SUBLİNGUAL NONSENDROMİK SOLİTER NÖROFİBROM OLGU SUNUMU

Year 2018, , 117 - 122, 01.03.2018

Mehmet Emrah Ceylan , Gözde Ceylan Mustafa Tunç

https://doi.org/10.17343/sdutfd.306838

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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