Primitive neuroectodermal tumor/Ewing’s sarcoma of the urinary bladder: a case report and its molecular diagnosis

Abstract

We report a rare case of primitive neuroectodermal tumor/Ewing's sarcoma (PNET/ES) arising from the urinary bladder. A 65-year-old man presented with hematuria and dysuria. Computed tomography revealed an enlarged invasive tumor at the base of the bladder. No additional abnormal findings were disclosed by other diagnostic imaging methods. The surgical specimens showed small round cell tumor with positive staining for MIC2 gene product (CD99). EWS-FLI1 fusion transcripts were detected by reverse transcriptase polymerase chain reaction and direct sequencing, confirming the diagnosis of PNET/ES. The patient developed swollen pelvic lymph nodes as well as multiple lung metastases at 8 months postoperatively. No effective results could be obtained even with systemic chemotherapy consisting of vincristine, ifosfamide, doxorubicin and etoposide (VIDE) based on the EUROpean Ewing tumour Working Initiative of National Groups 1999 (EURO-E.W.I.N.G. 99) multinational trial. The patient died of acute superior mesenteric artery thrombosis at 22 months postoperatively. PNET/ES could have been included in past cases of small cell carcinoma because of the difficulty in its differential diagnosis. Exact diagnosis is crucial for deciding the treatment strategy for rare bladder tumors consisting of small round cells.

The Ewing’s Sarcoma Family of Tumors of Urinary Bladder: A Case Report and Review of the Literature

“…Ewing's sarcoma/Primitive neuroectodermal tumor (ES/PNET) can also rarely occur in visceral organs such as the liver, testis, uterus, kidney, ovary, parotid gland, lung, pancreas, urinary bladder, rectum, heart and gall bladder (2,3). In the literature, only 15 cases of ES/PNET of the urinary bladder have been reported (4)(5)(6)(7)(8)(9)(10). We present here another case of primary ES/ PNET in the urinary bladder, which was locally invasive and had no metastases at the time of diagnosis.…”

Primitive neuroectodermal tumor of urinary bladder

“…Radical cystectomy was performed in 6 of 18 cases (33.3%), lung metastasis was detected in 1 of 6 cases; of these, 5 patients received postoperative chemotherapy, and the treatment of the last one was not reported. [ 4 , 11 , 20 – 23 ] Of the 6 patients who underwent combined therapy, 1 patient (17%) died 14 months after the surgery, and the mean survival period after the surgery was at least 22.5 months (14–36 months). TURBT or partial cystectomy was performed in 11 of the remaining 12 cases (91.7%), [ 3 , 6 , 8 – 10 , 12 , 15 , 16 , 24 , 25 ] pelvic or retroperitoneal lymph node metastasis was detected in 5 of 11 cases, and the treatment of the last one was not reported (Table 1 ).…”

Prostatic sarcoma of the Ewing family in a 33-year-old male – A case report and review of the literature

“…Existing data suggests similar outcome of adult extraosseous Ewing sarcoma compared to that of bone-confined disease in terms of response to multi-modality treatment and the prognostic factors influencing treatment success [17] , [18] . As for the field of urology, individual cases of extraosseous Ewing sarcomas have also been reported in the urinary bladder, kidneys, adrenal glands and even the penis [19] , [20] , [21] , [22] . To our knowledge only nine other cases of (peri-)prostatic Ewing sarcoma are known worldwide being first described in 2003 [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] .…”