Introduction: Craniopharyngiomas are rare epithelial tumors that arise from remnants of Rathke's pouch. Although benign histologically, these tumors frequently shorten life and effect significant morbidity due to their perilous proximity to eloquent neural and vascular structures. Accordingly, craniopharyngiomas are treated surgically. Alternatives in surgical approach, goal for extent of tumor excision, and use of adjuvant radiation delineate treatment options. Consensus as to optimal management remains elusive in light of conflicting data regarding rates of tumor recurrence and of significant morbidty following different surgical management modalities.
Methods: Utilizing a large single-surgeon case series of adult patients, we review overall survival, recurrence and visual morbidity following surgery in pursuit of GTR, and without adjuvant RT. Results are compared to published ranges to appreciate the role of aggressive resection without RT in management. A total of 40 patients were selected for review.
Results: Average age was 50 + 17 years with follow-up of 85 + 9.5 months. Overall survival was 87.5%, within the reported range of 83.0-92.7%, and 1 and 3 year survival rates were slightly better than reported ranges at 95% and 92.5%, respectively. Recurrence rate was 17.5%, in the lower end of the reported range, though significantly reduced in those who received GTR at 4% vs STR at 41.7%, (p=0.004). RFS in patients at 2 and 5 years were 90.9% and 75.0%, respectively, both above published ranges. Preopt visual symptoms were present in 92.5% of reviewed patients; 15% demonstrated a worsening of visual symptoms postopt, a rate less than reported. Remarkably, 78.4% of those with preopt symptoms reported an improvement in visual symptoms.
Conclusions: These findings suggest that the pursuit of GTR of craniopharyngioma by an experienced surgeon, without use of adjuvant radiation therapy, is an appropriate strategy for treatment in adult patients. Furthermore, this strategy may allow for resolution of tumor induced visual deficits.
Patient Care: Better inform surgeons and neuro-oncologist as to the optimal management of craniopharyngioma.
Learning Objectives: 1. To appreciate the difficulty and variability in treatment of this disease, utilizing variable goals of resection and use of adjuvant radiation, as well as to appreciate the correspondingly broad ranges of reported outcomes.
2. To appreciate to success of surgery with the intention of aggressive gross total resection in the hand of an experienced surgeon without adjuvant radiation, with regard to outcome measures compared to published series.
3. To appreciate the potential of this surgical treatment paradigm to restore more functional vision in the majority of patients who present with visual deficits.
References: 1. Michael E Sughrue et al., “Endocrinologic, Neurologic, and Visual Morbidity After Treatment for Craniopharyngioma,” Journal of Neuro-Oncology 101, no. 3 (June 10, 2010): 463–476, doi:10.1007/s11060-010-0265-y.
2. B E Zacharia et al., “Incidence, Treatment and Survival of Patients with Craniopharyngioma in the Surveillance, Epidemiology and End Results Program,” Neuro-Oncology 14, no. 8 (July 30, 2012): 1070–1078, doi:10.1093/neuonc/nos142.
3. Isaac Yang et al., “Craniopharyngioma: a Comparison of Tumor Control with Various Treatment Strategies,” Neurosurgical FOCUS 28, no. 4 (April 2010): E5, doi:10.3171/2010.1.FOCUS09307.
4. N Karavitaki, “Craniopharyngiomas,” Endocrine Reviews 27, no. 4 (March 16, 2006): 371–397, doi:10.1210/er.2006-0002.