Friday, July 9, 2010

Craniopharyngioma

Epidemiology
3% of all pediatric brain tumors (~100 cases/year in US)
Peak age 5 - 14 years but can occur in adults

Pathology
Embryonic remnant of Rathke's pouch
Most common histology "adamantinomatous"...unfortunately this does not make you into Wolverine

Imaging
Mixed cystic and solid suprasellar mass
Usually calcified
Cyst often waxes and wanes during and after treatment

Clinical
Diabetes insipidus
Growth failure
Visual field deficits
Cognitive/behavioral changes
Large tumors can compress 3rd ventricle and cause hydrocephalus, increased ICP

Surgery
Complete resection --> 80-90% tumor control with a very high rate of complications (DI, hypothalamic insufficiency)

XRT
Indicated if resection is incomplete (true for most patients)
Rx 54 - 55.8Gy
Margin to PTV = 1.3 - 1.5cm
Cyst re-expansion occurs in up to 50% of pts receiving RT
Other side effects: cognitive deficit (worse in younger patients, larger tumors, more surgeries, +hydrocephalus)
Expected control rates for subtotal resection + adjuvant RT: 3-yr EFS ~90%

Further reading/Sources
St. Jude has the answers (no, really!): PMID 16506496

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