Objective: To describe the gross and histological characters of MRKHS patients’ ovaries and assess their ovarian reserve directly for future pregnancy. Design: A prospective case-control study. Setting and population: A prospective case-control study involving 67 MRKH syndrome patients and 17 ovarian biopsies (with age-matched controls), from January 2017 to February 2020. Methods: We observed the gross appearance of 67 Chinese MRKHS patients during laparoscopically assisted neovagina creation surgery. Seventeen ovarian biopsies were obtained and compared with age-matched controls for the histological characters. Twelve biopsy specimens underwent a complete serial section and calculation of the primordial follicular density. Main Outcome Measures: Follicle density, morphology in ovarian cortex biopsies. Results: 5/67 MRKHS patients’ ovaries had abnormal gross appearance, including streak gonads and bilateral porcelain white ovaries. We observed corpus luteum or corpus albicans on 5/8 patients under 20 years old, but none of the controls. None of the biopsies from MRKHS type Ⅱ patients showed corpus luteum or corpus albicans. Two type Ⅰ patients presented with compromised ovarian reserve, and significant increase of atretic follicles and corpus albicans. A significant inverse correlation was observed between primordial follicular density and age in 12 patients aged 16 to 25 years old. Conclusion: The ovaries of MRKHS patients have higher rates of abnormalities regarding gross appearance and histological characters. Tweetable abstract: It is suggested that early preparation for fertility reserve and incorporated as a part of the treatment when reconstructing the neovagina in MRKH syndrome patients due to premature declination of the ovarian reserve.

Background Little is known about the oncologic results of patients with low-grade endometrial stromal sarcoma (LGESS) with fertility preservation. This study was to investigate the prognosis of fertility-sparing surgeries and relevant obstetrical outcomes. Methods All eligible patients in the study center were retrospectively reviewed if they consented to surgical treatment for primary LGESS from February 2012 to June 2019 in the study center. Follow-up of fertility and oncologic outcomes was carried out until June 1, 2020. Results Among 135 patients accepted surgical therapy for primary uterine LGESS, 21 (15.6%) and 42 (31.1%) had fertility-sparing surgery and ovarian preservation, respectively. After a median follow-up of 38.5 (range 5-98) months, differences existed in the recurrent rates among patients with and without fertility-sparing surgery (47.6% versus 15.8%, p=0.002), or with and without ovarian preservation (42.9% versus 10.8%, p<0.001). Fertility-sparing surgery and ovarian preservation significantly increased the recurrent risks in the whole cohort (hazard ratio [HR] 3.5 and 5.2, 95% confidential interval [CI] 1.6-7.6 and 2.4-11.3, p=0.002 and <0.001) and in stage I patients (HR 2.6 and 3.1, 95% CI 1.0-6.4 and 1.3-7.4, p=0.041 and 0.011). No factor was found to be associated with increased mortality risk. Among 9 patients attempting pregnancy, 7 (77.8%) achieved 8 live births. Three advanced stage patients with fertility-sparing surgery all experienced recurrence without successful conception. Conclusion Fertility-sparing surgery for LGESS results in a significantly increased risk of recurrence but not of mortality, and has promising results in terms of live births in stage I patients.