Last Updated: March 27, 2020
- Intracranial germ cell tumor often occurring in the pineal region (extragonadal seminoma/dysgerminoma)
- WHO grade II (pure germinoma) or grade III (syncytiophoblastic giant cells)
- Associated with Down and Klinefelter syndromes and NF-1
- Polygonal primitive germ cells and lymphocytic infiltrates are common microscopic features
- Single or multiple tumors may occur
- Multiple tumors are considered metastatic rather than synchronous in the United States
- Children and young adults usually afflicted (90% <20 years)
- Marked male gender predilection (10-33:1) in pineal-region germinomas
- Females more commonly afflicted with suprasellar germinomas
- Common presenting signs/symptoms
- Pineal-region: headache due to mass effect on tectum and ventricular obstruction at the cerebral aqueduct, Parinaud syndrome (upgaze palsy)
- Suprasellar: endocrine dysfunction (diabetes insipidus, precocious puberty), visual field defects
- Laboratory findings: increased serum/CSF β-HCG
- Treatment: biopsy, low-dose radiation ± chemotherapy
- Positive prognosticators include mild elevation of β-HCG and pure (WHO II) germinoma histology
- Invasion and CSF dissemination commonly occur
- Mass within pineal or suprasellar region at or near midline
- Engulfs pineal gland, may accelerate pineal calcification
- Basal ganglia uncommonly involved
- Single or multiple locations (metastatic)
- Often infiltrates into ventricles, midbrain, thalamus; ± CSF dissemination
- Lobulated, hyperdense mass
- ± Cysts, hemorrhage
- Avid, homogeneous enhancement on contrast-enhanced CT ± CSF dissemination
- T1WI: iso- to hyperintense; normal posterior pituitary “bright spot” may be absent in suprasellar germinoma
- T2WI: iso- to hyperintense; foci of hemorrhage appear hypointense; hyperintense areas of cysts or necrosis
- FLAIR: mildly hyperintense
- DWI: diffusion restriction due to hypercellularity
- T1WI+C: avid, homogeneous enhancement including areas of CSF dissemination and parenchymal invasion
- MR spectroscopy: elevated Cho, decreased NAA
- MRI without and with IV contrast including brain and spine to detect metastases
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD
Jinguji S, et al. Factors affecting functional outcomes in long-term survivors of intracranial germinomas: a 20-year experience in a single institution. J Neurosurg Pediatr. 2013;11:454-463.
Liang L, et al. MRI of intracranial germ-cell tumours. Neuroradiology. 2002;44:382-388.
Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed.). Philadelphia, PA: Elsevier, 2016.
Sawamura Y. WHO histological classification of tumors of the central nervous system: germ cell tumors (WHO, 1993). Intracranial Germ Cell Tumors. 1998:3–4.
Ueno T, et al. Spectrum of germ cell tumors: from head to toe. Radiographics. 2004;24:387-404.
Vasiljevic A, et al. Histopathology of pineal germ cell tumors. Neurochirurgie. 2015;61(2-3):130-137.
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