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Malignant Rhabdoid Tlimor of Brain: An Aggressive Clinical Entity

Published online by Cambridge University Press:  18 September 2015

B.J. Fisher ,
J. Siddiqui ,
D. MacDonald ,
A.E. Cairney ,
D. Ramsey ,
D. Munoz  and
R. Del Maestro
B.J. Fisher*
Affiliation:
Department of Radiation Oncology, London Regional Cancer Centre, London
J. Siddiqui
Affiliation:
Department of Neurosurgery, London Health Sciences Centre, London
D. MacDonald
Affiliation:
Department of Neurology, London Health Sciences Centre, London
A.E. Cairney
Affiliation:
Department of Paediatrics, London Health Sciences Centre, London
D. Ramsey
Affiliation:
Department of Pathology, London Health Sciences Centre, London
D. Munoz
Affiliation:
Department of Pathology, London Health Sciences Centre, London
R. Del Maestro
Affiliation:
Department of Neurosurgery, London Health Sciences Centre, London
*
London Regional Cancer Centre, 790 Commissioners Road East, London, Ontario, Canada N6A 4L6
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Abstract

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Objective: We report three patients with malignant rhabdoid tumor (MRT) of the brain, two children and an adult. There were three purposes to this report: to describe the clinical course in an adult with MRT; to describe the interesting histopathological metamorphosis of one of the tumors; and to report the outcome of the treatment regimens we used in order to help guide future treatment. Since these tumors are quite rare it is important to continue to try new regimens in the search for effective therapy rather than to repeat ineffective ones. Method: Report of three patients. Results: The clinical course in all three patients was typical of these aggressive neoplasms in that chemotherapy and radiotherapy were ineffective in modifying the rapid deterioration leading to death. Conclusions: MRT can occur in adults. Autopsy in one patient showed that the tumor seemed to undergo an evolution in appearance when compared with the original pathology specimen from craniotomy. Administration of systemic therapy should be prompt and include intrathecal chemotherapy.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 23 , Issue 4 , November 1996 , pp. 257 - 263
Copyright
Copyright © Canadian Neurological Sciences Federation 1996

References

1.Briner, J, Bannwart, F, Kleihues, P, et al. Malignant small cell tumor of the brain with intermediate filaments – a case of a primary cerebral rhabdoid tumor. Paediatr Pathol 1985; 3 (abst): 117–118.Google Scholar
2.Sotelo-Avila, C, Gonzales-Crussi, F, deMello, D, et al. Renal and extrarenal rhabdoid tumors in children: a clinicopathologic study of 14 patients. Semin Diagn Pathol 1986; 3: 151–163.Google ScholarPubMed
3.Kapur, S, Patterson, K.Primary rhabdoid tumor of the cerebellum. Paediatr Pathol 1986; 5: 110.Google Scholar
4.Biggs, PJ, Garen, PD, Powers, , Garvin, AJ.Malignant rhabdoid tumor of the central nervous system. Hum Pathol 1987; 18: 332–337.CrossRefGoogle ScholarPubMed
5.Jakate, SM, Marsden, HB, Ingram, L.Primary malignant rhabdoid tumor of the brain: CT characteristics. Virchows Arch (A) 1988; 412: 393–397.CrossRefGoogle Scholar
6.Ho, PSP, Lee, WH, Chen, CY, et al. Primary malignant rhabdoid tumor of the brain: CT characteristics. J Comput Assist Tomogr 1990; 14: 461–463.CrossRefGoogle ScholarPubMed
7.Chou, SM, Anderson, JS.Primary CNS malignant rhabdoid tumor (MRT): report of two cases and review of literature. Clin Neuropathol 1991; 10: 1–10.Google ScholarPubMed
8.Agranovich, AL, Ang, LC, Griebel, RW, et al. Malignant rhabdoid tumor of the central nervous system with subarachnoid dissemination. Surg Neurol 1992; 37: 410–414.CrossRefGoogle ScholarPubMed
9.Hanna, SL, Langston, JW, Parham, DM, Douglas, EC.Primary malignant rhabdoid tumor of the brain: clinical, imaging, and pathologic findings. Am J Neuroradiol 1993; 14: 107–115.Google ScholarPubMed
10.Velasco, NM, Brown, JA, Kini, J, Ruppert, ES.Primary congenital rhabdoid tumor of the brain with neoplastic hydranencephaly. Child’s Nerv Syst 1993; 9: 185–190.CrossRefGoogle ScholarPubMed
11.Muller, M, Hubbard, SL, Provias, J, et al. Malignant rhabdoid tumor of the pineal region. Can J Neurol Sci 1994; 21: 273–277.CrossRefGoogle Scholar
12.Weeks, DA, Malott, RL, Zuppan, CW, Liwnicz, BH, Beckwith, JB.Primitive cerebral tumor with rhabdoid feature: a case of phenotypic rhabdoid tumor of the central nervous system. Ultrastruct Pathol 1994; 18: 23–28.CrossRefGoogle ScholarPubMed
13.Perilongo, G, Sutton, K, Czaykowski, D, Gusnard, D, Biegel, J.Rhabdoid tumor of the central nervous system. Med Pediatr Oncol 1991; 19: 310–317.CrossRefGoogle ScholarPubMed
14.Cossu, A, Massarelli, G, Manetto, V, et al. Rhabdoid tumors of the central nervous system: report of three cases with immunocyto-chemical and ultrastructural findings. Virchows Archiv A Pathol Anat 1993; 422: 81–85.CrossRefGoogle Scholar
15.Satoh, H, Gioshi, J, Sogabe, T, et al. Primary malignant rhabdoid tumor of the central nervous system case report and review of the literature. Surg Neurol 1993; 40: 429–430.CrossRefGoogle ScholarPubMed
16.Bouffet, E, Frappaz, D, Dolbeau, D, et al. Successful treatment for metastatic supratentorial malignant rhabdoid tumor. J Neurooncol 1993; 17: 65–70.CrossRefGoogle ScholarPubMed
17.Horn, M, Schlote, W, Lerch, K, et al. Malignant rhabdoid tumor: primary intracranial manifestation in an adult. Acta Neuropathol 1992; 83: 445–448.CrossRefGoogle ScholarPubMed
18.Olson, T, Bayar, E, Kosnik, M, et al. Successful treatment of disseminated central nervous system malignant rhabdoid tumor. J Ped Hematol Oncol 1995; 17: 71–77.CrossRefGoogle ScholarPubMed
19.Gonzalez-Cruss, F, Goldschmidt, R, Hseuh, W, et al. Infantile sarcoma with intracytoplasmic filamentous inclusions: distinctive tumor of possible histiocytic origin. Cancer 1981; 49: 2365.3.0.CO;2-I>CrossRefGoogle Scholar
20.Beckwith, JB, Palmer, NF.Histopathology and prognosis of Wilms’ tumor. Cancer 1978; 41: 1937–1948.3.0.CO;2-U>CrossRefGoogle ScholarPubMed
21.Tsuneyoshi, M, Daimaru, T, Hashimoto, H.Malignant soft tissue neoplasms with the histologic features of renal rhabdoid tumors: an ultrastructural and immunohistochemical study. Human Pathology 1985; 16: 1235.CrossRefGoogle ScholarPubMed
22.Parham, DM, Peiper, SC, Rabincheaux, G.Malignant rhabdoid tumor of the liver: evidence for epithelial differentiation. Arch Pathol Lab Med 1988; 112: 61–64.Google ScholarPubMed
23.Lynch, HT, Skurin, SB, Dahms, BB.Paravertebral malignant rhabdoid tumor in infancy: in vitro studies of a familial tumor. Cancer 1983; 52: 290–296.3.0.CO;2-L>CrossRefGoogle ScholarPubMed
24.Tsokos, M, Gregory, K, Ramo, SC.Malignant rhabdoid tumor of the kidney and soft tissues. Arch Pathol Lab Med 1989; 113: 115–120.Google ScholarPubMed
25.Haas, J, Palmer, N, Weinberg, A, Beckwith, J.Ultrastructure of malignant rhabdoid tumor of the kidney. Hum Pathol 1983; 12: 481–492.Google Scholar
26.Molenaar, WM, Jansson, DS, Gould, VE, et al. Molecular markers of primitive neuroectodermal tumors and other central nervous system tumors. Lab Invest 1989; 61: 635–643.Google Scholar
27.Ota, S, Crebbe, DC, Tran, TN, Traich, TJ, Shimada, H.Malignant rhabdoid tumor. A study of two established cell lines. Cancer 1993; 71: 2862–2872.3.0.CO;2-D>CrossRefGoogle ScholarPubMed
28.Bonnin, JM, Rubenstein, LT, Palmer, NF, Beckwirth, JB.The association of embryonal tumors originating in the kidney and brain: a report of seven cases. Cancer 1984; 54: 2137–2146.3.0.CO;2-D>CrossRefGoogle Scholar
29.Biegel, JA, Rorke, LB, Packer, RJ, Emanuel, BS.Monosomy 22 in rhabdoid or atypical tumors of the brain. J Neurosurg 1990; 73: 710–714.CrossRefGoogle ScholarPubMed
30.Dowdy, SF, Fasching, CL, Araujo, D, et al. Suppression of tumorgeneicity in Wilms’ tumor by the p15.5-p14 region of chromosome 11. Science 1991; 254–293.CrossRefGoogle Scholar
31.Garvin, AJ, Regg, , Tamowski, BI, Hazen-Martin, DJ, Sens, DA.The G401 cell line, utilized for studies of chromosomal changes in Wilms’ tumor, is derived from a rhabdoid tumor of the kidney. Am J Pathol 1993; 142: 375–380.Google ScholarPubMed
32.Bouffet, E, Franpez, D, Dalbeau, D, et al. Successful treatment for a metastatic supratentorial malignant rhabdoid tumor. J Neurooncol 1993; 17: 65–70.CrossRefGoogle ScholarPubMed