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Clinicopathologic Characteristics of Endometrial Cancer in Lynch Syndrome: A French Multicenter Study
Léa Rossi1, Marie-Aude Le Frere-Belda, Pierre Laurent-Puig
1*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.
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.
