with DTC (age range, 3-18 years) was collected from 65 clinical institutions in Germany. Characteristics of 80 females and 34 males were evaluated and the influ-1 Clinic for Nuclear Medicine and Tumor Center ence of age, gender, histology, multicentric growth, tumor stage, and lymph node University of Würzburg, Würzburg, Germany. involvement on distant metastases was tested using multivariate discriminant anal-2 Clinic for Pediatrics, University of Lübeck, Lü-ysis. Comparison between groups was performed using the Student's t test and beck, Germany. chi-square test. Correlation between incidence and age was assessed by linear regression analysis. RESULTS. The overall incidence of thyroid carcinoma in females was higher than in males, with a peak of female/male ratio occurring at puberty. The incidence of DTC correlated with age in females õ 16 years (correlation coefficient [r] Å 0.84; P Å 0.0006), which was more pronounced in children with papillary thyroid carci-noma (PTC) (r Å 0.83; P Å 0.006) but not in those with follicular thyroid carcinoma (FTC) (r Å 0.20; P Å 0.16). FTC was associated with less advanced disease (P Å 0.009), fewer lymph nodes involved (P Å 0.007), and fewer metastases (P Å 0.02) compared with PTC. Males tended to have a higher risk for distant metastases. However, statistical analysis failed to reach a level of significance (P Å 0.08). Multi-variate analysis revealed tumor stage as the only powerful factor (P Å 0.02) correlated with distant metastasis.

No abstract

Pheochromocytoma (PCC) in children is rare, genetically not well described, and often related to a poor prognosis. We detected genomic imbalances in all 14 tumors from children analyzed by comparative genomic hybridization. A combinatorial loss of chromatin from 3p and 11p was a common feature in 10 of 14 (72%) patients, which was a result of either a loss of a total chromosome 3 and a total chromosome 11 in 6 of 10 patients, or confined deletions of their p arms in 4 of 10 patients. All patients exhibiting a loss of 3p and 11p carried VHL mutations. The VHL mutations were constitutive in 9 cases and somatic and restricted to tumor DNA in the remaining tumor. On the other hand, VHL mutations were absent in 4 patients, 2 who had other familial syndromes (NF1, SDHD) and 2 with unknown etiology. Our data show that the pattern of imbalances in the tumor DNA of PCC patients strongly correlated with an underlying familial VHL mutation. Furthermore, we show that true sporadic PCC is rare in childhood. Thus, children with PCC should be checked for a related predisposing gene. This would also identify familial syndrome patients requiring long-term monitoring for other syndrome-related malignancies.

Since only 35% of the clinics answered this retrospective analysis cannot give any statement about the incidence of MET in children. As to the prognosis thyroid cancer seems to have a favourable prognosis in childhood and adolescence. In contrast, metastatic adrenocortical cancer is incurable in this age group. Carcinoids of the appendix can be treated curatively by appendectomy. To better understand the biology of MET in children and adolescence and to achieve a better prognosis for some types of these tumours, much more data are needed. For these reason a multicenter prospective therapy study for childhood MET seems to be necessary.

It can be concluded that in children and adolescents: 1. The incidence of papillary thyroid cancer is higher in females than males, with a peak at puberty. 2. The only significant factor associated with distant metastases is the advanced tumor stage. 3. Childhood thyroid cancer is frequently associated with lymph-node involvement, distant metastases and advanced tumor stage. 4. Papillary childhood thyroid cancer is more aggressive than follicular type.