Association of cerebellopontine angle atypical teratoid/rhabdoid tumors with acute facial nerve palsy in infants.
Atypical teratoid/rhabdoid tumors (AT/RTs) are highly malignant CNS tumors found almost exclusively in childhood. Although essentially universally fatal when incompletely resected, prompt diagnosis followed by early chemoradiation can improve outcomes. An AT/RT can occur extraaxially at the cerebellopontine angle (CPA) and cause acute cranial nerve deficits as the presenting sign. The authors report a series of 3 children who presented with isolated acute facial nerve palsies and in whom subsequent diagnosis of a CPA AT/RT was made. The authors propose that in young children whose presenting symptom is an acute facial nerve palsy with a CPA tumor, AT/RT should be highly suspected.
Department of Neurological Surgery, George Washington University;
Alan
A
Siu
Michaela
M
Lee
Robert
R
Rice
John S
JS
Myseros
D000208:Acute Disease; D002528:Cerebellar Neoplasms / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D002530:Cerebellopontine Angle / Q000473:pathology*; D003937:Diagnosis, Differential; D005158:Facial Paralysis / Q000209:etiology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D018335:Rhabdoid Tumor / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D013724:Teratoma / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D017211:Treatment Failure