Last Updated: March 27, 2020
- Slow-growing pineal parenchymal tumor
- WHO grade I or II
- Arises from pineocytes or pineocyte precursors
- Well-differentiated tumor without mitoses or necrosis
- Small, uniform cells arranged in sheets with intervening septae are typical microscopic features
- Mass effect on adjacent structures without invasion
- Affects all ages (mean 35-40 years old)
- No gender predilection
- Commonly presents with signs/symptoms of increased intracranial pressure secondary to 3rd and lateral ventricular obstructive hydrocephalus
- Headache, nausea, vomiting, and altered mental status
- Parinaud syndrome (upgaze palsy)
- Treatment: surgical resection; CSF shunts for hydrocephalus
- Prognosis: Better prognosis than pineoblastoma; 5-year survival 85-100%
- Peripherally-calcified, well-circumscribed pineal region mass
- Usually smaller size than pineoblastomas (<3 cm), less invasion of adjacent parenchyma
- Calcification and cysts are often present
- ± Obstructive hydrocephalus due to compression at the cerebral aqueduct
- Rare intraventricular extension, CSF dissemination or parenchymal invasion
- Well-circumscribed, iso- to hypodense pineal mass
- ± Peripheral calcification
- Moderate enhancement on contrast-enhanced CT
- T1WI: hypo- to isointense
- T2WI: hyperintense
- FLAIR: hyperintense
- T2*GRE/SWI/GRE: black signal blooming in foci of calcification at the periphery of the tumor
- T1WI+C: variable enhancement
- MRI without and with IV contrast, CT to detect calcification
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD
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