Delayed Diagnosis of Enhancing Posterior Fossa Tumors Mimicking the Tela Choroidea of the Fourth Ventricle

Abstract

Posterior fossa brain tumors are common in children. Symptoms typically develop when the tumors have reached sufficient size to cause compression of adjacent neural structures or cause obstructive hydrocephalus. Many tumors in this region originate from the tela choroidea and choroid plexus of the fourth ventricle. Enhancement of the fourth ventricular tela choroidea and choroid plexus is uncommon in children, and when such enhancement is present, it may represent early tumor growth. A 5-year-old girl with a history of congenital nystagmus, for whom initial work-up was reported as negative, presented again several years later with headache, nausea, and vomiting. She was found to have a large posterior fossa lesion on repeat neuroimaging that was retrospectively seen on the first neuroimaging scan as prominent enhancement in the region of the fourth ventricular choroid plexus. The second patient presented with congenital nystagmus and a lingual tremor and was found to have a slowly growing lesion situated in the fourth ventricle. Initial imaging was read as nodularly enhancing tela choroidea, but subsequent scans revealed enlargement of the lesion. The first patient underwent gross total resection, and neuropathology was consistent with an atypical teratoid rhabdoid tumor. The patient has done well with postoperative adjuvant therapies. In the second patient, resection of the lesion revealed ependymoma; the patient has done well after adjuvant radiation therapy. Pediatric patients who have enhancing tela choroidea or choroid plexus without an obvious mass lesion of the fourth ventricle may harbor early tumors. Surveillance imaging in these patients may be warranted given the aggressive nature of certain posterior fossa tumors in children. Failure to recognize abnormal enhancement patterns in this region may lead to delayed diagnosis.