Acta Neuropathologica

, Volume 110, Issue 3, pp 306–311 | Cite as

Pilocytic astrocytoma presenting as primary diffuse leptomeningeal gliomatosis: report of a unique case and review of the literature

  • Georg Bohner
  • Florian Masuhr
  • Roland Distl
  • Juri Katchanov
  • Randolf Klingebiel
  • Rolf Zschenderlein
  • Andreas von Deimling
  • Frank K. H. van LandeghemEmail author
Case Report


We describe a 25-year-old male patient with primary diffuse leptomeningeal gliomatosis (PDLG) presenting with gait ataxia, positive Lhermitte’s sign, double vision, and right abducens nerve palsy. Spinal magnetic resonance imaging showed extended intradural, extramedullary, contrast-enhancing masses with compression of the myelon. Spinal leptomeningeal biopsy revealed a pilocytic astrocytoma WHO grade I. Despite chemotherapy with vincristin and carboplatin, the patient died 2 months after admission. A thorough autopsy showed no evidence for primary neoplasms in brain, spine and optic nerve. Sequence analysis of tumor protein 53 gene (TP53) revealed a missense mutation in exon 5, and expression of phosphatase and tensin homolog (mutated in multiple advanced cancers 1) (PTEN) protein was not detected, which may have contributed to astrocytoma development. To our knowledge, this is the first definitive case of pilocytic astrocytoma presenting as PDLG.


Pilocytic astrocytoma Primary diffuse leptomeningeal gliomatosis TP53 



We thank Karl Riabowol for critically reading and discussing our manuscript. We thank Petra Matylewski and Ulrike Lass for excellent technical assistance.


  1. 1.
    Ashworth B, Gordon A (1994) Leptomeningeal gliomatosis. J Neurol Neurosurg Psychiatry 57:471–473PubMedGoogle Scholar
  2. 2.
    Baborie A, Dunn EM, Bridges LR, Bamford JM (2001) Primary diffuse leptomeningeal gliomatosis predominantly affecting the spinal cord: case report and review of the literature. J Neurol Neurosurg Psychiatry 70:256–258CrossRefPubMedGoogle Scholar
  3. 3.
    Bailey O (1936) Relation of glioma of the leptomeninges to neuroglia nests: report of a case of astrocytoma of the leptomeninges. Arch Pathol 21:584–600Google Scholar
  4. 4.
    Bailey P, Robitaille Y (1985) Primary diffuse leptomeningeal gliomatosis. Can J Neurol Sci 12:278–281PubMedGoogle Scholar
  5. 5.
    Beauchesne P, Pialat J, Duthel R, Barral FG, Clavreul G, Schmitt T, Laurent B (1998) Aggressive treatment with complete remission in primary diffuse leptomeningeal gliomatosis—a case report. J Neurooncol 37:161–167CrossRefPubMedGoogle Scholar
  6. 6.
    Bhrany D, Murohy MG, Horenstein S, Silbert SW (1974) Diffuse periventricular and meningeal glioma. Acta Neuropathol 30:243–249CrossRefPubMedGoogle Scholar
  7. 7.
    Carpentier AF, Sanson M, Kujas M, Giroud M, Poisson M, Delattre JY (1994) [Primary meningeal gliomatosis]. Rev Neurol (Paris) 150:232–235Google Scholar
  8. 8.
    Cheng Y, Pang JC, Ng HK, Ding M, Zhang SF, Zheng J, Liu DG, Poon WS (2000) Pilocytic astrocytomas do not show most of the genetic changes commonly seen in diffuse astrocytomas. Histopathology 37:437–444CrossRefPubMedGoogle Scholar
  9. 9.
    Cooper IS, Kernohan JW (1951) Heterotopic glial nests in the subarachnoid space; histopathologic characteristics, mode of origin and relation to meningeal gliomas. J Neuropathol Exp Neurol 10:16–29PubMedGoogle Scholar
  10. 10.
    Dietrich PY, Aapro MS, Rieder A, Pizzolato GP (1993) Primary diffuse leptomeningeal gliomatosis (PDLG): a neoplastic cause of chronic meningitis. J Neurooncol 15:275–283CrossRefPubMedGoogle Scholar
  11. 11.
    Fayet G, Bertout-Lemercier C, Gugenheim M, Auffray E, Jardel-Bouissiere J, Lacroix C, Cuilliere P, Lhuillier de Cordoze M, Feve JR (1994) [Leptomeningeal gliomatosis. 2 clinicopathological cases]. Rev Neurol (Paris) 150:700–703Google Scholar
  12. 12.
    Gilmer-Hill HS, Ellis WG, Imbesi SG, Boggan JE (2000) Spinal oligodendroglioma with gliomatosis in a child. Case report. J Neurosurg Spine 92:109–113Google Scholar
  13. 13.
    Giordana MT, Bradac GB, Pagni CA, Marino S, Attanasio A (1995) Primary diffuse leptomeningeal gliomatosis with anaplastic features. Acta Neurochir (Wien) 132:154–159Google Scholar
  14. 14.
    Havlik DM, Becher MW, Nolte KB (2001) Sudden death due to primary diffuse leptomeningeal gliomatosis. J Forensic Sci 46:392–395PubMedGoogle Scholar
  15. 15.
    Heye N, Iglesias JR, Tonsen K, Graef G, Maier-Hauff K (1990) Primary leptomeningeal gliomatosis with predominant involvement of the spinal cord. Acta Neurochir (Wien) 102:145–148Google Scholar
  16. 16.
    Ho KL, Hoschner JA, Wolfe DE (1981) Primary leptomeningeal gliomatosis: symptoms suggestive of meningitis. Arch Neurol 38:662–666PubMedGoogle Scholar
  17. 17.
    Jänisch W, Engel U, Leonhardt T (1991) Diffuse primary leptomeningeal gliomatosis. Zentralbl Pathol 137:523–530]PubMedGoogle Scholar
  18. 18.
    Kastenbauer S, Danek A, Klein W, Yousry TA, Bise K, Reifenberger G, Pfister HW (2000) Primary diffuse leptomeningeal gliomatosis: unusual MRI with non-enhancing nodular lesions on the cerebellar surface and spinal leptomeningeal enhancement. J Neurol Neurosurg Psychiatry 69:385–388CrossRefPubMedGoogle Scholar
  19. 19.
    Kitahara M, Katakura R, Wada T, Namiki T, Suzuki J (1985) Diffuse form of primary leptomeningeal gliomatosis. Case report. J Neurosurg 63:283–287PubMedGoogle Scholar
  20. 20.
    Kobayashi M, Hara K, Nakatsukasa M, Murase I, Toya S (1996) Primary spinal leptomeningeal gliomatosis presenting visual disturbance as the initial symptom: case report. Acta Neurochir (Wien) 138:480–481Google Scholar
  21. 21.
    Korein J, Feigin I, Shapiro MF (1957) Oligodendrogliomatosis with intracranial hypertension. Neurology 7:589–594PubMedGoogle Scholar
  22. 22.
    Lang FF, Miller DC, Pisharody S, Koslow M, Newcomb EW (1994) High frequency of p53 protein accumulation without p53 gene mutation in human juvenile pilocytic, low grade and anaplastic astrocytomas. Oncogene 9:949–954PubMedGoogle Scholar
  23. 23.
    Leproux F, Melanson D, Mercier C, Michaud J, Ethier R (1993) Leptomeningeal gliomatosis: MR findings. J Comput Assist Tomogr 17:317–320PubMedGoogle Scholar
  24. 24.
    Louis DN, Deimling A von, Chung RY, Rubio MP, Whaley JM, Eibl RH, Ohgaki H, Wiestler OD, Thor AD, Seizinger BR (1993) Comparative study of p53 gene and protein alterations in human astrocytic tumors. J Neuropathol Exp Neurol 52:31–38PubMedGoogle Scholar
  25. 25.
    Mawrin C, Kirches E, Schneider-Stock R, Scherlach C, Vorwerk C, Deimling A von, Landeghem F van, Meyermann R, Bornemann A, Müller A, Romeike B, Stoltenburg-Didinger G, Wickboldt J, Pilz P, Dietzmann K (2003) Analysis of TP53 and PTEN in gliomatosis cerebri. Acta Neuropathol 105:529–536Google Scholar
  26. 26.
    Ng HK, Poon WS (1998) Primary leptomeningeal astrocytoma. Case report. J Neurosurg 88:586–589PubMedGoogle Scholar
  27. 27.
    Ng HK, Poon WS (1999) Diffuse leptomeningeal gliomatosis with oligodendroglioma. Pathology 31:59–63CrossRefPubMedGoogle Scholar
  28. 28.
    Ohgaki H, Eibl RH, Schwab M, Reichel MB, Mariani L, Gehring M, Petersen I, Holl T, Wiestler OD, Kleihues P (1993) Mutations of the p53 tumor suppressor gene in neoplasms of the human nervous system. Mol Carcinog 8:74–80PubMedGoogle Scholar
  29. 29.
    Park JS, Noort S van den, Kim RC, Walot I, Licht H (1996) Primary diffuse leptomeningeal gliomatosis with signs of increased intracranial pressure and progressive meningeal enhancement on MRI. J Neuroimaging 6:250–254PubMedGoogle Scholar
  30. 30.
    Paulino AC, Thomas C, Slomiany DJ, Suarez CR (1999) Diffuse malignant leptomeningeal gliomatosis in a child: a case report and review of the literature. Am J Clin Oncol 22:243–246CrossRefPubMedGoogle Scholar
  31. 31.
    Payan H, Toga M, Berard M, Hassoun J (1968) [Diffuse meningeal gliomatosis of the neuraxis]. Ann Anat Pathol 13:253–262Google Scholar
  32. 32.
    Pingi A, Trasimeni G, Di Biasi C, Gualdi G, Piazza G, Corsi F, Chiappetta F (1995) Diffuse leptomeningeal gliomatosis with osteoblastic metastases and no evidence of intraaxial lesions. AJNR Am J Neuroradiol 16:1018–1020PubMedGoogle Scholar
  33. 33.
    Poisson M, Stragliotto G, Davila G, Duyckaerts C, Delattre JY (1995) [Gliomatous meningitis of hemispheric tumors. Study of 22 cases in adults]. Rev Neurol (Paris) 151:177–189Google Scholar
  34. 34.
    Ramsay DA, Goshko V, Nag S (1990) Primary spinal leptomeningeal astrocytoma. Acta Neuropathol 80:338–341CrossRefPubMedGoogle Scholar
  35. 35.
    Rasheed BK, Stenzel TT, McLendon RE, Parsons R, Friedman AH, Friedman HS, Bigner DD, Bigner SH (1997) PTEN gene mutations are seen in high-grade but not in low-grade gliomas. Cancer Res 57:4187–4190PubMedGoogle Scholar
  36. 36.
    Rees JH, Balakas N, Agathonikou A, Hain SF, Giovanonni G, Panayiotopoulos CP, Luxsuwong M, Revesz T (2001) Primary diffuse leptomeningeal gliomatosis simulating tuberculous meningitis. J Neurol Neurosurg Psychiatry 70:120–122CrossRefPubMedGoogle Scholar
  37. 37.
    Rogers LR, Estes ML, Rosenbloom SA, Harrold L (1995) Primary leptomeningeal oligodendroglioma: case report. Neurosurgery 36:166–169PubMedGoogle Scholar
  38. 38.
    Rubio MP, Deimling A von, Yandell DW, Wiestler OD, Gusella JF, Louis DN (1993) Accumulation of wild type p53 protein in human astrocytomas. Cancer Res 53:3465–3467PubMedGoogle Scholar
  39. 39.
    Shuangshoti S (1996) Primary diffuse leptomeningeal glioblastoma multiforme of brainstem and spinal cord clinically mimicking meningitis: case report and review of literature. J Med Assoc Thai 79:403–408PubMedGoogle Scholar
  40. 40.
    Singh M, Corboy JR, Stears JC, Kleinschmidt-DeMasters BK (1998) Diffuse leptomeningeal gliomatosis associated with multifocal CNS infarcts. Surg Neurol 50:356–362; discussion 362CrossRefPubMedGoogle Scholar
  41. 41.
    Sumi SM, Leffman H (1968) Primary intracranial leptomeningeal glioma with persistant hypoglycorrhachia. J Neurol Neurosurg Psychiatry 31:190–194PubMedGoogle Scholar
  42. 42.
    Von Deimling A, Fimmers R, Schmidt MC, Bender B, Fassbender F, Nagel J, Jahnke R, Kaskel P, Duerr EM, Koopmann J, Maintz D, Steinbeck S, Wick W, Platten M, Muller DJ, Przkora R, Waha A, Blumcke B, Wellenreuther R, Meyer-Puttlitz B, Schmidt O, Mollenhauer J, Poustka A, Stangl AP, Lenartz D, Ammon K von (2000) Comprehensive allelotype and genetic anaysis of 466 human nervous system tumors. J Neuropathol Exp Neurol 59:544–558PubMedGoogle Scholar
  43. 43.
    Wada C, Kurata A, Hirose R, Tazaki Y, Kan S, Ishihara Y, Kameya T (1986) Primary leptomeningeal ependymoblastoma. Case report. J Neurosurg 64:968–973PubMedGoogle Scholar
  44. 44.
    Watanabe K, Peraud A, Gratas C, Wakai S, Kleihues P, Ohgaki H (1998) p53 and PTEN gene mutations in gemistocytic astrocytomas. Acta Neuropathol 95:559–564CrossRefPubMedGoogle Scholar
  45. 45.
    Wolbach S (1907) Congenital rhabdomyoma of the heart. Report of a case associated with multiple nests of neuroglia tissue in the meninges of the spinal cord. J Med Res 16:495–519Google Scholar

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Georg Bohner
    • 1
  • Florian Masuhr
    • 2
  • Roland Distl
    • 3
  • Juri Katchanov
    • 2
  • Randolf Klingebiel
    • 1
  • Rolf Zschenderlein
    • 2
  • Andreas von Deimling
    • 3
  • Frank K. H. van Landeghem
    • 3
    Email author
  1. 1.Department of Radiology, Neuroradiology SectionCharité Medical CenterBerlinGermany
  2. 2.Department of NeurologyCharité Medical CenterBerlinGermany
  3. 3.Institute of NeuropathologyCharité Medical CenterBerlinGermany

Personalised recommendations