Carcinoma ex pleomorphic adenoma CXPA, a rare epithelial malignancy arising from a primary or recurrent benign pleomorphic adenoma, accounts for 11.% of all malignant salivary gland neoplasms. It is difficult to diagnose preoperatively. often poses a diagnostic challenge to clinicians and pathologists Treatment involves an ablative surgical procedure with neck dissection followed by radiotherapy. We aim to investigate the impact of postoperative radiotherapy on improving disease-free survival.

A 39-year-old Libyan male presented with painless swelling near the angle of the right mandible four months ago. FNA Cytology showed a benign pleomorphic adenoma. A total parotidectomy with VII CN preservation was done in September 2022. The histopathological features were consistent with carcinoma EX pleomorphic adenoma, a widely invasive salivary duct carcinoma grade III with < 1mm(close)margins staged PT1 N0 M0. The immunohistochemistry revealed the negative expression of ER and PR assays.

In December 2022, he received adjuvant radiation to the tumor bed (66 GY) in 33 fractions over 6 weeks based on the VMAT technique. 12-month follow-up, the patient showed no evidence of local or regional disease recurrence or distant metastasis.

Radical surgery, followed by adjuvant radiotherapy, should be considered the standard of care for a patient, with significant improvement in 5-year locoregional control. and in general, salivary gland neoplasms respond poorly to chemotherapy and are currently indicated only for palliative sitting. More prospective data is needed to establish a role for hormonal therapy and molecularly targeted therapies.

CXPA is an uncommonly aggressive malignancy that, if associated with regional metastasis, invariably leads to mortality. Total resection of the tumor, followed by adjuvant radiotherapy, should be considered the standard of care for a patient with significantly improved 5-year locoregional control. Early and prompt diagnosis, followed by aggressive surgical intervention and adjuvant radiotherapy for patients with carcinoma ex pleomorphic adenoma, can enhance their survival rates.