Abstract
BackgroundThe Ewing sarcoma family of tumors comprises a group of high-grade small round cell tumors, including Ewing sarcoma of bone, extra-skeletal Ewing sarcoma, peripheral primitive neuro-ectodermal tumor (PNET), and Askin tumor (thoraco-pulmonary PNET). They are more prevalent in young children and adolescents. Ewing's Sarcoma is an aggressive tumor majorly described in bones. Primary renal Ewing's sarcoma is an extremely rare entity, characterized by a very aggressive course, with very few reported cases in the literature.Case presentationWe present an 18-year-old girl who presented with sudden onset left flank pain and hematuria. The patient had histopathology-proven primary renal Ewing's sarcoma, which was initially misdiagnosed as renal cell carcinoma on imaging.ConclusionsOwing to its non-specific radiological appearance, a high index of suspicion and a systematic approach is essential for detection of renal Ewing’s Sarcoma.
Highlights
The Ewing sarcoma family of tumors comprises a group of high-grade small round cell tumors, including Ewing sarcoma of bone, extra-skeletal Ewing sarcoma, peripheral primitive neuro-ectodermal tumor (PNET), and Askin tumor
We report the case of an eighteen-year girl with biopsy-proven extraskeletal Ewing’s sarcoma in the kidney, which was indistinguishable from renal cell carcinoma (RCC) on imaging
No specific imaging features are seen in imaging and it is impossible to differentiate renal Ewing’s from much more prevalent RCC by imaging alone
Summary
The Ewing sarcoma family of tumors comprises a group of high-grade small round cell tumors, including Ewing sarcoma of bone, extra-skeletal Ewing sarcoma, peripheral primitive neuro-ectodermal tumor (PNET), and Askin tumor (thoraco-pulmonary PNET). Conclusions: Owing to its non-specific radiological appearance, a high index of suspicion and a systematic approach is essential for detection of renal Ewing’s Sarcoma. Background All Ewing sarcoma family of tumors (ESFT) are characterized by small round blue cells on histology and show similar chromosomal translocations [1]. Rarer still is its manifestation as a primary renal mass, with a reported incidence of slightly more than 100 cases globally [3].
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