Cara C Cockerill1, Brian C Gross2, Stephanie Contag3, Sarah Rein4, Eric J Moore5, Kerry D Olsen6, Laura J Orvidas7. 1. Mayo Clinic Department of Otorhinolaryngology, Head and Neck Surgery, 200 First St SW, Rochester, MN, 55905, USA. Electronic address: Cockerill.Cara@mayo.edu. 2. Mayo Clinic Department of Otorhinolaryngology, Head and Neck Surgery, 200 First St SW, Rochester, MN, 55905, USA. Electronic address: Briangross81@gmail.com. 3. Mayo Clinic Department of Otorhinolaryngology, Head and Neck Surgery, 200 First St SW, Rochester, MN, 55905, USA. Electronic address: Wise.Stephanie@mayo.edu. 4. Mayo Clinic Department of Otorhinolaryngology, Head and Neck Surgery, 200 First St SW, Rochester, MN, 55905, USA. Electronic address: reinx046@umn.edu. 5. Mayo Clinic Department of Otorhinolaryngology, Head and Neck Surgery, 200 First St SW, Rochester, MN, 55905, USA. Electronic address: Moore.Eric@mayo.edu. 6. Mayo Clinic Department of Otorhinolaryngology, Head and Neck Surgery, 200 First St SW, Rochester, MN, 55905, USA. Electronic address: Olsen.Kerry@mayo.edu. 7. Mayo Clinic Department of Otorhinolaryngology, Head and Neck Surgery, 200 First St SW, Rochester, MN, 55905, USA. Electronic address: Orvidas.Laura@mayo.edu.
Abstract
OBJECTIVE: To evaluate the presentation, treatments and outcomes in pediatric patients with salivary gland malignancies. STUDY DESIGN: Retrospective chart review (1950-2012), Prospective phone interview. METHODS: Patients ≤18 years old with a salivary gland malignancy treated at our institution were identified. Patients were also contacted by phone for a follow up survey. RESULTS: Fifty-six patients were identified. Tumor origin was 88% parotid (n = 49), 5% (n = 3) submandibular and 7% (n = 4) minor salivary glands. Time from onset of symptoms to diagnosis was over one year (mean = 14.4 years). Fifteen out of 52 patients with major gland malignancy had a locoregional recurrence and local recurrences were almost all after initial enucleation. Two of these patients died of disease (overall disease specific survival = 96%). Three out of 4 patients with minor gland malignancy had a local recurrence and two patients with high grade pathology developed metastases and died of their disease (overall survival = 50%). On long term follow up survey in 13 patients (25%), 100% reported normal facial movement and 54% described symptoms of Frey's syndrome, which is higher than other reported series in children. Recurrence was noted up to 45 years after initial treatment. CONCLUSIONS: The majority of malignant pediatric salivary gland tumors are low grade and have excellent survival, especially if found at an early stage. Minor salivary gland malignancies, particularly high grade, have a worse prognosis. Long term mild Frey's syndrome can be expected in approximately half of patients. We advocate a need for long term follow up and increased awareness among providers to diagnose these patients earlier.
OBJECTIVE: To evaluate the presentation, treatments and outcomes in pediatric patients with salivary gland malignancies. STUDY DESIGN: Retrospective chart review (1950-2012), Prospective phone interview. METHODS:Patients ≤18 years old with a salivary gland malignancy treated at our institution were identified. Patients were also contacted by phone for a follow up survey. RESULTS: Fifty-six patients were identified. Tumor origin was 88% parotid (n = 49), 5% (n = 3) submandibular and 7% (n = 4) minor salivary glands. Time from onset of symptoms to diagnosis was over one year (mean = 14.4 years). Fifteen out of 52 patients with major gland malignancy had a locoregional recurrence and local recurrences were almost all after initial enucleation. Two of these patients died of disease (overall disease specific survival = 96%). Three out of 4 patients with minor gland malignancy had a local recurrence and two patients with high grade pathology developed metastases and died of their disease (overall survival = 50%). On long term follow up survey in 13 patients (25%), 100% reported normal facial movement and 54% described symptoms of Frey's syndrome, which is higher than other reported series in children. Recurrence was noted up to 45 years after initial treatment. CONCLUSIONS: The majority of malignant pediatric salivary gland tumors are low grade and have excellent survival, especially if found at an early stage. Minor salivary gland malignancies, particularly high grade, have a worse prognosis. Long term mild Frey's syndrome can be expected in approximately half of patients. We advocate a need for long term follow up and increased awareness among providers to diagnose these patients earlier.
Authors: Simone de Campos Vieira Abib; Chan Hon Chui; Sharon Cox; Abdelhafeez H Abdelhafeez; Israel Fernandez-Pineda; Ahmed Elgendy; Jonathan Karpelowsky; Pablo Lobos; Marc Wijnen; Jörg Fuchs; Andrea Hayes; Justin T Gerstle Journal: Ecancermedicalscience Date: 2022-02-17