Case Report: A rare pediatric case of secretory carcinoma of the parotid gland with high-grade components misdiagnosed as pleomorphic adenoma
By
Li Xu
Huihua He
Honglin Yan
Xiaoli Tian
Yongfei Tang
Jingping Yuan
May 29, 2026
Clinical Scorecard: Pediatric Case Study: Misdiagnosis of High-Grade Secretory Carcinoma of the Parotid Gland as Pleomorphic Adenoma
At a Glance
Category Detail
Condition High-Grade Secretory Carcinoma of the Parotid Gland
Key Mechanisms ETV6::NTRK3 gene fusion associated with translocation t(12;15)(p13;q25)
Target Population Pediatric patients, particularly those under 16 years of age
Care Setting Pediatric oncology and surgical settings
Key Highlights
Secretory carcinoma is an extremely rare malignant neoplasm in children, accounting for less than 1% of salivary gland cancers. Preoperative diagnosis is challenging due to similarities with pleomorphic adenoma. Intraoperative frozen section suggested a low-grade malignant tumor. Histopathological examination revealed high-grade features and positive immunohistochemical markers. Patient remained disease-free at 29 months follow-up after surgical resection.
Guideline-Based Recommendations
Diagnosis
Comprehensive histopathological and molecular analyses are essential for accurate diagnosis.
Management
Surgical resection is the primary treatment; adjuvant therapy may not be necessary in localized cases.
Monitoring & Follow-up
Regular follow-up is required to monitor for recurrence or residual disease.
Risks
Misdiagnosis can lead to inappropriate management and unfavorable clinical outcomes.
Patient & Prescribing Data
Children with salivary gland tumors, particularly those misdiagnosed with pleomorphic adenoma.
Surgical resection alone may be sufficient for localized high-grade secretory carcinoma.
Clinical Best Practices
Utilize fine-needle aspiration cytology cautiously due to potential for misdiagnosis. Involve a multidisciplinary team for complex cases. Consider intraoperative frozen section for real-time pathological assessment.
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