Abstract Details

Title Superficial Siderosis Associated with Central Neurocytoma

Topic Neuro-oncology

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 7-008

Objective We describe a patient who presented to the Emergency Department (ED) for recurrent falls and hearing loss and was later diagnosed with Central Neurocytoma (CNC).

Background CNC is a rare primary brain tumor that typically occurs in younger patients (mean age at diagnosis is 29 years). CNC, a WHO grade II brain neoplasm, is a slow-growing, usually intraventricular tumor. The most frequent presentation of CNC is nausea, vomiting, and blurry vision, which are due to increased intracranial pressure secondary to obstructive hydrocephalus. Superficial Siderosis (SS) is a common complication of hemorrhagic intraventricular tumors, particularly ependymoma and oligodendroglioma. The low-grade bleeding into the cerebrospinal fluid leads to hemosiderin deposition in the subpial layers of the brain and the spinal cord. The cardinal features in SS are sensorineural hearing loss and cerebellar ataxia, which occur in approximately 90% of cases.

Design/Methods A 32-year-old African-American man presented to the ED after a fall. He had a 5-year history of progressive hearing loss and gait instability but had not saught medical advice. In the few weeks prior to his visit to the ED, he had started to experience blurry vision, frequent falls, and speech changes. The neurologic exam revealed bilateral severe hearing loss, cerebellar ataxia, and dysarthria.

Results Brain MRI showed a large heterogeneously enhancing cystic and solid mass in the left lateral ventricle abutting the septum pellucidum, severe cerebellar atrophy, and diffuse hypointense signal consistent with SS. Subtotal tumor resection (STR) via left frontal transcallosal craniotomy approach was performed. The pathology was consistent with CNC. Despite the STR, the symptoms related to SS have remained the main cause of the patient’s disability.

Conclusions Superficial siderosis can be rarely associated with central neurocytoma. It can augment the disability resulting from the tumor itself and is definitely harder to treat.

Authors/Disclosures
Mina Lobbous, MD (Cleveland Clinic Foundation) PRESENTER	Dr. Lobbous has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Servier . Dr. Lobbous has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals . Dr. Lobbous has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Telix pharmaceuticals . Dr. Lobbous has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Springworks Pharmaceuticals . Dr. Lobbous has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Springworks Pharmaceuticals . Dr. Lobbous has received publishing royalties from a publication relating to health care.
Paula P. Warren, MD (The Headache Specialists, PLLC)	No disclosure on file
Mohamed Kazamel, MD (UAB)	Dr. Kazamel has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alnylam Pharamceuticals. Dr. Kazamel has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Akcea Therapeutics.

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