A Rare Case of Diffuse Neurofibroma of Maxillary Sinus

Abstract

Neurofibroma is a benign slow growing tumour of the peripheral nerve sheath; frequently associated with neurofibromatosis type I. Diffuse neurofibroma of maxillary sinus is an exceedingly rare tumour. We report a case of a 17-year-old male patient presented in the ENT department with a painless swelling of the left side of the face for the last one year. CT scan revealed a soft tissue tumour with involvement of predominantly left premaxillary region. It also showed bilateral maxillary, right ethmoidal and sphenoid sinusitis with DNS and bilateral inferior turbinate hypertrophy. FNAC of the swelling showed cytomorphological features suggestive of benign mesenchymal lesion possibly benign nerve sheath tumour. Tumour excision was done. On histopathological examination, it consisted of elongating spindle cells with poorly defined, pale eosinophilic cytoplasm and tapering wavy nuclei in a fibromyxoid matrix. Many foci of meisnerian differentiation were identified. Dendritic cells containing melanin pigments were also found. The features were consistent with diffuse neurofibroma. This case proves how aggressive the tumor of this type may be, showing few clinical signs. Despite the rareness of this disease, pathologists should keep this diagnosis in mind within the range of tumours of the paranasal sinuses.