Prevalence and clinicopathological characteristics of lipomatous neurofibromas in neurofibromatosis 1: An investigation of 229 cutaneous neurofibromas and a systematic review of the literature.

Autor: Rozza-de-Menezes RE; Department of Pathology, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil.; Neurofibromatosis National Center (Centro Nacional de Neurofibromatose, CNNF), Rio de Janeiro, Brazil., Brum CAI; Department of Pathology, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil., Gaglionone NC; Graduate Program in Pathology, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil., de Sousa Almeida LM; Neurofibromatosis National Center (Centro Nacional de Neurofibromatose, CNNF), Rio de Janeiro, Brazil.; Graduate Program in Pathology, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil., Andrade-Losso RM; Neurofibromatosis National Center (Centro Nacional de Neurofibromatose, CNNF), Rio de Janeiro, Brazil.; Graduate Program in Pathology, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil., Paiva BVB; School of Biomedicine, Universidade Federal Fluminense, Niterói, Brazil., Faveret PLS; Department of General and Specialized Surgery, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil., da Silva AV; Breast Surgery Service, Antônio Pedro University Hospital, Universidade Federal Fluminense, Niterói, Brazil., Siqueira OHK; Department of General and Specialized Surgery, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil., Riccardi VM; The Neurofibromatosis Institute, La Crescenta, California., Cunha KS; Department of Pathology, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil.; Neurofibromatosis National Center (Centro Nacional de Neurofibromatose, CNNF), Rio de Janeiro, Brazil.; Graduate Program in Pathology, School of Medicine, Universidade Federal Fluminense, Niterói, Brazil.
Jazyk: angličtina
Zdroj: Journal of cutaneous pathology [J Cutan Pathol] 2018 Oct; Vol. 45 (10), pp. 743-753. Date of Electronic Publication: 2018 Aug 01.
DOI: 10.1111/cup.13315
Abstrakt: Background: Lipomatous neurofibroma (Lnf) is a histopathological variant with adipocytes noted among cells of cutaneous neurofibromas. We aimed to investigate the prevalence and clinicopathological features of Lnfs of neurofibromatosis 1 (NF1)-associated cutaneous neurofibromas and to review the literature systematically. We also evaluated the expression of leptin (a hormone involved in lipid metabolism) in neurofibromas to better understand the pathogenesis of Lnfs.
Methods: A prospective histologic study was conducted on 229 cutaneous neurofibromas from 85 NF1 individuals. Leptin expression was immunohistochemically evaluated in 111 cutaneous neurofibromas. To systematically review the literature, two authors independently performed literature searches without year restriction.
Results: Forty (17.5%) neurofibromas were lipomatous. Lnfs were significantly larger lesions and associated with females. Eighteen (7.9%) of all neurofibromas had multinucleated floret-like giant cells, and these were associated with Lnfs. All neurofibromas expressed leptin. We systematically reviewed 13 articles. Three large studies investigated Lnfs mainly in sporadic neurofibromas and suggested that 0.3% to 8.0% of tumors (NF1 and sporadic) are Lnfs.
Conclusion: In NF1, Lnfs are common, mainly in larger tumors and women. All cutaneous NF1-neurofibromas express leptin. It is unknown if the expression of leptin accounts for the lipomatous variant, but it may have a role in the pathogenesis of cutaneous neurofibroma.
(© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
Databáze: MEDLINE
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