The Role of Radiation Therapy in Pediatric Mucoepidermoid Carcinomas of the Salivary Glands

Department of Radiation Oncology, Cancer Center Antoine-Lacassagne, Institut Universitaire de la Face et du Cou, University Nice Sophia-Antipolis, Nice, France. Electronic address: .
The Journal of pediatrics (Impact Factor: 3.74). 11/2012; 162(4). DOI: 10.1016/j.jpeds.2012.09.045
Source: PubMed

ABSTRACT OBJECTIVE: To investigate the role of radiation therapy in rare salivary gland pediatric mucoepidermoid carcinoma (MEC). STUDY DESIGN: A French multicenter retrospective study (level of evidence 4) of children/adolescents treated for MEC between 1980 and 2010 was conducted. RESULTS: Median age of the 38 patients was 14 years. Parotid subsite, low-grade, and early primary stage tumors were encountered in 81%, 82%, and 68% of cases, respectively. All except 1 patient were treated by tumoral surgical excision, and 53% by neck dissection (80% of high grades). Postoperative radiation therapy and chemotherapy were performed in 29% and 11% of cases. With a median 62-month follow-up, overall survival and local control rates were 95% and 84%, respectively. There was 1 nodal relapse. Lower grade and early stage tumors had better survival. Postoperative radiation therapy and chemotherapy were associated with similar local rates. Patients with or without prior cancer had similar outcomes. CONCLUSIONS: Pediatric salivary gland MEC carries a good prognosis. Low-intermediate grade, early-stage tumors should be treated with surgery alone. Neck dissection should be performed in high-grade tumors. Radiation therapy should be proposed for high grade and/or advanced primary stage MEC. For high-grade tumors without massive neck involvement, irradiation volumes may be limited to the primary area, given the risk of long-term side effects of radiation therapy in children. Pediatric MEC as second cancers retain a similar prognosis. Long-term follow-up is needed to assess late side effects and second cancers.

1 Follower
  • [Show abstract] [Hide abstract]
    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.ProceduresDiagnostic/therapeutic guidelines were developed and shared among Italian pediatric oncology/surgical centers.ResultsSeventeen 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. Pediatr Blood Cancer © 2014 Wiley Periodicals, Inc.
    Pediatric Blood & Cancer 11/2014; 61(11). DOI:10.1002/pbc.25139 · 2.56 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: We report the case of a patient whose main complaint was swelling on the right side of the mandible when he presented to the Ear, Nose and Throat (ENT) Service. Imaging studies revealed a large homogeneous, multilocular, expansive lesion in the body of the right mandibular ramus. The lesion was poorly enhanced following intravenous contrast injection. The patient was treated with hemimandibular surgical resection, fibula free flap reconstruction and adjuvant radiotherapy. Currently, the patient is disease free and free of posttreatment complications.
    Case Reports in Oncology 01/2014; 7(3):732-738. DOI:10.1159/000368825
  • [Show abstract] [Hide abstract]
    ABSTRACT: The management of pediatric salivary gland cancer (SGC) remains a challenge, and long-term outcome data are lacking for these rare tumors. The heterogeneity of histopathological features in 24 different SGCs further complicates unequivocal treatment recommendations across different age groups. This review aims to outline the management guidelines for pediatric SGC. Compared with adults, pediatric SGC is more often localized to the primary site and of low-grade histology. Surgery remains the treatment of choice accompanied by oncological treatment in selected patients. Elective neck dissection seems unnecessary as the occurrence of occult metastases is rare. This review provides information for clinicians to manage pediatric SGC highlighting the need for individually based treatment decisions at multidisciplinary head and neck centers.
    Current opinion in otolaryngology & head and neck surgery 01/2014; DOI:10.1097/MOO.0000000000000026 · 1.39 Impact Factor