Natasha D Dombrowski1, Nikolaus E Wolter2, Alexandria L Irace1, Michael J Cunningham3, Jennifer W Mack4, Karen J Marcus5, Sara O Vargas6, Antonio R Perez-Atayde6, Caroline D Robson7, Reza Rahbar8. 1. Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, MA, 02115, United States. 2. Department of Otolaryngology, Hospital for Sick Children, Toronto, ON, Canada; Department of Otolaryngology - Head and Neck Surgery, Faculty of Medicine, University of Toronto, Toronto, ON, Canada. 3. Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, MA, 02115, United States; Department of Otolaryngology, Harvard Medical School, Boston, MA, 02115, United States. 4. Department of Pediatric Hematology/Oncology, Dana-Farber Cancer Institute, Boston, MA, 02115, United States; Department of Hematology/Oncology, Boston Children's Hospital, Boston, MA, 02115, United States; Department of Pediatrics, Harvard Medical School, Boston, MA, 02115, United States. 5. Division of Radiation Oncology, Boston Children's Hospital, Boston, MA, 02115, United States; Department of Radiation Oncology, Harvard Medical School, Boston, MA, 02115, United States. 6. Department of Pathology, Boston Children's Hospital, Boston, MA, 02115, United States; Department of Pathology, Harvard Medical School, Boston, MA, 02115, United States. 7. Department of Radiology, Boston Children's Hospital, Boston, MA, 02115, United States; Department of Radiology, Harvard Medical School, Boston, MA, 02115, United States. 8. Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, MA, 02115, United States; Department of Otolaryngology, Harvard Medical School, Boston, MA, 02115, United States. Electronic address: Reza.rahbar@childrens.harvard.edu.
Abstract
INTRODUCTION: Mucoepidermoid carcinoma is a rare malignant salivary gland neoplasm in the pediatric population. Few studies have discussed best practice with respect to diagnosis and treatment in children. OBJECTIVE: To present our institution's experience with the evaluation and management of pediatric mucoepidermoid carcinoma of the head and neck. METHODS: Retrospective chart review of patients under 20 years of age diagnosed with mucoepidermoid carcinoma of the head and neck between 1998 and 2017. Data assessed includes demographics, clinical presentation, imaging examinations, histopathology, treatment, complications, local recurrence, distant metastasis, and follow-up. RESULTS: Sixteen patients (10 female, 6 male) were identified with a median age of 12.9 (IQR: 10.9-15.0) years. Tumors were located within the parotid gland (n = 11, 68.8%), accessory lobe of the parotid gland (n = 2, 12.5%), palate (n = 2, 12.5%), and submandibular region (n = 1, 6.3%). In 9 patients (56.3%) a neoplastic etiology was suspected based on the clinical and/or radiographic findings and confirmed pathologically on biopsy or excision. All patients were treated surgically and five patients required adjuvant radiotherapy. One patient had recurrence requiring re-excision. Seven patients (43.8%) had transient facial paresis post-operatively and one had Frey syndrome. Median follow-up time was 59.7 months (IQR: 18.9-99.3). CONCLUSION: The malignant nature of mucoepidermoid carcinoma requires comprehensive, multidisciplinary management. Imaging and tissue sampling by fine needle aspiration give clinicians the best insight into location and nature of the mass. Complete surgical excision with attention to preservation of facial nerve and achieving negative margins is desired.
INTRODUCTION:Mucoepidermoid carcinoma is a rare malignant salivary gland neoplasm in the pediatric population. Few studies have discussed best practice with respect to diagnosis and treatment in children. OBJECTIVE: To present our institution's experience with the evaluation and management of pediatric mucoepidermoid carcinoma of the head and neck. METHODS: Retrospective chart review of patients under 20 years of age diagnosed with mucoepidermoid carcinoma of the head and neck between 1998 and 2017. Data assessed includes demographics, clinical presentation, imaging examinations, histopathology, treatment, complications, local recurrence, distant metastasis, and follow-up. RESULTS: Sixteen patients (10 female, 6 male) were identified with a median age of 12.9 (IQR: 10.9-15.0) years. Tumors were located within the parotid gland (n = 11, 68.8%), accessory lobe of the parotid gland (n = 2, 12.5%), palate (n = 2, 12.5%), and submandibular region (n = 1, 6.3%). In 9 patients (56.3%) a neoplastic etiology was suspected based on the clinical and/or radiographic findings and confirmed pathologically on biopsy or excision. All patients were treated surgically and five patients required adjuvant radiotherapy. One patient had recurrence requiring re-excision. Seven patients (43.8%) had transient facial paresis post-operatively and one had Frey syndrome. Median follow-up time was 59.7 months (IQR: 18.9-99.3). CONCLUSION: The malignant nature of mucoepidermoid carcinoma requires comprehensive, multidisciplinary management. Imaging and tissue sampling by fine needle aspiration give clinicians the best insight into location and nature of the mass. Complete surgical excision with attention to preservation of facial nerve and achieving negative margins is desired.