Elephantiasis neuromatosa. A light, immunohistochemical and electron microscopic study

• Published in: Virchows Archiv A Pathological Anatomy and Histopathology Vol. 404; no. 4; pp. 427 - 434
• Main Authors: Holck, S, Medgyesi, S, Darre, E, Lassen, M
• Format: Journal Article
• Language: English
• Published: Germany 01-01-1984
• Subjects: Adolescent; Female; Histocytochemistry; Humans; Immunochemistry; Lipomatosis - complications; Microscopy, Electron; Myelin Sheath - pathology; Neurofibromatosis 1 - complications; Neurofibromatosis 1 - metabolism; Neurofibromatosis 1 - pathology; Peripheral Nervous System Neoplasms - complications; Peripheral Nervous System Neoplasms - metabolism; Peripheral Nervous System Neoplasms - pathology; S100 Proteins - metabolism
• ISSN: 0174-7398; 1432-2307
• DOI: 10.1007/BF00695226

An example of elephantiasis neuromatosa, an unusual variant of neurofibromatosis, coexistent with lipomatosis is presented. This dual cell population produced a diffuse swelling of the gluteal sulcus of a young female. The neurogenous origin of the spindle cell component was substantiated by immunoreactivity for S-100 protein as well as by the fine structural observation of a regular basement membrane coating and a profusion of Luse bodies. As far as can be ascertained, this is the first reported immunohistochemical and ultrastructural study of this particular variant of peripheral nerve sheath lesion.