Mehmet Emrah CEYLAN, Gözde CEYLAN, Mustafa TUNÇ

SUBLİNGUAL NONSENDROMİK SOLİTER NÖROFİBROM OLGU SUNUMU

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.

Anahtar Kelimeler:

Sublingual, Nörofibrom, Nörofibromatozis

A CASE REPORT OF SUBLINGUAL NON-SYNDROMIC SOLITARY NEUROFIBROMA

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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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.