Carcinoma Ex Pleomorphic Adenoma: Case Report and Options for Systemic Therapy

Abstract

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.

<p>Anlotinib is effective in the treatment of advanced carcinoma ex pleomorphic adenoma of the submandibular gland</p>

“…In 2009, Lüers JC et al13 retrospectively analyzed 22 patients with CXPA of the parotid gland and showed that patients receiving chemotherapy even had a lower 5-year overall survival rate (50%) than those receiving no chemotherapy (63%). However, in a case report of CXPA from a Canadian group, chemotherapy with CAP (cyclophosphamide, doxorubicin, and cisplatin) result in an excellent clinical and radiologic response 14. Therefore, the role of chemotherapy in CXPA is still unclear.…”

Carcinoma ex pleomorphic adenoma of the trachea: A case report

“…The pathogenesis of CXPA is unknown. Chooback et al[5] proposed a role for the deficiency of some genes, such as those on chromosomal arms 8q, 12q, and 17p. Kim et al[6] suggested that the accumulation of genetic instabilities was the main cause of malignant transformation in pleomorphic carcinoma.…”

Challenges in the Diagnosis of Myoepithelial-Rich Salivary Gland Neoplasms

“…Adjuvant radiation is typically reserved for patients with stage III/IV disease 99 . At present, there is no well-defined role for systemic therapy 99,100 . It should be noted that the distinction between de novo and ex-PA malignancies is not merely semantic.…”