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.

Epidemiology
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.

They are the most common tumour of the pineal region accounting for approximately
50% of all tumours, and the majority (73-86%) 10 of intracranial germ cell tumour.

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.

Clinical presentation
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.

Pathology
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%
o more frequently seen in Asian populations
 Both pineal and suprasellar region: 6-13% 11

Radiographic features
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.

CT
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
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

 T2
o isointense or slightly hyperintense to adjacent brain
o may have areas of cyst formation
o may have areas of haemorrhage (low signal)
o have a predilection for invading adjacent brain (oedema)
o 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.
Differential diagnosis
The differential can depend on location:

 for lesions in the pineal region: refer to the pineal mass article
 for lesions in the suprasellar region: refer to the suprasellar mass article

Referensi

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