Giant craniopharyngiomas in children: short- and long-term implications

Craniopharyngiomas (CPs) are histologically benign tumors, yet they may carry an unfavorable prognosis. "Giant" tumors are associated with worse prognosis. Our aim was to evaluate the impact of tumor's size on different short- and long-term clinical factors.

Between 2002 and 2012, a total of 36 consecutive CP patients less than 18 years of age and with at least 12 months of post-operative follow-up (FU) underwent a total of 54 operations for excision of CPs. Gross total resection (GTR) was the goal for all the first surgical resections. Twenty-seven patients were identified as eligible for inclusion in this study. Data were retrospectively collected by reviewing pertinent clinic/office notes and inpatient records as well as pre- and post-operative imaging. Long-term neurosurgical, ophthalmological, and endocrinological outcomes were obtained from records of the most recent FU office visit. Statistical analysis was performed to compare data from patients with tumors greater than or equal to 4.5 cm (nine patients) to those with smaller ones (<4.5 cm; 18 patients).

Mean age at the time of surgery was 5.4 years (median 5 years, range 1.3-15.3 years) for patients in the large-tumor group (LTG) and 8.9 years (median 9.6 years, range 2.1-17.1 years) for the small-tumor group (STG). Average duration of follow-up was 82.1 and 105.4 months for LTG and STG patients, respectively. There was a noticeable difference in the rate of emergent surgeries between the two groups (33.3 vs. 5.5 % in the LTG and STG, respectively; p = 0.055) as well as in recurrent surgeries (RR = 3.76; CI = 95 %, 1.793-7.877) and radiotherapy (RR = 2, 95 % CI 0.775-5.154). Rates of residual tumor on both initial post-operative imaging and last FU imaging were significantly increased in LTG patients (44.5 vs. 27.7 % and 66.6 vs. 16.6 %; respectively). Progression-free survivals (PFS) assessed at 2 and 5 years were 33.3 vs. 73.3 % (RR = 2.2, 95 % CI = 0.171-1.202) and 33.3 vs. 53.3 % (RR = 1.6, 95 % CI 0.221-1.765) in favor of smaller tumors. No difference in 2-, 5-, and 10-year overall survival was found. We found no significant difference in mean BMI at last follow-up between the two groups (23.83 ± 4.86 and 27.33 ± 8.09, respectively; p = 0.27), although significantly more patients in the LTG had shorter stature (mean height SDS -1.72 ± 1.88 and -0.17 ± 1.08 in LTG and STG patients, respectively; p = 0.027).

Tumor's size has significant impact on the management of CP in children. It affects both short-term factors (initial symptoms, urgency of surgical resection, extent of resection, and perioperative morbidity) as well as long-term parameters (PFS, rate of adjuvant treatments/recurrent surgeries, and metabolic/endocrinal/ophthalmological and functional outcomes). We think that a proper, world-wide accepted definition of what is "large," "giant," or even "monstrous" CP should be established. This will enable carrying multi-institutional studies on a larger group of patients, allowing further determining the importance of tumor's size in the management and outcome of craniopharyngiomas in children.