The Turkish Journal of Pediatrics 2006 , Vol 48 , Num 2
Plexiform neurofibroma of the tongue: a case report of a child
1Departments of Oto-Rhino-Laryngology, Head and Neck Surgery, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey
2 Departments of Oto-Rhino-Laryngology, Head and Neck Surgery, Mustafa Kemal University Faculty of Medicine, Antakya, Hatay, Turkey
3 Departments of Pathology, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey
Güneri EA, Akoğlu E, Sütay S, Ceryan K, Sağol Ö, Pabuçcuoğlu U. Plexiform neurofibroma of the tongue: a case report of a child. Turk J Pediatr 2006; 48: 155-158.

A three-year-old girl with a lingual plexiform neurofibroma treated by total excision is presented. Despite their occurrence in the head and neck region, neural sheath tumors are rarely encountered in the oral cavity. It is reported that 4-7% of patients affected by neurofibromatosis display oral manifestations. Neurofibromatosis is characterized by café-au-lait spots and cutaneous neurofibromas. Plexiform neurofibroma is said to be indicative of von Recklinghausen’s disease (VRD) even though it may be the only manifestation of the disease. Generally, surgical resection represents the treatment of choice and the diagnosis can only be confirmed after histological examination. Affected patients need regular follow-up to detect malignant degeneration, an early recurrence or appearance of other manifestations of VRD. Keywords : plexiform neurofibroma, tongue, child, von Recklinghausen, schwannoma, neurofibroma

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