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胚系BRCA2突变的晚期胰腺腺泡细胞癌对奥拉帕利的反应:一例报告。

Response of germline BRCA2-mutated advanced pancreatic acinar cell carcinoma to olaparib: A case report.

作者信息

Li Mao, Mou Yu, Hou Shengzhong, Cao Dan, Li Ang

机构信息

Department of Pancreatic Surgery.

Department of Abdominal Oncology, West China Hospital, Sichuan University, Chengdu, China.

出版信息

Medicine (Baltimore). 2018 Nov;97(45):e13113. doi: 10.1097/MD.0000000000013113.

Abstract

RATIONALE

Pancreatic acinar cell carcinoma (PACC) is a relatively rare malignancy of the exocrine pancreas. BRCA2, a cancer susceptibility gene, has been widely studied in breast and ovarian carcinomas as mutation carriers for this gene are at a high risk for cancer development. Olaparib, an oral poly(adenosine diphosphate-ribose) polymerase (PARP) inhibitor, has been approved for the treatment of ovarian cancer with any BRCA 1/2 mutations. Herein, we report the first case of a germline BRCA2-mutated unresectable advanced PACC patient who responded well to olaparib treatment.

PATIENT CONCERNS

A 59-year-old male with a family history of cancer presented with a persistent epigastric dull pain for 3 months.

DIAGNOSIS

The patient was diagnosed with advanced PACC based on computed tomography (CT) scan, laparotomy, and pathology.

INTERVENTIONS

Exploratory laparotomy, intratumoral brachytherapy by radioiodine-125 seeds, modified FOLFIRINOX chemotherapy, and targeted therapy with olaparib were administered.

OUTCOMES

The patient responded well to olaparib until the occurrence of severe adverse drug reactions, he died as a result of multiple organ failure with an overall survival period of 12 months.

LESSONS

As a PARP inhibitor, olaparib has remarkable curative effect not only on breast and ovarian cancers, but also on other malignancies with BRCA mutations. Patients with advanced cancer could benefit from active targeted therapy with improvement in overall survival and quality of life.

摘要

理论依据

胰腺腺泡细胞癌(PACC)是一种相对罕见的胰腺外分泌恶性肿瘤。BRCA2是一种癌症易感基因,在乳腺癌和卵巢癌中已得到广泛研究,因为该基因的突变携带者患癌风险很高。奥拉帕利是一种口服聚(腺苷二磷酸 - 核糖)聚合酶(PARP)抑制剂,已被批准用于治疗任何BRCA 1/2突变的卵巢癌。在此,我们报告首例携带种系BRCA2突变的不可切除晚期PACC患者,该患者对奥拉帕利治疗反应良好。

患者情况

一名有癌症家族史的59岁男性,出现上腹部持续性隐痛3个月。

诊断

根据计算机断层扫描(CT)、剖腹手术及病理检查,该患者被诊断为晚期PACC。

干预措施

实施了剖腹探查术、125碘粒子瘤内近距离放疗、改良FOLFIRINOX化疗以及奥拉帕利靶向治疗。

结果

患者对奥拉帕利反应良好,直至出现严重药物不良反应,最终因多器官功能衰竭死亡,总生存期为12个月。

经验教训

作为一种PARP抑制剂,奥拉帕利不仅对乳腺癌和卵巢癌有显著疗效,对其他携带BRCA突变的恶性肿瘤也有疗效。晚期癌症患者可从积极的靶向治疗中获益,从而提高总生存期和生活质量。

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