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

Abstract

Background: Only 15 cases of Ewing's Sarcoma (EWS) family of tumors of urinary bladder have been documented in the literature to date. Case Report: We presented here a 38 year-old female with primary urinary bladder EWS with no distant metastases. She had presented with macroscopic hematuria and had undergone transurethral resection of the tumor within the following week. Microscopic examination revealed a tumor diffusely infiltrating the lamina propria and muscularis propria under an intact urothelium, which was composed of small round blue cells with scant cytoplasm, monotonous round or oval nuclei, stippled chromatin and small nucleoli. Immunohistochemistry showed strong vimentin, synaptophysin and membranous CD99 expression by the tumor. Fluorescent in situ hybridization analysis displayed the EWSR1 rearrangement. Radical cystectomy with total abdominal hysterectomy and bilateral salpingo-oophorectomy, extended lymph node dissection, and ileal conduit were performed. As adjuvant chemotherapy, she received vincristine, doxorubicin, cyclophosphamide and mesna, alternating with courses of etoposide, iphosphamide and mesna. She is alive and well with no evidence of disease 14 months after the surgery. Conclusion: Surgery supported with chemotherapy should be considered as an option, especially in advanced Ewing's sarcoma family of tumors of urinary bladder.

Primary adult primitive neuroectodermal tumor of the bladder

“…Most patients with PNETs have a young age at onset. However, according to the 9 cases of bladder PNETs (Table 1 ) currently available in the literature, [ 8 – 16 ] this type of PNET is predominant in middle-aged and elderly individuals, with an average onset age of 53.5 years; in 60% of the cases, including the present one (6/10), the patients were older than 60 years. This type of bladder tumor is often overlooked by clinicians, because the patient's age and tumor site show inconsistencies with the onset characteristics of PNETs.…”

“…Currently, surgery is the main treatment for PNETs, and it should be performed in patients who can tolerate it. [ 8 , 9 , 11 – 16 ] The prognosis of patients whose tumors can be completely removed (complete RO) is better than that of those who undergo palliative surgery. [ 8 – 16 ] Postoperative chemotherapy is the conventional treatment for adult bladder PNETs; vincristine, adriamycin, cyclophosphamide, epirubicin, cisplatin, etoposide, docetaxel, dexamethasone, doxorubicin, and mesna have been used as chemotherapeutic agents in such cases.…”

Primitive neuroectodermal tumor of urinary bladder

“…Recent evidence showed that immunodeficiency might be a risk factor for PNET of the bladder. [ 4 ] A review of the literature revealed that out of 5 (28%) patients who had immunodeficiency before the diagnosis of PNET of the urinary bladder, 1 patient received immunosuppressive drugs after renal transplantation and 4 patients underwent chemotherapy for other malignant tumors. Molecular tests by fluorescence in situ hybridization (FISH) or reverse transcriptase–polymerase chain reaction (RT-PCR) revealed that the Ewing sarcoma/friend leukemia integration-1 ( EWS/FLI-1 ) fusion gene was detected in some immunodeficient patients with PNET.…”

“…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 ).…”

Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade’s Experience From a Single Centre in India

“…Further ESFTs in bladder and prostate noted in this cohort were rarely reported. [42][43][44][45][46][47] Metastatic disease involving lung, lymph nodes, and several organs has been described 12,48,49 ; however, primary ESFTs of breast, [50][51][52] lung, [53][54][55] intestine, 56,57 and brain 22,[58][59][60][61][62] were rarely reported.…”