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A Case Series of Plexiform Neurofibroma: The Unusual Presentations and Surgical Challenges
Hui Yuan Lam1, Shawaltul Akhma Bt Harun Nor Rashid2
1Reconstructive Science Unit, Hospital Universiti Sains Malaysia, Kelantan, MYS.
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.
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