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CNS involvement of Langerhans cell histiocytosis. Report of 23 surgically treated cases

  • Neurooncology
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Abstract

We present 23 cases of Langerhans cell histiocytosis with central nervous system (CNS) involvement. The major complaints were a mass on the cranial vault in fifteen (65 %), visual disturbance in four (16 %), polyurea-polydipsia in three (13 %), and progressive weakness in all extremities in one patient (4 %). Neurological examination revealed no abnormality in sixteen patients (70 %), cranial nerve palsy, visual field defect and optic atrophy in six (26 %) and paraparesis in one (4 %). Tumoral mass was found to be located on the cranial vault (65 %), in the suprasellar region (21 %) and in the spinal column (8 %). The cranium and spinal column were both involved in one patient. All patients underwent surgery; craniectomy with grossly total tumor excision plus cranioplasty (65 %), craniotomy with subtotal tumor excision (26 %), and vertebrectomy with grafting (13 %) were performed. The clinical radiological and histopathological features, as well as therapeutical considerations are discussed and the pertinent literature is reviewed.

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References

  1. Al-Rodhan NRF, O Al-Mefty, JT Godwin, JT Godwin, JR Jinkins, JL Fox: Hystiocytosis X of the spinal cord: a case report. Neurosurgery 19 (1986) 837–840

    Google Scholar 

  2. Anthony DC, JA Tucker: Histiocytosis X affecting the Nervous System. In: Wilkins RH, SS Rengachary (eds): Neurosurgery Update II. Mc Graw-Hill, New York, 1991

    Google Scholar 

  3. Baghdassarian SA, HF Shammas: Eosinophilic granuloma of orbit. Ann Ophthalmol 9 (1977) 1247–51

    Google Scholar 

  4. Bollini G, JL Jouve, JC Gentet, M Jacquemier, JM Bouyala: Bone Lesions in Histiocytosis X. J Pediatric Orthopedics 11 (1991) 469–477

    Google Scholar 

  5. Cassady JR: Current role of radiation therapy in the management of histiocytosis X. Hematol Oncol Clin North Am 1 (1987) 123–129

    Google Scholar 

  6. Chu T, GL D'Angio, B Favara, S Ladisch, M Nesbit, J Pritchard: Histiocytosis X syndrome in children. Lancet 24 (1987) 208–9

    Google Scholar 

  7. Cunningham MJ, H Curtin, L Butkiewicz: Histiocytosis X of the temporal bone: CT findings. J Comp Assist Tomogr 12 (1988) 70–74

    Google Scholar 

  8. Çataltepe O, A Çolak, S Sağlam, B Önol: Unusual Location of Histiocytosis X: presenting as a petrous apex syndrome. J Clin Neuro-ophthalmology 11 (1991) 31–34

    Google Scholar 

  9. Dickinson LD, SM Farhat: Eosinophilic granuloma of the cervical spine. Surg Neurol 35 (1991) 57–63

    Google Scholar 

  10. Enriquez P, DL Dahlin, AB Hayles, ED Henderson: Histiocytosis X: a clinical study. Proc Staff Meet Mayo Clin 42 (1967) 88–99

    Google Scholar 

  11. Gaines P, JCN Chan, CS Cockram: Histiocytosis X involving the thyroid and hypothalamus. Postgrad Med J 67 (1991) 680–682

    Google Scholar 

  12. Graif M, JM Pennock: MR imaging of histiocytosis X in the central nervous system. AJNR 7 (1986) 21–23

    Google Scholar 

  13. Itoh H, S Waga, T Kojima, T Hosshina: Solitary Eosinophilic Granuloma in the Frontal Lobe: Case Report. Neurosurgery 30 (1992) 295–298

    Google Scholar 

  14. Kepes JJ, M Keper: Predominantly cerebral forms of histiocytosis X: a reappraisal of “Gagel's hypothalamic granuloma”, “granuloma infiltrans of the hypothalamus” and “Ayala's disease” with a report of four cases. Acta Neuropathol (Berl) 14 (1969) 77–98

    Google Scholar 

  15. Komp DM, J Herson, KA Starling et al: A staging system for histiocytosis X: a Southwest Oncology Group Study. Cancer 47 (1981) 798–800

    Google Scholar 

  16. Komp DM: Long term sequelae of histiocytosis X. Am J Pediatr Hematol Oncol 3 (1981) 163–8

    Google Scholar 

  17. Lichtenstein L: Histiocytosis X: integration of eosinophilic granuloma of bone, “Letterer-Siwe disease” and “Schüller-Christian disease” as related manifestations of a single nosologic entity. Arch Pathol 56 (1953) 84–102

    Google Scholar 

  18. Livingstone PA: Differential diagnosis of radiolucent lesions of the temporal bone. Radiol Clin N Am 12 (1974) 571–583

    Google Scholar 

  19. Mc Cumber MW, PN Hoffman, GS Wand, JI Epstein, WE Beschorner, R Green. Ophthalmic Involvement in aggressive histiocytosis X. Ophthalmology 97 (1990) 22–27

    Google Scholar 

  20. Miyachi S, T Kobayashi, T Takahashi, K Saito, Y Hashizume, K Sugita: An Intracranial Mass Lesion in Systemic Xantogranulomatosis: Case Report. Neurosurgery 27 (1990) 822–826

    Google Scholar 

  21. Oberman HA: Idiopathic histiocytosis: a clinicopathologic study of 40 cases and review of the literature on eosinophilic granuloma of bone, Hand-Schüller-Christian disease and Letterer-Siwe disease. Pediatrics 28 (1961) 307–327

    Google Scholar 

  22. O'Sullivan RM, M Sheehan, KJ Poskitt, DA Graeb, AC Chu, GF Joplin: Langerhans Cell Histiocytosis of Hypothalamus and Optic Chiasm: CT and MR Studies. J Comput Assist Tomogr 15 (1991) 52–55

    Google Scholar 

  23. Rawlings CE, RH Wilkins: Solitary Eosinophilic Granuloma of the Skull. Neurosurgery 15 (1984) 155–161

    Google Scholar 

  24. Starling KA: Chemotherapy of histiocytosis X. Hematol Oncol Clin North Am 1 (1987) 119–122

    Google Scholar 

  25. Sweet RM, AH Kornblut, WS Hyams. Eosinophilic granuloma in the temporal bone. Laryngoscope 89 (1979) 1545–1552

    Google Scholar 

  26. Turgut M, Ö Gürçay: Multifocal histiocytosis X of bone in two adjacent vertebrae causing paraplegia. Aust N Z J Surg 62 (1992) 241–244

    Google Scholar 

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Authors and Affiliations

  1. Hacettepe University School of Medicine, Ankara, Turkey

    Deniz Belen & Osman E. Özcan

  2. Inönü University School of Medicine, Malatya, Turkey

    Ahmet Çolak

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  1. Deniz Belen
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  2. Ahmet Çolak
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  3. Osman E. Özcan
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Belen, D., Çolak, A. & Özcan, O.E. CNS involvement of Langerhans cell histiocytosis. Report of 23 surgically treated cases. Neurosurg. Rev. 19, 247–252 (1996). https://doi.org/10.1007/BF00314840

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