[Craniopharyngiomas]

Abstract

Craniopharyngiomas are rare benign epithelial tumors, arising from the pituitary stalk or gland and developing in the sellar and suprasellar region, affecting both adults and children. Incidence is 0.5 to 2 new cases per million inhabitants. Clinical features essentially include visual disturbances, endocrine deficiencies, and neurological signs. Initial signs are often visual loss and increased intracranial pressure in children, growth and pubertal delay in teenagers, visual disturbances or cognitive impairment in adults. Diagnosis is made on MRI and CT scan, demonstrating a sellar or suprasellar tumor, heterogeneous, with frequent calcifications. Craniopharyngiomas can be classified depending on their locations from the sella, the diaphragma sellae, and upon their origins from the pituitary stalk or the infundibulum. They can also be classified depending on the location from the optic chiasm and the third ventricle. This classification allows surgical series comparison, which is of importance since developments and extensions of the tumor can explain surgical difficulties. The management of this lesion is still controversial. Because it is an extra-cerebral benign lesion, the ideal goal of treatment should be complete tumor removal with improvement of altered visual functions, minimal deterioration of endocrine function, and no neuropsychological impairment. But the situation of the tumor, its relationship with third ventricle, hypothalamus, optic tract, vascular structures make its removal often difficult. However, great progresses have been realized in surgical treatment, resulting in a dramatic improvement of the prognosis of craniopharyngiomas. Nowadays, one can expect total removal in 60 to 70% of patients, subtotal removal in 20 to 30%, and partial removal in 10%. When total removal is impossible, radiotherapy may reduce the risk of a poor evolution. Recurrences are a problem in 15% of patients with total removal, 35% in subtotal removal, 70% in partial removal. If radiotherapy has not been performed as first treatment, it is efficient in 80% of recurrences. Long term follow up is necessary in these patients, due to medical management of endocrine, visual and psychological problems, and risk of late recurrence. With close involvement in this management, most of patients may enjoy a good outcome, 80% being able to return to normal active life.