GCT/NGGCT

Question 1

What is the most common pineal tumor in children and adolescents

Answer 1

Germinomas

Question 2

What is the average age of germinomas

Answer 2

10-12 year of age

Question 3

In what racial group are they more common in?

Answer 3

seen more in Asians

Question 4

Where do germinomas arise from

Answer 4

They arise from the proximal 3rd ventricular structures and their path of natural spread is along the subepndymal ventricular lining of the 3rd and 4th ventricles

Question 5

What % and type of germinomas are multifocal

Answer 5

pure germinomas

Question 6

What % will have CSF spread at diagnosis

Answer 6

10-15%

Question 7

Describe the workup for germinomas

Answer 7

If located in the suprasellar region: diabetes insipidus, neuroendocrine anomalies, such as precocious puberty, visual defecits

If pineal location: Parinaud’s syndrome (upward gaze), convergence nystagmus

H&P
Neurolgical exam
MRI brain and spine
serum makers: AFP and Beta HCG
LP serum markers: AFP and Beta HCG

Question 8

When should a biopsy be done

Answer 8

If suspicious for a germinomas but markers are not elevated

Question 9

What should be done if there is an urgent need for hydrocephalus management

Answer 9

external drain or a 3rd ventriculostomy ( no shunts)

Question 10

What not use shunts

Answer 10

SHunts can cause seeding

Question 11

Should surgery be done

Answer 11

NO, surgery in the pineal region is highly morbid
Biopsy is all that is needed

Question 12

What labs are elevated in germinomas

Answer 12

Mild elevation of Beta HCG
AFP is never elecated

Question 13

What labs are elevated in different types of nongerminomas

Answer 13

If AFP is elevated: It is always a non germinomatous germ cell tumor
If beta HCG is moderately elevated, meaning >100- then NGGCT