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Lingual schwannoma
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     1 Department of Otolaryngology, Head and Neck Surgery, Istanbul University, School of Medicine, Turkey

    2 Department of Pathology, Istanbul University, School of Medicine, Turkey

    Abstract

    Schwannomas or neurilemmomas are benign, slow growing, usually solitary and encapsulated tumor, originating from Schwann cells of the nerve sheath. Intraoral schwannoma accounts for 1% of head and neck region and are commonly seen at the base region of tongue. Most of the few such reports in the literature, have described schwannomas that occurred in the tongue. In this article, we report a rare case of lingual schwannoma involving the anterior of tongue, in a young individual, in whom the lesion was completely excised via an intra oral approach.

    Keywords: Schwannoma, tongue, mass.

    Introduction

    Schwannomas or neurilemmomas are benign, slow growing, usually solitary and encapsulated tumor, originating from Schwann cells of the nerve sheath.[1],[2],[3],[4] First, it was identified by Virchow in 1908.[5] Approximately 25 to 40% of all schwannomas, are seen in the head and neck region.[6],[7] Intraoral schwannoma accounts for 1% of all head and neck region tumors[7],[8],[9] and are commonly seen at the base region of tongue.[7],[10],[11] We report a patient with a schwannoma of the anterior part of the tongue, that was excised intraorally.

    Case Report

    A 7-year old male patient presented with an 8 month's history of swelling, at the anterior mobile part of the tongue. The patient described that the mass was very small at onset and gradually became larger. The swelling was associated with pain and tenderness in swallowing, at the time of admittion. Intraoral examination revealed an elastic, non-tender, smooth, visible and a well-demarcated mass of 25 mm in diameter, on the anterior tip of the tongue [Figure - 1]a and b. No lymph nodes were enlarged and the remainder of the head and neck examination revealed no other lesions. The patient's medical history was unremarkable. We didn't need to do radiological investigations, because the mass was easily visible and palpable, at the anterior mobile part of the tongue. The lesion was excised with a small border of clinically uninvolved surrounding tissue, intraorally. Macroscopically, the entire lesion was removed with its capsule. The postoperative course was uneventful. The mobility of the tongue was good. Histopathological examination of surgical specimen showed a schwannoma [Figure - 2],[Figure - 3]. Immunohistochemistry shows positive staining for S-100 protein, Leu 7 antigen, vimentin and glial fibrillary acid protein. Based on these findings, a histopathological diagnosis of benign schwannoma of the tongue was made. The patient has been followed up for 5 years and there has been no evidence of recurrence.

    Discussion

    Schwannomas or neurilemmomas are benign, slow growing, usually solitary and encapsulated tumor, originating from Schwann cells of the nerve sheath.[1],[2],[3],[4] Approximately 25 to 40% of all schwannomas are seen in the head and neck region.[6],[7] Intraoral schwannoma accounts for 1% of all head and neck region tumors.[7],[8],[9] and commonly seen at the base region of tongue.[7],[10],[11] Identification of the originating nerve may be difficult. In more than 50% of intraoral lesions, it is not possible to differentiate between tumors of the lingual, hypoglossal and glossopharyngeal nerves.[12] It can be seen alone, or is associated with Von Recklinghousen disease.[13] Etiology is stil unknown and the disease is generally asymptomatic.[14] In general, it starts as a capsulated nodule and grows slowly. If it invades submucosal areas, it leads to pain and discomfort.[15],[16] Malignant transformation is mentioned in 8-10% of the cases.[13] The tumor develops in patients of all ages, without an obvious preference for either sex.[4],[7] The presenting feature of a tongue schwannnoma, is usually a tumor mass. Other symptoms include dyspnea or dysphagia and depend on the location of tumor.[10] Histopathologically, the tumor tissue consists of so-called Antoni A and B type cells. Type A tissue shows densely packed, elongated spindle cells, while type B tissue has a more myxoid consistency. In addition, hemorrhage from adjacent tissue, necrosis, hyalinization and cystic degeneration, may also occur.[17] Antoni A zone has paralelly formed thin reticulin fibres, fusiform shaped cells and curled nucleus. In general, the zone includes a variety of different cells without apparent borders, amongst their cytosols. Amongst the sheats, there are acellular eosinophilic bodies called as verocay bodies, formed by thin cytoplasmic fibres.[14],[18]

    Magnetic resonance imaging (MRI) is superior to other imaging modalities, for examination of the base of tongue. On MRI, a schwannoma is smooth and well-demarcated. This tumor is isointense to muscle on T1-weighted images and homogeneously hyperintense on T2-weighted images.[20]

    The differantial diagnosis of lingual schwannoma, includes malignant lesions such as squamous cell carcinomas and sarcomas and such benign lesions as granular cell tumors, salivary gland tumors, schwannomas, leiomyomas, rhabdomyomas, lymphangiomas, hemangiomas, (epi) dermoid cysts, lipomas, inflammatory lesions and lingual thyroid.[21] Clinically, a schwannoma is indistinguishable from other encapsulated benign tumors.[22] Treatment consists of complete surgical excision.[10]

    References

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