SUBLİNGUAL NONSENDROMİK SOLİTER NÖROFİBROM OLGU SUNUMU

Year 2018, Volume: 25 Issue: 1, 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

References

• 1. Chick G, Hollevoet N, Victor J, Bianchi S. The role of imaging in isolated benign peripheral nerve tumors: A practical review for surgeons. Hand Surg Rehabil. 2016 Oct;35(5):320-329.
• 2. Louis DN. et al The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016 Jun;131(6):803-20. doi: 10.1007/s00401-016-1545-1.
• 3. Mahalle A, Reddy MGS, Kheur SM, Bagul N, Ingle Y. Solitary Non Syndromic Oral Plexiform Neurofibroma: a Case Report and Review of Literature. Dent Shiraz UnivMed Sci., 2016 September; 17(3 Suppl): 293-296.
• 4. Longo F, Maremonti P, Mangone GM, De Maria G, Califano L. Midline (dermoid) cysts of the floor of the mouth: report of 16 cases and review of surgical techniques. Plast Reconstr Surg. 2003 Nov;112(6):1560-5.
• 5. Kim DH, Murovic JA, Tiel RL, Kline DG. Operative outcomes of 546 Louisiana State University Health Sciences Center peripheral nerve tumors. Neurosurg Clin N Am 2004;15:177–92.
• 6. Abreu E, Aubert S, Wavreille G, Gheno R, Canella C, Cotten A. Peripheral tumor and tumor-like neurogenic lesions. Eur J Radiol. 2013 Jan;82(1):38-50.
• 7. Rodriguez FJ, Folpe AL, Giannini C, Perry A. Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems. Acta Neuropathol. 2012 Mar;123(3):295-319
• 8. Krol EM, El-Fanek H, Borruso J. Solitary Neurofibroma with Malignant Transformation: Case Report and Review Of Literature. Conn Med. 2015 Apr;79(4):217-9.
• 9. Kamra HT, Dantkale SS, Birla K, Sakinlawar PW, Bharia PH Plexiform neurofibroma in the submandibular gland along with small diffuse neurofibroma in the floor of the mouth but without neurofibromatosis-1: a rare case report. Ecancermedicalscience. 2013 May 2;7:313.
• 10. Bhattacharyya, Indraneel, and D. Cohen. "Oral neurofibroma." (2009). Oral Neurofibroma: Background, Pathophysiology, Epidemiology
• 11. Marocchio LS, Oliveira DT, Pereira MC, Soares CT, Fleury RN. Sporadic and multiple neurofibromas in the head and neck region: A retrospective study of 33 years. Clin Oral Investig. 2007;11:165–9.
• 12. H. Papadopoulos, N. Zachariades, and A. P. Angelopoulos, “Neurofibroma of the mandible: review of the literature and report of a case,” International Journal of Oral Surgery, vol. 10, no. 4, pp. 293–297, 1981.
• 13. Chao YT, Li WY, Chu PY. Postradiation Tumor in the Floor of Mouth.JAMA Otolaryngol Head Neck Surg. 2015 Jul;141(7):663-4.
• 14. Antoszczyk S, Rabkin SD. Prospect and progress of oncolytic viruses for treating peripheral nerve sheath tumors. Expert Opin Orphan Drugs. 2016;4(2):129-138.
• 15. Al-Omran MK, Al-Khamis AN, Malik AK. Neurosciences (Riyadh). 2006 Jan;11(1):53-5. Solitary neurofibroma of the floor of the mouth.
• 16. Maruyama M, Fushiki H, Watanabe Y. Solitary neurofibroma of the floor of the mouth: a case report. Case Rep Otolaryngol. 2011;2011:967896.