Schwannomatosis Involving Peripheral Nerves: A Case Report

Abstract

Schwannomatosis or neurilemmomatosis has been used to describe patients with multiple nonvestibular schwannomas with no other stigmata of neurofibromatosis type-2 (NF-2). In our case, schwannomatosis, multiple schwannomas were present in a 21-yr-old woman with no stigmata or family history of NF-1 or NF-2. She had no evidence of vestibular schwannoma or other intracranial tumors. Multiple peripheral tumors were found in the carotid space of the neck, and soft tissue of posterior shoulder, lower back, ankle and middle mediastinum. All of those tumors were completely limited to the right side of the body. All surgically removed tumor specimens in this patient proved to be schwannomas.

Isolated Schwannoma of the Upper Eyelid Margin in a 50-year-old Male

“…In Schwannomatosis (neurilemmomatosis), multiple non-vestibular Schwannomas are observed with no other stigmatas of NF type 1 or NF type 2. 4 Clinico-pathologic variants of Schwannoma include conventional Schwannoma, cellular Schwannoma, and melanotic Schwannoma. 5 Microscopically, they may demonstrate a biphasic pattern, and areas of highly cellular (Antoni type A) and myxoid matrix (Antoni type B) may be observed.…”

“…The most important feature for diagnosis of a Schwannoma is still its strong reactivity to S100 protein in immunochemistry. 1,2,3,4,5,6,7 …”

Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

“…• Confusion with NF2, presumptive schwannomatosis, 4 and familial schwannomatosis [23][24][25] • Two studies 2,26 were performed before the release of the 2005 consensus statement, 1 and other studies did not ensure the patients met the criteria for definite schwannomatosis, particularly regarding the lack of systematic brain MRI to formally eliminate NF2, [26][27][28] being < 18 years of age at diagnosis, 25,[29][30][31][32][33] and the presence of intracranial lesions 34,35 • Two studies were original articles with no individual descriptive cases 1,36 Approximately 10 to 15% of patients with schwannomatosis have a family history. 17,37 The remaining 85 to 90% have sporadic disease.…”

Schwannome du nerf axillaire (à propos d’un cas)

“…Quel que soit le siège de la tumeur, l'examen clinique recherche une douleur, une tuméfaction et/ou un syndrome irritatif [1,4]. L'IRM oriente le diagnostic en mettant en évidence une tumeur arrondie en hypersignal ayant l'aspect d'une cible prenant le gadolinium [2,4,6]. L'angio-IRM permet d'étudier les rapports avec les vaisseaux avoisinants.…”