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Intracranial lipomatous hamartomas (Intracranial “lipomas”)

A study of 13 cases including combinations with medulloblastoma, colloid and epidermoid cysts, angiomatosis and other malformations

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Summary

13 cases of intracranial lipomatous hamartomas are presented, 12 of which were incidentally found at autopsy. Only one case, verified by biopsy, showed progressive focal symptoms and lipomatous infiltration of the acoustic nerve. 5 lipomatous hamartomas were located in the cisterna ambiens region, 3 in the cerebellopontine angle, 2 in the hypothalamic and 1 in the callosal regions. 2 cases had multiple intracranial adipose tissue masses. Extracranial malformations were present in 4 cases, whereas 8 observations showed (micro)dysgenesias of brain and meningeal tissues surrounding the lipomatous hamartoma (mixed neuroglial-mesenchymal tissue, glial dystopias, aberrant nerve fibres, “hypertrophic” nerve bundles, peripheral myelin, micropolygyria, cerebellar microdysgenesias). 5 cases are described in detail: 1. Pontine lipomatous hamartoma combined with body malformations, frontobasal epidermoid cyst, tentorial osteoma and cerebellar microdysgenesias. 2. Cerebellar medulloblastoma associated with fibrolipomatous meningeal hamartoma and cerebellar micropolygyria. 3. Large lipomatous hamartoma of the callosal region encircling a colloid cyst. 4. Multiple meningeal fatty nodules associated with facial malformations, cerebellar dysgenesias and tentorial osteoma. 5. Intracranial hamartomatosis with lipo-fibro-angiomatosis of meninges, megadolichobasilaris and multiple “subependymomas”. The origin of intracranial fatty tissue is discussed; intracranial lipomatous hamartoma should be regarded as a complex malformation, affecting both the cerebral ectomesenchyma and brain parenchyma, and classified as true hamartoma (tumour-like, but primarily non-neoplastic malformation). The term “lipoma”, implicating a neoplastic character, should be abandoned. Links to phakomatoses are suggested.

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References

  1. Bailey, P., Bucy, P. C.: The origin and nature of meningeal tumors. Amer. J. Cancer15, 15–54 (1931)

    Google Scholar 

  2. Boström, E.: Über die pialen Epidermoide, Dermoide und Lipone und duralen Dermoide. Zbl. Path.8, 1–98 (1897)

    Google Scholar 

  3. Capon, A., Flament-Durand, J., Potvliege, R.: Deux cas de lipome du corps calleux. Acta neurol. psychiat. belg.66, 9–28 (1966)

    Google Scholar 

  4. Demus, H.: Neue Gesichtspunkte zur Entstehung der pialen Lipome. Ein Beitrag zur Klinik, Pathologie, Entstehungsweise und Herkunft der Fettgeschwülste des Zentralnervensystems. Arch. Psychiat. Nervenkr.209, 426–442 (1967)

    Google Scholar 

  5. Dragojevié, S., Mehraein, P., Bock, H.-J.: Beobachtung eines Lipoms der Temporalregion mit Rindemmißbildung und Epilepsie. Klinisch-pathologische Studie. Arch. Psychiat. Nervenkr.217, 335–342 (1973)

    Google Scholar 

  6. Duckett, S., Claireaux, A. E., Pearse, A. G. E.: Histoenzymatic study of fetal medulloblastoma with associated congenital malformations. Neurology (Minneap.)16, 283–287 (1966)

    Google Scholar 

  7. Durity, F. A., Dolman, C. L., Moyes, P. D.: Ganglioneuroblastoma of the cerebellum. Case report. J. Neurosurg.28, 270–273 (1968)

    Google Scholar 

  8. Feigin, I., Cravioto, H.: A histochemical study of myelin. A difference in the solubility of the glycolipid components in the central and peripheral nervous system... Neuropath. exp. Neurol.20, 245–254 (1961)

    Google Scholar 

  9. Ghatak, N. R., Hirano, A., Doron, Y., Zimmerman, H. M.: Remyelination in multiple sclerosis with peripheral type myelin. Arch. Neurol. (Chic.)29, 262 (1973)

    Google Scholar 

  10. Giuffrè, R., Gambacorta, D.: Lipoma of the spinal cord. Case report. J. Neurosurg.35, 335–337 (1971)

    Google Scholar 

  11. Gold, L. H. A., Kieffer, S. A., Peterson, H. O.: Lipomatous invasion of the spinal cord associated with spinal dysraphism. Myelographic evaluation. Amer. J. Roentgenol.107, 479–485 (1969)

    Google Scholar 

  12. Griepentrog, F., Pauly, H.: Intra- und extrakranielle, frühmanifeste Medulloblastome bei erbgleichen Zwillingen. Zbl. Neurochir.17, 129–140 (1957)

    Google Scholar 

  13. Gullotta, F.: Vergleichende Untersuchungen zur Morphologie und Genese der sogenannten Medulloblastome. Acta neuropath. (Berl.)8, 76–83 (1967)

    Google Scholar 

  14. Gullotta, F.: The specific localisation of mesenchymal tumours of CNS. Proc. VIth Internat. Congr. Neuropath. (Paris), pp. 974–975. Paris: Masson 1970

    Google Scholar 

  15. Gullotta, F., Kersting, G.: The ultrastructure of medulloblastoma in tissue culture. Virchows Arch., Abt. A, Path. Anat.356, 111–118 (1972)

    Google Scholar 

  16. Haberland, C., Perou, M.: Encephalocraniocutaneous lipomatosis. A new example of ectomesodermal dysgenesis. Arch. Neurol. (Chic.)22, 144–155 (1970)

    Google Scholar 

  17. Haglid, K. G., Stavrou, D., Rönnbäck, L., Carlsson, C.-A., Weidenbach, W.: The S-100 protein in water-soluble and pentanol-extractable form in normal human brain and tumors of the human nervous system. A quantitative study. J. neurol. Sci.20, 103–111 (1973)

    Google Scholar 

  18. Harvey, S. C., Burr, H. S.: The development of the meninges. Arch. Neurol. Psychiat. (Chic.)15, 545–567 (1926)

    Google Scholar 

  19. Harvey, S. C., Burr, H. S., VanCampenhout, E.: Development of the meninges. Further experiments. Arch. Neurol. Psychiat. (Chic.)29, 683–690 (1933)

    Google Scholar 

  20. Henschen, F.: Tumoren des Zentralnervensystems und seiner Hüllen. In: Handb. spez. path. Anat. Histol., Bd. 13/3, S. 413–1040. Berlin-Göttingen-Heidelberg: Springer 1955

    Google Scholar 

  21. Hirano, A., Zimmerman, H. M., Levine, S.: Electron microscopic observations of peripheral myelin in a central nervous system lesion. Acta neuropath. (Berl.)12, 348–365 (1969)

    Google Scholar 

  22. Hörstadius, S.: The neural crest. Its properties and derivatives in the light of experimental research. London: Oxford University Press 1950

    Google Scholar 

  23. Huber, K., Hammer, B., Seitelberger, F.: Ein operiertes intrakranielles Lipom im Dach des 3. Ventrikels. Wien. Z. Nervenheilk.7, 104–114 (1953)

    Google Scholar 

  24. Jellinger, K.: Einige morphologische Aspekte der Multiphen Sklerose. Wien. Z. Nervenheilk., Suppl. II, 12–37 (1969)

    Google Scholar 

  25. Jellinger, K., Sunder-Plassmann, M.: Connatal intracranial tumours. Neuropädiatrie (Stuttg.)4, 46–63 (1973)

    Google Scholar 

  26. Kadin, M. E., Rubinstein, L. J., Nelson, J. S.: Neonatal cerebellar medulloblastoma originating from the fetal external granular layer. J. Neuropath. exp. Neurol.29, 583–600 (1970)

    Google Scholar 

  27. Kane, W., Aronson, S. M.: Gangliogliomatous maturation in cerebellar medulloblastoma. Acta neuropath. (Berl.)9, 273–279 (1967)

    Google Scholar 

  28. Kernohan, J. W.: Tumors of congenital origin. In: Pathology of the nervous system, J. Minckler, Ed., Vol. 2, 1927–1937. New York: McGraw-Hill 1971

    Google Scholar 

  29. Koehl, R. H., Solitare, G. B., Heffner, R. R., Jr.: Lipomatous hamartoma involving the midbrain and cerebellum of a mentally retarded man. J. ment. Defic. Res.14, 227–234 (1970)

    Google Scholar 

  30. Krainer, L.: Die Hirn-und Rückenmarkslipome. Virchows Arch. path. Anat.295, 107 bis 142 (1935)

    Google Scholar 

  31. Kramer, W.: Lesions of the central nervous system in multiple neurofibromatosis. Psychiat. Neurol. Neurosurg.74, 349–368 (1971)

    Google Scholar 

  32. Landolt-Weber, U. M.: Ultrastruktur einer Kolloidcyste des dritten Ventrikels. Acta neuropath. (Berl.)26, 59–70 (1973)

    Google Scholar 

  33. List, C. F., Holt, J. F., Everett, M.: Lipoma of the corpus callosum: A clinico-pathologic study. Amer. J. Roentgenol.55, 125–134 (1946)

    Google Scholar 

  34. Machacek, F.: Lipom des Balkens und symmetrische Plexuslipome. Wien. klin. Wschr.78, 390–392 (1966)

    Google Scholar 

  35. Manganiello, L. O. J., Daniel, E. F., Hair, L. Q.: Lipoma of the corpus callosum. Case report. J. Neurosurg.24, 892–894 (1966)

    Google Scholar 

  36. Matakas, F., Cervós-Navarro, J., Gullotta, F.: The ultrastructure of medulloblastomas. Acta neuropath. (Berl.)16, 271–284 (1970)

    Google Scholar 

  37. McLean, A. J.: Intracranial tumors. In: Handb. Neurol, Bd. 14, S. 131–241. Berlin: Springer 1936

    Google Scholar 

  38. Nippe, M.: Traumatisch entstandenes Gliosarkom mit Lipom des Gehirns. Frankfurt. Z. Path.11, 466–471 (1912)

    Google Scholar 

  39. Nordin, W. A., Tesluk, H., Jones, R. K.: Lipoma of the corpus callosum. Arch. Neurol. Psychiat. (Chic.)44, 300–307 (1955)

    Google Scholar 

  40. Ostertag, B.: Die hyperplastischen Hamartome des Hypothalamus und ihre allgemeinpathologische Bedeutung. Acta neuropath. (Berl.)4, 107–125 (1964)

    Google Scholar 

  41. Rogers, H. M., Long, D. M., Chou, S. N., French, L. A.: Lipomas of the spinal cord and cauda equina. J. Neurosurg.34, 349–354 (1971)

    Google Scholar 

  42. Rubinstein, L. J.: Tumors of the central nervous system. In: Atlas of tumor pathology, ser. 2, fasc. 6. Washington, D.C.: AFIP 1972

    Google Scholar 

  43. Rubinstein, L. J.: Cytogenesis and differentiation of primitive central neuroepithelial tumors. J. Neuropath. exp. Neurol.31, 7–26 (1972)

    Google Scholar 

  44. Rubinstein, L. J.: Personal communication (1972)

  45. Rubinstein, L. J., Northfield, D. W. C.: The medulloblastoma and the so-called “arachnoidal cerebellar sarcoma”. A critical re-examination of a nosological problem. Brain87, 379–412 (1964)

    Google Scholar 

  46. Russel, D. S., Rubinstein, L. J.: Pathology of tumours of the nervous system, 3rd ed. London: Edward Arnold Ltd. 1971

    Google Scholar 

  47. Sabouraud, O., Pecker, J., Simon, P., Chatel, M.: Lipomes du corps calleux. Données angiographiques et discussion pathogénique de leur séméiologie clinique. Rev. neurol.117, 557–570 (1967)

    Google Scholar 

  48. Scherer, E.: Über die pialen Lipome des Gehirns. Beitrag eines Falles von ausgedehnter meningealer Lipomatose einer Großhirnhemisphäre bei Mikrogyrie. Z. ges. Neurol.154, 45–61 (1936)

    Google Scholar 

  49. Schmid, A. H.: A lipoma of the cerebellum. Acta neuropath. (Berl.)26, 75–80 (1973)

    Google Scholar 

  50. Stefan, H.: Lipom lokalisiert im Kleinhirnbrückenwinkel als Nebenbefund einer tuberkulösen Meningitis mit histologischem Befund. Z. ges. Neurol.145, 445–453 (1933)

    Google Scholar 

  51. Tanaka, K.: Rare intracranial tumors. Folia psychiat. neurol jap.5, 167–175 (1952)

    Google Scholar 

  52. Thomas, J. E., Miller, R. H.: Lipomatous tumors of the spinal cord. A study of their clinical range. Proc. Mayo Clin.48, 393–400 (1973)

    Google Scholar 

  53. Treip, C. S.: A congenital medulloepithelioma of the midbrain. J. Path. Bact.74, 357–363 (1957)

    Google Scholar 

  54. Vonderahe, A. R., Niemer, W. T.: Intracranial lipoma. A report of four cases. J. Neuropath. exp. Neurol.3, 344–354 (1944)

    Google Scholar 

  55. Vuia, O.: Multiple lipomata. Krabbe disease. In: Handbook of clinical neurology, P. J. Vinken and G. W. Bruyn, Eds., Vol. 14, pp. 405–413. Amsterdam: North-Holland 1972

    Google Scholar 

  56. Wassermann, F.: Die Fettorgane des Menschen. Entwicklung, Bau und systematische Stellung des sogenannten Fettgewebes. Z. Zellforsch.3, 235–328 (1926)

    Google Scholar 

  57. Willis, R. A.: The pathology of the tumours of children. Edinburgh-London: Oliver & Boyd 1962

    Google Scholar 

  58. Wolbach, S. B., Millet, J. A. P.: Diffuse subdural lipomatosis of the spinal cord in an infant. Boston med. Sci. J.168, 681–686 (1913)

    Google Scholar 

  59. Wolpert, S. M., Carter, B. L., Ferris, E. J.: Lipomas of the corpus callosum. An angiographic analysis. Amer. J. Roentgenol.115, 92–99 (1972)

    Google Scholar 

  60. Yalcin, S., Fragoyannis, S.: Intracranial lipoma. Case report. J. Neurosurg.24, 895–897 (1966)

    Google Scholar 

  61. Zelengurov, V. M., Galaiko, R. A.: Lipoma of the brain. Arch. Pat. (Moscow)12, 62–64 (1969) (in Russian)

    Google Scholar 

  62. Zettner, A., Netsky, M. G.: Lipoma of the corpus callusom. J. Neuropath. exp. Neurol.19, 305–319 (1960)

    Google Scholar 

  63. Zülch, K. J.: Biologie and Pathologie der Hirngeschwülste. In: Handb. Neurochir., Bd. 3, S. 1–702. Berlin-Heidelberg-New York: Springer 1956

    Google Scholar 

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Budka, H. Intracranial lipomatous hamartomas (Intracranial “lipomas”). Acta Neuropathol 28, 205–222 (1974). https://doi.org/10.1007/BF00719025

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