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PerineuriomD36.1
Synonym(s)
HistoryThis section has been translated automatically.
Pulitzer and Reed 1985
DefinitionThis section has been translated automatically.
Rare, benign tumor originating from the perineural cells, occurring in the dermis, subcutis as well as in the deep soft tissue.
2 main types are distinguished:
- intraneural perineurioma
- extraneural perineurioma (synonym: storiform perineural fibroma; digital Pacini neuroma)
Clinical featuresThis section has been translated automatically.
Intraneural perineuriomas occur as painless, well defined, nodular strands along peripheral nerves of extremities. Extraneural perineuriomas are found as soft, painless, subcutaneous or deep-seated nodules along the trunk or extremities. The tumors are benign. Isolated publications on malignant perineuriomas exist.
HistologyThis section has been translated automatically.
Intraneural perineuriomas show well-demarcated, onion-skin-like EMA-positive cell formations arranged around a nerve trunk. The center is sometimes calcified(psammoma bodies).
Extraneural pernineuriomas show well demarcated proliferations of spindle-like, also histiocytoid or epithelioid, vimentin, EMA, laminin and irregular CD34-positive cells. These are not reactive for S-100 protein.
Histologic variants include:
- sclerosing perineurioma (dermal solitary papule in the hand area of young adults with spindle-like to epithelioid cell formations (EMA-, Claudin-1-,GLUT1-positive, negative for S-100 protein) surrounded by hyalized stroma).
- reticular perineurioma (reticular arrangement of tumor convolutes)
Note(s)This section has been translated automatically.
An older term was: Localized mononeuropathy or hypertrophic neuropathy
LiteratureThis section has been translated automatically.
- Huang Y et al (2014) Intraneurally malignant perineurioma: a casereport and review of literature. Int J Clin Exp Pathol 7:4503-4507.
- Kadu S et al. (2016) Mesenchymal tumors of the skin. In: Cerroni L et al. histopathology of the skin. Springer-Verlag Berlin Heidelberg New-York S 807-808
- Kaku Yet al (2015) clinicopathologic and immunohistochemical study of 7 cases of sclerosing perineurioma. At J Dermatopathol 37:122-128.
Toussaint-Caire S et al (2015) Sclerosing acral skin perineurioma: clinicopathologic study of ten cases (eight classical and two with xanthomatous changes). Gac Med Mex 151:299-305.