Abstract
Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a childhood malignancy, typically occurring in the bone and rarely in any other part of the body. We herein present a case of ES/PNET of the renal pelvis. A 37-year-old male patient presented with a chief complaint of pain in the left flank and gross hematuria. The tumor had caused moderate hydronephrosis, and ureteroscopic biopsy findings were highly suspicious of sarcoma. Subsequently, radical nephroureterectomy was performed. On the basis of the pathological and cytogenetic findings, a final diagnosis of primary ES/PNET of left renal pelvis was made. Adjuvant chemotherapy with adriamycin and ifosfamide was initiated as ES/PNET often exhibits aggressive biological behavior. The patient was disease-free at his last regular follow-up visit 18 months after the surgery. To our knowledge, this is the first reported case of primary ES/PNET of the renal pelvis.
Highlights
Ewing sarcoma/primitive neuroectodermal tumor (ES/ PNET) is the second most common form of bone malignancy occurring in childhood [1]
To help establish the biological nature of primary ES/PNET of the renal pelvis, we present the clinical, diagnostic, and therapeutic aspects of this rare neoplasm along with a review of the literature
During the past two decades, nine cases of primary sarcoma of the renal pelvis have been described in the English literature: six of leiomyosarcoma, two of rhabdomyosarcoma, and one of cystic embryonal sarcoma (Table 1) [2-10]
Summary
Ewing sarcoma/primitive neuroectodermal tumor (ES/ PNET) is the second most common form of bone malignancy (after osteosarcoma) occurring in childhood [1]. To help establish the biological nature of primary ES/PNET of the renal pelvis, we present the clinical, diagnostic, and therapeutic aspects of this rare neoplasm along with a review of the literature. Case presentation A 37-year-old male patient was admitted with a chief complaint of intermittent pain in the left plank and gross hematuria. His past medical history and findings of a physical examination were unremarkable. The tumor cells stained positive for CD99 (Figure 2), CD56, S-100, and vimentin; focally positive for epithelial membrane antigen and Ki-67; and negative for Bcl-2, cytokeratin 7, cytokeratin 20, desmin, WT-1, muscle specific actin, and Syn. On the basis of these findings, a pathological diagnosis of primary ES/PNET of the left renal pelvis was made. The patient was in excellent physical and mental condition without recurrence or metastasis as confirmed by careful follow-up at 18 months postoperatively
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