Long-term results of treatment for craniopharyngioma in children.
Results of the treatment of 27 children with craniopharyngioma are reported. A subfrontal pterional approach was used in 55.6% of cases, a transsphenoidal and a transcallosal or transcortical approach in 25.9% and 18.5% of cases, respectively. Radical removal was the goal of surgery and was achieved in 70.8% of cases treated as primary surgery. The operative mortality was 3.7% and was due to hypothalamic failure. Most (81.4%) patients were followed up, for a mean of 7 years. Patients were evaluated according to a functional evaluation scale and outcome categories proposed by us. The scale takes account of tumor (recurrences and their eventual evolution); visual functions; endocrine functions (mainly hypothalamic), attainment of endocrine balance and drug regimen; headache; and psychosocial function. Recurrences were observed in 17.6% of patients treated with radical surgery and in 42.8% of those treated with limited surgery plus radiotherapy. A progressive amelioration of visual, endocrine and neuropsychosocial functions from the intervention to follow-up was observed in the majority of patients. Complete tumor excision was associated in 85% of cases with a low score on the functional scale, reflecting a high functional performance. Adequate substitution therapy maintained endocrine balance in 81% of patients. Since the intervention a progressive decrease in the number and dosages of medications has been observed. The majority of patients were again able to lead a normal social life. Small stature, obesity, headache, and emotional and sexual disturbances were frequent cause of long-term disability even despite adequate drug regimens. The functional evaluation scale we propose is a simple and effective tool that can be easily used during routine evaluation of patients with craniopharyngioma.
Villani
R M
RM
Institute of Neurosurgery, University of Milan, Ospedale Maggiore Policlinico, IRCCS, Italy.
Tomei
G
G
Bello
L
L
Sganzerla
E
E
Ambrosi
B
B
Re
T
T
Giovanelli Barilari
M
M
D000293:Adolescent; D001932:Brain Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D002648:Child; D003397:Craniopharyngioma / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local; D011183:Postoperative Complications; D012189:Retrospective Studies; D013997:Time Factors; D014057:Tomography, X-Ray Computed