Plexiform Schwannoma of the Tongue in a Pediatric Patient with Neurofibromatosis Type 2: A Case Report and Review of Literature.

Samir M Amer,Cecilia G Clement,Harold S Pine,Aijan Ukudeyeva,Gerald A Campbell

doi:10.1155/2018/9814591

Samir M Amer, Cecilia G Clement + Show 3 more

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https://doi.org/10.1155/2018/9814591

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Abstract

Introduction Plexiform schwannoma is a rare variant of schwannoma that accounts for only 5% of all schwannomas. Herein, we present a rare case of plexiform schwannoma of the tongue in a pediatric patient with neurofibromatosis type 2 (NF2). Case Presentation A 13-year-old female presented with a growing left-sided tongue mass. The patient has a past medical history of NF2. The tongue mass was excised and histopathological examination revealed a spindle cell tumor with multinodular growth pattern, with Verocay bodies' formation. Tumor cells were strongly positive for S-100 protein and negative for smooth muscle actin (SMA), and EMA highlighted perineural fibroblasts surrounding tumor nodules. These findings were diagnostic of plexiform schwannoma. Conclusion Plexiform schwannoma of the tongue is an extremely rare tumor seen in patients with NF2. Clinical examination and histopathological evaluation are important for diagnosis of plexiform schwannoma.

Highlights

Plexiform schwannoma is a rare variant of schwannoma that accounts for only 5% of all schwannomas
Up to 40% of all schwannomas are located in the head and neck region, of which 1% are found inside the oral cavity, frequently at the base of the tongue, the tumor can occur anywhere else inside the mouth [1, 2]
This variant accounts for only 5% of all schwannomas, with most cases occurring in the skin and subcutaneous tissue and showing a predilection for the head and neck region, similar to ordinary schwannomas [3, 4]

Summary

Introduction

Schwannoma, known as neurilemmoma, is a benign tumor of Schwann cells in the neural sheath of myelinated nerves throughout the body [1]. Up to 40% of all schwannomas are located in the head and neck region, of which 1% are found inside the oral cavity, frequently at the base of the tongue, the tumor can occur anywhere else inside the mouth [1, 2]. Plexiform schwannoma is a rare variant of schwannoma, characterized by a multinodular (plexiform) intraneural growth pattern [3] This variant accounts for only 5% of all schwannomas, with most cases occurring in the skin and subcutaneous tissue and showing a predilection for the head and neck region, similar to ordinary schwannomas [3, 4]. We present a rare case of plexiform schwannoma of the tongue in a pediatric patient with NF2

Case Presentation

Discussion

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Conclusion