An unusual lid/orbital tumour in infants

Abstract

Purpose To present 3 cases of an unusual soft tissue lesion in infants with clinicopathological correlation. Methods Case 1: A 2‐year‐old boy presented with a rapid growing lesion in his right lateral orbit. Case 2: A 5‐year‐old girl with a left superior orbital lesion close to lacrimal sac. Both cases had clinical appearances of a dermoid and excision was performed. Case 3: A 1‐year‐old boy presented with a 6 week history of an enlarging right medial canthal mass. CT scan showed a greatly expanded nasolacrimal duct. Endoscopy revealed a polypoid lesion and multiple biopsies were taken. Results The specimens from cases 1 and 2 consisted of firm nodular tissue measuring up to 30 and 18mm in diameter respectively. Several biopsies (2‐6mm) were submitted from case 3. In all cases histology showed plump spindle cells arranged in interweaving fascicles in a collagenous stroma. Variable amount of myxoid change was present. There was no necrosis or mitotic activity. Diffuse SMA and focal CD68 positivity were encountered. Negative markers were: CD34, S100, neurofilament, desmin, MyoD1 and myogenin. The overall features were those of a nodular fasciitis. Conclusion Only four cases of orbital nodular fasciitis have been reported in children younger than 5 years. We report a series of further three cases in this age group, two masquerading as a dermoid, raising the awareness of this entity as a clinical differential diagnosis of rapidly growing lid/orbital lesions in infants.