Salivary gland carcinomas in children and adolescents: The Italian TREP project experience

Stefano Chiaravalli, Marco Guzzo, Gianni Bisogno, Maria Debora De Pasquale, Roberta Migliorati, Francesco De Leonardis, Paola Collini, Michela Casanova, Giovanni Cecchetto, Andrea Ferrari

Research output: Contribution to journalArticlepeer-review

Abstract

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 cases. Adjuvant radiotherapy was given to 6 patients. The overall prognosis was good: only one patient with a huge high-grade tumor experienced disease progression and died of the disease. The other 16 patients were alive in first continuous remission 1-8 years after diagnosis. In 4/17 cases, the salivary gland carcinoma was a second tumor occurring 6-9 years after another primary cancer. Conclusions: This is the first reported prospective national cooperative series of pediatric salivary gland carcinoma patients. Compliance with the TREP recommendations was high. These tumors are rarely managed by pediatric oncologists/surgeons. A broader international cooperation and better networking with otolaryngologists and head-neck surgeons expert on adult salivary gland carcinomas would be advisable.

Original languageEnglish
Pages (from-to)1961-1968
Number of pages8
JournalPediatric Blood and Cancer
Volume61
Issue number11
DOIs
Publication statusPublished - Nov 1 2014

Keywords

  • Adolescents
  • Carcinoma
  • Children
  • Rare pediatric tumors
  • Salivary glands
  • TREP project

ASJC Scopus subject areas

  • Oncology
  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Medicine(all)

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