Revision 7 for 'Central nervous system germinoma'
Germinomas (also known as dysgerminoma, or extra-gonadal seminoma) account for only 1-2.5% of intracranial tumours. They arise from germ-cells and typically arise in the the midline, relating to the third ventricle:
- pineal gland: 80-90%
- suprasellar mass: 50%
- paraventricular parts of the basal ganglia and thalamus: 5-10%
- more frequently seen in Asian populations
Demographics and clinical presentation
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).
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. When the thalami and basalganglia are involved, then presentation is often delayed with a larger tumour at diagnosis.
Prognosis is good, with over 90% 5 year survival with chemotherapy and radiotherapy.
Germinomas are soft tissue density, enhancing masses. When present in the pineal region they appear to "engulf" normal pineal tissue and associated calcification, in contrast to pineocytomas, and pineoblastomas which are described as "exploding" the foci of calcification. Cystic components are common, found in upto 45% of cases.
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 close followup is required to identify potentially very suble abnormal pituitary stalk ehnancement 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 in only ~10% of children between 11 and 14 years of age.
Treatment and prognosis
The mainstay of treatment is with radiotherapy which offers a 90% long term cure rate. Leptomeningeal seeding or spillage during surgery are thought to be poor prognostic factors. Imaging of the entire neuraxis is therefore recommended prior to irradiation.
Depends on location.