7.Clinicopathological features and prognosis of 117 cases of fumarate hydratase-deficient uterine leiomyomas
Wennian YU ; Siyi PAN ; Cong WANG ; Zhendong HUANG ; Xianglian WANG ; Shu ZHU ; Yan DING ; Xiuli WANG
Chinese Journal of Obstetrics and Gynecology 2025;60(6):450-460
Objective:To explore the clinicopathological characteristics and prognosis of fumarate hydratase-deficient uterine leiomyoma (FH-dUL).Methods:Clinical data and follow-up information for 117 patients with FH-dUL diagnosed through surgical pathology and immunohistochemistry in the First Affiliated Hospital of Nanjing Medical University from January 2020 to December 2024, were collected. A control group of 130 patients with common uterine leiomyomas was also included. The differences between the two groups in clinical, imaging, and pathological characteristics were compared. Additionally, recurrence rates, fertility outcomes for FH-dUL patients, and the incidence of renal cancer in FH germline mutation carriers were monitored.Results:(1) Comparison of clinicopathological characteristics: the median age of 117 FH-dUL patients was 35 years, and the median age at first diagnosis of uterine leiomyomas was 29 years, both significantly younger than the control group (41 and 36 years; both P<0.01). The FH-dUL group showed significantly higher incidences of uterine myomectomy, multiple leiomyomas, diffusion restriction on pelvic magnetic resonance imaging diffusion weighted imaging, and typical pathological features (candelabra-like vessels, bizarre nuclei, cytoplasmic eosinophilic globules, perinuclear halo, cellular atypia) and higher ultrasound blood flow score (all P<0.05). Of the 30 FH-dUL patients who underwent genetic testing, 9 had germline mutations, 3 had somatic mutations, and 6 had mutations of unclear origin. Among the 9 FH gene germline mutation patients, 2 had already developed renal cell carcinoma. (2) Recurrence analysis: among the 56 patients who underwent uterine myomectomy, 22 (39.3%, 22/56) experienced recurrence during follow-up, compared to 12 (21.8%, 12/55) of the 55 patients in the control group, the difference between the two groups was statistically significant ( P=0.046). Multivariate binary logistic regression analysis showed that cellular leiomyomas ( OR=9.489, 95% CI: 1.740-51.755; P=0.009) and multiple uterine leiomyomas ( OR=10.709, 95% CI: 1.354-84.683; P=0.025) were significant risk factors for recurrence in FH-dUL. (3) Fertility analysis: among the 66 FH-dUL patients who underwent fertility-preserving surgery, 16 had the intention to have fertility desire, only 2 (2/16) completed their fertility plans during follow-up. Conclusions:Clinicopathological features and imaging features help to differentiate FH-dUL from common type uterine fibroids, but lack specificity, and the diagnosis of FH-dUL is based on immunohistochemistry. The recurrence rate after resection of FH-dUL is high, and cellular and multiple leiomyomas are important predictors of recurrence. It is crucial to perform genetic testing, genetic counseling, drug treatment to prevent recurrence, fertility guidance, and long-term comprehensive management after surgery for FH-dUL management.
8.Application of CA 125 elimination rate constant K score in prognostic forecast of patients undergoing interval debulking surgery for high grade serous ovarian cancer
Huidong LIU ; Haili WU ; Linlin MA ; Ying CUI ; Shaowei WANG ; Guihua SHEN
Chinese Journal of Obstetrics and Gynecology 2025;60(6):461-468
Objective:To investigate the predictive value of the cancer antigen 125 (CA 125) elimination rate constant K (KELIM) score for no visible residual disease (R0) and prognosis in high-grade serous ovarian carcinoma (HGSOC) patients undergoing neoadjuvant chemotherapy (NACT)+interval debulking surgery (IDS). Methods:A retrospective analysis was conducted on 78 HGSOC patients treated with NACT+IDS at Beijing Hospital, from June 2014 to June 2024. The KELIM score was calculated, and its predictive value for R0 resection, chemotherapy response score (CRS), platinum-free interval (PFI), progression-free survival (PFS) time, and overall survival (OS) time was analyzed.Results:(1) The mean age at diagnosis was (61.9±9.9) years. The mean KELIM score was 1.1±0.4, with 44 patients having KELIM score≥1 and 34 having KELIM score <1. (2) Patients with KELIM score ≥1 had significantly higher rates of R0 resection (84% vs 56%; P=0.006), CRS3 grading (41% vs 0; P<0.001), and PFI ≥6 months (84% vs 53%; P=0.04) compared to those with KELIM score <1. Additionally, the median PFS time (18.7 vs 13.2 months; P<0.001) and OS time (34.8 vs 29.9 months; P=0.007) were significantly longer in the KELIM score ≥1 group. Chemosensitivity: patients with PFI <6 months had a significantly lower median KELIM score than those with PFI ≥6 months (0.8 vs 1.2; P=0.005). Surgical outcome: patients achieving R0 resection had a significantly higher median KELIM score than those without R0 (1.2 vs 0.7; P<0.001). (3) Univariate analysis identified non-R0 resection, CRS3 grading, lack of poly adenosine diphosphate ribose polymerase (PARP) inhibitor maintenance therapy, and KELIM score <1 as significant risk factors for OS time (all P<0.05). Multivariate analysis confirmed non-R0 resection ( HR=3.78,95% CI: 1.13-12.66; P=0.031), no PARP inhibitor maintenance ( HR=7.41,95% CI:1.82-30.15; P=0.005), and KELIM score <1 ( HR=5.14,95% CI:1.41-18.72; P=0.013) as independent risk factors for OS time. Conclusions:The KELIM score may serve as a predictive marker for chemosensitivity, R0 resection, PFS time, and OS time in HGSOC patients undergoing NACT+IDS. KELIM score<1 is an independent risk factor for OS.
9.Endometriosis-associated ovarian mesonephric-like adenocarcinoma:a clinicopathological analysis of 9 cases
Hua WANG ; Jing LIU ; Yu CHENG ; Shunni WANG ; Fangfang ZHONG ; Weiyong GU
Chinese Journal of Obstetrics and Gynecology 2025;60(6):469-476
Objective:To investigate the clinical and pathological characteristics, treatment and prognosis of endometriosis (EM)-associated ovarian mesonephric-like adenocarcinoma (MLA).Methods:Clinical and pathological data were collected from nine patients diagnosed with EM-associated ovarian MLA at the Obstetrics and Gynecology Hospital of Fudan University between January 2022 and December 2024. Histological slides were re-reviewed, immunohistochemical examination and molecular testing were performed, and patient follow-up was conducted.Results:(1) Clinical characteristics: the median age of the nine patients was 54 years (range: 38-69 years). All patients presented with a pelvic mass; five cases also reported abdominal pain. Tumor location included five cases in the right ovary, two in the left ovary, and two involving both ovaries. International Federation of Gynecology and Obstetrics (FIGO) staging showed 3 cases at stage Ⅰ, 4 at stage Ⅱ, and 2 at stage Ⅲ. (2) Pathological features: gross examination revealed mixed solid-cystic masses with solid areas appearing gray-white or yellow-brown; the median maximum tumor diameter was 9.0 cm (range: 2.6-13.0 cm). Microscopically, tumors exhibited various architectural patterns, including tubular, glandular, papillary, slit-like, sex cord-like, glomeruloid, and solid structures, with tubular and glandular patterns being most common. Tumor cells demonstrated mild to moderate nuclear atypia. Of the 11 tumor foci in the 9 cases, 8 showed coexistence of MLA with other tumor components, such as endometrioid carcinoma, borderline endometrioid or borderline seromucinous tumors. In 1 case of MLA mixed with a borderline endometrioid tumor, both components exhibited squamous metaplasia. Immunohistochemistry showed variable expression of GATA-binding protein 3, thyroid transcription factor-1, CD 10, and calretinin, with positive rates of 9/11, 8/11, 5/11, and 3/6, respectively. Two tumor foci (2/11) exhibited focal expression of estrogen receptor and progesterone receptor. All cases displayed wild-type p53 expression. Molecular testing via next-generation sequencing in five patients revealed pathogenic mutations in the KRAS gene (5/5), with 3 cases (3/5) harboring additional pathogenic mutations in other genes. (3) Treatment and prognosis: all patients underwent surgery, supplemented by chemotherapy and (or) targeted therapy. Five patients underwent comprehensive staging surgery, four received cytoreductive surgery, and one patient received targeted therapy. The median follow-up duration was 7 months (range: 2-27 months). Three patients (3/9) experienced recurrence, and no deaths were reported during the follow-up period. Conclusions:EM-associated ovarian MLA demonstrates diverse morphological patterns and frequently coexists with other tumor types. Accurate diagnosis relies on an integrated evaluation of histomorphology, immunohistochemistry, and molecular testing. The primary treatment for EM-associated ovarian MLA is surgery, followed by adjuvant chemotherapy. Patients harboring pathogenic KRAS p.G12C mutations may benefit from targeted therapies. Ovarian MLA is an aggressive tumor, prone to recurrence in the short term, and has a poor prognosis.
10.Analysis of risk factors for severe postpartum hemorrhage during vaginal delivery of twin pregnancy
Xiaoyue GUO ; Pengbo YUAN ; Yuan WEI ; Yangyu ZHAO
Chinese Journal of Obstetrics and Gynecology 2025;60(4):260-267
Objective:To investigate the risk factors for severe postpartum hemorrhage (SPPH) during vaginal delivery of twin pregnancy.Methods:A retrospective analysis was conducted on clinical data from twin pregnancies ≥28 weeks′ gestation undergoing vaginal delivery at Peking University Third Hospital between January 2016 and December 2023. The twin pregnant women were divided into the SPPH group (postpartum hemorrhage ≥1 000 ml within 24 hours) with 22 cases and the non-SPPH group with 171 cases. The differences between the two groups were compared and the risk factors for SPPH were analyzed.Results:(1) The incidence of SPPH during vaginal delivery in twin pregnancies was 11.4% (22/193). The causes of SPPH included 12 cases (54.5%, 12/22) of simple uterine atony, 4 cases (18.2%, 4/22) of uterine atony combined with vaginal lacerations after forceps delivery, and 6 cases (27.3%, 6/22) of uterine atony combined with placental factors. (2) The age and postpartum hospital stay in the SPPH group were significantly higher than those in the non-SPPH group (all P<0.05). Compared to the non-SPPH group, the proportion of hypertensive disorders in pregnancy, accreta placenta implantation, and anemia in the SPPH group were significantly increased, and the birth weight of newborn 1st, the sum of the birth weights of two newborns, the duration of the second stage of labor, and the proportion of labor followed induction were also significantly increased (all P<0.05). (3) Multivariate analysis showed that age ≥38 years ( OR=16.785, 95% CI: 2.679-105.166; P=0.003), the second stage of labor ≥90 minutes ( OR=9.670, 95% CI: 2.532-36.930; P=0.001), hypertensive disorders in pregnancy ( OR=5.945, 95% CI: 1.702-20.761; P=0.005), and anemia ( OR=8.048, 95% CI: 2.086-31.049; P=0.002) were independent risk factors for SPPH in twin pregnancies during vaginal delivery. Conclusions:Anemia should be actively corrected during twin pregnancy. For twin pregnant women with advanced age, hypertensive disorders in pregnancy, or other risk factors of SPPH, if vaginal delivery is chosen, attention should be paid to the management of labor duration, dynamic assessment of the risk of postpartum hemorrhage, and proactive measures should be taken to ensure a smooth vaginal delivery and effectively reduce the incidence of SPPH.

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