Astrocytic gliomas are the most common pediatric brain tumors; however, nonbrainstem glioblastomas are extremely rare compared with their adult counterparts. Little information is available on the clinical significance of various molecular markers in pediatric grade IV astrocytomas. The current study was focused on the molecular analysis and clinico-pathological correlations in a set of 44 tumor samples obtained from pediatric patients with nonbrainstem glioblastomas. Fluorescence in situ hybridization (FISH) with a set of 10 commercial chromosome probes (1p36, 1q25, centromere (CEP)7, EGFR, CEP9, 9p21/p16, CEP10, 10q23/PTEN, 19p13, and 19q13) was performed. Disclosed molecular abnormalities, in descending order of frequency, included polysomy 7 (72%), loss of 10q23 (61%), loss of 9p21 (52%), loss of 1p36 (41%), gain of 1q25 (25%), polysomy 9 (16%), EGFR amplification (9%), loss of 19q13 (5%), polysomy 19 (5%), and codeletion 1p36/19q13 (2%). The overall survival time was markedly shorter only for those patients whose lesions harbored deletion of 10q23/ PTEN locus (log-rank test; P ¼ 0.00007). By multivariate analysis, only loss of 10q23 locus reached an independent level of prognostic value (hazard ratio ¼ 2.88; P ¼ 0.01). There were no significant differences in patient survival for other molecular abnormalities. In conclusion, a FISH analysis of 10q23 dosage should be recommended as an ancillary laboratory method that allows further clinical subdivision of pediatric glioblastomas.

A few children with craniopharyngioma grew normally after surgery, in spite of being GH-deficient. This growth phenomenon, which is usually accompanied by obesity, was more common in patients with suprasellar tumour and is likely to be associated with the effect of IGF-1 bioavailability, which, in turn, may be modulated largely by insulin.

A review is given on the operative management of anterior third ventricle tumours, with special emphasis on the selection of the approach, the postoperative results and complications. The review is based on our own experiences with 337 cases and 340 operations, among them 198 craniopharyngiomas, 80 gliomas, 23 colloid cysts, 11 ependymomas, and 25 others. The tumours can be approached through the lamina terminals or transcallosally or using a combination of both of these approaches. The approach through the lamina terminalis is useful only in rather small tumours, because it does not allow a sufficient revision of the upper and posterior third ventricle compartments. For larger tumours the transcallosal approach is preferable. We have abandoned the approach through the anterior horn of the lateral ventricle, because it does not allow an equally good vision of both sides of the third ventricle. In some of the craniopharyngioma cases it was necessary to combine the transcallosal and subfrontal approaches in order to achieve total or subtotal tumour resection. In craniopharyngiomas total or subtotal tumour removal was possible in 66% of the children and 59% of the adult patients, with a mortality higher in adults (30%) than in children (18.5%). The main causes of complications in craniopharyngiomas were acute disturbances of hypothalamic circulation and function, with water-electrolyte imbalance and other signs of diencephalic insufficiency. In glioma cases an additional important cause of complications has been haemorrhage into the remaining tumour parts. Colloid cysts could be exstirpated without mortality, using the transcallosal approach.(ABSTRACT TRUNCATED AT 250 WORDS)

Thyrotropinomas (TSHomas) are rare pituitary adenomas, particularly in childhood. We present here the case of an 11-year-old boy with type 1 autoimmune polyglandular syndrome (APS1) and TSHoma which was diagnosed by elevated thyroid - stimulating hormone and thyroid hormones levels without evident clinical signs of hyperthyroidism. He was underwent partial resection of the tumor via transsphenoidal approach and subsequently radiation therapy. Consequently, 1 year after radiotherapy, the patient developed growth hormone deficiency, three and half years after radiation became euthyroid, and five and half years after treatment - hypothyroid. This is the first case of the coexistence of these two rare endocrine diseases in one patient.

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