Garcin syndrome resulting from a giant cell tumor of the skull base in a child

Abstract 

Garcin syndrome is characterized by a progressive ipsilateral involvement of cranial nerves, culminating in paralysis of all or at least seven of them, without sensory or motor long-tract disturbance, with no intracranial hypertension, and with osteoclastic involvement in the skull base on radiographic computed tomography. Giant cell tumor is a primary bone tumor rarely affecting the skull base. An 8-year-old female presented with a 3-month history of increasingly worsening right otalgia, tinnitus, hearing loss, right facial numbness, and diplopia. She was admitted with a 2-week history of swallowing difficulties, voice change, and right shoulder pain. Neurologic examination disclosed unilateral paralysis of the right fifth through twelfth cranial nerves, with no other abnormal neurologic findings. Skull radiographic computed tomography revealed lytic lesions in the right temporal petrous portion. Computed tomographic scan indicated a destructive mass involving the right greater wing of the sphenoid bone and temporal petrous apex. Magnetic resonance imaging demonstrated a tumor arising from the temporosphenoidal region, infiltrating neither the brain nor the brainstem. No hydrocephalus was observed. Biopsy revealed giant cell tumor. Posterior treatment consisted of radiotherapy. At an 8-year follow-up, the patient was well but with functional sequelae. There is no magnetic resonance imaging evidence of tumor growth. No other giant cell tumor presenting as Garcin syndrome is known to have been reported.