Author(s): Zmorda Fatma El Messioui, Mouna Ounaies, Khairi Saibi, Nouha Baba, Saied Baccari and Lamjed Tarhouni

Abstract: Introduction: Neurofibromatosis and schwannomatosis are a group of related tumour suppressor syndromes characterised by an increased incidence of tumours of the nerve sheath. Schwannomatosis involving the nerves of a single limb is a rare clinicopathologic entity, distinguished from neurofibromatosis type 2 (NF2). The clinical similarities between the two aforementioned conditions may cause diagnostic difficulties. The main diagnostic criterion in favour of NF2 in this case is the presence of bilateral vestibular nerve schwannoma, which is mainly revealed by hearing loss. Case report: A 41 year-old male underwent surgery three times in 2012, 2019 and 2023 for schwannomas of the fibular nerve, the medial plantar nerve, the saphenous nerve and lastly the sciatic nerve of the right lower limb. Anatomopathological examination confirmed the diagnosis of schwannoma every time. He was relieved of pain and paraesthesia after all his surgeries. The patient was diagnosed with schwannomatosis based on the recurrence of multiple schwannomas of the right lower limb over the course of 11 years and the lack of clinical signs of bilateral hearing loss, tinnitus or balance loss in favour of vestibular tumours characteristic of the NF2.Conclusion: In cases of multiple schwannomas with no clinical or radiological signs of concomitant bilateral vestibular nerve schwannomas, the diagnosis of schwannomatosis is very likely. It becomes definite after eliminating all criteria for the diagnosis of NF2, including first-degree family history of Von Recklinghausen’s disease.

DOI: 10.22271/27080056.2024.v6.i1b.81

Pages: 67-71 | Views: 358 | Downloads: 134

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