Evidence Based Questions Flashcards
Estimate the 10-yr LC with Sg alone vs. Sg + postop RT for craniopharyngioma.
Sg (GTR + STR) alone ∼42%; Sg + RT ∼84%. (Stripp DC et al., IJROBP 2004)
Estimate the 10-yr LC with adj RT vs. salvage RT.
Similar rates. Both ∼83%–84%. (Stripp DC et al., IJROBP 2004) RT can be deferred for children <5–7 yo after Sg.
What is the typical response rate to intralesional bleomycin?
Limited data, ∼65% ORR (29% CR). Median PFS is 1.8 yrs. (Hukin J et al., Cancer 2007)
Is there a dosimetric advantage to protons vs. photon therapy?
Yes. Compared to IMRT, proton therapy reduces integral dose to the brain and body. (Beltran C et al., IJROBP 2012; Boehling NS, IJROBP 2012)
What important Tx consideration is needed when treating with protons for craniopharyngioma?
Cyst dynamics. Frequent imaging is necessary when treating with protons (or any conformal techniques) to ensure adequate coverage of the cysts. (Winkfield KM et al., IJROBP 2009) Merchant et al. showed that pts with more frequent cyst surveillance during RT had a lower rate of progression. (IJROBP 2013)
What factors have been shown to correlate with inf LC in craniopharyngioma?
Size > 5 cm (Joint Center data: Hetelekidis S et al., IJROBP 1993) and RT dose < 55 Gy (Pittsburgh data: Varlotto JM et al., IJROBP 2002)
What study proposed a risk-stratification scheme to guide the aggressiveness/extent of Sg for craniopharyngioma?
A French study by Puget et al. showed significant reductions in endocrine and hypothalamic dysfunction if pts were stratified prospectively before Sg based on the degree of hypothalamic involvement: grades 0–1, attempt GTR; grade 2, STR (+ RT if >5 yo, observe if <5 yo). (J Neurosurg 2007)
What is the 10-yr OS of pts with craniopharyngioma?
10-yr OS is 70%–92%.
What is the 20-yr OS of pts with craniopharyngioma?
The 20-yr OS is 76%.
What are the 10-yr LC rates for pts with craniopharyngioma (by Tx)?
GTR/STR alone is about 42%.
Sg + RT is ∼85%.
Adj RT vs. Salvage RT both 83%–84%.
Are clinical outcomes better with aggressive total resection or limited resection followed by RT?
This is controversial. Retrospective data and systematic reviews of the literature suggest GTR versus STR + adjuvant RT have similar OS and LC, but GTR may cause more endocrine dysfunction.
Can RT be reserved for salvage treatment?
Most likely. Retrospective data from the University of Pennsylvania found that LC was worse with surgery alone versus surgery+adjuvant RT, but after accounting for the surgery alone pts who ultimately received salvage RT, LC and OS were comparable. Furthermore, retrospective data from the UK demonstrated similar outcomes among 87 pts treated with adjuvant RT versus salvage RT.
What are the late effects after treatment?
Craniopharyngioma originates in a highly sensitive area of the brain, particularly in children, and late effects are common given the long natural history of the disease. Diabetes insipidus is common after aggressive surgical resection. Neuropsychological changes including disinhibition, perseveration, attention and memory deficits are common. Endocrine effects including GH abnormalities are common in children. Additional effects of treatment near the hypothalamus include hypothalamic obesity, sleep disturbance, and defective thirst sensation. Visual impairment can occur from treatment or tumor progression. Stroke can occur due to proximity to the carotid artery and due to microvascular changes. Moyamoya syndrome (microvascular ischemia of the basal ganglia) is less common. Second malignancy (meningioma and others) can also occur.
What is the overall expected survival and the prognostic features for patients with craniopharyngioma?
Overall, the prognosis is very good, with 5- and 10-year overall survival rates of 89% to 96% and 85% to 91%, respectively, and local control rates of 63% and 53% to 75%. Poor prognostic factors include age ≤5 years, severe hydrocephalus, ≥3.5 cm tumor height in midline, presence of signs of hypothalamic disturbance, intraoperative complications, and removal of tumor observed to be adherent to hypothalamus.
De Vile, CJ, Grant, DB, Kendall, BE, et al. Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted?. J Neurosurg. 1996;85:73-81.
