Pediatr Blood Cancer 2014;61:1961–1968
Salivary Gland Carcinomas in Children and Adolescents: The Italian TREP Project Experience Stefano Chiaravalli, MD,1 Marco Guzzo, MD,2 Gianni Bisogno, MD,3 Maria Debora De Pasquale, MD,4 Roberta Migliorati, MD,5 Francesco De Leonardis, MD,6 Paola Collini, MD,7 Michela Casanova, MD,1 Giovanni Cecchetto, MD,8 and Andrea Ferrari, MD1* Background. Salivary gland carcinomas are extremely rare in pediatric age. We report the clinical features of a series of children/ adolescents with salivary gland carcinomas prospectively registered in the Italian TREP (Rare Tumors in Pediatric Age) project. Procedures. Diagnostic/therapeutic guidelines were developed and shared among Italian pediatric oncology/surgical centers. Results. Seventeen patients were registered between 2000 and 2012, representing 19% of the cases expected to be seen based on epidemiological data. Tumors arose mainly in the parotid gland (14 cases). In most cases they were low-grade tumors (14 cases), often with a favorable clinical presentation, and low-stage disease. All patients underwent surgical resection, achieving histologically free margins in 9/17 cases. Thirteen of the 14 patients with parotid gland tumors had parotidectomy (10 total, 3 superficial), while one had a tumorectomy. Postoperative facial nerve lesions were reported in two
Key words:
# 2014 Wiley Periodicals, Inc.
adolescents; carcinoma; children; rare pediatric tumors; salivary glands; TREP project
INTRODUCTION Salivary gland carcinomas are extremely rare in pediatric age. Epidemiological research based on the North American populationbased Surveillance, Epidemiology and End Results (SEER) database identified 263 children and adolescents (aged 0–19 years) registered from 1973 to 2006, while more than 12,000 adult cases were collected during the same period [1]. Epidemiological studies have identified an annual incidence between 0.8 [1] and 1.4 [2] per million population under 20 years old. Various retrospective reports have been published on childhood salivary gland tumors, but they all included only a few cases of malignant epithelial tumor [3–28]. Like other very rare pediatric neoplasms, salivary gland carcinomas can be considered “orphan” diseases, meaning that: (a) there is a paucity of clinical and biological details about them and their clinical behavior, and many pediatric oncologists and surgeons may be unaware of the treatment options available; (b) no specific clinical or scientific organizations have been established to support their clinical management and related research; (c) it is very difficult to conduct clinical trials on them, and this makes it hard to develop evidence-based treatment guidelines, so their treatment is usually individualized; and (d) dedicated financial resources are limited. In 2000, with the aim to improving the clinical management of such tumors and stimulating research, a national-scale cooperative initiative called the TREP project (Tumori Rari in Eta` Pediatrica [Rare Tumors in Pediatric Age]) was launched in Italy, dedicated to “pediatric solid malignancies with an annual incidence
Salivary Gland Carcinomas in Children and Adolescents: The Italian TREP Project Experience Stefano Chiaravalli, MD,1 Marco Guzzo, MD,2 Gianni Bisogno, MD,3 Maria Debora De Pasquale, MD,4 Roberta Migliorati, MD,5 Francesco De Leonardis, MD,6 Paola Collini, MD,7 Michela Casanova, MD,1 Giovanni Cecchetto, MD,8 and Andrea Ferrari, MD1* Background. Salivary gland carcinomas are extremely rare in pediatric age. We report the clinical features of a series of children/ adolescents with salivary gland carcinomas prospectively registered in the Italian TREP (Rare Tumors in Pediatric Age) project. Procedures. Diagnostic/therapeutic guidelines were developed and shared among Italian pediatric oncology/surgical centers. Results. Seventeen patients were registered between 2000 and 2012, representing 19% of the cases expected to be seen based on epidemiological data. Tumors arose mainly in the parotid gland (14 cases). In most cases they were low-grade tumors (14 cases), often with a favorable clinical presentation, and low-stage disease. All patients underwent surgical resection, achieving histologically free margins in 9/17 cases. Thirteen of the 14 patients with parotid gland tumors had parotidectomy (10 total, 3 superficial), while one had a tumorectomy. Postoperative facial nerve lesions were reported in two
Key words:
# 2014 Wiley Periodicals, Inc.
adolescents; carcinoma; children; rare pediatric tumors; salivary glands; TREP project
INTRODUCTION Salivary gland carcinomas are extremely rare in pediatric age. Epidemiological research based on the North American populationbased Surveillance, Epidemiology and End Results (SEER) database identified 263 children and adolescents (aged 0–19 years) registered from 1973 to 2006, while more than 12,000 adult cases were collected during the same period [1]. Epidemiological studies have identified an annual incidence between 0.8 [1] and 1.4 [2] per million population under 20 years old. Various retrospective reports have been published on childhood salivary gland tumors, but they all included only a few cases of malignant epithelial tumor [3–28]. Like other very rare pediatric neoplasms, salivary gland carcinomas can be considered “orphan” diseases, meaning that: (a) there is a paucity of clinical and biological details about them and their clinical behavior, and many pediatric oncologists and surgeons may be unaware of the treatment options available; (b) no specific clinical or scientific organizations have been established to support their clinical management and related research; (c) it is very difficult to conduct clinical trials on them, and this makes it hard to develop evidence-based treatment guidelines, so their treatment is usually individualized; and (d) dedicated financial resources are limited. In 2000, with the aim to improving the clinical management of such tumors and stimulating research, a national-scale cooperative initiative called the TREP project (Tumori Rari in Eta` Pediatrica [Rare Tumors in Pediatric Age]) was launched in Italy, dedicated to “pediatric solid malignancies with an annual incidence
