A rare pure embryonal rhabdomyosarcoma of the urinary bladder in an adult successfully managed with neoadjuvant chemotherapy and surgery: a case report

Ahsaini, Mustapha; Ouattar, Khalid; Azelmad, Hamid; Mellas, Soufiane; Ammari, Jallal Eddine; Tazi, Mohammed Fadl; Fassi, Mohammed Jamal El; Farih, Moulay Hassan; Sekal, S.; Harmouch, Taoufiq

doi:10.1186/s13256-018-1870-1

Cited by 5 publications

(3 citation statements)

References 16 publications

(17 reference statements)

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“…The therapeutic options include total or partial cystectomy, transurethral resection of the bladder, chemotherapy and radiotherapy. 2 , 4 In our case, RMS was diagnosed at the metastatic stage, which is why we opted for chemotherapy after TURB, which allowed the cessation of hematuria. In case of localized RMS, the gold standard remains radical cystectomy associated with bilateral lymph node dissection.…”

Section: Discussionmentioning

confidence: 79%

“…3 This radical surgery may be preceded by neo-adjuvant chemotherapy. 4 Radiation therapy adjuvant to surgery gives excellent results in case of embryonal RMS of the bladder in children. 5 Partial cystectomy can be proposed in young subjects who have a desire to father a child, when the tumor is small (less than 3 cm) and located at the level of the dome but it is imperative to ensure healthy surgical margins.…”

Section: Discussionmentioning

confidence: 99%

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Adult bladder rhabdomyosarcoma: A case report

Rahoui

Ouanes²,

Marrak³

et al. 2022

Urology Case Reports

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Section: Discussionmentioning

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Adult bladder rhabdomyosarcoma: A case report

Rahoui

Ouanes²,

Marrak³

et al. 2022

Urology Case Reports

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“…In literature, there is a paucity of successful reports of neoadjuvant chemotherapy and adjuvant radiotherapy for adult RMS. 17,18 From 1989 to 2009, Wang et al published a case series of 25 adult soft tissue prostate tumors (six were RMS) demonstrating that chemotherapy did not improve overall survival. 19 Given our findings, it is plausible that specific subtypes of soft tissue tumors in the prostate, like RMS, may be responsive to chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

Epidemiology and survival outcome of adult kidney, bladder, and prostate rhabdomyosarcoma: A SEER database analysis

Patel

Hensel

et al. 2020

Rare Tumors

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Rhabdomyosarcoma (RMS) is rare in adulthood, accounting for 2%–5% of adult soft tissue tumors, and less than 20% occur in genitourinary organs. Given its rarity, survival data on adult kidney, bladder, and prostate RMSs is limited. In this population-based analysis, we performed an analysis of all adult RMS cases reported in Surveillance, Epidemiology, and End Results (SEER) database to understand prognostic factors among kidney, bladder, and prostate RMS. A query of the SEER database was performed from 1973 to 2016 for patients >18 of age with RMS. The final cohort consisted of 14 kidney, 35 bladder, and 21 prostate RMS cases in the adult population. Demographic, treatment, and survival data were obtained. Analysis was performed using Fisher’s exact test, survival analysis, and model. The median (range) age of diagnosis for adult bladder RMS was 65 years old (19–84) compared to 52.5 (28–68) and 42 (19–87) for kidney and prostate ( p = 0.007). About 78.6% of patients underwent surgical intervention. Five-year overall survival (OS) for adult kidney, bladder, and prostate RMS are 17.1% (2.9–41.6%), 22.2% (9.4–38.4%), and 33.0 (12.8–55.0%), respectively. OS was not statistically associated with primary site ( p = 0.209). On multivariable analysis, compared to adult bladder RMS, kidney RMS had a higher incidence of mortality (HR: 2.16, 95% CI 1.03–4.53, p = 0.041). Incidence of mortality from prostate RMS was not significantly different from bladder RMS (HR: 0.70, 95% CI 0.30–1.65, p = 0.411). Extent of disease (HR: 5.17, 95% CI 2.09–12.79, p < 0.001) and older age (HR 1.03, 95% CI 1.01–1.04, p = 0.002) were adverse prognostic factors for OS. Overall survival at 5 years for adult kidney, bladder, and prostate RMS is poor. Localized disease and younger age are prognostic factors for improved outcomes in adult RMS. Hence, early diagnosis and intervention appear paramount to improved survival for this rare malignancy in adulthood.

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Small cell neuroendocrine carcinoma and poorly differentiated rhabdomyosarcomas of the urinary bladder in adults—A comparative analysis in favor of a common histogenesis

Bahlinger,

Stoehr,

Hartmann

et al. 2024

Virchows Arch

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Rhabdomyosarcoma (RMS) of the urinary bladder in adults and elderly is an exceptionally rare neoplasm that displays poorly differentiated solid (alveolar-like) small cell pattern, frequently indistinguishable from small cell neuroendocrine carcinoma (SCNEC). However, the histogenesis of RMS and SCNEC and their inter-relationship have not been well studied and remained controversial. We herein analyzed 23 SCNEC and 3 small round cell RMS of the bladder for neuroendocrine (synaptophysin + chromogranin A) and myogenic (desmin + myogenin) marker expression and for TERT promoter mutations. In addition, the RMS cohort and one SCNEC that was revised to RMS were tested for gene fusions using targeted RNA sequencing (TruSight Illumina Panel which includes FOXO1 and most of RMS-related other genes). Overall, significant expression of myogenin and desmin was observed in one of 23 original SCNEC justifying a revised diagnosis to RMS. On the other hand, diffuse expression of synaptophysin was noted in 2 of the 4 RMS, but chromogranin A was not expressed in 3 RMS tested. TERT promoter mutations were detected in 15 of 22 (68%) SCNEC and in two of three (67%) assessable RMS cases, respectively. None of the four RMS cases had gene fusions. Our data highlights phenotypic and genetic overlap between SCNEC and RMS of the urinary bladder. High frequency of TERT promoter mutations in SCNEC is in line with their presumable urothelial origin. In addition, the presence of TERT promoter mutation in 2 of 3 RMS and lack of FOXO1 and other gene fusions in all 4 RMSs suggest a mucosal (urothelial) origin, probably representing extensive monomorphic rhabdomyoblastic transdifferentiation in SCNEC.

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A rare pure embryonal rhabdomyosarcoma of the urinary bladder in an adult successfully managed with neoadjuvant chemotherapy and surgery: a case report

Cited by 5 publications

References 16 publications

Adult bladder rhabdomyosarcoma: A case report

Adult bladder rhabdomyosarcoma: A case report

Epidemiology and survival outcome of adult kidney, bladder, and prostate rhabdomyosarcoma: A SEER database analysis

Small cell neuroendocrine carcinoma and poorly differentiated rhabdomyosarcomas of the urinary bladder in adults—A comparative analysis in favor of a common histogenesis

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