Childhood Craniopharyngioma: Post-operative Long-term Follow-up And Analysis

BackgroundCraniopharyngiomas are common congenital intracranial neoplasms, thought to be derived from Rathke's pouch epithelium. It accounts for 4%-6% of all primary intracranial neoplasms and represents 9%-13% of all childhood intracranial tumors. Although histologically benign, their proximity to critical neurovascular structures, particularly the hypothalamus and the visual pathways, as a calcificated tumor with adhesions to the surrounding tissues, imparts significant challenge to neurosurgeons. For hundreds of years, numerous investigators have been working through this issue, without ideal treatment protocol because of its complicated biological behavior. Furthemore, poor quality of survival is associated with hypothalamic involvement and recurrence in craniopharyngioma. Improvement in Operation approach, risk-adapted neurosurgical method and radiotherapeutic treatment strategy remains controversial.ObjectivesThe aim of this study is aim to explore the relationship between surgical approaches and total or sub-total removal of craniopharyngioma by discussing the protection of pituitary stalk during operation and the quality of survival, so as to assess an ideal method of surgical strategies to craniopharyngioma.Methods111 cases of childhood craniopharyngioma were treated in our hospital during 2001 to 2008 with pathology confirmation. By collecting clinical data to retrospective analysis and long-term follow-up, this study reveals the discrepancy in different surgical approaches, protection of pituitary stalk during operation, and irradiation protocol.ResultsAll the cases treated surgically, total, subtotal and partial tumor removal was obtained in 45,30 and 36 patients, respectively. Pituitary stalk was found in 59 cases, in which 48 cases with protection and 11 with disjunction.85 cases (83.6%) were followed up with a term of 2 years to 9 years (mean 63.1 months) while missing 18 cases.2 cases found dead during follow up.ConclusionTotal, subtotal or partial removal of craniopharyngioma is irrelevant to surgical approaches. Trans-longitudinal-fissure approach and Key-hole approach take advantage in exposing the pituitary stalk, while trans-sphenoidal approach can rarely detect the same structure. Pituitary stalk preservation may not be related to increased recurrence rates. Total removal should be achieved with all neurosurgeons' effort during operation to avoid recurrence. Protection of the pituitary stalk seems to have no statistical significance to long-term diabetes insipidus comparing to disjunction of that structure. Recurrence can be postponed but not prevented by irradiation. Recurrence takes place even if total removal plus irradiation. During the follow-up, we found that lots of patients suffered from dwarfism and no secondary sexual characters development, which might be regarding to the Age of Onset, nature of craniopharyngioma, operation devastation, or irradiation injury, urging for further investment.