Objective:

To report a rare case of high-grade secretory carcinoma of the parotid gland in a pediatric patient and highlight the diagnostic challenges associated with its preoperative identification, emphasizing its significance in pediatric oncology.

Key Findings:

• The tumor exhibited small, monomorphic epithelial cells with eosinophilic cytoplasm and secretory granules.
• Histological features included solid areas, nuclear enlargement, discernible mitotic figures, and positive PAS staining.
• Fluorescence in situ hybridization confirmed the presence of an ETV6::NTRK3 gene fusion.

Interpretation:

The case illustrates the potential for misdiagnosis of secretory carcinoma as pleomorphic adenoma due to overlapping clinical and imaging characteristics, highlighting the need for increased awareness among clinicians.

Limitations:

• The rarity of secretory carcinoma in children may limit pathologists' experience and familiarity with its presentation, impacting diagnostic accuracy.
• No standardized clinical guidelines exist for managing secretory carcinoma in pediatric patients, complicating treatment decisions.

Conclusion:

The successful management of this case underscores the importance of comprehensive histopathological and molecular analyses in accurately diagnosing salivary gland tumors, reinforcing the need for clinician awareness of this rare condition.