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Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor

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Abstract

Atypical teratoid/rhabdoid tumors (AT/RT) are highly malignant lesions of childhood that carry a very poor prognosis. AT/RT can occur in the central nervous system (CNS AT/RT) and disease in this location carries an even worse prognosis with a median survival of 7 months. In spite of multiple treatment regimens consisting of maximal surgical resection (including second look surgery), radiation therapy (focal and craniospinal), and multi-agent intravenous, oral and intrathecal chemotherapy, with or without high-dose therapy and stem cell rescue, only seven long-term survivors of CNS AT/RT have been reported, all in patients with newly diagnosed disease. For this reason, many centers now direct such patients, particularly those under 5 years of age, or those with recurrent disease, towards comfort care rather than attempt curative therapy. We now report on four children, two with newly diagnosed CNS AT/RT and two with progressive disease after multi-agent chemotherapy who are long term survivors (median follow-up of 37 months) using a combination of surgery, radiation therapy, and intensive chemotherapy. The chemotherapy component was modified from the Intergroup Rhabdomyosarcoma Study Group (IRS III) parameningeal protocol as three of the seven reported survivors in the literature were treated using this type of therapy. Our four patients, when added to the three reported survivors in the literature using this approach, suggest that patients provided this aggressive therapy can significantly alter the course of their disease. More importantly, we report on the first two survivors after relapse with multi-agent intravenous and intrathecal chemotherapy treated with this modified regimen.

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References

  1. RJ Packer JA Biegel S Blaney J Finlay JR Geyer R Heideman J Hilden AJ Janss L Kun G Vezina LB Rorke M Smith (2002) ArticleTitleAtypical teratoid/rhabdoid tumor of the central nervous system: report on workshop J Pediatr Hematol Oncol 24 IssueID5 337–342

    Google Scholar 

  2. J Briner F Bannwart P Kleihues B Odermatt R Janzer U Willi E Boltshauser (1985) ArticleTitleMalignant small cell tumor of the brain with intermediate filaments – a case of a primary cerebral rhabdoid tumor Pediatr Pathol 3 117–118

    Google Scholar 

  3. IB Lefkowitz LB Rorke RJ Packer LN Sutton KR Siegel RJ Katnick (1987) ArticleTitleAtypical teratoid tumor of infancy: definition of an entity Ann Neurol 22 448–449

    Google Scholar 

  4. PC Burger IT Yu T Tihan HS Friedman DR Strother JL Kepner PK Duffner LE Kun EJ Perlman (1998) ArticleTitleAtypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study Am J Surg Pathol 22 IssueID9 1083–1092

    Google Scholar 

  5. LB Rorke RJ Packer JA Biegel (1996) ArticleTitleCentral nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity J Neurosurg 85 IssueID1 56–65

    Google Scholar 

  6. JM Hilden J Watterson DC Longee CL Moertel ME Dunn J Kurtzberg BW Scheithauer (1998) ArticleTitleCentral nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature J Neurooncol 40 IssueID3 265–275

    Google Scholar 

  7. E Weiss B Behring J Behnke HJ Christen A Pekrun CF Hess (1998) ArticleTitleTreatment of primary malignant rhabdoid tumor of the brain: report of three cases and review of the literature [see comments] Int J Radiat Oncol Biol Phys 41 IssueID5 1013–1019

    Google Scholar 

  8. M Weinblatt J Kochen (1992) ArticleTitleRhabdoid tumor of the central nervous system Med Pediatr Oncol 20 IssueID3 258

    Google Scholar 

  9. TA Olson E Bayar E Kosnik AB Hamoudi KJ Klopfenstein RS Pieters FB Ruymann (1995) ArticleTitleSuccessful treatment of disseminated central nervous system malignant rhabdoid tumor J Pediatr Hematol Oncol 17 IssueID1 71–75

    Google Scholar 

  10. YS Yim DH Mahoney SuffixJr. DG Oshman (1991) ArticleTitleHemiparesis and ischemic changes of the white matter after intrathecal therapy for children with acute lymphocytic leukemia Cancer 67 IssueID8 2058–2061

    Google Scholar 

  11. K Phanthumchinda T Intragumtornchai V Kasantikul (1991) ArticleTitleStroke-like syndrome, mineralizing microangiopathy, and neuroaxonal dystrophy following intrathecal methotrexate therapy Neurology 41 IssueID11 1847–1848

    Google Scholar 

  12. S Kastenbauer M Wiesmann HW Pfister (2000) ArticleTitleCerebral vasculopathy and multiple infarctions in a woman with carcinomatous meningitis while on treatment with intrathecal methotrexate J Neurooncol 48 IssueID1 41–45

    Google Scholar 

  13. S Constantini F Umansky R Nesher M Shalit (1987) ArticleTitleTransient blindness following intracranial pressure changes in a hydrocephalic child with a V-P shunt Childs Nerv Syst 3 IssueID6 379–381

    Google Scholar 

  14. NJ Newman (1994) ArticleTitleBilateral visual loss and disc edema in a 15-year-old girl Surv Ophthalmol 38 IssueID4 365–370

    Google Scholar 

  15. AG Kulkarni AP Amte NS Brid SR Yadav (1998) ArticleTitleBilateral optic atrophy with hydrocephalus Postgrad Med J 74 IssueID872 369–370

    Google Scholar 

  16. DM Albert VG Wong ES Henderson (1967) ArticleTitleOcular complications of vincristine therapy Arch Ophthalmol 78 IssueID6 709–713

    Google Scholar 

  17. RL Byrd TM Rohrbaugh RB Raney SuffixJr. DG Norris (1981) ArticleTitleTransient cortical blindness secondary to vincristine therapy in childhood malignancies Cancer 47 IssueID1 37–40

    Google Scholar 

  18. LB Rorke R Packer J Biegel (1995) ArticleTitleCentral nervous system atypical teratoid/rhabdoid tumors of infancy and childhood J Neurooncol 24 IssueID1 21–28

    Google Scholar 

  19. J Lutterbach J Liegibel D Koch A Madlinger H Frommhold A Pagenstecher (2001) ArticleTitleAtypical teratoid/rhabdoid tumors in adult patients: case report and review of the literature J Neurooncol 52 IssueID1 49–56

    Google Scholar 

  20. JA Biegel B Fogelgren LM Wainwright JY Zhou H Bevan LB Rorke (2000) ArticleTitleGermline INI1 mutation in a patient with a central nervous system atypical teratoid tumor and renal rhabdoid tumor Genes Chromosomes Cancer 28 IssueID1 31–37 Occurrence Handle1:STN:280:DC%2BD3c3gsVWnsw%3D%3D Occurrence Handle10738300

    CAS  PubMed  Google Scholar 

  21. CW Roberts SA Galusha ME McMenamin CD Fletcher SH Orkin (2000) ArticleTitleHaploinsufficiency of Snf5 (integrase interactor 1) predisposes to malignant rhabdoid tumors in mice Proc Natl Acad Sci USA 97 IssueID25 13796–13800

    Google Scholar 

  22. AL Agranovich LC Ang RW Griebel NL Kobrinsky N Lowry SP Tchang (1992) ArticleTitleMalignant rhabdoid tumor of the central nervous system with subarachnoid dissemination Surg Neurol 37 IssueID5 410–414

    Google Scholar 

  23. J Arrazola I Pedrosa R Mendez C Saldana BW Scheithauer A Martinez (2000) ArticleTitlePrimary malignant rhabdoid tumour of the brain in an adult Neuroradiology 42 IssueID5 363–367

    Google Scholar 

  24. B Behring W Bruck HH Goebel J Behnke A Pekrun HJ Christen HA Kretzschmar (1996) ArticleTitleImmunohistochemistry of primary central nervous system malignant rhabdoid tumors: report of five cases and review of the literature Acta Neuropathol (Berl) 91 IssueID6 578–586

    Google Scholar 

  25. JA Biegel LB Rorke RJ Packer BS Emanuel (1990) ArticleTitleMonosomy 22 in rhabdoid or atypical tumors of the brain J Neurosurg 73 IssueID5 710–714

    Google Scholar 

  26. PJ Biggs PD Garen JM Powers AJ Garvin (1987) ArticleTitleMalignant rhabdoid tumor of the central nervous system Hum Pathol 18 IssueID4 332–337

    Google Scholar 

  27. KS Caldemeyer RR Smith B Azzarelli JC Boaz (1994) ArticleTitlePrimary central nervous system malignant rhabdoid tumor: CT and MR appearance simulates a primitive neuroectodermal tumor Pediatr Neurosurg 21 IssueID4 232–236

    Google Scholar 

  28. CH Chang N Ramirez WA Sakr (1989) ArticleTitlePrimitive neuroectodermal tumor of the brain associated with malignant rhabdoid tumor of the liver: a histologic, immunohistochemical, and electron microscopic study Pediatr Pathol 9 IssueID3 307–319

    Google Scholar 

  29. YW Chi WH Lee RC Hwang HB Liao SN Cheng ML Chu TY Chou PS Ho (1989) ArticleTitleIntracranial malignant rhabdoid tumor: report of one case Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi 30 IssueID5 316–322

    Google Scholar 

  30. SM Chou JS Anderson (1991) ArticleTitlePrimary CNS malignant rhabdoid tumor (MRT): report of two cases and review of literature Clin Neuropathol 10 IssueID1 1–10

    Google Scholar 

  31. RD Cohn Y Frank AE Stanek P Kalina (1995) ArticleTitleMalignant rhabdoid tumor of the brain and kidney in a child: clinical and pathologic features Pediatr Neurol 13 IssueID1 65–68

    Google Scholar 

  32. A Cossu G Massarelli V Manetto G Viale F Tanda L Bosincu P Iuzzolino S Cossu R Padovani V Eusebi (1993) ArticleTitleRhabdoid tumours of the central nervous system. Report of three cases with immunocytochemical and ultrastructural findings Virchows Arch A Pathol Anat Histopathol 422 IssueID1 81–85

    Google Scholar 

  33. YN Chung KC Wang SH Shin N Kim JG Chi KS Min BK Cho (2002) ArticleTitlePrimary intracranial atypical teratoid/rhabdoid tumor in a child: a case report J Korean Med Sci 17 IssueID5 723–726

    Google Scholar 

  34. T Dang M Vassilyadi J Michaud C Jimenez EC Ventureyra (2003) ArticleTitleAtypical teratoid/rhabdoid tumors Childs Nerv Syst 19 IssueID4 244–248

    Google Scholar 

  35. P Mot ParticleDe P Demaerel G Wilms S Gool ParticleVan R Sciot (2003) ArticleTitleImaging features of multifocal primary rhabdoid tumour of the central nervous system with meningeal and spinal metastases Pediatr Radiol 33 IssueID4 275–277

    Google Scholar 

  36. E Guler A Varan F Soylemezoglu Kudret Caglar F Ba A Demirkazik M Buyyukpamuk (2001) ArticleTitleExtraneural metastasis in a child with atypical teratoid rhabdoid tumor of the central nervous system J Neurooncol 54 IssueID1 53–56

    Google Scholar 

  37. SL Hanna JW Langston DM Parham EC Douglass (1993) ArticleTitlePrimary malignant rhabdoid tumor of the brain: clinical, imaging, and pathologic findings AJNR Am J Neuroradiol 14 IssueID1 107–115

    Google Scholar 

  38. PS Ho WH Lee CY Chen TY Chou YC Wang MJ Sun WY Liou (1990) ArticleTitlePrimary malignant rhabdoid tumor of the brain: CT characteristics J Comput Assist Tomogr 14 IssueID3 461–463

    Google Scholar 

  39. M Horn W Schlote KD Lerch WI Steudel D Harms E Thomas (1992) ArticleTitleMalignant rhabdoid tumor: primary intracranial manifestation in an adult Acta Neuropathol (Berl) 83 IssueID4 445–448

    Google Scholar 

  40. RP Hasserjian RD Folkerth RM Scott DE Schofield (1995) ArticleTitleClinicopathologic and cytogenetic analysis of malignant rhabdoid tumor of the central nervous system J Neurooncol 25 IssueID3 193–203

    Google Scholar 

  41. E Izycka-Swieszewska M Debiec-Rychter B Wasag A Wozniak D Gasecki K Plata-Nazar J Bartkowiak J Lasota J Limon (2003) ArticleTitleA unique occurrence of a cerebral atypical teratoid/rhabdoid tumor in an infant and a spinal canal primitive neuroectodermal tumor in her father J Neurooncol 61 IssueID3 219–225

    Google Scholar 

  42. SM Jakate HB Marsden L Ingram (1988) ArticleTitlePrimary rhabdoid tumour of the brain Virchows Arch A Pathol Anat Histopathol 412 IssueID4 393–397

    Google Scholar 

  43. DN Korones SP Meyers A Rubio C Torres LS Constine (1999) ArticleTitleA 4-year-old girl with a ventriculoperitoneal shunt metastasis of a central nervous system atypical teratoid/rhabdoid tumor Med Pediatr Oncol 32 IssueID5 389–391

    Google Scholar 

  44. DT Manhoff LB Rorke AT Yachnis (1995) ArticleTitlePrimary intracranial atypical teratoid/rhabdoid tumor in a child with Canavan disease Pediatr Neurosurg 22 IssueID4 214–222

    Google Scholar 

  45. JF Martínez Lage A Nieto J Sola R Domingo TR Costa M Poza (1997) ArticleTitlePrimary malignant rhabdoid tumor of the cerebellum Childs Nerv Syst 13 IssueID7 418–421

    Google Scholar 

  46. M Muller SL Hubbard J Provias M Greenberg LE Becker JT Rutka (1994) ArticleTitleMalignant rhabdoid tumour of the pineal region Can J Neurol Sci 21 IssueID3 273–277

    Google Scholar 

  47. A Munoz A Carrasco MJ Munoz J Esparza (1995) ArticleTitleCranial rhabdoid tumor with marginal tumor cystic component and extraaxial extension AJNR Am J Neuroradiol 16 IssueID8 1727–1728

    Google Scholar 

  48. J Pimentel R Silva T Pimentel (2003) ArticleTitlePrimary malignant rhabdoid tumors of the central nervous system: considerations about two cases of adulthood presentation J Neurooncol 61 IssueID2 121–126

    Google Scholar 

  49. G Perilongo L Sutton D Czaykowski D Gusnard J Biegel (1991) ArticleTitleRhabdoid tumor of the central nervous system Med Pediatr Oncol 19 IssueID4 310–317

    Google Scholar 

  50. F Proust A Laquerriere B Constantin MM Ruchoux JP Vannier P Freger (1999) ArticleTitleSimultaneous presentation of atypical teratoid/rhabdoid tumor in siblings J Neurooncol 43 IssueID1 63–70

    Google Scholar 

  51. D Reinhardt J Behnke-Mursch E Weiss HJ Christen J Kuhl M Lakomek A Pekrun (2000) ArticleTitleRhabdoid tumors of the central nervous system Childs Nerv Syst 16 IssueID4 228–234

    Google Scholar 

  52. W Roggendorf (1991) ArticleTitlePrimative neuroectodermal tumor with focal rhabdoid component Clin Neuropathol 10 106

    Google Scholar 

  53. H Satoh J Goishi T Sogabe T Uozumi K Kiya K Migita (1993) ArticleTitlePrimary malignant rhabdoid tumor of the central nervous system: case report and review of the literature Surg Neurol 40 IssueID5 429–434

    Google Scholar 

  54. C Sotelo-Avila F Gonzalez-Crussi D deMello C Vogler WM Gooch Suffix3rd G Gale R Pena (1986) ArticleTitleRenal and extrarenal rhabdoid tumors in children: a clinicopathologic study of 14 patients Semin Diagn Pathol 3 IssueID2 151–163

    Google Scholar 

  55. ME Velasco JA Brown J Kini ES Ruppert (1993) ArticleTitlePrimary congenital rhabdoid tumor of the brain with neoplastic hydranencephaly Childs Nerv Syst 9 IssueID3 185–190

    Google Scholar 

  56. SB Wharton C Wardle JW Ironside WH Wallace JA Royds DW Hammond (2003) ArticleTitleComparative genomic hybridization and pathological findings in atypical teratoid/rhabdoid tumour of the central nervous system Neuropathol Appl Neurobiol 29 IssueID3 254–261

    Google Scholar 

  57. DA Weeks RL Malott CW Zuppan BH Liwnicz JB Beckwith (1994) ArticleTitlePrimitive cerebral tumor with rhabdoid features: a case of phenotypic rhabdoid tumor of the central nervous system Ultrastruct Pathol 18 IssueID1–2 23–28

    Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Oncology, Pediatric Neuro-Oncology, Dana-Farber Cancer Institute, Rm # SW331, 44 Binny Street, Boston, MA, 02115, USA

    Mary Ann Zimmerman, Christopher D. Turner, Susan N. Chi, Christine Chordas & Mark W. Kieran

  2. Division of Hematology/Oncology, Children’s Hospital, USA

    Mary Ann Zimmerman, Christopher D. Turner, Susan N. Chi, Christine Chordas & Mark W. Kieran

  3. Department of Neurosurgery, Children’s Hospital, USA

    Liliana C. Goumnerova, Mark Proctor & R. Michael Scott

  4. Department of Radiation Oncology, Children’s Hospital, USA

    Karen Marcus

  5. Department of Neurology, Children’s Hospital, Boston, MA, USA

    Scott L. Pomeroy

Authors
  1. Mary Ann Zimmerman
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  2. Liliana C. Goumnerova
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  3. Mark Proctor
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  4. R. Michael Scott
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  5. Karen Marcus
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  6. Scott L. Pomeroy
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  7. Christopher D. Turner
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  8. Susan N. Chi
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  10. Mark W. Kieran
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Correspondence to Mark W. Kieran.

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Ann Zimmerman, M., Goumnerova, L.C., Proctor, M. et al. Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. J Neurooncol 72, 77–84 (2005). https://doi.org/10.1007/s11060-004-3115-y

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