Patients and Methods: We analyzed 135 primary operated craniopharyngioma (CP) patients and 75 CP patients, received radiotherapy/radiosurgery in 2005-2012. Neurologic, endocrine, visual functions, quality of life (QofL) and neuroimaging data before and after treatment were assessed.
Patients were divided in 2 groups according to CP location: 48,5% endosellar (ESCP), and 51,5% suprasellar (SSCP). Surgical treatment included tumor excision (total in 34,9%, subtotal in 25,7%, and partial in 24,8% cases), transnasal cyst evacuation in 10,1%, Omaya implantation in 4,5%.
Results: 5-year PFS after total resection was 79%, it was significantly (P<0.01) higher then after nonradical resection - 20% (after subtotal resection 4%, partial resection 37%, transnasal cyst aspiration 27%, and Omaya implantation 0%). 5-year PFS after subtotal or partial tumor resection followed by irradiation was 86% - similar as after radical tumor excision.
Endocrine function depended from tumor location. Patients with ESCP had more prominent pituitary deficiency before surgery (20% panhypopituitarism), than patients with SSCP (4.5% panhypopituitarism). Tumor resection caused endocrine status deterioration: 80% after surgery had panhypopituitarism and diabetes insipidus (DI).
Anterior pituitary deterioration significantly more often occurred after total tumor resection (P<0.01), than after other surgical procedures. DI occurred after total resection more often too, though it was not significant (P=0.07). The incidence of new hormone deficit after subtotal, partial resection or after transnasal cyst evacuation didn't differ. The was no endocrine deterioration after Omaya implantation.
15/75 patients before radiation had partially preserved anterior pituitary function. Surprisingly, but only in 1/15 cases irradiation induced new hormone deficit adrenal insufficiency 3.5 years after irradiation.
QoL score didn't correlate with sex, height SDS, DI, anterior pituitary hormone deficiency, or visual function. There was significant correlation between QoL score and age at surgery (R=0.4, P<0.01), and QoL score and BMI SDS (R= -0.3, P=0.001). BMI SDS slightly increased after ESCP excision (from -0.1 to 0,3, difference is NS). In patients with SSCP BMI SDS significantly increased after total resection (from 0.06 to 1.6, P<0.001), and subtotal resection (from 0.5 to 1.5, P=0.005), and not significantly increased after partial resection or Omaya implantation (from 0.5 to 1.0, P=0.3). In SSCP group QoL score was significantly higher after partial resection/Omaya followed by irradiation, than after total resection (P=0.01)
Conclusion: Optimal treatment for patients with ESCP with panhypopituitarism before surgery is total tumor excision. The optimal management of SSCP with risk of hypothalamic involvment is limited surgery, followed by irradiation.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology