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Primary malignant rhabdoid tumours of the central nervous system: An immunohistochemical and ultrastructural study

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Summary

Three cases of primary rhabdoid tumour of the CNS (RT-CNS) are presented. In case 1 a hemispheric tumour developed in a 10.5 months old girl, who survived for 6 months after incomplete resection, radio- and polychemotherapy. Case 2 was a 4 years and 8 months old boy with a large IIIrd ventricle tumour, who died of leptomeningeal tumour dissemination 7 months after diagnosis despite radiotherapy. In case 3 a pineal mass occurring in a 14 month old female was radioresistant and totally exstirpated. The child died due to tumour recurrence two months later. Autopsy examination revealed widespread leptomeningeal dissemination. All three cases fulfilled light and electron microscopic criteria of RT-CNS including abundant eosinophilic cytoplasm, vesicular nuclei with large nucleoli and conspicuous anti-vimentin positive filaments. Extensive immunohistochemical studies showed expression of epithelial (EMA, KL1), macrophage (alpha-1 antichymotrypsin), neuro-ectodermal (GFAP, NSE, β-tubulin III) and myogenic markers (desmin, actin). Different stress proteins (alpha-B crystallin, HSP70) were also expressed. Tumour cells showed a proliferation (MIB1) index of 28.4% (case 1) and 33.4% (case 2). From our study it can be concluded that RT-CNS reveals significant immuno-morphological heterogeneity thus supporting the view that it is not a specific pathological entity but merely a phenotypic appearance of different neoplasms, some of which are linked to primitive neuro-ectodermal tumours.

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References

  1. Agranovich AL, Ang LC, Griebel RW, Kobrinsky NL, Lowry N, Tchang SP (1992) Malignant rhabdoid tumour of the central nervous system with subarachnoid dissemination. Surg Neurol 37: 410–414

    PubMed  Google Scholar 

  2. Beckwith JB, Palmer NF (1978) Histopathology and prognosis of Wilms tumour. Results from the first national Wilms tumour study pathology center. Human Pathol 14: 481–492

    Google Scholar 

  3. Behring B, Brück W, Goebel HH, Behnke J, Pekrun A, Christen HJ, Kretzschmar HA (1996) Immunohistochemistry of primary central nervous system malignant rhabdoid tumours: report of five cases and review of the literature. Acta Neuropathol 91: 578–586

    PubMed  Google Scholar 

  4. Bergmann M, Kuchelmeister K, Schmid KW, Kretzschmar HA, Schröder R (1996) Different variants of frontotemporal dementia: a neuropathological and immunohistochemical study. Acta Neuropathol 92: 170–179

    PubMed  Google Scholar 

  5. Bergmann M (1993) Motor neuron disease/amyotrophic lateral sclerosis — lessons from ubiquitin. Pathol Res Pract 189: 902–912

    PubMed  Google Scholar 

  6. Biegel JA, Rorke LB, Packer RJ, Emanuel BS (1990) Monosomy 22 in rhabdoid or atypical tumours of the brain. J Neurosurg 73: 710–714

    PubMed  Google Scholar 

  7. Biggs PJ, Garen PD, Powers JM, Garvin AJ (1987) Malignant rhabdoid tumour of the central nervous system. Hum Pathol 18: 332–337

    PubMed  Google Scholar 

  8. Bonnin JM, Rubinstein LJ, Palmer NF, Beckwith JB (1984) The association of embryonal tumours originating in the kidney and in the brain. A report of seven cases. Cancer 54: 2137–2146

    PubMed  Google Scholar 

  9. Bouffet E, Frappaz D, Dolbeau D, Sobol H, Jouvet A, Carrie C, Pondarre C, Brunat-Mentigny M, Philip T, Mottolese C (1993) Successful treatment for a metastatic supratentorial malignant rhabdoid tumour. J Neurooncol 17: 65–70

    PubMed  Google Scholar 

  10. Briner J, Bannwart F, Kleihues P, Odermatt B, Janer R, Willi U, Boltshauser E (1985) Malignant small cell tumour of the brain with intermediate filaments — a case of a primary cerebral rhabdoid tumour. Pediatr Pathol 3: 117–118

    Google Scholar 

  11. Chi Y-W, Lee W-H, Hwang R-C, Liao H-B, Cheng S-N, Chu M-L, Chou T-Y, Ho PSP (1989) Intracranial malignant rhabdoid tumour: report of one case. Acta Paed Sin 30: 316–322

    Google Scholar 

  12. Chou SM, Anderson JS (1991) Primary CNS malignant rhabdoid tumour (MRT): report of two cases and review of the literature. Clin Neuropathol 10: 1–10

    PubMed  Google Scholar 

  13. Cossu A, Masarelli G, Manetto V, Viale G, Tanda F, Bosincu L, Iuzzolino P, Cossu S, Padovini R, Eusebi V (1993) Rhabdoid tumours of the central nervous system — report of three cases with immunocytochemical and ultrastructural findings. Virch Arch A Pathol Anat 422: 81–85

    Google Scholar 

  14. Gonzalez-Crussi F, Goldschmidt RA, Hsueh W, Trujillo YP (1982) Infantile sarcoma with intracytoplasmic filamentous inclusions. Cancer 49: 2365–2375

    PubMed  Google Scholar 

  15. Hamos JE, Oblas B, Pulaski-Salo D, Welch WJ, Bole DG, Drachman DA (1991) Expression of heat shock proteins in Alzheimer's disease. Neurology 41: 345–350

    PubMed  Google Scholar 

  16. Hanna SL, Langston JW, Parham DM, Douglass EC (1993) Primary malignant rhaboid tumour of the brain: clinical, imaging, and pathologic findings. AJNR Am J Neuroradiol 14: 107–115

    PubMed  Google Scholar 

  17. Ho PSP, Lee WH, Chen CY, Chou TY, Wang YC, Sun MJ, Liou WY (1990) Primary malignant rhabdoid tumour of the brain: CT characteristics. J Comp Assist Tomogr 14: 461–463

    Google Scholar 

  18. Horn M, Schlote W, Lerch KD, Steudel WI, Harms D, Thomas E (1992) Malignant rhabdoid tumour: primary intracranial manifestation in an adult. Acta Neuropathol 83: 445–448

    PubMed  Google Scholar 

  19. Jakate SM, Marsden HB, Ingram L (1988) Primary rhaboid tumour of the brain. Virchow Arch 412: 393–397

    Google Scholar 

  20. Kapur S, Patterson K, Chandra R (1985) Primary rhabdoid tumour of the cerebellum (abstract). Pediatr Pathol 3: 101

    Google Scholar 

  21. Kato S, Hirano A, Kato M, Herz F, Ohama E (1993) Comparative study on the expression of stress response protein (srp) 72, srp 27, aB-crystallin and ubiquitin in brain tumours. An immunohistochemical investigation. Neuropathol Appl Neurobiol 19: 436–442

    PubMed  Google Scholar 

  22. Kchir N, Limam R, Haouet S, Chatti S, Chadly A, Khaldi M, Boubaker S, Zitouna M (1993) Tumeur rhabdoide maligne du systeme nerveux central. A propos d'une observation avec etudes immunohistochimique et ultrastructurale, et revue de la literature. Arch Anat Cytol Pathol 41: 102–106

    PubMed  Google Scholar 

  23. Kepes JJ, Moral LA (1991) Malignant rhabdoid tumours (MRT-S) of the central nervous system (CNS) and their morphological features seen in other CNS neoplasms. J Neuropathol Exp Neurol 50: 362

    Google Scholar 

  24. Lowe J, Mayer RJ, Landon M (1993) Ubiquitin in neurodegenerative diseases. Brain Pathol 3: 55–65

    PubMed  Google Scholar 

  25. Molenaar WM, Jansson DS, Gould VE, Rorke LB, Franke WW, Lee VMY, Packer RJ, Trojanowski JQ (1989) Molecular markers of primitive neuroectodermal tumours and other pediatric central nervous system tumours — monoclonal antibodies to neuronal and glial antigens distinguish subsets of primitive neuroectodermal tumours. Lab Invest 61: 635–643

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  27. Olson TA, Bayar E, Kosnik E, Hamoudi AB, Klopfenstein KJ, Pieters RS, Ruymann FB (1995) Successful treatment of disseminated central nervous system malignant rhabdoid tumour. J Pediatr Hematol Oncol 17: 71–75

    PubMed  Google Scholar 

  28. Papadakis V, Vlachopapadopoulou EA, Levine L (1995) Rhabdoid tumour of the kidney with humoral hypercalcemia and parathyroid hormone-related protein production. Med Pediatr Oncol 24: 133–136

    PubMed  Google Scholar 

  29. Parham DM, Weeks DA, Beckwith JB (1994) The clinicopathologic spectrum of putative extrarenal rhabdoid tumours — an analysis of 42 cases studied with immunohistochemistryor electron microscopy. Am J Surg Pathol 18: 1010–1029

    PubMed  Google Scholar 

  30. Perilongo G, Sutton L, Czaykowski D, Gusnard D, Biegel J (1991) Rhabdoid tumour of the central nervous system. Med Pediatr Oncol 18: 310–317

    Google Scholar 

  31. Roggendorf W (1991) Primitive neuroectodermal tumour with focal rhabdoid component. Abstract. Clin Neuropathol 10: 106

    Google Scholar 

  32. Rorke LB, Packer R, Biegel J (1995) Central nervous system atypical teratoid/rhabdoid tumours of infancy and childhood. J Neurooncol 24: 21–28

    PubMed  Google Scholar 

  33. Rosemberg S, Menezes Y, Sousa MR, Plese P, Ciquini O (1994) Primary malignant rhabdoid tumour of the spinal dura. Clin Neuropathol 13: 221–224

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  35. Sharifah NA, Yun K (1994) Malignant rhabdoid tumour of the kidney expresses insulin-like growth factor II transcripts. Pathology 26: 134–137

    PubMed  Google Scholar 

  36. Soini Y, Miettinen M (1989) Alpha-1 antitrypsin and lysozyme. Their limited significance in fibrohistiocytic tumours. Am J Clin Pathol 91: 515–521

    PubMed  Google Scholar 

  37. Sotelo-Avila C, Gonzalez-Crussi F, deMello MD, Vogler C, Gooch WM, Gale G, Pena R (1986) Renal and extrarenal rhabdoid tumours in children: a clinicopathologic study of 14 patients. Semin Diagn Pathol 3: 151–163

    PubMed  Google Scholar 

  38. Tokunaga T, Shigemori M, Nobuaki N, Kuramoto S, Nakano R, Sasaguri Y, Irie K, Morimatsu M (1991) Primary rhabdoid tumour of the cerebellum in a child — clinicopathological, immunohistochemical and ultrastructural studies of a case report. Nerv Syst Child

  39. Velasco ME, Brown JA, Kini J, Ruppert ES (1993) Primary congenital rhabdoid tumour of the brain with neoplastic hydranencephaly. Childs Nerv Syst 9: 185–190

    PubMed  Google Scholar 

  40. Weeks DA, Malott RL, Zuppan CW, Liwnicz BH, Beckwith JB (1994) Primitive cerebral tumour with rhabdoid features: a case of phenotypic rhabdoid tumour of the central nervous system. Ultrastruct Pathol 18: 23–28

    PubMed  Google Scholar 

  41. Weinblatt M, Kochen J (1992) Rhabdoid tumour of the central nervous system. Med Pediatr Oncol 20: 258

    PubMed  Google Scholar 

  42. Weyman C, Dolson L, Kedar A (1993) Section of vasointestinal peptide by a primary liver tumour with rhabdoid features. J Surg Oncol 54: 267–270

    PubMed  Google Scholar 

  43. Wick MR, Ritter JH, Dehner LP (1995) Malignant rhabdoid tumours: a clinicopathologic review and conceptual discussion. Sem Diagn Pathol 12: 233–248

    Google Scholar 

  44. Wilson D, Dempsey RJ, John WJ (1990) Rhabdoid tumour of the conus medullaris. J Neuropathol Exp Neurol 49: 339

    Google Scholar 

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Bergmann, M., Spaar, H.J., Ebhard, G. et al. Primary malignant rhabdoid tumours of the central nervous system: An immunohistochemical and ultrastructural study. Acta neurochir 139, 961–969 (1997). https://doi.org/10.1007/BF01411306

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