Malignant Extrarenal Rhabdoid Tumor of the Bladder: 9-Year Survival After Chemotherapy and Partial Cystectomy

Chang, Joseph H.; Dikranian, Armen H.; Johnston, W. H.; Storch, Susan K.; Hurwitz, Roger A.

doi:10.1097/01.ju.0000107014.40195.cf

Cited by 19 publications

(12 citation statements)

References 2 publications

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“…Good outcomes in MRT may be the result of an aggressive surgical approach [8,9], and the degree of surgical resection appears to be one of the most important factors in predicting prognosis [10]. Anticancer agents we gave as the adjuvant chemotherapy (doxorubicin, vincristine, cyclophosphamide, ifosfamide, and etoposide) were almost the same ones used unsuccessfully in the above case [7].…”

Section: Discussionmentioning

confidence: 94%

Continuous remission in an infant with chest wall malignant rhabdoid tumor after relapse

Hosoi

Iehara

Tsuchiya

et al. 2007

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 94%

Continuous remission in an infant with chest wall malignant rhabdoid tumor after relapse

Hosoi

Iehara

Tsuchiya

et al. 2007

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 99%

“…However, interestingly, 2 reported cases of pure MRT of the bladder with long-term follow-up have had good outcomes [11,12]. Overall, however, the typical published 5-year overall survival rates based on retrospective case series range from 15% to 36% [1,11,20]. The relationship between younger age (younger than 36 months) and poorer outcomes in MRT/AT/RT, regardless of location of the primary tumor, is increasingly well established [1,9,21,22].…”

Section: Discussionmentioning

confidence: 99%

Molecularly confirmed primary malignant rhabdoid tumor of the urinary bladder: implications of accurate diagnosis

Savage

Linn

McDonough

et al. 2012

Annals of Diagnostic Pathology

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Malignant rhabdoid tumors (MRTs) are well recognized in the kidney and extrarenal sites such as soft tissues, retroperitoneum, and bladder but are classified as atypical teratoid/rhabdoid tumors in the central nervous system. The unifying features of both extracranial MRT and atypical teratoid/rhabdoid tumors are the exon deletions/mutations of the SMARCB1 (SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1) gene in 22q11.23 and resulting loss of SMARCB1/INI1 (integrase interactor 1) protein expression by immunohistochemistry. We herein report a case of extrarenal rhabdoid tumor confined to the bladder in a 3-year-old child, diagnosed by histopathology and confirmed by immunohistochemical and molecular studies. This is only the fourth molecularly proven primary MRT of the bladder to be reported. The patient’s peripheral blood was negative for the deletions observed in the tumor, thereby confirming a sporadic origin for the tumor. Given the possible dismal outcome, urgency for definitive diagnosis to institute intensive multimodality therapy, histopathologic differential diagnosis with rhabdomyosarcoma and urothelial carcinoma with rhabdoid features, and lack of consensus management guidelines, oncologists, urologists, and pathologists must be aware of this entity. Evaluation for a germ line SMARCB1 alteration may greatly aid risk stratification and family planning.

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“…Two of these cases, both 4-year-old children, were alive at 2 and 9 years at follow up, respectively. 4,5 The National Wilms Tumour Study showed survival increased with age in patients with rhabdoid tumours, but this 30% 1-year survival pure malignant rhabdoid tumour of the bladder Warren et al…”

Section: Discussionmentioning

confidence: 99%

Pure malignant rhabdoid tumour of the bladder

Warren

Oxley²,

Koupparis

2014

CUAJ

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We present the case of a 17-year-old girl with pure malignant rhabdoid tumour of the bladder treated with a multimodal approach. She is recurrence-free at her 1-year follow-up. Case reportWe present the case of a 17-year-old female who presented with lethargy, haemoglobin of 41 g/L and an abdominal ultrasound showing a 5-cm bladder mass. She had no hematuria.Endoscopic examination demonstrated a solid tumour resembling a transitional cell carcinoma and 42 g were resected. The tumour was poorly differentiated showing immunohistochemical positivity for pan cytokeratin, CD34, WT1 and CD56. This was interpreted as a poorly differentiated carcinoma with neuroendocrine features. The slides were sent for a second opinion to the pediatric pathologist at the Bristol Royal Infirmary and Great Ormond Street, UK. Further immunohistochemistry showed loss of INI1/BAF47 and positive vimentin staining. This was diagnostic of a malignant rhabdoid tumour (MRT). There was no evidence of deletion of the HIRA region of 22q11, assessed using in situ hybridization, although small deletions may be missed using this technique.A staging computed tomography scan and magnetic resonance imaging (MRI) post-resection revealed localized disease. She was reviewed at both the pediatric and adult bladder cancer multidisciplinary meetings where a decision for a multimodal therapy was made.She received 2 cycles of doxorubicin, ifosfamide, carboplatin and etopside, and a subsequent MRI demonstrated good response.She underwent a robotic uterine, cervical and vaginal sparing cystectomy and intracorporeal formation of an ileal conduit. The decision to spare her vagina, cervix and uterus was deemed feasible from an oncological point of view and desirable for her age. The choice of reconstruction was limited by the need to keep the pelvis clear for subsequent radiotherapy. The ovaries were transposed out of the pelvis and marked with metal clips to enable future identification. Access to the vesical pedicles and bladder was achieved through 2 windows on both sides; the first between the medial umbilical ligament, round ligament and the pelvic side wall, and the second through the broad ligament inferior to the round ligament. Vaginal preservation was achieved through an incision made superiorly to the cervix with the plane of dissection between bladder and vagina guided by a vaginal swabstick. Finally, an omental flap was developed and attached to the pelvic side wall, the round ligament and to the superior surface of the uterus to prevent small bowel falling into the radiation field postoperatively. She had an uneventful recovery and was discharged on postoperative day 4. The specimen confirmed a 10-mm MRT with invasion through muscle into fat, clear surgical margins and all 17 lymph nodes were negative (pT3aN0MO).Three weeks following surgery, she started a combination of radiotherapy and a total of 9 cycles of high-dose alkylating chemotherapy: vincristine, cyclophosphamide, actinomycin D, ifosfamide, carboplatin and etopside. MRI scans of the...

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Malignant Extrarenal Rhabdoid Tumor of the Bladder: 9-Year Survival After Chemotherapy and Partial Cystectomy

Cited by 19 publications

References 2 publications

Continuous remission in an infant with chest wall malignant rhabdoid tumor after relapse

Continuous remission in an infant with chest wall malignant rhabdoid tumor after relapse

Molecularly confirmed primary malignant rhabdoid tumor of the urinary bladder: implications of accurate diagnosis

Pure malignant rhabdoid tumour of the bladder

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