Case Report: A rare pediatric case of secretory carcinoma of the parotid gland with high-grade components misdiagnosed as pleomorphic adenoma - Scorecard - 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 Scorecard: Pediatric Case Study: Misdiagnosis of High-Grade Secretory Carcinoma of the Parotid Gland as Pleomorphic Adenoma

At a Glance

CategoryDetail
ConditionHigh-Grade Secretory Carcinoma of the Parotid Gland
Key MechanismsETV6::NTRK3 gene fusion associated with translocation t(12;15)(p13;q25)
Target PopulationPediatric patients, particularly those under 16 years of age
Care SettingPediatric 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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