Tarun Guha1, Bonapart Chowdhury 2, Rakesh Tripura3

A 60-year-old male presented to ENT OPD with a swelling on the left side of face for past 30 years. This was insidious in onset, slowly progressive, not associated with pain, visual disturbance, bleeding from the swelling, facial deformity or impairment of hearing. He had a small ulcer over the swelling for past 2 months and hunchback for past 20 years. He had no history of diabetes mellitus, hypertension, tuberculosis, bronchial asthma, radiation exposure, trauma, surgery in the past or any other intervention.
His personal history was non-contributory with no history of substance abuse or industrial exposure. None of his family members ever suffered from neurofibromatosis or haemangioma or any other congenital anomalies. His general physical examination revealed pallor and kyphosis at upper lumbar region without any lymphadenopathy, café au lait spot or inguinal freckles. His systemic examination was within normal limits and there were no Lisch nodules.
On local examination, the tumour was dark brown multinodular soft tissue mass approximately 12 × 8 cm2 involving left zygoma, preauricular groove, 2 cm above ramus, the mandible, left angle of mouth, nasolabial fold and infraorbital margin. It was firm, noncompressible with a non-bleeding small ulcer with inverted edge having purulent discharge. No visible blood vessels or any pulsation or bruit. The entire tumour was mobile at subcutaneous plane. ENT and neck was within normal limits.
Routine blood examination revealed normocytic normochromic anaemia with HBsAg positive. X-ray LS spine suggested lumbar kyphosis and other routine investigations and pure tone audiometry were within normal limits.
FNAC from the tumour yielded blood and fibrous tissue, thus inconclusive. Radiology supported a superficial tumour.
With a preoperative differential diagnosis of neurofibroma, fibroma and haemangioma, this case is rare because of the adult-onset multinodularity with atypical pattern, gigantic size and diagnostic surprise.