Adherence to the guidelines and the pathological diagnosis of high-risk gastrointestinal stromal tumors in the real world
Adherence to the guidelines and the pathological diagnosis of high-risk gastrointestinal stromal tumors in the real world
复制标题
DOI：
10.1007/s10120-019-00966-4
复制
发表时间：
2020-01-01
期刊：
影响因子：
7.4
通讯作者：
Hirota, Seiichi
中科院分区：
文献类型：
--
作者：
Nishida, Toshirou;Sakai, Yoshiharu;Hirota, Seiichi
背景基于指南和专业病理学家的病理诊断的多学科方法对改善GIST患者的预后和生活质量至关重要。本研究检查了对指南的遵守情况和高危GIST病理诊断的一致性。患者和方法在2012年12月至2015年12月期间招募的541例高危GIST患者中，534例患者在采用KIT和DOG1 IHC进行中心病理学检查以及KIT和PDGFRA基因分型后进行了分析。结果534例患者中，432例（81%）接受伊马替尼辅助治疗，起始剂量为400或300 mg/d。多因素分析表明，年龄（HR 0.71; 95% CI 0.58 - 0.88），肿瘤大小（HR> 10 cm vs <5 cm，3.87; 95% CI 1.72 - 8.74），有丝分裂（HR> 10 vs <5，3.54; 95% CI 1.84 - 6.79）、肿瘤破裂（HR 3.69; 95% CI 1.43 - 9.52）和体能状态（HR 0.55; 95% CI 0.31 - 0.99）与辅助治疗独立相关。在当地诊断的534例高危GIST中，19例肿瘤（3.6%）被诊断为非GIST，其他93例（18.1%）GIST被中心病理学重新分类为低风险类别。在10例非GIST患者和8例PDGFRA D842V突变患者中，分别有4例（40%）和3例（38%）患者在收到中心病理学结果后继续治疗。结论高危GISTs患者对指南的依从性和病理诊断的一致性较好。中枢病理学可能有助于改善诊断，但可能需要进一步完善。
Background A multidisciplinary approach based on guidelines and pathological diagnosis by specialized pathologists are important for improving the prognosis and QoL of GIST patients. This study examined the adherence to the guidelines and the concordance of the pathological diagnosis of high-risk GISTs. Patients and methods Among 541 patients with high-risk GISTs recruited to the prospective registry between Dec. 2012 and Dec. 2015, 534 patients were analyzed after central pathology with KIT and DOG1 IHC and genotyping of KIT and PDGFRA. Results Of the 534 patients, 432 (81%) received imatinib adjuvant therapy at a starting dose of 400 or 300 mg/day. Multivariate analysis indicated that age (HR 0.71; 95% CI 0.58-0.88), tumor size (HR for > 10 cm vs < 5 cm, 3.87; 95% CI 1.72-8.74), mitosis (HR for > 10 vs < 5, 3.54; 95% CI 1.84-6.79), tumor rupture (HR 3.69; 95% CI 1.43-9.52) and performance status (HR 0.55; 95% CI 0.31-0.99) were independently related to adjuvant therapy. Among the 534 high-risk GISTs diagnosed locally, 19 tumors (3.6%) were diagnosed as non-GISTs, and the other 93 (18.1%) GISTs were reclassified into lower risk categories by central pathology. Among 10 patients with non-GISTs and 8 patients with PDGFRA D842V mutations, 4 (40%) and 3 (38%) patients, respectively, continued the therapy after receiving the central pathology results. Conclusions The adherence to guidelines and the concordance of pathological diagnoses were comparatively good for high-risk GISTs. Central pathology may contribute to improved diagnosis, but further refinements may be required.