Giant dumbbell C2C3 neurofibroma invading prebulbar cistern: Case report and literature review

Neurofibromatosis 1 (NF1) has a broad spectrum of clinical manifestations, most typically involving café-au-lait spots and skin neurofibromas. Only 2% of patients with NF1 have symptomatic spinal tumors. A patient with a previous diagnosis of NF1 presented with cervicalgia, dysphagia/mild dysphonia,...

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Published inSurgical neurology international Vol. 10; p. 77
Main Authors Serrano, Julia Pinheiro Martinez, Neves, Maick Willen Fernandes, Marchi, Cassiano, Nakasone, Fabio Jundy, Maldaun, Marcos Vinicius Calfat, de Aguiar, Paulo Henrique Pires, Scappini, Jr, Wilson
Format Journal Article
LanguageEnglish
Published United States Scientific Scholar 10.05.2019
Subjects
Case Report
Dumbell neurofibroma
Neurofibromatosis 1
Giant neurofibroma
Spinal tumors
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Summary:Neurofibromatosis 1 (NF1) has a broad spectrum of clinical manifestations, most typically involving café-au-lait spots and skin neurofibromas. Only 2% of patients with NF1 have symptomatic spinal tumors. A patient with a previous diagnosis of NF1 presented with cervicalgia, dysphagia/mild dysphonia, gait alteration, and progressive hypoesthesia involving all four limbs. The magnetic resonance documented a giant dumbbell neurofibroma arising between the C2 and C3 levels which extended toward the foramen magnum, causing medullary and bulbar compression. The major challenge of surgical management was the enormous size and location this C2-C3 (5 cm × 4 cm × 5.1 cm) lesion. Compression of the foramen magnum attributed to a dumbbell giant spinal neurofibroma at the C2C3 level resulting in prebulbar cisterns should be among the differential diagnostic considerations for patients presenting with tetraparesis and underlying NF1.
ISSN:2229-5097
2152-7806
2152-7806
DOI:10.25259/SNI-76-2019