Chondroid lesion of the parotid gland: chondroid pleomorphic adenoma or extraskeletal chondroma?

Chondroid lesions of the parotid gland are exceptionally rare. The differential diagnosis includes pleomorphic adenoma with extensive chondroid differentiation and extraskeletal chondromas. Clinically, they present as slow-growing, painless, well-circumscribed masses. A 70-year-old asymptomatic male was referred after incidental detection of a parapharyngeal mass on brain MRI. Clinical and endoscopic evaluation revealed left oropharyngeal and nasopharyngeal bulging. Neck MRI showed a well-defined, predominantly cystic lesion in the deep lobe of the left parotid gland, with peripheral nodularity, high T2 signal, high apparent diffusion coefficient (ADC 1 ) values, and minimal enhancement. Following ultrasound guided fine needle aspiration (FNA 2 ) pleomorphic adenoma was considered. The patient underwent partial parotidectomy and histopathology showed a chondroid lesion with degenerative changes. RNA sequencing revealed a HMGA2::WIF1 fusion. The tumor was completely excised with close margins. Postoperative recovery was uneventful. The presence of an HMGA2::WIF1 fusion favors a diagnosis of chondroid pleomorphic adenoma rather than a true extraskeletal chondroma, although a true chondroma cannot be fully excluded. Complete surgical excision with preservation of the facial nerve is the standard treatment. Recurrence remains a consideration; therefore annual imaging follow-up can be considered, taking into account tumor characteristics and surgical factors. Chondroid lesions of the parotid gland are rare and diagnostically challenging. The identification of an HMGA2::WIF1 fusion, characteristic of pleomorphic adenoma but not reported in chondromas, supports classification as a chondroid pleomorphic adenoma. These findings question whether salivary gland chondromas constitute a distinct entity and highlight the value of molecular analysis in this differential diagnosis.