Chooback N, Shen Y, Jones M, Kasaian K, Martin M, Ng T, Thomson T, Marra M, Laskin J, Ho C
Department of Medical Oncology, BC Cancer Agency.
BC Cancer Research Centre.
Curr Oncol. 2017 Jun;24(3):e251-e254. doi: 10.3747/co.24.3588. Epub 2017 Jun 27.
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.
最常见的涎腺良性肿瘤是多形性腺瘤。多形性腺瘤恶变，即多形性腺瘤恶变癌（cxpa），发生率为6%。治疗重点在于手术切除和放疗；然而，罕见病例需要全身治疗。我们报告了一例60岁女性左腮腺cxpa患者，其局部复发性疾病需要全身治疗。治疗方案以关于恶性涎腺肿瘤的文献以及肿瘤的全基因组和转录组测序为指导。该患者在病程中接受了多种全身治疗药物，环磷酰胺-阿霉素-顺铂提供了最佳控制效果（部分缓解）。包括索拉非尼和维莫德吉在内的基因组和转录组导向治疗的临床获益有限。cxpa中的恶变是一个复杂过程，针对单一肿瘤途径的治疗可能不足以控制疾病。
