| Literature DB >> 35308189 |
Hui Yuan Lam1, Shawaltul Akhma Bt Harun Nor Rashid2.
Abstract
Neurofibromatosis type one (NF-1) is an autosomal dominant neurocutaneous disorder also known as Von Recklinghausen disease. Plexiform neurofibroma is a rare kind of NF-1 where the neurofibroma originates from nerve sheath cells or subcutaneous peripheral nerves. It is pathognomonic of NF-1, and isolated occurrence is relatively rare. We reported three cases of solitary plexiform neurofibromas with an unusual presentation. We have two cases of spontaneous bleeding isolated plexiform neurofibroma that have never been reported in the literature. Neither one of them showed signs and symptoms associated with the neurofibromatosis spectrum. This unusual presentation poses substantial challenges in diagnosis and management.Entities:
Keywords: autosomal dominant neurocutaneous disorder; isolated; neurofibromatosis; plexiform neurofibromatosis; von recklinghausen disease
Year: 2022 PMID: 35308189 PMCID: PMC8919432 DOI: 10.7759/cureus.23141
Source DB: PubMed Journal: Cureus ISSN: 2168-8184