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Abstract Details

CNS non-germinomatous germ cell tumor causing obstructive hydrocephalus, Parinaud’s syndrome, and secondary Parkinsonism
Neuro-oncology
P3 - Poster Session 3 (5:30 PM-6:30 PM)
7-016
NA
CNS germ cell tumors occur at an incidence of 0.10 per 100,000 in the United States, belonging to a spectrum of extragonadal germ cell malignancies thought to arise from aberrant rostral-to-caudal migration during embryogenesis. In the brain, the majority are located in the pineal and suprasellar regions. Response to treatment depends on the degree of differentiation and recent data indicate that six alternating cycles of carboplatin/etoposide and ifosfamide/etoposide offer the best result for non-germinomatous germ cell tumors (NGGCT).
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A 23-year-old man with history of hepatic abscess complicated by portal vein thrombus and secondary splenomegaly being treated with warfarin presented in early June 2018 with headache, blurry vision, and fatigue. Head CT and gadolinium-enhanced MRI revealed a left thalamic mass with compression of the cerebral aqueduct, requiring an immediate ventriculoperitoneal shunt for obstructive hydrocephalus. He was discharged and re-presented in late June 2018 with worsening encephalopathy, Parinaud’s syndrome, and secondary Parkinsonism. A stereotactic biopsy was performed with pathology showing a NGGCT with yolk sac component. His serum and cerebrospinal fluid AFP were 1011 ng/ml and 677.9 ng/ml, respectively, an increase from 115.8 ng/ml and 0.6 ng/ml in early June. He was initiated on six alternating cycles of carboplatin/etoposide and etoposide/ifosfamide, with an appropriate decrease in AFP and tumor size. The patient’s clinical course was complicated initially by shunt malfunction and hydrocephalus, requiring repeated ventriculostomies, which later resolved when the tumor responded to treatment. He also received carbidopa-levodopa for secondary Parkinsonism and intensive rehabilitation to regain his motor functions.


This case demonstrates positive response of a pineal region NGGCT to alternating cycles of carboplatin/etoposide and ifosfamide/etoposide in a young adult. Complications that developed include obstructive hydrocephalus, Parinaud’s syndrome, and secondary Parkinsonism, which can all be addressed with appropriate interventions.


Authors/Disclosures
Sydni Cole, MD
PRESENTER
Dr. Cole has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Aquifer.
Sasmit Sarangi, MD, MBBS (Rhode Island Hospital/Brown University) The institution of Dr. Sarangi has received research support from Fore biotherapeutics.
No disclosure on file
Eric T. Wong, MD, FAAN (Rhode Island Hospital) Dr. Wong has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novocure. Dr. Wong has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ZaiLab. Dr. Wong has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Turning Point Therapeutics. Dr. Wong has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Imvax. The institution of Dr. Wong has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novocure. The institution of Dr. Wong has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Turning Point Therapeutics. The institution of Dr. Wong has received research support from Novocure. Dr. Wong has received intellectual property interests from a discovery or technology relating to health care. Dr. Wong has received publishing royalties from a publication relating to health care.