Neurofibroma D36.16

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Definition
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Most frequent neurogenic tumour, which occurs solitary or as part of neurofibromatosis .

Classification
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A distinction is made between:
  • Diffuse (superficially located) neurofibroma
  • Plexiform (encapsulated, usually deep dermal or subcutaneous) neurofibroma
  • Pigmented neurofibroma (diffuse or plexiform)
  • Mixed neurofibroma (diffuse and plexiform).

Clinical features
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  • Solitary or multiple, 0.2-0.5 cm large or monstrous, sack-shaped polyploid, skin-coloured to bluish, broad or stalked, conspicuously soft tumours, possibly with wrinkling or as a dewlap-like structure.
  • The bell-button phenomenon is typical: Tumours which protrude the skin can be pushed back with a finger through a hernia-like dermal gap. The plexiform (encapsulated) neurofibromas can be palpated as strand-like, rough structures in the depth of the dermis or subcutis.

Histology
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Cell-rich, almost homogeneous accumulation of myelinated and non-myelinated nerve fibres, modified Schwann cells, fibroblasts, macrophages, mast cells, collagenous and elastic fibres and weakly basophilic mucoidal basic substance without recognisable cell boundaries.

Differential diagnosis
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Therapy
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In case of functionally or cosmetically disturbing tumours, excision if necessary.

Literature
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  1. Babovic S et al (2003) Liposuction: a less invasive surgical method of debulking plexiform neurofibromas. Dermatol Surgery 29: 785-787
  2. Filippo G et al (1989) Solitary Plexiform Neurofibroma. Dermatologica 179: 84-86
  3. Renshaw A et al (2003) Massive plexiform neurofibroma with associated meningo-encephalocoele and occipital bone defect presenting as a cervical mass. Br J Plast Surgery 56: 514-517

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Last updated on: 29.10.2020