Malignant Craniopharyngioma: The Effect of Radiation Therapy on Malignant Transformation and Chemotherapy on Survival

Background: Malignant craniopharyngioma is a rare tumor with few published case reports. It can form de novo or transform from a benign variant and is associated with a dismal survival rate. We reviewed the literature for all published cases and studied the effect of radiation on the rate of malignant transformation. We analyzed the effect of chemotherapy on survival.

Methods: We reviewed the literature from 1980 onward with various search engines, such as Medline, EMBASE, and Web of Science, and identified 31 case reports, one of which was excluded. Statistical analysis using the SAS software was conducted, and a significant value was identified if p was < 0.05. Categorical variables were compared using the Chi-square analysis for parametric data and Fisher's exact test for nonparametric data. Quantitative variables were compared using the student t-test for parametric data and Wilcoxon's sum of rank for nonparametric data.

Results: There was an equal male to female distribution. The average age at malignant diagnosis is 31.11 years (±15.16) and 12.19 years (±8.41) for the average interval of benign tumor progression to malignancy. The most common clinical presentation was visual loss and/or field deficits in 26/30 (86%). Almost 11/30 patients (37%) had endocrinological deficits, with panhypopituitarism as the most common in 8/11 (73%). Fourteen patients received radiation before malignant transformation (47%) and demonstrated slowing down of malignant transformation (p = 0.0074). Gross total resection was achieved in 2/30 patients. The average time to mortality postoperatively is 5.3 months ± 4.3. Ten patients received chemotherapy, and 5 were alive at last follow-up (p = 0.046).

Conclusion: Despite the poor outcome of malignant craniopharyngiomas, radiation therapy may slow down the malignant transformation. Additionally, chemotherapy may confer a favorable survival.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021