Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement.

OBJECT: The current treatment of craniopharyngiomas is evolving into one of a multimodal approach in which the aim is disease control and improved preservation of quality of life (QOL). To date, an appropriate classification system with which to individualize treatment is absent. The objectives of this study were to identify preoperative prognostic factors in patients with craniopharyngiomas and to develop a risk-based treatment algorithm. METHODS: The authors reviewed data obtained in a retrospective cohort of 66 children (mean age 7.4 years, mean follow-up period 7 years) who underwent resection between 1984 and 2001. Postoperative recurrence rates, vision status, and endocrine function were consistent with those reported in the literature. The postoperative morbidity was related to hypothalamic dysfunction. The preoperative magnetic resonance imaging grade, clinically assessed hypothalamic function, and the sugeon's operative experience (p = 0.007, p = 0.047, p = 0.035, respectively) significantly predicted poor outcome. Preoperative hypothalamic grading was used in a prospective cohort of 22 children (mean age 8 years, mean follow-up period 1.2 years) treated between 2002 and 2004 to stratify patients according to whether they underwent gross-total resection (GTR) (20%), complete resection avoiding the hypothalamus (40%), or subtotal resection (STR) (40%). In cases in which residual disease was present, the patient underwent radiotherapy. There have been no new cases of postoperative hyperphagia, morbid obesity, or behavioral dysfunction in this prospective cohort. CONCLUSIONS: For many children with craniopharyngiomas, the cost of resection is hypothalamic dysfunction and a poor QOL. By using a preoperative classification system to grade hypothalamic involvement and stratify treatment, the authors were able to minimize devastating morbidity. This was achieved by identifying subgroups in which complete resection or STR, performed by an experienced craniopharyngioma surgeon and with postoperative radiotherapy when necessary, yielded better overall results than the traditional GTR.

Tectal plate lesions in children.

OBJECT: The authors characterized the clinical course of tectal plate lesions in a group of pediatric patients to identify the prognostic factors at presentation that predict progression, in an attempt to differentiate tectal hamartomas from tumors. METHODS: A retrospective review was conducted of the management of tectal plate lesions in children since the advent of magnetic resonance (MR) imaging at the authors' hospital (1984-2003). The lesion volume seen on MR images, the clinical and radiological features at presentation, and the clinical course of the population were analyzed for correlations. Forty children with tectal lesions presented in the typical delayed fashion (mean 8.5 months) with symptoms referable to hydrocephalus (93%). Fourteen children whose tumors demonstrated radiological progression (enlargement, contrast enhancement, or cystic change) were treated surgically. Histologically, 80% of the surgically treated lesions were low grade (with the other 20% consisting of one dysplasia, one high-grade tumor, and one unidentified tumor). Five patients required a second operation and one required a third. One patient died of a high-grade astrocytoma after undergoing surgery and radiotherapy; the other 39 patients remain clinically stable. The only factor predictive of tumor enlargement was lesion volume at presentation (p = 0.002). Distribution analysis revealed three subgroups based on lesion volume (< 4, 4-10, and > 10 cm3), which correlated with the clinical course of the disease. CONCLUSIONS: Children with tectal lesions should undergo contrast-enhanced MR imaging and volume assessment at the time of presentation. After hydrocephalus has been managed with endoscopic third ventriculostomy, these children require prolonged, close clinical and radiological surveillance. Lesions with a volume less than 4 cm3 were likely to be hamartomas and followed a predominantly benign course, with few atypical cases progressing. All large lesions, defined as having a volume greater than 10 cm3 at presentation, eventually required treatment, and all were histologically determined to be tumors. An argument is made for earlier treatment of larger lesions with the aim of improving outcome.