Summary
A case of symmetrical neurofibroma with onion bulbs in various stages of development and progression to microneurinomas is presented. Immunohistochemistry with differentiation and growth factor markers as well as electron microscopy showed a Schwann cell origin of the concentrically arranged cells. The onion bulbs differed from those of hypertrophic neuropathy by their more compact structure. A partial expression of cellular proliferation markers in the onion bulbs was consistent with a multifocal proliferative activity, confirming the neoplastic nature of the lesion.
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Baker DL, Molenaar WM, Trojanowski JQ, Evans AE, Ross AH, Rorke LB, Packer RJ, Lee VM-Y, Pleasure D (1991) Nerve growth factor receptor expression in peripheral and central neuroectodermal tumors, other pediatric brain tumors, and during development of the adrenal gland. Am J Pathol 139:115–122
Bailey P, Herrmann JD (1938) The role of the cells of Schwann in the formation of tumors of the peripheral nerves. Am J Pathol 14:1–37
Bielschowsky M (1922) Familiäre hypertrophische Neuritis und Neurofibromatose. J Psychol Neurol 29:182–205
Bilbao JM, Khoury NJS, Hudson AR, Briggs J (1984) Perineurioma (localized hypertrophic neuropathy). Arch Pathol Lab Med 108:557–560
Chang Y, Weinberger M, Horoupian DS (1991) Hypertrophic interstitial neuropathy (HIN) of trigeminal nerve (abstract). J Neuropathol Exp Neurol 50:350
Chesa PG, Rettig WJ, Thomson TM, Old LJ, Melamed MR (1988) Immunohistochemical analysis of nerve growth factor receptor expression in normal and malignant human tissues. J Histochem Cytochem 36:383–389
Dyck PJ (1984) Interited neuronal degeneration and atrophy affecting peripheral motor, sensory, and autonomic neurons. In: Dyck PJ, Thomas PK, Bunge RP (eds) Peripheral neuropathy, 2nd edn, vol 2. Saunders, Philadelphia, pp 1600–1655
Erlandson RA (1985) Peripheral nerve sheath tumors. Ultrastruct Pathol 9:113–122
Gerdes J, Lemke H, Baisch H, Wacker HH, Schwab U, Stein H (1984) Cell cycle analysis of a cell proliferation-associated human nuclear antigen defined by the monoclonal antibody Ki-67. J Immunol 133:1710–1715
Hall PA, Levison DA, Woods AL, Yu CCW, Kellock DB, Watkins JA, Barnes DM, Gillett CE, Camplejohn R, Dover R, Waseem NH, Lande DP (1990) Proliferating cell nuclear antigen (PCNA) immunolocalization in paraffin sections: an index of cell proliferation with evidence of deregulated expression in some neoplasms. J Pathol 162:285–294
Hirose T, Sano T, Hizawa K (1986) Ultrastructural localization of S-100 protein in neurofibroma. Acta Neuropathol (Berl) 69:103–110
Imaginário J da G, Coelho B, Tomé F, Sales Luis ML (1964) Nevrite interstitielle hypertrophique monosymptomatique. J Neurol Sci 1:340–347
Johnson PC, Kline DG (1989) Localized hypertrophic neuropathy: possible perineurial barrier defect. Acta Neuropathol 77:514–518
Kerkhoff H, Jennekens FGI, Troost D, Veldman H (1991) Nerve growth factor receptor immunostaining in the spinal cord and peripheral nerves in amyotrophic lateral sclerosis. Acta Neuropathol 81:649–656
Krücke W (1942) Zur Histopathologie der neuralen Muskelatrophie, der hypertrophischen Neuritis und Neurofibromatose. Arch Psychiatr Nervenkr 115:180–236
Lallemand RC, Weller RO (1973) Intraneural neurofibromas involving the posterior interosseous nerve. J Neurol Neurosurg Psychiatry 36:991–996
Louis DN, Edgerton S, Thor AD, Hedley-Whyte ET (1991) Proliferating cell nuclear antigen and Ki-67 immunohistochemistry in brain tumors: a comparative study. Acta Neuropathol 81:675–679
Masson P (1956) Tumeurs humaines, 2nd edn. Maloine, Paris, pp 947–954
Mitsumoto H, Wilbourn AJ, Goren H (1980) Perineurioma as the cause of localized hypertrophic neuropathy. Muscle Nerve 3:403–412
Morris GE, Mathews MB (1989) Regulation of proliferating cell nuclear antigen during the cell cycle. J Biol Chem 264:13856–13864
Ohnishi A, Nada O (1972) Ultrastructure of the onion bulb-like lamellated structure observed in a case of von Recklinghausen's disease. Acta Neuropathol (Berl) 20:258–263
Perentes E, Rubinstein LJ (1987) Recent applications of immunoperoxidase histochemistry in human neurooncology. An update. Arch Pathol Lab Med 111:796–812
Perentes E, Nakagawa Y, Ross GW, Stanton C, Rubinstein LJ (1987) Expression of epithelial membrane antigen in perineurial cells and their derivatives. Acta Neuropathol (Berl) 75:160–165
Prior R, Reifenberger G, Wechsler W (1989) Nerve growth factor receptor in tumours of the human nervous system: immunohistochemical analysis of receptor expression and tumour growth fraction. Pathol Res Pract 185:332–338
Reed RJ, Harkin JC (1983) Tumors of the peripheral nervous system. In: Atlas of tumor pathology, 2nd series, fascicle 3, supplement. Armed Forces Institute of Pathology, Washington, D.C., pp S25-S27
Ross GW, Miller JQ, Persing JA, Urich H (1989) Hemimegalencephaly, hemifacial hypertrophy and intracranial lipoma: a variant of neurofibromatosis. Neurofibromatosis 2:69–77
Schober R, Vogeley KT, Urich H, Hölzle E, Wechsler W (1992) Vascular permeability changes in tumours of the peripheral nervous system. Virchows Arch [A] 420:59–64
Sporn MB, Todaro GJ (1980) Autocrine secretion and malignant transformation of cells. N Engl J Med 303:878–880
Thomas PK, Landon DN, King RHM (1984) Diseases of the peripheral nerves. In: Adams JH, Corsellis JAN, Duchen LW (eds) Greenfield's neuropathology, 4th edn. Arnold, London, pp 807–920
Urich H (1976) Diseases of peripheral nerves. In: Blackwood W, Corsellis JAN (eds) Greenfield's neuropathology, 3rd edn. Arnold, London, pp 688–770
Urich H (1984) Pathology of tumors of cranial nerves, spinal roots and peripheral nerves. In: Dyck PJ, Thomas PK, Bunge RP (eds) Peripheral neuropathy, 2nd edn, vol 2. Saunders, Philadelphia, pp 2253–2299
Weller RO, Cervós-Navarro J (1977) Pathology of peripheral nerves. Butterworths, London, pp 150–152
Woods AL, Hall PA, Shepherd NA, Hanby AM, Waseem NH, Lane DP, Levison DA (1991) The assessment of proliferating cell nuclear antigen (PCNA) immunostaining in primary gastrointestinal lymphomas and its relationship to histological grade, S+G2+M phase fraction (flow cytometric analysis) and prognosis. Histopathology 19:21–27
Yaeger MJ, Koestner A, Marushige K, Marushige Y (1991) The reverse transforming effects of nerve growth factor on five human neurogenic tumor cell lines: in vitro results. Acta Neuropathol 83:72–80
Yasuda T, Sobue G, Mitsuma T, Takahashi A, Hashizume Y (1989) Nerve growth factor receptor immunoreactivity in human benign peripheral nerve sheath tumor. Acta Neuropathol 77:591–598
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Schober, R., Reifenberger, G., Kremer, G. et al. Symmetrical neurofibroma with Schwann cell predominance and focal formation of microneurinomas. Acta Neuropathol 85, 227–232 (1993). https://doi.org/10.1007/BF00227773
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DOI: https://doi.org/10.1007/BF00227773