Management benign T - Craniopharyngiomas

Where are they found + from what?

1. Near pituitary gland

2. From cell growth in foetal dev - not migrated to correct areas.

2 Types cranipharyngiomas

1. Adamantinomas CRP

2. Papillary CRP

Adamantinomas CRP

• G1

• Cystic mass

• Children peak (5-14) and adult peak (50-74)

• Mutation - CTNNB1

Papillary CRP

• G1

• Adults 5-70

• Mutation - BRAF

Both types

• Vey rare

• 1-4% paed cases

• No familial link

• High recurrence - 50%

CRP pres symptoms (5)

1. Headaches - Raised ICP/Nauseas + vomiting

2. Visual disturbances -  Temporal hemianopsia (loss vis field) - caused by optic chiasm compression

3. Hormone deficient - GHD/Erectile dysfunction

4. Weight gain

5. Fatigue

Investigations

1. MRI ± CT

2. Visual examination

3. Endocrine evaluation - hormone profile

Management - surgery + risks

• Endoscopic endonasal transphenoidal (EEA) OR craniotomy

• Risk CSF leak - runny nose

• Safe maximal resection preferred over GTR

• High recurrence rates

• Good visualisation preservation

Management - RT criteria

• Post op to reduce rate recurrence

• Unable have surgery

Fractionated Stereotactic RT tech and DOSE/F

• VMAT

• 50-55Gy/30-33F/6-6.5W

SRS

• 9-12Gy/1F

• Careful as risk of rad induced optic neuropathy

• But Frac RT is favoured

Proton beam therapy

• 100% 5yr survival in paeds

• Dec in cognitive decline

• Red in induced optic neuropathy

• Red in sec malignancies