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.
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ABSTRACT: Ewing sarcoma (ES) of the bladder is extremely rare. Here we report the successful treatment of the youngest case of ES arising from bladder encountered to date and a literature review. A 10-year-old boy who presented with polyuria and lower abdominal swelling was found to have a 13 cm mass arising from the bladder localized to the center of the lower abdomen. Biopsy confirmed ES. Following chemotherapy, the mass shrank to 5 cm and was found to be localized to the right side of the bladder with invasion of the right vas deferens. One-third of the right side of the bladder and part of the right vas deferens were resected. No viable cancer cells were detected in the resected specimen, and the surgical margins were reported to be negative. The patient is currently well with no recurrence or metastases after 11 months. There are 12 cases of Ewings sarcoma arising from the bladder reported in the English literature; two cases in children. Our case will be the third pediatric case and the youngest.Pediatric Surgery International 07/2014; · 1.06 Impact Factor
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ABSTRACT: Mesenchymal tumours of the urinary bladder and prostate are infrequent neoplasms. The body of literature is growing with isolated case reports and short series, and the majority of cases are benign neoplasms. Other than stromal tumour of uncertain malignant potential and prostatic stromal sarcoma, both neoplasms derived from the specific prostatic stroma, the mesenchymal neoplasms in these locations are identical to their counterparts seen in other organs. However, the limited amount of tissue generated by biopsy and rarity of mesenchymal lesions in these sites create unique diagnostic difficulties, while correct classification of the neoplasm often bears significant impact on prognosis and therapeutic strategy. In this review we summarise the diagnostic features, focus on the differential diagnosis, and highlight the potential diagnostic pitfalls of mesenchymal tumours of the bladder and prostate.Pathology 12/2012; · 2.62 Impact Factor