Genitourinary Rhabdomyosarcoma

Kaefer, Martin; Rink, Richard C.

doi:10.1016/s0094-0143(05)70095-1

Cited by 17 publications

(8 citation statements)

References 81 publications

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“…Whereas the prognosis of untreated rhabdomyosarcomas is poor, modern multimodal therapy concepts have been quite successful [14]. Surgery, chemotherapy, and radiotherapy are applied, depending on the tumor stage [25]. In a review by Bale et al [2], 72% of patients with urinary bladder rhabdomyosarcomas survived 5 years, while 11 of 31 children (28%) with primary rhabdomyosarcoma died after combined therapy.…”

Section: Rhabdomyosarcomamentioning

confidence: 99%

Nonepithelial neoplasms of the urinary bladder

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2001

Virchows Arch

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Nonepithelial tumors are rare in the urinary bladder, but their exact classification is very important in the differential diagnosis between these tumors and epithelial lesions. In the new WHO classification and in the third series of the Armed Forces Institute of Pathology (AFIP) "Atlas of Tumor Pathology" on urinary bladder tumors, various mesenchymal tumors, mixed epithelial and mesenchymal tumors and myofibroblastic proliferations are summarized. In the following we will describe the histology, immunohistology, and cytogenetics of nonepithelial tumors and lesions.

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Section: Rhabdomyosarcomamentioning

confidence: 99%

Nonepithelial neoplasms of the urinary bladder

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2001

Virchows Arch

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“…It is the most common bladder neoplasm in children under 10 years of age and the median age of children presenting with B/P RMS is 5 years (10). Male to female ratio is (1.4:1) and it is more often found in Caucasians than African-Americans (2.3:1) [ 11 , 12 ].…”

Section: Rhabdomyosarcomamentioning

confidence: 99%

“…The increased incidence in siblings of children with central nervous system tumors and adrenocortical carcinoma and the familial aggregations of RMS with other sarcomas, breast tumors, and brain neoplasms are suggestive of a possible genetic factor [ 12 ].…”

Section: Rhabdomyosarcomamentioning

confidence: 99%

Genetics of Bladder Malignant Tumors in Childhood

Zangari¹,

Zaini²,

Gulìa³

2015

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Bladder masses are represented by either benign or malignant entities. Malignant bladder tumors are frequent causes of disease and death in western countries. However, in children they are less common. Additionally, different features are found in childhood, in which non epithelial tumors are more common than epithelial ones. Rhabdomyosarcoma is the most common pediatric bladder tumor, but many other types of lesions may be found, such as malignant rhabdoid tumor (MRT), inflammatory myofibroblastic tumor and neuroblastoma. Other rarer tumors described in literature include urothelial carcinoma and other epithelial neoplasms. Rhabdomyosarcoma is associated to a variety of genetic syndromes and many genes are involved in tumor development. PAX3-FKHR and PAX7-FKHR (P-F) fusion state has important implications in the pathogenesis and biology of RMS, and different genes alterations are involved in the pathogenesis of P-F negative and embryonal RMS, which are the subsets of tumors most frequently affecting the bladder. These genes include p53, MEF2, MYOG, Ptch1, Gli1, Gli3, Myf5, MyoD1, NF1, NRAS, KRAS, HRAS, FGFR4, PIK3CA, CTNNB1, FBXW7, IGF1R, PDGFRA, ERBB2/4, MET, BCOR. Malignant rhabdoid tumor (MRT) usually shows SMARCB1/INI1 alterations. Anaplastic lymphoma kinase (ALK) gene translocations are the most frequently associated alterations in inflammatory myofibroblastic tumor (IMT). Few genes alterations in urothelial neoplasms have been reported in the paediatric population, which are mainly related to deletion of p16/lnk4, overexpression of CK20 and overexpression of p53. Here, we reviewed available literature to identify genes associated to bladder malignancies in children and discussed their possible relationships with these tumors.

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“…The presenting symptoms of ERMP published in literature are often related to urethral obstruction. Radiologic evaluation of genitourinary organs and potential intra-abdominal metastatic sites can be accomplished with computed tomography (CT) of the abdomen and pelvis [5] [6]. Recently, the improved contrast resolution of magnetic resonance imaging (MRI) has offered new potential for staging primary tumors.…”

Section: Discussionmentioning

confidence: 99%

“…Recently, the improved contrast resolution of magnetic resonance imaging (MRI) has offered new potential for staging primary tumors. In fact, the use of longitudinal T1-weighted images in MRI reduces false impressions of tumor spread by reducing the partial-volume averaging effect encountered in CT scanning [5]. However, the radiological differential diagnosis with prostate adenocarcinoma can be very difficult.…”

Section: Discussionmentioning

confidence: 99%

Adult Primary Prostate Embryonal Rhabdomyosarcoma: Report of a Case and Revue of Literature

Amaadour¹,

Tahiri²,

Ameurtesse³

et al. 2014

JCT

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Introduction: Primary embryonal rhabdomyosarcoma of prostate (ERMP) is an extremely rare pathological and clinical entity in adults. It's characterized by extensive loco-regional spread and a tendency to metastasize by blood stream and regional lymphatics. In adult patients, data available on the management of ERMP are limited and mostly derived from the pediatric clinical trials on rhabdomyosarcoma. Case Report: We report a case of group III ERMP approached by initial surgery followed by the administration of three courses of vincristine, doxorubicin and Cyclophosphamide regimen. The patient succumbed to disease complications three months after the original diagnosis. Conclusion: The most appropriate therapeutic approach for adults with ERMP remains questionable. Further studies are needed to improve the understanding of biological behavior of ERMP in adults and to define the best therapeutic strategy in locally advanced disease.

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Genitourinary Rhabdomyosarcoma

Cited by 17 publications

References 81 publications

Nonepithelial neoplasms of the urinary bladder

Nonepithelial neoplasms of the urinary bladder

Genetics of Bladder Malignant Tumors in Childhood

Adult Primary Prostate Embryonal Rhabdomyosarcoma: Report of a Case and Revue of Literature

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