Rhabdoid Tumor (Rhabdoid Tumors)

A rare but highly lethal childhood tumor found almost exclusively in infants. Histopathologically, it resembles RHABDOMYOSARCOMA but the tumor cells are not of myogenic origin. Although it arises primarily in the kidney, it may be found in other parts of the body. The rhabdoid cytomorphology is believed to be the expression of a very primitive malignant cell. (From Holland et al., Cancer Medicine, 3d ed, p2210)

Also Known As:

Networked: 293 relevant articles (11 outcomes, 26 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Wilms Tumor (Wilm's Tumor)
3.	Neoplasm Metastasis (Metastasis)
4.	Medulloblastoma
5.	Renal Cell Carcinoma (Grawitz Tumor)

Experts

1.	Biegel, Jaclyn A: 13 articles (02/2015 - 01/2002)
2.	Frühwald, Michael C: 10 articles (09/2014 - 04/2007)
3.	Hasselblatt, Martin: 9 articles (09/2014 - 10/2006)
4.	Kalpana, Ganjam V: 9 articles (03/2013 - 01/2002)
5.	Leuschner, Ivo: 6 articles (09/2014 - 02/2010)
6.	Schneppenheim, Reinhard: 6 articles (09/2014 - 02/2010)
7.	Judkins, Alexander R: 6 articles (10/2011 - 05/2005)
8.	Houghton, Peter J: 6 articles (09/2011 - 05/2008)
9.	Kolb, E Anders: 6 articles (07/2010 - 05/2008)
10.	Reynolds, C Patrick: 6 articles (07/2010 - 05/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Rhabdoid Tumor:

1.	Doxorubicin (Adriamycin)FDA LinkGeneric 09/01/2014 - "Malignant rhabdoid tumor of the kidney: significantly improved response to pre-operative treatment intensified with doxorubicin." 01/01/1981 - "Rhabdoid tumor of the kidney: complete remission induced by cis-platinum and adriamycin." 01/01/2013 - "The histone deacetylase inhibitor SAHA acts in synergism with fenretinide and doxorubicin to control growth of rhabdoid tumor cells." 07/01/2015 - "Assuming an undifferentiated esophageal adenocarcinoma a palliative chemotherapy with 5-FU/folinic acid, oxaliplatin, and docetaxel (FLOT) was initiated which was changed towards a combination of doxorubicin and ifosphamide as immunohistochemistry of the primary and the liver metastases revealed a rhabdoid tumor. " 02/01/2002 - "We evaluated the in vitro sensitivity of four malignant rhabdoid tumor (MRT) cell lines to six chemotherapeutic agents: 5-fluororuacil, vincristine, carboplatin, doxorubicin, etoposide, and paclitaxel. "
2.	Cisplatin (Platino)FDA LinkGeneric 01/30/2015 - "Cisplatin-selected resistance is associated with increased motility and stem-like properties via activation of STAT3/Snail axis in atypical teratoid/rhabdoid tumor cells." 05/01/2009 - "There were eight patients, including four with medulloblastoma (MB), three with atypical teratoid/rhabdoid tumor (AT/RT) and one with supratentorial primitive neuroectodermal tumor, whose tumors recurred after surgery and radiotherapy, with or without conventional intravenous cisplatin-based chemotherapy. " 05/01/2008 - "Cisplatin was evaluated against 23 cell lines, and 40 xenografts representing brain tumors, neuroblastoma, rhabdoid tumors, sarcoma, Wilms tumor, and acute lymphoblastic leukemia (ALL). " 01/01/1981 - "Rhabdoid tumor of the kidney: complete remission induced by cis-platinum and adriamycin." 01/01/2014 - "Preclinical high-dose acetaminophen with N-acetylcysteine rescue enhances the efficacy of cisplatin chemotherapy in atypical teratoid rhabdoid tumors."
3.	Histone Deacetylase InhibitorsIBA 01/01/2009 - "Imprinted CDKN1C is a tumor suppressor in rhabdoid tumor and activated by restoration of SMARCB1 and histone deacetylase inhibitors." 01/01/2009 - "Induction of autophagy in malignant rhabdoid tumor cells by the histone deacetylase inhibitor FK228 through AIF translocation." 01/01/2012 - "In vivo efficacy of the histone deacetylase inhibitor suberoylanilide hydroxamic acid in combination with radiotherapy in a malignant rhabdoid tumor mouse model." 02/01/2012 - "The aim of this study was to investigate the effect of the combination therapy with the histone deacetylase inhibitor suberoylanilide hydroxamic acid (SAHA) and irradiation on atypical teratoid rhabdoid tumors and osteosarcoma compared to normal tissue cell lines. " 01/01/2013 - "The histone deacetylase inhibitor SAHA acts in synergism with fenretinide and doxorubicin to control growth of rhabdoid tumor cells."
4.	Biological Markers (Surrogate Marker)IBA 10/01/2015 - "JWiTS-1 (1996-2005) was analyzed, and JWiTS-2 (2005-2014) is now under analysis; the following problems have been identified and used to decide future study protocol: (i) there has been a decline in survival rate for patients with rhabdoid tumor of the kidney (RTK) and new treatment strategies are required; (ii) the survival rate for bilateral Wilms tumors (BWT) has improved, but results for renal preservation are unsatisfactory; (iii) the prognosis of stage IV favorable nephroblastoma is very good, suggesting that the current protocols provide overtreatment, particularly for patients with lung metastasis; and (iv) no effective biological risk factors exist for predicting the outcome of Wilms tumor, and a study of the genetic changes of these tumors is necessary to determine biological markers for use in risk classification. " 06/01/2005 - "To further distinguish the underlying pathogenesis and to identify biological markers for clinical use, an atypical teratoid/rhabdoid tumor-derived cell line was established and its gene expression pattern analyzed in comparison to the human astrocyte SVG12 cell line and the human DAOY medulloblastoma cell line using a complementary DNA microarray method. " 01/01/2014 - "This review debates the importance of miRNAs in the biology of central nervous systemembryonal tumors focusing on medulloblastoma and atypical teratoid/rhabdoid tumors and highlights the advantages as well as the limitations of their prospective application as biomarkers and candidates for molecular therapeutic targets. "
5.	vorinostat (suberoylanilide hydroxamic acid)FDA Link 01/01/2012 - "We explored the effect as well as the radiosensitizing properties of suberoylanilide hydroxamic acid (SAHA) in vivo in a malignant rhabdoid tumor (MRT) mouse model. " 02/01/2012 - "Suberoylanilide hydroxamic acid affects γH2AX expression in osteosarcoma, atypical teratoid rhabdoid tumor and normal tissue cell lines after irradiation." 01/01/2012 - "In vivo efficacy of the histone deacetylase inhibitor suberoylanilide hydroxamic acid in combination with radiotherapy in a malignant rhabdoid tumor mouse model." 02/01/2012 - "The aim of this study was to investigate the effect of the combination therapy with the histone deacetylase inhibitor suberoylanilide hydroxamic acid (SAHA) and irradiation on atypical teratoid rhabdoid tumors and osteosarcoma compared to normal tissue cell lines. " 04/15/2007 - "The authors evaluated the cytotoxicity of histone deacetylase inhibitors (HDI) [MS-275, SAHA, TSA, M344, M360, D85, SW55, SW187 and valproic acid (VPA)] on 13 embryonal tumor cell lines [4 medulloblastomas, 5 neuroblastomas, 2 atypical teratoid/rhabdoid tumors (AT/RT), and 2 malignant rhabdoid tumors of the kidney (RTK)] in MTT assay. "
6.	flavopiridolIBA 01/15/2008 - "Flavopiridol is effective in inducing cell cycle arrest and cytotoxicity in rhabdoid tumors. " 01/01/2010 - "Potent inhibition of rhabdoid tumor cells by combination of flavopiridol and 4OH-tamoxifen." 01/15/2008 - "Flavopiridol is potentially a novel chemotherapeutic agent for rhabdoid tumors." 01/15/2008 - "Because cdk inhibitors have not been used for preclinical or clinical testing on rhabdoid tumors, we investigated the effect of flavopiridol, a pan-cdk inhibitor with promising clinical activity, on rhabdoid tumors. " 01/15/2008 - "Rhabdoid tumor growth is inhibited by flavopiridol."
7.	Acetylcysteine (Siran)FDA LinkGeneric 01/01/2014 - "Preclinical high-dose acetaminophen with N-acetylcysteine rescue enhances the efficacy of cisplatin chemotherapy in atypical teratoid rhabdoid tumors."
8.	Acetaminophen (Paracetamol)FDA LinkGeneric 01/01/2014 - "Preclinical high-dose acetaminophen with N-acetylcysteine rescue enhances the efficacy of cisplatin chemotherapy in atypical teratoid rhabdoid tumors."
9.	MicroRNAs (MicroRNA)IBA 03/01/2014 - "The impact of let-7b miRNA on HMGA2 expression and the malignant potential of human rhabdoid tumor cell G401 (SMARCB1(-/-)) were investigated by antisense inhibition and ectopic overexpression studies. " 11/01/2013 - "Study of the gene expression and microRNA expression profiles of malignant rhabdoid tumors originated in the brain (AT/RT) and in the kidney (RTK)." 03/01/2014 - "Frequent overexpression of HMGA2 in human atypical teratoid/rhabdoid tumor and its correlation with let-7a3/let-7b miRNA." 12/01/2012 - "Amplification of the miRNA cluster at 19q13.42 has been identified previously as a genetic hallmark for ETMR, but an immunohistochemistry-based assay for clinical routine diagnostics [such as INI-1 for atypical teratoid rhabdoid tumor (AT/RT)] is still lacking. " 06/01/2014 - "In addition, when malignant rhabdoid tumor with SMARCB1 gene alterations and proximal type and conventional type epithelioid sarcoma with no SMARCB1 gene alterations were compared, 56 microRNAs were isolated as being significantly different (ANOVA, P<0.05). "
10.	DNA (Deoxyribonucleic Acid)IBA 06/01/2001 - "Genetic study showed a homozygous mutation in the tumoral DNA and a constitutional heterozygous mutation, as it was demonstrated in atypical teratoid rhabdoid tumors and in renal and extra-renal rhabdoid tumors." 11/01/1998 - "Malignant rhabdoid tumor of the vulva: case report with cytological, immunohistochemical, ultrastructural and DNA ploidy studies and a review of the literature." 01/01/2015 - "We report a case of atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) in an adult and its immunological phenotype and chromosomal DNA imbalance characteristics, as detected by comparative genomic hybridization (CGH). " 10/01/2002 - "Association between DNA content and tumor suppressor gene expression and aggressiveness of atypical teratoid/rhabdoid tumors." 01/01/2001 - "Mutations of the p53 gene in malignant rhabdoid tumors of soft tissue and the kidney: immunohistochemical and DNA direct sequencing analysis."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 09/01/2014 - "Despite the very young age of the patient, the initially desperate situation of RTPS1, and the synchronous localization of congenital rhabdoid tumors, intensive chemotherapy was well tolerated; the child is still in complete remission 5 years following diagnosis. " 07/01/2010 - "Atypical teratoid or rhabdoid tumors: improved outcome with high-dose chemotherapy." 03/01/2005 - "Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy." 04/01/2015 - "All children with malignant rhabdoid tumors should be treated initially with intensive chemotherapy." 01/01/2015 - "A 7-year-old girl with stage IIA extrarenal rhabdoid tumor near the left cubital fossa received preoperative chemotherapy and surgical resection with median nerve reconstruction followed by postoperative high-dose chemotherapy. "
2.	Radiotherapy 02/01/2014 - "Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. " 05/01/2014 - "Radiotherapy (RT) is a promising option in rhabdoid tumors but its application in infants remains controversial. " 09/01/2012 - "Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis." 03/15/2006 - "To assess outcomes and prognostic factors in radiotherapy of pediatric central nervous system atypical teratoid/rhabdoid tumor (AT/RT). " 03/15/2006 - "Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience)."
3.	Adjuvant Chemotherapy 08/01/2009 - "Improved survival in patients with breast rhabdoid tumors with multi-agent adjuvant chemotherapy combined with irradiation." 06/01/2009 - "The histological diagnoses of these tumors were astrocytoma, n = 3; pineocytoma, n = 2; teratoma, n = 1; ependymoma, n = 1; atypical teratoid/rhabdoid tumor, n = 1; glioblastoma, n = 1; medulloblastoma, n = 1; and choroid plexus papilloma, n = 1. Surgical resection was performed in eight patients, and adjuvant chemotherapy was administered to all except one patient with choroid plexus papilloma. "
4.	Nephrectomy 10/01/2009 - "This report describes a 4-month-old girl with malignant rhabdoid tumor of the kidney (MRTK), who developed CA after left nephrectomy without lymphadenectomy, and who was successfully treated conservatively with enteral therapy. " 09/01/2013 - "Nephrectomy specimen revealed clear cell carcinoma with sarcomatoid and rhabdoid tumor cells. " 10/01/2009 - "Chylous ascites after nephrectomy without lymphadenectomy for malignant rhabdoid tumor of the kidney: A rare occurrence and literature review." 01/01/2014 - "We selected a total of 204 nephrectomy cases in this study, including 163 cases of renal cell carcinoma; 37 cases of urothelial carcinoma; 4 cases from other categories (Wilms tumor, primary diffuse large B-cell lymphoma, angiolipoma, rhabdoid tumor). " 04/01/1999 - "At review of the nephrectomy slides, the diagnoses were: Wilms tumor (16 patients), with anaplasia in one case, rhabdoid tumor (2 patients), neuroblastoma (2 patients), mesoblastic nephroma (2 patients), clear cell sarcoma (1 patient), malignant teratoma (1 patient), and renal cell carcinoma (1 patient). "
5.	Transplants (Transplant) 01/01/2002 - "A detailed summary of the discussions regarding diagnostic studies and the roles for radiation therapy, chemotherapy, and bone marrow or stem cell transplant in the treatment of rhabdoid tumors will be published separately." 06/01/2008 - "Successful outcome with tandem myeloablative chemotherapy and autologous peripheral blood stem cell transplants in a patient with atypical teratoid/rhabdoid tumor of the central nervous system." 06/01/2003 - "High-dose chemotherapy followed by autologous stem cell transplant may be an effective front-line therapeutic approach for patients with metastatic rhabdoid tumor of the kidney."