Lynch syndrome-related non-endometrioid endometrial cancer: analysis of outcomes

2019 ◽  
Vol 30 (1) ◽  
pp. 56-61
Author(s):  
Giorgio Bogani ◽  
Maria Grazia Tibiletti ◽  
Maria Teresa Ricci ◽  
Ileana Carnevali ◽  
Viola Liberale ◽  
...  

ObjectiveWomen with Lynch syndrome have a risk up to 40–60% of developing endometrial cancer, which is higher than their risk of developing colorectal or ovarian cancer. To date, no data on the outcomes of patients with Lynch syndrome diagnosed with non-endometrioid endometrial cancer are available. The goal of this study was to evaluate the outcome of patients with Lynch syndrome diagnosed with non-endometrioid endometrial cancer.MethodsData from consecutive patients diagnosed with Lynch syndrome and with a histological diagnosis of non-endometrioid endometrial cancer were retrospectively collected in two referral institutes in Italy. A case–control comparison (applying a propensity matching algorithm) was performed in order to compare patients with proven Lynch syndrome and controls. Inclusion criteria were: (a) histologically-proven endometrial cancer; (b) detection of a germline pathogenic variant in one of the MMR genes; (c) adequate follow-up. Only carriers of pathogenic or likely pathogenic variants (ie, class 5 and 4 according to the InSiGHT classification) were included in the study. Survival outcomes were assessed using KaplanMeier and Cox models.ResultsOverall, 137 patients with Lynch syndrome were collected. Mean patient age was 49.2 (10.9) years. Genes involved in the Lynch syndrome included MLH1, MSH2, and MSH6 in 43%, 39%, and 18% of cases, respectively. The study population included 27 patients with non-endometrioid endometrial cancer, who were matched 1:2 with patients with sporadic cancers using a propensity matching algorithm. After a median follow-up of 134 months (range 1–295), 2 (7.4%) of the 27 patients developed recurrent disease (3 and 36 months) and subsequently died of disease (7 and 91 months). Patients diagnosed with Lynch syndrome experienced better disease-free survival (HR 7.86 (95% CI 1.79 to 34.5); p=0.006) and overall survival (HR 5.33 (95% CI 1.18 to 23.9); p=0.029) than controls.ConclusionsNon-endometrioid endometrial cancer occurring in patients with Lynch syndrome might be associated with improved oncologic outcomes compared with controls. Genetic/molecular profiling should be investigated in order to better understand the mechanism underlying the prognosis.

2018 ◽  
Vol 104 (1) ◽  
pp. 60-65 ◽  
Author(s):  
Hulya Ayik Aydin ◽  
Tayfun Toptas ◽  
Selen Bozkurt ◽  
Elif Pestereli ◽  
Tayup Simsek

Purpose: Despite the common occurrence of adenomyosis in endometrial cancer (EC), there is a paucity and conflict in the literature regarding its impact on outcomes of patients. We sought to compare outcomes of patients with endometrioid type EC with or without adenomyosis. Methods: A total of 314 patients were included in the analysis. Patients were divided into 2 groups according to the presence or absence of adenomyosis. Adenomyosis was identified in 79 patients (25.1%). A propensity score-matched comparison (1:1) was carried out to minimize selection biases. The propensity score was developed through multivariable logistic regression model including age, stage, and tumor grade as covariates. After performing propensity score matching, 70 patients from each group were successfully matched. Primary outcome of the study was disease-free survival (DFS), and the secondary outcomes were overall survival (OS) and disease-specific survival (DSS). Results: Median follow-up time was 61 months for the adenomyosis positive group and 76 months for the adenomyosis negative group. There were no statistically significant differences in 3- and 5-year DFS, OS, and DSS rates between the 2 groups. Five-year DFS was 92% vs 88% (hazard ratio [HR] 1.54 [0.56-4.27]; p = 0.404), 5-year OS was 94% vs 92% (HR 1.60 [0.49-5.26]; p = 0.441), and 5-year DSS was 94% vs 96% (HR 2.51 [0.46-13.71]; p = 0.290) for patients with and without adenomyosis, respectively. Conclusions: Coexistent adenomyosis in EC is not a prognostic factor and does not impact survival outcomes.


2019 ◽  
Vol 29 (1) ◽  
pp. 77-85 ◽  
Author(s):  
Su Hyun Chae ◽  
Seung-Hyuk Shim ◽  
Sun Joo Lee ◽  
Ji Young Lee ◽  
Soo-Nyung Kim ◽  
...  

ObjectiveHormonal management is an alternative treatment for preserving fertility in patients with presumed early stage endometrioid endometrial cancer. This study aimed to define the pregnancy and oncologic outcomes and factors of successful conception after hormone therapy for endometrioid endometrial cancer.MethodsWe retrospectively analyzed patients presumed to have stage IA, grade 1–2 endometrioid endometrial cancer who underwent fertility-sparing treatment. Concurrent medroxyprogesterone and levonorgestrel-release intra-uterine devices were used for treatment. The pregnancy outcomes and oncologic outcomes were compared between the pregnant and non-pregnant groups.ResultsSeventy-one patients presumed to have stage IA, grade 1–2 endometrioid endometrial cancer had complete remission, and 49 of them tried to conceive. Twenty-two (44.9%) patients became pregnant; the total number of pregnancies was 30. These pregnancies resulted in seven abortions (23.3%), one pre-term birth (3.3%), and 20 full-term births (66.6%). The total live birth rate was 66.6 % (20/30). The median duration of hormonal treatment was 11.9 months (range 4–49) and 12.0 months (range 3–35) in the pregnant and non-pregnant groups, respectively. On multivariate analysis, age, body mass index, treatment duration, medroxyprogesterone dose, and number of dilatation and curettage biopsies were not significantly associated with pregnancy failure, but the association with grade (OR 6.2, 95% CI 1.0 to 38.9; P<0.05) was statistically significant. The median disease-free survival duration was 26 months (range 20–38) and 12 months (range 4–48) in the pregnant and non-pregnant groups, respectively (P<0.05, log-rank test).ConclusionsA lower grade might be a positive factor for future pregnancy. Moreover, successful pregnancy might be a factor in preventing recurrence.


2019 ◽  
Vol 29 (4) ◽  
pp. 705-710 ◽  
Author(s):  
Giorgio Bogani ◽  
Maria Teresa Ricci ◽  
Marco Vitellaro ◽  
Antonino Ditto ◽  
Valentina Chiappa ◽  
...  

ObjectiveLynch syndrome is a risk factor for developing endometrial carcinoma. Our aim was to evaluate the impact of gene-specific germline pathogenic variants on clinical features of patients affected by endometrial cancer.MethodsPatients with a diagnosis of endometrial cancer and with a germline pathogenic variant in mismatch repair genes were reviewed. Patients were classified on the basis of classes of risk according to the ESGO-ESGO-ESTRO (European Society of Medical Oncology/European Society of Gynaecological Oncology/European Society for Radiotherapy and Oncology) guidelines. One-way analysis of variance (ANOVA) and Kruskal-Wallis test were performed to compare three groups of continuous parametric and non-parametric variables, respectively. χ2 test was used to analyze proportions.ResultsOverall, 68 patients with endometrial cancer and Lynch syndrome were evaluated. Ten (14.7%) patients were excluded because of absence of information about the gene involved in Lynch syndrome, thus leaving 58 (85.3%) patients available for the final analysis. MLH1, MSH2, and MSH6 pathogenic variants were observed in 19 (32.7%), 33 (56.9%), and six (10.3%) patients, respectively. Mean±SD age at endometrial cancer diagnosis was 51±6.4, 43.5±7.4, and 60.3±8.8 years (p=0.0002). Prevalence of non-endometrioid endometrial cancer was 15.7%, 24.2%, and 0% in the MLH1, MSH2, and MSH6 groups, respectively (p=0.345). According to the ESMO-ESGO-ESTRO classification, low, intermediate, and high risk endometrial cancer accounted for 47.3%, 10.5%, and 42.1% of the MLH1 group, 57.6%, 3%, and 39.4% of the MSH2 group, and 50%, 50%m and 0% of the MSH6 group (p=0.009).ConclusionsPatients with MLH1 and MSH2 pathogenic variants are at a higher risk of early onset of endometrial cancer than patients with MSH6 pathogenic variants.


Cancers ◽  
2020 ◽  
Vol 12 (4) ◽  
pp. 969
Author(s):  
Guido Alberto Massimo Tiberio ◽  
Vittorio Ferrari ◽  
Zeno Ballarini ◽  
Giovanni Casole ◽  
Marta Laganà ◽  
...  

Background. This study explores the impact of Hypertermic Intra PEritoneal Chemotherapy (HIPEC) on adrenocortical carcinoma (ACC) management through a safety analysis completed by a preliminary evaluation of survival performances. Methods. Retrospective chart review of 27 patients submitted to surgical treatment completed by HIPEC for primary (SP, 13 patients) or recurrent (SR, 14 patients, 17 treatments) ACC. Safety was evaluated by means of procedural morbidity and mortality. Survival performances included multiple end points: local/peritoneal disease-free survival (l/pDFS), overall progression-free survival (OPFS), and overall survival (OS). Results. In the SP group, mortality was nil and morbidity was 46% (major 23%). At a median follow-up of 25 months, the median value for all the different survival measures had not been reached. Mortality was also nil in the SR group. However, morbidity was 77% (major 18%). Median l/pDFS and OPFS were 12 ± 4 and 8 ± 2 months, respectively. At a median follow-up of 30 months, median OS had not been reached. Conclusion. Surgery and HIPEC is an invasive procedure. Its employment in the surgery for primary setting deserves attention as it may affect oncologic outcomes positively. Its value in the management of recurrences seems less appreciable, albeit it may find its place in the multimodal management of a rare disease for which multiple therapeutic options do not yet exist.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e15573-e15573
Author(s):  
Nadire Kucukoztas ◽  
Selim Yalcin ◽  
Samed Rahatli ◽  
Ozlem Ozen ◽  
Nihan Haberal ◽  
...  

e15573 Background: Stage IC patients are at an increased risk of recurrence and overall worse prognosis compared with stage IA and IB patients. Adjuvant chemoherapy is utilized based on specific pathologic factors. The objective of this study is to evaluate treatment outcomes at a single institution in patients with 1988 FIGO stage IC endometrial adenocarcinoma. Methods: Records of the patients with FIGO stage IB (formerly IC) endometrial cancer were retrospectively evaluated. All patients were initially treated surgically with comprehensive staging lymphadenectomy. Results: A total of 85 patients were included. Patient and tumor characteristics are shown in the table. Median age of the patients was 60 (range 27-95). Fifty-nine patients had at least one co-morbid disease. Complete surgical staging including pelvic and paraaortic lymph node dissection was performed in all the patients. Sixteen patients (19%) received adjuvant chemotherapy, including 6 patients with serous cancer and one patient with small cell cancer. Paclitaxel/carboplatin was the preferred regimen in Median follow up was 30 months (range 10-61 months). Seven patients (8%) relapsed and 4 patients (5%) died on follow up. 5 year disease free survival was 89% and overall survival was 95%. One of the 16 patients (6.2%) who received chemotherapy and 6 of the 69 patients (8.7%) who did not receive relapsed/died on follow up. Survival analysis was not performed because of the low number of events in both groups. Conclusions: We found similar rates of recurrence and death with previous studies in stage IC endometrial cancer. Complete surgical staging is the mainstay of treatment. Marginally lower recurrence rate in chemotherapy treated patients delineate the need for prospective randomized data addressing the role of adjuvant systemic therapy in early-stage patients with endometrial adenocarcinoma. [Table: see text]


Cancers ◽  
2020 ◽  
Vol 12 (12) ◽  
pp. 3571
Author(s):  
Paolo Casadio ◽  
Mariangela La Rosa ◽  
Andrea Alletto ◽  
Giulia Magnarelli ◽  
Alessandro Arena ◽  
...  

Endometrial cancer (EC) is the fourth largest female cancer in Europe and North America. In 5% of cases, the diagnosis is made in women who wish to become pregnant. In our retrospective study, we reported our experience about fertility sparing treatment of G1 endometrioid endometrial cancer (G1 EEC) or atypical endometrial hyperplasia/endometrial intraepithelial neoplasm (AEH/EIN) in young women desiring pregnancy treated in our Center. Conservative treatment was based on operative hysteroscopy and hormone therapy with megestrol acetate (160 mg/die for 9 months). For the first time we included women with G1 EEC with minimal myometrial infiltration. The minimum follow-up period was two years and consisted of serial outpatient hysteroscopies with endometrial biopsies. Among the 36 women with G1 EEC we observed one case of disease persistence and four recurrences and four recurrences among the 46 women diagnosed with AEH/EIN. To date, 35 live births were obtained in both groups. Our results advance the hypothesis that conservative treatment can represent a safe and feasible alternative to propose to young women with desire for pregnancy. Further randomized and multicentric studies are needed to arrive at unambiguous and standardized guidelines on the surgical and medical treatment of young women with EEC or AEH/EIN.


2012 ◽  
Vol 30 (5_suppl) ◽  
pp. 384-384 ◽  
Author(s):  
Sarah P. Psutka ◽  
Francis J. McGovern ◽  
Peter Mueller ◽  
W. Scott McDougal ◽  
Debra Gervais ◽  
...  

384 Background: Long-term oncologic outcomes for radiofrequency ablation (RFA) of renal cell carcinoma (RCC) are limited. The objective of this study was to assess the long-term oncological efficacy of RFA for treatment of renal cell carcinoma. Methods: Between 1998 and 2008, 311 biopsy-proven RCC were treated with RFA in 274 patients. Exclusion criteria included history of prior RCC or known metastatic RCC at time of RFA (n=92). 26 patients were lost to follow-up prior to their 6-month imaging study. We retrospectively reviewed the long-term oncologic outcomes for 193 patients. Mean follow-up was 4.6 yrs (range 1–12, SD 2.3). Results: Median age was 71 years (IQR: 63 –79 years). Median Charlson Score was 5.46 (IQR: 5–6). Median size of tumor treated was 3 cm (IQR: 2–3.9 cm, range 1–7.1cm) and 64 of these tumors (33%) were endophytic. Tumor breakdown by stage was T1a: n=153 (79%), T1b: n=37 (19%), and T2: n=3 (2%). Initial treatment success rate was 89%. There were 6 local recurrences (3%) in 4 patients with T1b disease and 2 patients with T2 disease with an average time-to-recurrence of 2.9 years (SD 0.7). 95% of patients with T1a RCC were disease free at last follow-up, in comparison to 81% of those with T1b and 33% of those with T2 disease (p=0.008). At last follow-up 178 (92%) patients were disease-free. 16 (8.2%) developed metastatic disease and 4 patients (2%) died of RCC. Mean disease-free survival was 4.3 years (SD 2.4). Conclusions: In patients who are poor surgical candidates, RFA results in durable local control and a low risk of disease recurrence in T1 RCC. Higher stage, however, correlates with a decreased disease free survival and alternate treatments should be considered when counseling these patients.


2020 ◽  
Author(s):  
Hong Yang ◽  
Jiabo Di ◽  
Ming Cui ◽  
Jiadi Xing ◽  
Chenghai Zhang ◽  
...  

Abstract Background: Neoadjuvant chemoradiotherapy (CRT) can downstage rectal carcinoma, resulting in superior resectability, better local control and survival benefits. However, it is unclear whether patients treated with CRT and those who did not have similar outcomes at the same pathological stage. This study aimed to investigate the long-term outcomes of ypT1-3N0 mid-low rectal cancer who received neoadjuvant CRT followed by total mesorectal excision (TME) compared with pT1-3N0 rectal cancer immediately managed with surgery. Methods: We retrospectively enrolled 180 patients with pT1-3N0 or ypT1-3N0 rectal cancer located within 10cm from the anal edge who underwent TME between 2009 and 2015. Of these patients, 63 received neoadjuvant CRT, while 117 underwent radical proctectomy without preoperative therapy. The disease-free survival (DFS) and cancer-specific survival (CSS) were compared between the two groups. Results: Within a median follow-up time of 65 months, the 5-year DFS was lower in the CRT group than the non-CRT group (74.9% vs. 92.6%, P=0.001), and the 5-year CSS presented a similar trend as well (89.6 % vs. 97.1%, P=0.054). By subgroup analysis, the difference in DFS and CSS was mainly caused by the difference between ypT3N0 and pT3N0 disease (71.1% vs. 96.1%, P<0.001 and 90.9% vs. 100%, P=0.029, respectively). However, patients with ypT1-2N0 had an analogous prognosis to those with pT1-2N0 disease (77.9% vs. 89.0%, P=0.225 and 88.1% vs. 94.2%, P=0.292, respectively). Multivariate analysis indicated that neoadjuvant CRT was not an independent predictor of DFS. Conclusion: After neoadjuvant CRT followed by TME, patients with ypT1-2N0 rectal cancer had an analogous prognosis to those with initial pT1-2N0 disease, whereas patients with ypT3N0 rectal cancer had worse prognosis compared with that of pT3N0 disease.


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