Büchsenschütz K, Veit J A, Schuler P J, Thierauf J, Laban S, Fahimi F, Bankfalvi A, Lang S, Sauerwein W, Hoffmann T K
Klinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Halschirurgie, Universitätsklinikum Essen, Essen.
Klinik für Hals-, Nasen-, Ohrenheilkunde, Universitätsklinikum Ulm, Ulm.
Laryngorhinootologie. 2014 Oct;93(10):657-64. doi: 10.1055/s-0034-1382024. Epub 2014 Oct 10.
The adenoid cystic carcinoma (ACC) is a neurotropic salivary gland tumor with a high blood-borne metastasis tendency. The treatment of choice for localized disease consists of radical surgical resection and, depending on resection status, adjuvant radiotherapy. Due to the high recurrence rate with limited local therapeutic options and frequent occurrence of distant metastases, one is confronted inevitably with the search for an adequate systemic therapy. ACC shows little response to a variety of chemotherapeutic agents, partial or complete remissions are extremely rare. Beside classical chemotherapies, immunotherapeutics and targeted therapies with more favorable side effect profiles were tested in trials, but due to the small number of patients, a definitive statement on the effectiveness can be hardly made. This results in the need for prospective multicenter studies that allow clear recommendations for systemic therapy of the tumor. The present paper gives an overview of the sub-cellular and genetic characteristics of ACC, which represent possible targets for systemic therapies and have partly already been included in running clinical trials.
腺样囊性癌(ACC)是一种具有高血行转移倾向的嗜神经性涎腺肿瘤。局限性疾病的首选治疗方法包括根治性手术切除,并根据切除情况进行辅助放疗。由于局部治疗选择有限且复发率高,以及远处转移频繁发生,人们不可避免地要寻找合适的全身治疗方法。ACC对多种化疗药物反应不佳,部分或完全缓解极为罕见。除了传统化疗外,免疫治疗和副作用较小的靶向治疗也在试验中进行了测试,但由于患者数量较少,很难对其有效性做出明确结论。这就需要进行前瞻性多中心研究,以便为该肿瘤的全身治疗提供明确建议。本文概述了ACC的亚细胞和遗传特征,这些特征是全身治疗的潜在靶点,部分已纳入正在进行的临床试验。