Long Term Sequelae of Pediatric Craniopharyngioma ? Literature Review and 20 Years of Experience

Abstract

Craniopharyngioma are rare histologically benign brain tumors that develop in the pituitary–hypothalamic area. They may invade nearby anatomical structures causing significant rates of neurological, neurocognitive, and endocrinological complications including remarkable hypothalamic damage. Information regarding long term implications of the tumors and treatment in the pediatric population is accumulating, and treatment goals appear to be changing accordingly. In this review we aim to present data regarding long term complications of craniopharyngioma in children and adolescents and our experience from a large tertiary center. Hypothalamic dysfunction was noted to be the most significant complication, adversely affecting quality of life in survivors. Obesity, fatigue, and sleep disorders are the most notable manifestations of this dysfunction, and treatment is extremely difficult. Changes in management in recent years show a potential for improved long term outcomes; we found a trend toward less aggressive surgical management and increasing use of adjuvant treatment, accompanied by a decrease in complication rates.

Management of Electrolyte and Fluid Disorders after Brain Surgery for Pituitary/Suprasellar Tumours

“…In a recent review of children with craniopharyngiomas, 17-27% have been reported to have diabetes insipidus [14]. CDI is also the most frequent central nervous system manifestation of Langerhans cell histiocytosis, occurring in 10-50% of all patients [15,16], while in intracranial germ cell tumours CDI occurs in 82% of cases [17].…”

“…In recent years, newer conservative surgical treatments like endoscopic transsphenoidal surgery and cyst decompression with or without intracystic treatment have been introduced to reduce morbidities. This has led to less postoperative endocrine complications like permanent CDI, now seen in 50-55% of patients after conservative surgery combined with radiation surgery [14], as compared to 60-90% of patients with aggressive surgery.…”

Craniopharyngioma – Pediatric Management

“…These results suggest that the patient's aggression was maintained by inadvertent social reinforcement. Taken together, these case studies suggest that cognitive rehabilitation approaches such as goal management therapy and functional behavioral analysis may be useful in supporting survivors of childhood craniopharyngioma adapt to and compensate for cognitive and psychosocial challenges (Cohen et al, 2011).…”

A rare presentation of craniopharyngioma: delayed puberty

“…Despite high survival rates (87–95% in recent series), quality of life is frequently impaired in long-term survivors owing to sequelae caused by the anatomical proximity of the tumour to the optic nerve, pituitary gland and hypothalamus, in many cases even entanglement with the hypothalamus 5 6. One of the serious quality-of-life complication of craniopharyngioma is a delayed puberty which is a problem that can manifest before and/or after a surgical treatment 7.…”