Last Updated: March 27, 2020
- Malignant, invasive pineal parenchymal tumor arising from embryonic pineocyte precursors (PNET)
- WHO grade IV
- Invades adjacent structures often including the cerebral aqueduct
- CSF dissemination common
- Hypercellularity and increased mitoses are characteristic features
- Associated with germline DICER1 mutations (DICER1 syndrome) and RB1 (bilateral retinoblastomas and pineoblastoma)
- Most commonly afflicts children in the 1st decade of life (mean age 3 years)
- No clear gender predilection
- Common presenting signs/symptoms
- Hydrocephalus: headache, nausea, vomiting, altered mental status, papilledema
- Parinaud syndrome (upgaze palsy)
- Absent serum tumor markers (differentiates from many germ cell tumors)
- Prognosis: poor prognosis; 5-year survival ~50%
- Lobulated, poorly-marginated pineal mass
- May be indistinguishable from lower grade pineal tumors
- Often larger and more invasive than pineocytomas
- Peripheral (“exploded”) calcifications common
- Commonly invades adjacent structures including cerebral aqueduct, corpus callosum, thalamus, brainstem, and vermis
- May see superior displacement of cerebral veins and mass effect on the tectum
- Heterogeneous density, irregular pineal mass
- Peripheral calcification
- Variable, patchy enhancement on contrast-enhanced CT
- T1WI: heterogeneously hypo- to isointense
- T2WI: heterogeneous signal; hyperintense peritumoral edema within the surrounding brain parenchyma common
- T1WI+C: heterogeneous enhancement
- DWI: diffusion restriction in solid component of mass common
- MR spectroscopy: elevated Cho, decreased NAA
- MRI without and with IV contrast including both brain and spine due to risk of CSF dissemination
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD
Cuccia V, et al. Pinealoblastomas in children. Childs Nerv Syst. 2006;22:577-585.
Gasparetto EL, et al. Diffusion-weighted MR images and pineoblastoma: diagnosis and follow-up. Arq Neuropsiquiatr. 2008;66:64-68.
Hirato J, et al. Pathology of pineal region tumors. J Neurooncol. 2001;54: 239-249.
Nakamura M, et al. Neuroradiological characteristics of pineocytoma and pineoblastoma. Neuroradiology. 2000;42:509-514.
Osborn AG, Salzman, KL, Jhaveri, MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier, 2016.
Reis F, et al. Neuroimaging in pineal tumors. J Neuroimaging. 2006;16:52-58.
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