Central nervous system germinoma
Intracranial germinomas, also known as dysgerminomas or extra-gonadal seminomas, are a type of germ cell tumour and are predominantly seen in paediatric populations. They tend to occur in the midline, either at the pineal region (majority) or along the floor of the third ventricle/suprasellar region.
Germinomas are tumours of young patients with a peak incidence of 10-12 years of age (90% of patients being younger than 20 at the time of diagnosis). They account for 3-5% of paediatric intracranial tumours but only 0.4-1% of intracranial tumours in adults 10.
Gender ratios are interestingly different depending on location. In the pineal region there is a marked male preponderance with a male to female ratio of 5-22:1 whereas in the suprasellar region, they are slightly more frequent in females with a male to female ratio of 1:1.3. Overall, due to the pineal region being most common, germinomas are seen more frequently in males.
Presentation depends on location, with compression of the tectal plate leading to obstructive hydrocephalus and Parinaud syndrome, whereas involvement on the pituitary infundibulum leads to diabetes insipidus (most common), hypopituitarism (common) or optic chiasm compression or signs of intracranial hypertension 11. When the thalami and basal ganglia are involved, the presentation is often delayed with a larger tumour at diagnosis.
Germinomas derive from germ-cells and typically arises in the midline, relating to the third ventricle 11:
- pineal gland: 80-90%, known as a pineal germinoma in this location
- suprasellar mass: 15-40%, termed a suprasellar germinoma in this location
- paraventricular parts of the basal ganglia and thalamus: 5-10%
- more frequently seen in Asian populations
- both pineal and suprasellar region: 6-13% 11
Germinomas are soft tissue density, enhancing masses. When present in the pineal region they appear to "engulf" the normal pineal tissue and can have associated central calcification, in contrast to pineocytomas, and pineoblastomas which are described as "exploding" the foci of calcification. Cystic components are commonly found in up to 45% of cases.
On CT, the high cellularity results in a degree to hyperdensity compared to adjacent brain. Usually, the mass enhances brightly.
When in the floor of the third ventricle it is typically seen filling and expanding the infundibular recess and supraoptic recess. Imaging may, however, be normal initially and if the diagnosis is suspected clinically (e.g. idiopathic hypothalamic diabetes insipidus) then a close follow-up is required to identify potentially very subtle abnormal pituitary stalk enhancement and thickening.
In the paediatric population presence of calcification in the pineal region is a useful marker of an underlying tumour, as no calcification of the pineal is seen in children below the age of 6.5 and only ~10% of children between 11 and 14 years of age.
MRI demonstrates a soft tissue mass, typically ovoid or lobulated in contour, engulfing the calcified pineal gland with the following signal characteristics 12:
- T1: isointense or slightly hyperintense to adjacent brain
- isointense or slightly hyperintense to adjacent brain
- may have areas of cyst formation
- may have areas of haemorrhage (low signal)
- have a predilection for invading adjacent brain (oedema)
- central calcification appears low signal (engulfed pineal gland)
- T1 C+ (Gd): vivid and homogeneous
Treatment and prognosis
The mainstay of treatment is with radiotherapy which offers ~85% (range 79-90%) long-term cure rate 10. Leptomeningeal seeding or spillage during surgery are thought to be poor prognostic factors. Imaging of the entire neuraxis is therefore recommended before irradiation.
Overall the prognosis is good, with over 90% 5-year survival with chemotherapy and radiotherapy.
The differential can depend on location:
- 1. Moon WK, Chang KH, Han MH et-al. Intracranial germinomas: correlation of imaging findings with tumor response to radiation therapy. AJR Am J Roentgenol. 1999;172 (3): 713-6. AJR Am J Roentgenol (abstract) - Pubmed citation
- 2. Hayashida Y, Hirai T, Korogi Y et-al. Pineal cystic germinoma with syncytiotrophoblastic giant cells mimicking MR imaging findings of a pineal cyst. AJNR Am J Neuroradiol. 2004;25 (9): 1538-40. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 3. Mootha SL, Barkovich AJ, Grumbach MM et-al. Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening, and the occult intracranial germinoma in children and adolescents. J. Clin. Endocrinol. Metab. 1997;82 (5): 1362-7. doi:10.1210/jc.82.5.1362 - Pubmed citation
- 4. Shibamoto Y, Sasai K, Oya N et-al. Intracranial germinoma: radiation therapy with tumor volume-based dose selection. Radiology. 2001;218 (2): 452-6. Radiology (full text) - Pubmed citation
- 5. Kilgore DP, Strother CM, Starshak RJ et-al. Pineal germinoma: MR imaging. Radiology. 1986;158 (2): 435-8. Radiology (abstract) - Pubmed citation
- 6. Cuccia V, Galarza M. Pure pineal germinomas: analysis of gender incidence. Acta Neurochir (Wien). 2006;148 (8): 865-71. doi:10.1007/s00701-006-0846-x - Pubmed citation
- 7. Takeuchi J, Handa H, Nagata I. Suprasellar germinoma. J. Neurosurg. 1978;49 (1): 41-8. doi:10.3171/jns.1978.49.1.0041 - Pubmed citation
- 8. Chang T, Teng MM, Guo WY et-al. CT of pineal tumors and intracranial germ-cell tumors. AJR Am J Roentgenol. 1989;153 (6): 1269-74. AJR Am J Roentgenol (abstract) - Pubmed citation
- 9. Banks KP, Brown SJ. AJR teaching file: solid masses of the pineal region. AJR Am J Roentgenol. 2006;186 (3): S233-5. doi:10.2214/AJR.05.0519 - Pubmed citation
- 10. Villano JL, Propp JM, Porter KR et-al. Malignant pineal germ-cell tumors: an analysis of cases from three tumor registries. Neuro-oncology. 2008;10 (2): 121-30. doi:10.1215/15228517-2007-054 - Free text at pubmed - Pubmed citation
- 11. Thapar K. Diagnosis and management of pituitary tumors. Humana Pr Inc. (2001) ISBN:0896034038. Read it at Google Books - Find it at Amazon
- 12. Tonn J, Westphal M. Neuro-Oncology of CNS Tumors. Springer. (2006) ISBN:3540258337. Read it at Google Books - Find it at Amazon
- 13. Osborn AG, Salzman KL, Barkovich AJ. Diagnostic Imaging. Lippincott Williams & Wilkins. (2009) ISBN:1931884722. Read it at Google Books - Find it at Amazon
Pineal region masses
The pineal region is anatomically complex and plays host to a number of unique masses and tumours as well as potentially affected by many entities seen more frequently elsewhere in the brain.
- cystic non-neoplastic lesions
- pineal parenchymal tumours
- germ cell tumours
- tumours also encountered in the pineal region
- pineal gland metastases
- vascular lesions
Pituitary region masses
- pituitary adenoma (commonest in the adult population)
- optic pathway glioma
- dermoid (CNS) / epidermoid / intracranial teratoma
- pituitary metastases
- granular cell tumour of the pituitary (pituitary choristoma)
- pilocytic astrocytoma of the neurohypophysis (infundibuloma)
- cellular infiltrates
- other lesions
Germ cell tumours
germ cell tumours
- general discussion
- germ cell tumours by location
- gonadal germ cell tumours
- ovarian germ cell tumour
- ovarian dysgerminoma
- non-seminomatous germ cell tumours
- ovarian embryonal cell carcinoma
- ovarian yolk sac tumour
- ovarian choriocarcinoma
- ovarian mixed germ cell tumour
- ovarian teratoma
- testicular germ cell tumour
- ovarian germ cell tumour
intracranial germ cell tumours
- intracranial germinoma
- non-germinomatous germ cell tumours
mediastinal germ cell tumours
- mediastinal germinoma
- mediastinal non-germinomatous germ cell tumours
- gonadal germ cell tumours