Age, stage, and radiotherapy, but not primary tumor site, affects the outcome of patients with malignant rhabdoid tumors

Sultan, Iyad; Qaddoumi, Ibrahim; Rodríguez‐Galindo, Carlos; Nassan, Anwar Al; Ghandour, Khalil; Al-Hussaini, Maysa

doi:10.1002/pbc.22285

Cited by 107 publications

(127 citation statements)

References 19 publications

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“…At 6 months a PET scan was performed and the patient had no evidence of disease. [5,6,[10][11][12][13][14][15][16][17][18][19][20][21]. In a review of the English literature, 14 cases of primary MRT involving the soft tissues of the head and neck were identified [11][12][13][14][15][16][17][18][19][20][21] (Table 1).…”

Section: Case Reportmentioning

confidence: 99%

“…One study by Sultan et al [10] found that radiation therapy, age at diagnosis (with children under 2 showing the worst prognosis) and tumor stage to be the most significant prognostic factors. Reports of overall survival rates of patients with MRT range from 20 to 33 % [5,10]. Distant metastasis has been reported and is more common in renal MRT [5,10].…”

Section: Case Reportmentioning

confidence: 99%

“…Reports of overall survival rates of patients with MRT range from 20 to 33 % [5,10]. Distant metastasis has been reported and is more common in renal MRT [5,10]. Interestingly, increased survival rates have been noted in children who received radiation therapy following resection of their tumor [5,10].…”

Section: Case Reportmentioning

confidence: 99%

“…Distant metastasis has been reported and is more common in renal MRT [5,10]. Interestingly, increased survival rates have been noted in children who received radiation therapy following resection of their tumor [5,10]. However, very few cases of MRT occurring in adults have been reported in the literature.…”

Section: Case Reportmentioning

confidence: 99%

“…These sites include the mediastinum, retroperitoneum, leg, bladder, liver, lung, abdomen, chest, foot, pelvis, parotid gland, central nervous system, parapharyngeal space, tongue, and gingiva [5,6,[10][11][12][13]. Tumors exhibiting rhabdoid differentiation act in an aggressive fashion and are correlated with a poor prognosis [5,6,12,13].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Malignant Rhabdoid Tumor of the Floor of Mouth: First Reported Case in the Oral Cavity of an Adult

Wetzel

Kerpel

Reich

et al. 2014

Head and Neck Pathol

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Malignant rhabdoid tumors (MRTs) are exceedingly rare lesions. To our knowledge, only 2 cases have been reported in the oral cavity, with both examples occurring in infants. The current case is the third reported case of MRT of the oral cavity and the first reported case to occur in an adult at this location. The following report describes the clinical, histologic and immunohistochemical features of this tumor.

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Section: Case Reportmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Malignant Rhabdoid Tumor of the Floor of Mouth: First Reported Case in the Oral Cavity of an Adult

Wetzel

Kerpel

Reich

et al. 2014

Head and Neck Pathol

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Recent insights into the SWI/SNF complex and the molecular mechanism of hSNF5 deficiency in rhabdoid tumors

et al. 2023

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Genetic information encoded by DNA is packaged in the nucleus using the chromatin structure. The accessibility of transcriptional elements in DNA is controlled by the dynamic structural changes of chromatin for the appropriate regulation of gene transcription. Chromatin structure is regulated by two general mechanisms, one is histone modification and the other is chromatin remodeling in an ATP‐dependent manner. Switch/sucrose nonfermentable (SWI/SNF) complexes utilize the energy from ATP hydrolysis to mobilize nucleosomes and remodel the chromatin structure, contributing to conformational changes in chromatin. Recently, the inactivation of encoding genes for subunits of the SWI/SNF complexes has been documented in a series of human cancers, accounting for up to almost 20% of all human cancers. For example, human SNF5 (hSNF5), the gene that encodes a subunit of the SWI/SNF complexes, is the sole mutation target that drives malignant rhabdoid tumors (MRT). Despite remarkably simple genomes, the MRT has highly malignant characteristics. As a key to understanding MRT tumorigenesis, it is necessary to fully examine the mechanism of chromatin remodeling by the SWI/SNF complexes. Herein, we review the current understanding of chromatin remodeling by focusing on SWI/SNF complexes. In addition, we describe the molecular mechanisms and influences of hSNF5 deficiency in rhabdoid tumors and the prospects for developing new therapeutic targets to overcome the epigenetic drive of cancer that is caused by abnormal chromatin remodeling.

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Improved 6‐year overall survival in AT/RT – results of the registry study Rhabdoid 2007

et al. 2016

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Atypical teratoid rhabdoid tumors (AT/RT) are characterized by mutations and subsequent inactivation of SMARCB1 (INI1, hSNF5), a predilection for very young children and an unfavorable outcome. The European Registry for rhabdoid tumors (EU-RHAB) was established to generate a common European database and to establish a standardized treatment regimen as the basis for phase I/II trials. Thus, genetic analyses, neuropathologic and radiologic diagnoses, and a consensus treatment regimen were prospectively evaluated. From 2005 to 2009, 31 patients with AT/RT from four countries were recruited into the registry study Rhabdoid 2007 and treated with systemic and intraventricular chemotherapy. Eight patients received high-dose chemotherapy, 23 radiotherapy, and 17 maintenance therapy. Reference evaluations were performed in 64% (genetic analyses, FISH, MLPA, sequencing) up to 97% (neuropathology, INI1 stain). Germ-line mutations (GLM) were detected in 6/21 patients. Prolonged overall survival was associated with age above 3 years, radiotherapy and achievement of a complete remission. 6-year overall and event-free survival rates were 46% (±0.10) and 45% (±0.09), respectively. Serious adverse events and one treatmentrelated death due to insufficiency of a ventriculo peritoneal shunt (VP-shunt) and consecutive herniation were noted. Acquisition of standardized data including reference diagnosis and a standard treatment schedule improved data quality Cancer MedicineOpen Access 1766

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Age, stage, and radiotherapy, but not primary tumor site, affects the outcome of patients with malignant rhabdoid tumors

Abstract: Our population-based study indicates that age at diagnosis, tumor stage, and use of radiotherapy favorably impact survival rates of patients with MRTs.

Cited by 107 publications

References 19 publications

Malignant Rhabdoid Tumor of the Floor of Mouth: First Reported Case in the Oral Cavity of an Adult

Malignant Rhabdoid Tumor of the Floor of Mouth: First Reported Case in the Oral Cavity of an Adult

Recent insights into the SWI/SNF complex and the molecular mechanism of hSNF5 deficiency in rhabdoid tumors

Improved 6‐year overall survival in AT/RT – results of the registry study Rhabdoid 2007

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