Rhabdoid tumor: the Irish experience 1986–2013

Uwineza, Alice; Gill, Harinder; Buckley, Patrick G.; Cormac, Owens; Capra, Michael; O'Sullivan, Catriona; McDermott, Michael; Brett, Francesca; Farrell, Michael; Pears, Jane; O’Sullivan, Maureen

doi:10.1016/j.cancergen.2014.05.015

Cited by 25 publications

(20 citation statements)

References 15 publications

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“…Between 10–15% of patients with MRTs present with primary CNS disease known as atypical teratoid/rhabdoid tumors (AT/RT) [4, 5]. Although MRTs were initially described as arising from the kidney and have been well described in the CNS, other cases have been identified in various locations, including the liver, lung, and soft tissues [6, 7]. MRTs, regardless of the anatomic site, tend to present at a young age and have an extremely aggressive course with dismal overall survival rates estimated near 23% [5].…”

Section: Introductionmentioning

confidence: 99%

Prognostic factors and survival in non-central nervous system rhabdoid tumors

Farber

Shukla

Lim

et al. 2017

Journal of Pediatric Surgery

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Introduction Non-central nervous system (non-CNS) rhabdoid tumors tend to present at a young age and have an extremely aggressive course, with dismal overall survival rates. Inactivation of the tumor suppressor gene SMARCB1 has been shown in rhabdoid tumors regardless of anatomic location, suggesting a common genetic basis. We retrospectively analyzed our institutional experience with non-CNS rhabdoid tumors to determine overall survival and prognostic variables. Methods We reviewed records of pediatric patients (age <22y) with non-CNS rhabdoid tumor at our institution between 1980 and 2014. Variables evaluated for correlation with survival included: age > or <1.5 years (median) at diagnosis, M1 status, and radiation therapy. The log-rank test was used to compare Kaplan-Meier probability distributions with P values adjusted for multiple testing using the false discovery rate approach. Results Nineteen consecutive patients (10 female) with histologically verified rhabdoid tumor were identified. Mean age at diagnosis was 3.2 years (median 1.5y, range 1.3mo–21.8y). Primary tumors were located in the kidney (n=10), head and neck (n=5), and in the liver, thigh, mediastinum and retroperitoneum (n=1 each). SMARCB1 expression was absent in all 10 patients tested. Eight patients had distant metastases at diagnosis. Median overall survival was 1.2 years. Age greater than the median and radiation therapy were associated with better outcome, with a median overall survival of 2.7 years (P=0.049 and P=0.003, respectively). Conclusion Survival rates for rhabdoid tumor remain poor, but prognosis is better in older children, regardless of primary tumor location. Because of its rarity, clinical trials with present agents are difficult to conduct. Further progress will require a focus on therapies targeted at tumor biology rather than anatomic location for non-CNS rhabdoid tumors.

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Section: Introductionmentioning

confidence: 99%

Prognostic factors and survival in non-central nervous system rhabdoid tumors

Farber

Shukla

Lim

et al. 2017

Journal of Pediatric Surgery

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“…In a recent European study of 25 pediatric cases, no special benefit was associated with any single therapy. 67 Proton beam therapy for brain MRT (atypical teratoid/rhabdoid tumors) extended progression-free survival in a series of 31 patients. 44 With regard to orbital cases, there is no organ-specific standardized treatment except to use protocols derived from experience at non-ophthalmic sites.…”

Section: Malignant Rhabdoid Tumormentioning

confidence: 99%

Malignant rhabdoid transformation of a longstanding, aggressive, and recurrent orbital angiomyxoma

Callahan

Stagner

Lee

et al. 2015

Survey of Ophthalmology

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“…Единого подхода в терапии рабдоидных опухолей нет и проанализировать преимущества отдельных режимов ПХТ невозможно из-за малого числа па-циентов, что также наглядно отражено в представ-ленных клинических случаях [53][54][55][56][57][58][59][60][61][62][63][64][65][66][67][68][69][70]. Показано, что на выживаемость влияет использование высоких доз алкилирующих агентов, в том числе антрациклино-вых антибиотиков и актиномицина D [9,80].…”

Section: результаты лечения злокачественных рабдоидных опухолейunclassified

Malignant rhabdoid tumors of soft tissues in children. Literature review

Телешова¹

2017

Ross. ž. det. gematol. onkol.

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Rhabdoid tumor: the Irish experience 1986–2013

Cited by 25 publications

References 15 publications

Prognostic factors and survival in non-central nervous system rhabdoid tumors

Prognostic factors and survival in non-central nervous system rhabdoid tumors

Malignant rhabdoid transformation of a longstanding, aggressive, and recurrent orbital angiomyxoma

Malignant rhabdoid tumors of soft tissues in children. Literature review

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