Isolated Plexiform Neurofibroma of the Tongue
An 11-year-old girl was admitted to the department of E.N.T. of our institute, with the complaint of inability to close the mouth because of a large, asymmetrical, protruding tongue. The girl had difficulty in swallowing, breathing, and clear speaking since early months of her life. On palpation, the right side of the tongue was hypertrophied and the movements of the tongue were normal, but she was unable to hold the tongue completely in the mouth because of its enormous size. The swelling was soft, nontender, nonreducible, and nonpulsatile on palpation. There was also slight generalized swelling of the right side of the base of the tongue. No neck glands were palpable and functions of V, VII, X, and XII cranial nerves were intact. A magnetic resonance image was obtained to better assess the base of tongue, which revealed a 3 × 3 × 2.5 cm heterogeneously enhancing mass with infiltration in the tongue and tongue base, this lesion was hypervascular and well-circumscribed. Under general anesthesia, a deep biopsy was taken from the tongue. Gross examination of the specimen revealed irregular surface which was soft in consistency []. Histopathological examination revealed well circumscribed multiple nodular lesions composed of elongated spindle wavy cells with muscle fibers in between, these wavy cells were arranged against a myxoid background. No atypia or increase in mitosis was seen [Figures and ]. An ophthalmologic examination was carried out which showed no eye lesions of neurofibromatosis (such as Lisch nodules in the iris, ephelides on eyelids, or ectopia lentis). The skin of the whole body was examined and no café au lait spots or frecklings of the axillary and inguinal region were observed. On plain radiograph, no bone pathology was found. After the diagnostic work-up, surgical treatment was planned. Under general anesthesia, the tumor was approached intraorally, and the biopsy report was consistent with plexiform neurofibroma. Postoperatively, a remarkable reduction in the size of the tongue was achieved. The movements of the tongue were preserved, and, in addition, a significant improvement in her speech was observed. The patient has been on follow-up for 10 months and no additional treatment has been necessary.