Role of Radiation Therapy in the Management of Craniopharyngiomas in Children and Young Adults
Reviewer: Heather Jones, MD
Last Modified: October 9, 2002
Presenter: D.C. Hsiung Stripp
Presenter's Affiliation: Department of Radiation Oncology, Hospital of the University of Pennsylvania
Type of Session: Poster
Treatment of craniopharyngioma has as its goal the reduction of the mass, restoration of neurologic function, prevention of recurrence, and prolongation of survival. Surgical resection, radiation, and combination surgery and radiation have been recommended. Some controversies still exist regarding the aggressiveness with which to pursue a complete resection and the role of radiation therapy in managing these tumors. The purpose of this single institution report was to evaluate the role of surgery, the extent of surgery and the role of radiation in the treatment of craniopharyngioma in children and young adults.
Materials and Methods
- Medical records of all patients with craniopharyngioma treated at the Hospital of the University of Pennsylvania (HUP) and Children's Hospital of Philadelphia (CHOP) between 1/74 and 7/01 were retrospectively reviewed.
- A total of 76 patients were identified.
- One patient was lost to follow up after surgery and was therefore not further evaluated. Histology was confirmed by a neuropathologist at CHOP.
- All patients received megavoltage radiation (median dose 54 Gy; range: 44-55.8 Gy).
- A resection was scored as gross total resection (GTR) based on the judgment of the neurosurgeon and/or post-operative imaging studies.
- Forty males and 35 females were analyzed in this study.
- Median age at of the study population diagnosis was 8.5 years (range: 1.5-24.8 years).
- Of the 75 evaluable patients, 57 received surgery alone as initial treatment while 18 received radiotherapy (XRT) following subtotal resection (STR).
- Median follow-up of surviving patients (n=68) was 7.6 years (range: 0.5-28.3 years). The 10-year actuarial OS rate was 86&, RFS was 46%, and LC rate was 51%
- Comparing those patients treated with surgery alone (n=57) with those treated with STR followed by post-op XRT (n=18), there was a significant difference in 10 year actuarial LC rates (42% vs. 81% respectively; p=0.001).
- There was no statistically significant difference in OS was noted between the two groups (89% vs. 72 % at 10 years; p=0.720) because XRT was highly effective as salvage therapy.
- The group of patients treated with XRT following relapse after surgery alone (n=22) had a similar 10 year actuarial LC as patients who received XRT immediately after STR (n=18) (77% versus 81%; p=0.801).
- For patients that had a GTR (n= 44) the 10 year actuarial LC rate of 42% with a median time to relapse of 85.5 months.
- Compared to the patients that had STR (n= 9), where the median time to relapse of was 3.5 months with 7 of 9 failing by one year.
Not all patients who undergo GTR for craniopharyngioma will relapse, and those who do may fail many years later. In patients that relapse, it is reasonable to reserve XRT as salvage therapy. The data would indicate that in patients who undergo STR, consideration should be given to administering post-op XRT as most of these patients will relapse within one year if no XRT is given. XRT is extremely effective in controlling craniopharyngiomas either when given at relapse or immediately after STR.
This is an important study as it is the largest experience to date in era of modern imaging.
Although debate exists regarding the extent to which total excision should be attempted, this report demonstrates that local tumor control and survival after subtotal removal, consisting of extensive resection or of limited biopsy and cyst aspiration and irradiation, is comparable with that achieved by radical excision. There is a body of literature that indicates that patients undergoing conservative treatment including biopsy and cyst drainage and irradiation appear to enjoy a better quality of life and demonstrate less psychosocial impairment than those initially treated with more extensive resections. We look forward to the outcomes study from the CHOP/HUP experience to further clarify these issues.
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