林奇综合征子宫内膜癌的临床病理特征：一项法国多中心研究
Clinicopathologic Characteristics of Endometrial Cancer in Lynch Syndrome: A French Multicenter Study.
作者信息
Rossi Léa, Le Frere-Belda Marie-Aude, Laurent-Puig Pierre, Buecher Bruno, De Pauw Antoine, Stoppa-Lyonnet Dominique, Canlorbe Geoffroy, Caron Olivier, Borghese Bruno, Colas Chrystelle, Delhomelle Hélène, Chabbert-Buffet Nathalie, Grandjouan Sophie, Lecuru Fabrice, Bats Anne-Sophie
机构信息
*Department of Gynaecological and Breast Cancer Surgery, Hôpital Européen Georges-Pompidou, Assistance Publique des Hopitaux de Paris (APHP); †Medical School, Paris Descartes University, Sorbonne Paris Cité; ‡Pathology and §Genetics, Hôpital Européen Georges-Pompidou; ∥Department of Tumour Biology, Curie Institute; ¶INSERM U830, University Paris Descartes, Sorbonne Paris Cité; and #Department of Obstetrics and Gynaecology, Tenon University Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Paris; **Department of Oncogenetics, Gustave Roussy Institute, Villejuif; and ††Institut Cochin, University Paris Descartes, Sorbonne Paris Cite, CNRS (UMR 8104); ‡‡INSERM U1016; §§University Paris Descartes, Sorbonne Paris Cite, Department of Gynaecology, Obstetrics, and Reproductive Medicine, Hôpital Cochin, Hopitaux Universitaires Paris Centre, AP-HP; ∥∥Department of Genetics, Pitié-Salpêtrière Hospital; ¶¶Genetics, Hôpital Saint-Antoine; ##Gynaecology and Obstetrics, Hôpital Tenon; and ***Department of Digestive Diseases and Oncogenetics, APHP, Cochin Hospital, Paris, France.
出版信息
Int J Gynecol Cancer. 2017 Jun;27(5):953-960. doi: 10.1097/IGC.0000000000000985.
BACKGROUND
Limited data exist on Lynch syndrome (LS)-related endometrial cancer (EC) features. Amsterdam criteria II, commonly used, have poor sensitivity for detection of LS, which is underdiagnosed.
AIM
The aim of this study was to describe the clinical and pathological features of LS-related EC among mutation-proven patients.
METHODS
We conducted a retrospective study from 1977 to 2013 in 5 hospitals. The inclusion criteria were patients who had a primary EC associated to LS proven by a germline mutation. We analyzed the clinical data and the pathology of the tumors. The patient management and the survival data were also collected.
RESULTS
Forty-nine patients (15 MLH1, 20 MSH2, 13 MSH6, 1 PMS2) were included. The mean age at diagnosis was 49.7 (SD, 10.5) years. The median body mass index was 22.6 kg/m. In 81.4% of cases, EC was the first cancer of the LS spectrum to occur. Endometrioid adenocarcinoma accounted for 89.2% of the EC, the lower uterine segment was involved in 25% of cases, and a synchronous ovarian cancer was present in 21.6% of patients. The tumors were grade 3 in 19.3% of cases and FIGO (International Federation of Gynecology and Obstetrics) stage I in 66.6% of cases. With a median follow-up of 58 months, 3 patients with conservative management developed a recurrence, and no patient died of EC.
CONCLUSIONS
The LS-associated EC is characterized by a young age at onset, a high prevalence of lower uterine segment involvement, and synchronous ovarian cancers. The prognosis of these cancers does not appear different from sporadic tumors.
背景
关于林奇综合征（LS）相关子宫内膜癌（EC）特征的数据有限。常用的阿姆斯特丹标准II对LS的检测敏感性较差，导致其诊断不足。
目的
本研究旨在描述经突变证实的LS相关EC患者的临床和病理特征。
方法
我们于1977年至2013年在5家医院进行了一项回顾性研究。纳入标准为通过种系突变证实患有与LS相关的原发性EC的患者。我们分析了肿瘤的临床数据和病理情况。还收集了患者的治疗管理和生存数据。
结果
纳入49例患者（15例MLH1、20例MSH2、13例MSH6、1例PMS2）。诊断时的平均年龄为49.7（标准差，10.5）岁。中位体重指数为22.6kg/m。在81.4%的病例中，EC是LS谱系中首个发生的癌症。子宫内膜样腺癌占EC的89.2%，25%的病例累及子宫下段，21.6%的患者存在同步性卵巢癌。19.3%的病例肿瘤为3级，66.6%的病例为国际妇产科联盟（FIGO）I期。中位随访58个月，3例接受保守治疗的患者出现复发，无患者死于EC。
结论
LS相关EC的特征为发病年龄较轻、子宫下段受累患病率高以及同步性卵巢癌。这些癌症的预后似乎与散发性肿瘤无异。
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