasm and a multimodal treatment with surgery, chemotherapy and radiotherapy is used for these rare cases. A high degree of care and improvements in diagnostic capabilities are required in order to identify this entity and avoid misdiagnosis.