Sellar and parasellar lesions - clinical outcome in 61 children

To evaluate clinical outcome in a 10-year consecutive series of children operated for sellar and parasellar tumors with special focus on neuropsychology and endocrinology.

We analyzed 61 children (30 female) under 18 years of age (mean age 9.9, range 1 month-17 years) operated between 2000 and 2010. Medical records were evaluated retrospectively; postoperative histologic diagnoses included 20 craniopharyngiomas, 17 gliomas, 6 pituitary adenomas and 18 rare tumor entities.

Of 61 patients, 58 (95%) were still alive at last follow-up. Three patients died, all due to progression of malignant rhabdoid tumors. Postoperative clinical morbidity consisted of endocrinological (66%), visual (60.7%) and other neurological deficits (55.9%) after a mean follow-up of 44 months. When compared to all other tumor entities in this series, craniopharyngiomas were associated with high rates of gross-total resection (p=0.008), frequent progression of residual tumor (p=0.005) scotomas (p=0.013), persistent diabetes insipidus (p<0.001), and panhypopituitarism (p<0.001). Surgically treated gliomas showed higher rates of motor weakness (p=0.004), double vision (p<0.001), and milder forms of endocrinopathy (single hormone deficits, p=0.02). In general, deterioration in school performance was associated with multiple surgeries (p=0.018) and radiotherapy (p=0.021).

Excellent overall survival in these patients is possible, however malignant rhabdoid tumors have a poor prognosis. Aggressive treatment is associated with significant morbidity. Children operated for craniopharyngioma showed an expected high rate of endocrine deterioration, whereas glioma patients had higher incidences of motor weakness and double vision. The treating physicians should be well aware of all these considerable postoperative deficits, especially when facing interdisciplinary management decisions, and for the informed consent discussions with the patient and the parents.