Case Report: A rare pediatric case of secretory carcinoma of the parotid gland with high-grade components misdiagnosed as pleomorphic adenoma - Report - MDSpire

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

  • 0 min

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Clinical Report: Misdiagnosis of High-Grade Secretory Carcinoma in a Child

Overview

This report details a case of high-grade secretory carcinoma of the parotid gland in a 12-year-old boy, initially misdiagnosed as pleomorphic adenoma. The patient underwent successful surgical resection and remained disease-free at 29 months follow-up.

Background

Secretory carcinoma (SC) of the parotid gland is a rare malignant neoplasm in children, often misdiagnosed due to its similarity to pleomorphic adenoma. Accurate diagnosis is critical, as misclassification can lead to inadequate treatment and poor outcomes. The lack of standardized clinical guidelines for managing SC in pediatric patients further complicates the clinical landscape.

Data Highlights

Revise to note qualitative findings are critical despite lack of numerical data.

Key Findings

  • The patient presented with a painless parotid mass, initially diagnosed via FNAC as pleomorphic adenoma.
  • Histopathological examination revealed features characteristic of high-grade secretory carcinoma, including nuclear enlargement and solid areas.
  • Immunohistochemical analysis confirmed the diagnosis with positive markers such as CK19 and SOX10.
  • Fluorescence in situ hybridization (FISH) identified an ETV6::NTRK3 gene fusion, a hallmark of secretory carcinoma.
  • The patient underwent superficial parotidectomy and functional neck dissection, with no adjuvant therapy required postoperatively.
  • At 29 months follow-up, the patient remained disease-free with preserved facial nerve function.

Clinical Implications

This case underscores the importance of comprehensive histopathological and molecular analyses in pediatric salivary gland tumors to avoid misdiagnosis. Clinicians should maintain a high index of suspicion for secretory carcinoma in children presenting with salivary gland masses, particularly when FNAC results are ambiguous.

Conclusion

Reiterate the importance of clinician awareness regarding rare pediatric tumors.

Related Resources & Content

  1. The Journal of Clinical Endocrinology & Metabolism, 2023 -- Evaluating Pediatric Patients with Potential Pheochromocytoma or Paraganglioma in Comparison to Neuroblastoma
  2. conexiant, 2023 -- Most Salivary Gland Neoplasms Found to Be Benign in Single-Center Study
  3. Acta Neuropathologica, 2010 -- Diagnostic Challenges in Oligodendroglial Tumors Exhibiting Ganglioglioma-like Features
  4. Frontiers in Oncology, 2026 -- Navigating the Diagnostic Pitfalls of Cystic and Solid Breast Masses: A Comparative Case Report of Metaplastic Carcinoma and Benign Lesions
  5. Practical pathological methods for reliable diagnosis of secretory carcinomas of the salivary gland | Discover Oncology | Springer Nature Link, 2025
  6. Radiotherapy of salivary gland tumours: 2025 update - ScienceDirect
  7. Efficacy and safety of entrectinib in children with extracranial solid or central nervous system (CNS) tumours harbouring NTRK or ROS1 fusions - PubMed
  8. Practical pathological methods for reliable diagnosis of secretory carcinomas of the salivary gland | Discover Oncology | Springer Nature Link
  9. Radiotherapy of salivary gland tumours: 2025 update - ScienceDirect
  10. Efficacy and safety of entrectinib in children with extracranial solid or central nervous system (CNS) tumours harbouring NTRK or ROS1 fusions - PubMed

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