SUBLİNGUAL NONSENDROMİK SOLİTER NÖROFİBROM OLGU SUNUMU

Abstract

GİRİŞ:

                Nörofibrom periferik sinir kılıfından köken alan yavaş büyüyen benign bir tümörüdür. Baş ve boyuna yerleşik nörofibromların yüzde 6’sı oral kavitede görülmektedir. Oral kavitede ağız tabanına yerleşik sendromik olmayan soliter nörofibrom oldukça az görülmektedir ve bu olgu sunumunun amacı nadir görülen bu hastalığın tanı ve tedavisine dikkat çekmektir.

OLGU SUNUMU:

                Otuz iki yaşında erkek hasta kliniğimize dil altında şişlik şikayetiyle başvurdu. Fizik muayenede ağız tabanında dili yukarı iten yaklaşık 3 cm büyüklükte üzeri normal mukoza ile kaplı kitle mevcuttu. Dil hareketleri normaldi ve tat duyusunda azalma tariflemiyordu. Sistemik muayenede herhangi bir cilt lezyonu veya iskelet anomalisi yoktu.

                Hastaya cerrahi tedavi önerildi. İntraoral yolla kitleye ulaşıldı, çevre dokulardan diseke edilerek çıkarıldı. Yapılan histolojik S-100 (+) olduğu izlenerek nörofibroma tanısı kesinleştirildi.

TARTIŞMA:

Nörofibromlar klasik olarak lokalize, diffüz ve pleksiform olarak üçe ayrılır. Lokalize nörofibrom genellikle soliter, sendromik olmayan bir kitledir. Genellikle 20-30 yaş aralığında tespit edilir ve intranöral, infiltratif olmayan tipte büyüme gösterir. Nörofibromun malign dönüşüm prevelansı yüzde 2-29 olarak bildirilmiştir. Uzun süredir mevcut kitlenin hızla büyümesi malign dönüşüm olabileceğini düşündürmelidir. Multipl nörofibromların varlığı NF1 tanısında önemlidir. Nörofibrom ön tanısı düşünüdüğünde hastanın genel tıbbi muayenesinde cafe au lait lekelerinin varlığı aranmalı ve hastadan detaylı bir özgeçmiş hikayesi alınmalıdır. Eşlik eden iskelet anomalileri, mukozal multipl nörofibrom varlığı multipl endokrin neoplazi tanısıyla genetik analiz gerektirebilir.

Sendromik olmayan nörofibromun schwann hücrelerinden veya perinöral fibroblastlardan köken aldığı düşünülmektedir. Periferik sinir kılıfı tümörlerinin tedavisi cerrahidir.

Oldukça nadir görülen bu hastalığın ağız tabanında şişlik şikayeti ile başvuran hastada ön tanılar arasında yer alması gerektiği, özellikle malign dönüşüm riski taşıyan bu kitlenin sendromik hastalıklarla ilişkili olabileceğinin bilinmesi önemlidir.

Keywords

• Sublingual,Nörofibrom,Nörofibromatozis

A CASE REPORT OF SUBLINGUAL NON-SYNDROMIC SOLITARY NEUROFIBROMA

Abstract

INTRODUCTION:

Neurofibromas located in head and neck constitute approximately twenty five percent of all neurofibromas and six percent of those are observed in oral cavity. Involvement of tongue, hard and soft palate, gingiva, mandibula, tongue base and rarely floor of the mouth are observed in oral cavity. (1)

Nonsyndromic solitary neurofibroma located in the flow of the mouth in oral cavity are observed quite low, and the purpose of this case report is to attract attention to the diagnosis and treatment of this rare disease.

CASE:

Thirty-two year-old male patient applied to our clinic with swelling under his tongue.  In physical examination, a mucosa covered mass was determined that is approximately 3 cm in size and pushing the tongue upwards. In the examination with bimanual palpation, mobile, painless and hard mass was observed to extend from the floor of the mouth to submandibular region.

Surgical treatment was recommended to the patient. The excision was decided to be performed under general anesthesia and through intraoral route. Diagnosis of neurofibroma was finalized since immunohistochemical study observed to be S-100 (+).

No findings suggesting neurofibramatosis or multiple endocrine neoplasia syndrome were determined in the repeated systemic examination. There were no cafe au lait spots on the body and family history for neurofibramatosis was negative.

CONCLUSION:

Localized neurofibromas are usually solitary, non-syndromic. It is usually detected in the age range of 20-30 years and shows intraneural, non-infiltrative type growth. The presence of multiple neurofibromas is important in NF1. When considering neurofibroma pre-diagnosis, the presence of cafe au lait spots should be sought in the general medical examination of the patient and a detailed resume story should be taken from the patient. Concomitant skeletal anomalies, presence of mucosal multiple neurofibromas, multiple endocrine neoplasia may require genetic analysis.

Non-syndromic neurofibroma is thought to originate from Schwann cells or from perineural fibroblasts. The treatment of peripheral nerve sheath tumors is surgery.

It is important for this very rare disease to be considered among pre-diagnoses for patients who have applied with swelling on the floor of their mouth, and especially to be recognized that this mass, which has a malignant transformation risk, may be associated with syndromic diseases.

Keywords

• Sublinggual,Neurofiroma,neurofibromatosis

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