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Background. The aim of the study was to charac- terize the distribution of anterior skull base (ASB) cancers in the pediatric population and to identify predictors of outcome. Methods. This was an international study of 1307 patients undergoing craniofacial surgery for malignant tumors; 6.4% (n 5 84) among these were � 21 years old. Results. The most common histologic type was sarcoma (40%), followed by squamous cell carcinoma (14%). Higher incidence of tumors was found in older children (7.5-21 years) compared to younger children. The 5-year disease-specific and overall survival rates were 61% and 55%, respectively (median follow-up of 30 months). The best prognosis was associated with low-grade sarcomas and the worst with salivary and squamous cell carcinomas. High-grade sarcoma represented an intermedi- ate risk group. On multivariate analysis, prior radiotherapy and histology were independent predictors of survival. Conclusion. Surgery for malignant tumors involving the ASB is feasible and safe in children. Histology is significant determi- nant of poor outcome in this population. V C 2008 Wiley Periodi

Intestinal-type adenocarcinoma (ITAC) of the nasal cavity and paranasal sinuses is an uncommon tumor associated with occupational exposure to dusts of different origin. Few investigations addressed molecular alterations in ITAC mainly focused on TP53, K-ras and H-ras gene mutations. The occurrence of TP53, p14(ARF) and p16(INK4a) deregulation and H-ras mutations was investigated in 21 consecutive and untreated ITACs cases, 17 with known professional exposure. No H-ras mutations were found. In patients with known exposure, cumulative evidence of TP53 or p14(ARF) alterations accounted for 88% and the evidence of p16(INK4a) alterations for 65%, respectively. TP53 mutations were present in 44% of the ITACs, consisted of G:C--A:T transitions in 86%, and involved the CpG dinucleotides in 50% of the cases. LOH at the locus 17p13 and an uncommon high rate of p53 stabilization were detected in 58% and 59% of the cases, respectively. p14(ARF)and p16(INK4a) promoter methylation accounted for 80% and 67% respectively, and LOH at the locus 9p21 occurred in 45% of the cases. Interestingly, all dust-exposed tumors with p16(INK4a) alterations shared TP53 or p14(ARF) deregulation. The present results show a close association of this occupational tumor with TP53, p14(ARF) and p16(INK4a) gene deregulation. Given the important role that these genes play in cell growth control and apoptosis, the knowledge of ITAC genetic profile may be helpful in selecting more tailored treatments.