Carcinoma ex pleomorphic adenoma: case report and options for systemic therapy

N. Chooback, Y. Shen, M. Jones, K. Kasaian, M. Martin, T. Ng, T. Thompson, M. Marra, J. Laskin, C. Ho


The most common benign salivary tumour is a pleomorphic adenoma. Transformation to malignancy, carcinoma ex pleomorphic adenoma (cxpa), occurs in 6% of cases. Management focuses on surgical resection and radiotherapy; however, rare cases require systemic management. We present the case of a 60-year-old woman with a cxpa of the left parotid gland who required systemic therapy for locally recurrent disease. Treatment options were guided by the literature concerning malignant salivary gland tumour and by whole-genome and transcriptome sequencing of the tumour. The patient received multiple systemic agents during the course of her disease, with cyclophosphamide–doxorubicin–cisplatin providing the best control (partial response). Genomeand transcriptome-directed therapy, including sorafenib and vismodegib, were utilized with limited clinical benefit. Malignant transformation in cxpa is a complex process, and therapy directed at a single tumour pathway might not be sufficient to control disease.


Carcinoma ex pleomorphic adenoma; chemotherapy; cyclophosphamide;, doxorubicin; cisplatin

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ISSN: 1198-0052 (Print) ISSN: 1718-7729 (Online)