Epithelial salivary glands neoplasms in children and adolescents: a forty-four-year experience

Epithelial neoplasms of salivary gland origin are relatively uncommon in children and adolescents. Over a 44-year period, there were 38 cases affecting children under 19 years of age in our Pediatric Hospital-Based Tumor Registry. Medical charts of 38 patients with epithelial neoplasms of salivary glands were reviewed. Data collected included demographic, clinical, and histological characteristics. Statistical analysis included descriptive statistics, Student t-test, and Kaplan-Meier method was used for survival analysis. The mean age was 11.8 years. There was a female preponderance of 1.9:1. The parotid gland was affected in most cases (65.8%). Twenty-seven patients had malignant tumors and eleven patients presented benign neoplasms. Pleomorphic adenoma was the most frequent benign tumor (7 out of 11) and mucoepidermoid carcinoma was the most frequent malignancy (17 out of 27). Five-year overall survival rate was 81.6% for patients with malignant tumors. Grade of differentiation was the only significant prognostic factor for patients with mucoepidermoid carcinomas. Epithelial salivary gland tumors are very rare in children. Surgery is the best option to achieve high cure rates and radiotherapy must have precise indications because of their long-term side effects in young age.

Experience with six children with fetal rhabdomyomatous nephroblastoma: review of clinical, biologic, and pathologic features

Fetal rhabdomyomatous nephroblastoma (FRN) is a rare variant of Wilms tumor. One hundred and thirty two children with kidney tumors were seen at our hospital from 1985 to 1993. Among them were 6 (4.5%) who had FRNs. Five were boys aged 8 months to 3 years; the girl was 17 months old. Three of the four with unilateral disease had tumors so large that they were considered unresectable at diagnosis. Five received pre-operative chemotherapy and three also received pre-operative radiation therapy. None of the tumors responded. Both patients with bilateral tumors died of progressive disease. Three of the four patients with unilateral disease followed for at least one year are alive for 1 to 10 years after diagnosis. FRN should be in the differential diagnosis of huge kidney tumors in children, and preoperative therapies escalated with caution since FRN is not responsive to treatment used for classic Wilms tumor.

Fetal rhabdomyomatous nephroblastoma: report of a case with unusual clinical behavior

The authors report a case of fetal rhabdomyomatous nephroblastoma that presented pulmonary metastases at diagnosis, a feature infrequently observed in this type of tumor.