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Rhabdoid Meningiomas: Histopathological Diagnosis

  • Ewa MatyjaEmail author
  • Wiesława Grajkowska
Chapter
Part of the Tumors of the Central Nervous System book series (TCNS, volume 11)

Abstract

Rhabdoid meningioma (RM) is an uncommon, aggressive variant of meningioma, designated as WHO grade III malignancy. It is characterized by a high risk to local recurrences, neoplastic dissemination and remote metastases. So far, the majority of reported cases of RMs have been found as secondary rhabdoid lesions in tumour recurrences.

The histological pattern of RM is characterized by the presence of neoplastic cells of distinctive rhabdoid morphology associated with polyphenotypic immunohistochemical profile. Rhabdoid cells usually stain diffusely for vimentin and S-100 protein and show focal expression of epithelial membrane antigen and cytokeratins. The tumour exhibits brisk mitotic activity and high MIB-1 labelling index.

The accurate histopathological diagnosis of rhabdoid subtype of meningioma is often difficult because of its similarity to other primary or secondary brain tumours that exhibit rhabdoid morphology. Particularly, the distinction from atypical teratoid/rhabdoid tumours (AT/RTs) and metastatic carcinomas is challenging and requires a wide spectrum of immunohistochemical and electron microscopic studies. The diagnosis of rhabdoid meningioma might be supported by evidence of SNF5 (INI1) protein expression, that allows to exclude AT/RT. Ultrastructural features characteristic for meningothelial cell phenotype i.e. interdigitating cell processes, numerous desmosomes and paranuclear whorls of intermediate filaments might be helpful in confirming the diagnosis.

Keywords

Rhabdoid Tumour Choroid Plexus Carcinoma Rhabdoid Cell Rhabdoid Feature Meningothelial Cell 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Al-Habib A, Lach B, Al-Khani A (2005) Intracerebral rhabdoid and papillary meningioma with leptomeningeal spread and rapid clinical progression. Clin Neuropathol 24:1–7PubMedGoogle Scholar
  2. Arrazola J, Pedrosa I, Mendez R, Saldana C, Scheithauer BW, Martinez A (2000) Primary malignant rhabdoid tumour of the brain in an adult. Neuroradiology 42:363–367PubMedCrossRefGoogle Scholar
  3. Biegel JA, Kalpana G, Knudsen ES, Packer RJ, Roberts CW, Thiele CJ, Weissman B, Smith M (2002) The role of INI1 and the SWI/SNF complex in the development of rhabdoid tumors: meeting summary from the workshop on childhood atypical teratoid/rhabdoid tumors. Cancer Res 62:323–328PubMedGoogle Scholar
  4. Cooper WA, Shingde M, Lee VK, Allan RS, Wills EJ, Harper C (2004) “Rhabdoid meningioma” lacking malignant features. Report of two cases. Clin Neuropathol 23:16–20PubMedGoogle Scholar
  5. Endo K, Tanaka S, Komagata M, Ikegami H, Kosaka T, Imakiire A, Serizawa H (2004) Rhabdoid transformation of recurrent meningioma in the cervical cord: a case report. J Orthop Sci 9:323–326PubMedCrossRefGoogle Scholar
  6. Hojo H, Abe M (2001) Rhabdoid papillary meningioma. Am J Surg Pathol 25:964–969PubMedCrossRefGoogle Scholar
  7. Horn M, Schlote W, Lerch KD, Steudel WI, Harms D, Thomas E (1992) Malignant rhabdoid tumor: primary intracranial manifestation in an adult. Acta Neuropathol 83:445–448PubMedCrossRefGoogle Scholar
  8. Inenaga C, Toyoshima Y, Mori H, Nishiyama K, Tanaka R, Takahashi H (2003) A fourth ventricle atypical teratoid/rhabdoid tumor in an infant. Brain Tumor Pathol 20:47–52PubMedCrossRefGoogle Scholar
  9. Jansen JC, Turner J, Sheehy J, Fagan PA (2003) Recurrent rhabdoid meningioma: case report. Skull Base 13:51–54PubMedCentralPubMedCrossRefGoogle Scholar
  10. Judkins AR, Mauger J, Ht A, Rorke LB, Biegel JA (2004) Immunohistochemical analysis of hSNF5/INI1 in pediatric CNS neoplasms. Am J Surg Pathol 28:644–650PubMedCrossRefGoogle Scholar
  11. Kawaguchi T, Kumabe T, Watanabe M, Tominaga T (2004) Atypical teratoid/rhabdoid tumour with leptomeningeal dissemination in an adult. Acta Neurochir 146:1033–1038PubMedGoogle Scholar
  12. Kepes JJ, Moral LA, Wilkinson SB, Abdullah A, Llena JF (1998) Rhabdoid transformation of tumor cells in meningiomas: a histologic indication of increased proliferative activity. Am J Surg Pathol 22:231–238PubMedCrossRefGoogle Scholar
  13. Kevasan S (2000) Meningioma with rhabdoid transformation. Singapore Med J 41:464–467Google Scholar
  14. Kim EY, Weon YC, Kim ST, Kim HJ, Byun HS, Lee JI, Kim JH (2007) Rhabdoid meningioma: clinical features and MR imaging findings in 15 patients. AJNR Am J Neuroradiol 28:1462–1465PubMedCrossRefGoogle Scholar
  15. Koenig MA, Geocadin RG, Kulesza P, Olivi A, Brem H (2005) Rhabdoid meningioma occurring in an unrelated resection cavity with leptomeningeal carcinomatosis. Case report. J Neurosurg 102:371–375PubMedCrossRefGoogle Scholar
  16. Louis DN, Scheithauer BW, Budka H, von Deimling A, Kepes JJ (2000) Meningiomas. In: Kleihues P, Cavenee WK (eds) WHO classification of tumours: pathology and genetics of tumours of the nervous system. IARC, Lyon, pp 176–184Google Scholar
  17. Lutterbach J, Liegibel J, Koch D, Madlinger A, Frommhold H, Pagenstecher A (2001) Atypical teratoid/rhabdoid tumors in adult patients: case report and review of the literature. J Neurooncol 52:49–56PubMedCrossRefGoogle Scholar
  18. Makuria AT, Rushing EJ, McGrail KM, Hartmann D-P, Azumi N, Ozdemirli M (2008) Atypical teratoid rhabdoid tumor (AT/RT) in adults: review of four cases. J Neurooncol 88:321–330PubMedCrossRefGoogle Scholar
  19. Martinez-Lage JF, Ferri Niguez B, Sola J, Perez-Espejo MA, Ros de San Pedro J, Fernandez-Cornejo V (2006) Rhabdoid meningioma: a new subtype of malignant meningioma also apt to occur in children. Childs Nerv Syst 22:325–329PubMedCrossRefGoogle Scholar
  20. Matyja E, Grajkowska W, Nauman P, Bonicki W, Bojarski P, Marchel A (2010) Necrotic rhabdoid meningiomas with aggressive clinical behavior. Clin Neuropathol 29:307–316PubMedCrossRefGoogle Scholar
  21. McMaster J, Ng T, Dexter M (2007) Intraventricular rhabdoid meningioma. J Clin Neurosci 14:672–675PubMedCrossRefGoogle Scholar
  22. Ota S, David CG, Crabbe DCG, Tran TN, Triche TI, Shimada H (1993) Malignant rhabdoid tumor. A study with two established cell lines. Cancer 71:2862–2872PubMedCrossRefGoogle Scholar
  23. Perry A (2006) Meningiomas. In: McLendon RE, Rosenblum NK, Bigner DD (eds) Russell and Rubinstein’s pathology of tumours of the nervous system, 7th edn. Hodder Arnold, London, pp 427–474CrossRefGoogle Scholar
  24. Perry A, Scheithauer BW, Stafford SL, Abell-Aleff PC, Meyer FB (1998) “Rhabdoid” meningioma: an aggressive variant. Am J Surg Pathol 22:1482–1490PubMedCrossRefGoogle Scholar
  25. Perry A, Fuller CE, Judkins AR, Dehner LP, Biegel JA (2005) INI1 expression is retained in composite rhabdoid tumors, including rhabdoid meningiomas. Mod Pathol 18:951–958PubMedCrossRefGoogle Scholar
  26. Perry A, Louis DN, Scheithauer BW, Budka H, von Deimling A (2007) Meningiomas. In: Louis DN, Ohgaki H, Weistler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system. IARC, Lyon, pp 163–172Google Scholar
  27. Pimentel J, Silva R, Pimentel T (2003) Primary malignant rhabdoid tumors of the central nervous system: considerations about two cases of adulthood presentation. J Neurooncol 61:121–126PubMedCrossRefGoogle Scholar
  28. Raisanen J, Biegel JA, Hatanpaa KJ, Judkins A, White CL, Perry A (2005) Chromosome 22q deletions in atypical teratoid/rhabdoid tumors in adults. Brain Pathol 15:23–28PubMedCrossRefGoogle Scholar
  29. Rezanko T, Tunakan M, Kahraman A, Sucu HK, Gelal F, Akkol I (2006) Primary rhabdoid tumor of the brain in an adult. Neuropathology 26:57–61PubMedCrossRefGoogle Scholar
  30. Rittierodt M, Tschernig T, Samii M, Walter GF, Stan AC (2001) Evidence of recurrent atypical meningioma with rhabdoid transformation and expression of pyrogenic cytokines in a child presenting with a marked acute-phase response: case report and review of the literature. J Neuroimmunol 120:129–137PubMedCrossRefGoogle Scholar
  31. Rorke LB, Packer RJ, Biegel JA (1996) Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 85:56–65PubMedCrossRefGoogle Scholar
  32. Saito A, Nakazato Y, Yoshii Y, Hyodo A, Harakuni T, Toita T, Ogawa K, Horikawa K, Terada Y, Kinjo S, Minei S (2001) Anaplastic meningioma with papillary, rhabdoid, and epithelial features: a case report. Brain Tumor Pathol 18:155–159PubMedCrossRefGoogle Scholar
  33. Santhosh K, Kesavadas C, Radhakrishnan VV, Thomas B, Kapilamoorthy TR, Gupta AK (2008) Rhabdoid and papillary meningioma with leptomeningeal dissemination. J Neuroradiol 35:286–291PubMedCrossRefGoogle Scholar
  34. Wakabayashi K, Suzuki N, Mori F, Kamada M, Hatanaka M (2005) Rhabdoid cystic papillary meningioma with diffuse subarachnoid dissemination. Acta Neuropathol 110:196–198PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2014

Authors and Affiliations

  1. 1.Department of Experimental and Clinical NeuropathologyMossakowski Medical Research Centre, Polish Academy of SciencesWarsawPoland

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