同时广泛伴有性索及平滑肌样分化是低级别子宫间质肉瘤（low-grade endometrial stromal sarcoma，LGESS）的一种少见的形态，这种肿瘤常会掩盖LGESS的形态学特征，具有很大的迷惑性，在病理诊断中非常容易引起误诊，需要引起病理医师的高度关注。本文报道1例LGESS伴有广泛性索及平滑肌样分化的病例，肿瘤大部区域表现为性索样结构（约70%），部分为平滑肌瘤样形态（约25%），仅局灶见到短梭形细胞，间质富于螺旋小动脉血管的区域，需考虑子宫内膜间质肿瘤的可能。免疫组织化学及荧光原位杂交检测结果均支持LGESS。结合本病例和相关文献复习，本文探讨了LGESS伴有性索及平滑肌分化的诊断及主要鉴别诊断要点，以提高对该肿瘤的认识，为肿瘤治疗及判断预后提供依据。.

To investigate the clinicopathological features, diagnosis, surgical treatment and prognosis of uterine tumors similar to ovarian sex cord tumors (UTROSCT). The clinical data, surgical approach, histopathological, and immunohistochemical features of 7 cases of UTROSCTs were retrospectively reviewed and followed up. All 4 patients were premenopausal women. The most common clinical presentation was menorrhagia (n = 4) followed by postmenopausal lower abdominal mass (n = 2) and postmenopausal bleeding (n = 1). Gynecological ultrasonography suggested uterine fibroids in 4 cases, adenomyosis with uterine fibroids in 2 cases, and an intrauterine mass in 1 case. Pelvic MRI was performed preoperatively in only 2 cases, and both indicated uterine fibroid degeneration, including 1 patient with suspected malignancy. Preoperative serum tumor markers were measured in 6 patients, and only 1 patient had elevated CA125 levels, up to 158 U/mL. Total hysterectomy with bilateral adnexectomy or salpingectomy was the most common treatment pattern (n = 6). The tumors were located within the myometrium (n = 4), submucosa (n = 1), and isthmus to external cervical os (n = 1), with a range of 2 to 12 (mean = 8) cm. Edema and degeneration were observed in 2 cases, and necrosis in 1 case. Postoperative follow-up ranged from 31 to 82 (mean = 43) months. Unfortunately, 1 patient died at 54 months of follow-up without undergoing hysterectomy. The remaining 6 cases showed no tumor recurrence or metastasis after surgery. Histological examination revealed a tumor composed of epithelioid tumor-like cells arranged in cords, trabeculae, and nests. All 7 tumors showed expression of 2 sex cord differentiation markers. Furthermore, all tumors expressed the smooth muscle marker, while epithelial marker CK (4/7). endometrial stromal marker CD10(0/7). The Ki-67 proliferation index was found to be <5% (5/7). The option of total hysterectomy may be considered for women who do not have any fertility requirements. However, for young women who desire to maintain their reproductive capacity, surgery to preserve the uterus may be an alternative, although it necessitates careful postoperative monitoring. In terms of follow-up monitoring, MRI is more suitable than ultrasound. The diagnosis of UTROSCT heavily relies on histopathological examination and immunohistochemical analysis.

卵巢恶性生殖细胞肿瘤主要受累人群为青少年、对国家人口政策和生育健康具有重要意义。卵巢恶性生殖细胞起源于发育异常、未凋亡的生殖细胞，目前，其病理学分型主要依据形态学进行分类，尚未纳入起源细胞的遗传学事件。依据对女性生殖细胞发育历程的新认识，本文将通过全面介绍和对比卵巢、睾丸及儿童生殖细胞肿瘤的研究进展，以期更加深入了解较为常见的几种卵巢恶性生殖细胞肿瘤的遗传学特征，进而辅助理解各亚型普通谱系分化的原因以及寻找用于临床诊断、监测的潜在靶点。.

暂无摘要。

Steroid cell tumors (SCTs) of the ovary are rare and understudied, and as such, uncertainties remain about their malignant potential, as well as clinicopathologic predictors of patient outcome. Based on a multi-institutional cohort of cases, we present findings from the largest study of SCT reported to date. Clinicopathologic data were documented on 115 cases of SCT that were assembled from 17 institutions. The median patient age was 55 years (range: 9 to 84). When measured, preoperative androgen levels were elevated in 84.2% (48/57) of patients. A total of 111 (96.5%) cases were classified as stage I (103 stage IA; 2 stage IB; 6 stage IC). The stage distribution for the remaining 4 patients was as follows: stage II (n = 1), III (n = 3; 1 IIIA, 1 IIIB, 1 IIIC). The median tumor size was 3 cm (range: 0.2 to 22). Cytologic atypia, microscopic tumor necrosis, microscopic tumor hemorrhage, and a mitotic index of >1 mitotic figure/10 high-power fields were present in 52% (60/115), 9.6% (11/115), 37% (43/115), and 19% (22/115) of cases, respectively. Of 115 patients, 7 (6.1%) recurred postexcision, 4 (3.5%) ultimately died of disease, and 10 (8.7%) either recurred, died of disease, or were advanced stage at presentation. The median duration to recurrence postresection was 33 months (range: 23 to 180). Four of the 7 recurrences were stage IA at baseline. Tumor size >4 cm, International Federation of Gynecology and Obstetrics (FIGO) stage ≥IB, tumor necrosis, and tumor hemorrhage were each significantly associated with reduced recurrence-free survival in log-rank tests and univariable Cox models, with age older than 65 years being of marginal significance (hazard ratio [HR]: 5.4, 95% CI: 1.0-30.0, P = 0.05). Multivariable analyses suggested that FIGO stage ≥IB (HR: 27.5, 95% CI: 2.6-290.5), and age older than >65 years (HR: 21.8, 95% CI: 1.6-303.9) were the only parameters that were independently associated with recurrence. Cross-section analyses showed that tumor necrosis, tumor hemorrhage, and larger tumor size were significantly associated with a FIGO stage ≥IB status, which bolstered the conclusion that they are not independent predictors of recurrence. In summary, <10% of SCTs are clinically malignant, a substantially lower frequency than has previously been reported in the literature. Clinicopathologic predictors of patient outcomes that are prospectively applicable in practice could not be definitively established. Recurrences may occur many years (up to 15 y in this study) after primary resection, even in stage IA cases.

Uterine tumors resembling ovarian sex cord tumors are rarely reported with limited imaging findings.The current study reported two case of uterine tumors resembling ovarian sex cord tumors and described the detailed MRI findings,which would provide valuable imaging evidence for the diagnosis of such tumors.
子宫类似卵巢性索瘤样肿瘤较为罕见,关于其影像报道也较为少见。现报道子宫类似卵巢性索瘤样肿瘤MRI表现2例,为临床提供诊断经验。.

The objective of this study was retrospectively to analyze the clinical characteristics and 18F-FDG PET/CT findings in Meigs syndrome (MS) patients. A total of 21 patients with MS induced by ovarian stromal tumors and 69 patients with pseudo-MS caused by ovarian cancer (OC-PMS) were subjected to evaluation using 18F-FDG PET/CT. Visual and semi-quantitative methods were employed to analyze the PET/CT findings. Visual analysis included recording whether the density of the primary tumor was uniform, whether there were cystic changes and calcifications, and the location of serous fluid accumulation. Semi-quantitative analysis involved the measurement of the tumor size, SUVmax, and SUVmean. No significant difference was observed in the size and density of primary tumors between the MS group and the OC-PMS group. However, the SUVmax and SUVmean of tumors in the MS group were found to be significantly lower than those in the OC-PMS group. The amount of serous cavity effusion caused by ovarian sex cord stromal tumors was found to be unrelated to the size of the tumor, SUVmax, and SUVmean but was positively correlated with the level of Ca125. MS patients have both benign ovarian tumors and ascites and/or pleural effusion, which may be accompanied by elevated Ca125 levels. This should be considered as one of the differential diagnoses for ovarian cancer. Understanding the PET/CT features of MS can facilitate the attainment of an accurate diagnosis before surgery.

Uterine tumor resembling ovarian sex cord tumor (UTROSCT) is a rare mesenchymal neoplasm that was recently reported to exhibit recurrent NCOA1-3rearrangement with the most frequent partners ESR1 and GREB1. In this study, the clinicopathological characteristics of 17 UTROSCT cases were summarized; among them, the fusion genes of 12 cases were retrospectively analyzed by targeted RNA sequencing. The mean age of our cohort was 47 years (19-67 y). Although the majority of UTROSCTs had clear boundaries on gross examination, microscopic infiltration into the myometrium was observed in 82.4 % of cases. The tumor cells showed diffuse, trabecular, nested, reticular, pseudopapillary, hollow and solid tubular patterns, expressing sex cord, epithelial, and myogenic markers. Six fusion genes, including ESR1::NCOA3 (n = 4), ESR1::NCOA2 (n = 2), ESR1::CITED2 (n = 2), GREB1::NCOA2 (n = 2), GREB1::NCOA1 (n = 1), and GREB1::NCOA3 (n = 1), were identified. The fusion genes of the three cases with recurrence and metastasis were GREB1::NCOA2, ESR1::NCOA3, and ESR1::CITED2. All 3 cases of recurrent tumors showed infiltrative growth, with moderate to severe dysplasia of tumor cells and different degrees of rhabdomyoid differentiation. This is the first report of the ESR1::CITED2 fusion genes in UTROSCT, and one of the two patients had recurrence and metastasis. Compared with UTROSCT withESR1 rearrangement, UTROSCT with GREB1 rearrangement was more common in elderly patientsand was more likely to present with intramural masses, less sex cord differentiation, poor prognosis, and relapse and metastasis.

暂无摘要。

卵巢黏液瘤是一种罕见的卵巢良性肿瘤，类似发生在软组织的黏液瘤，为第5版WHO女性生殖系统肿瘤分类中新增的肿瘤类型。本文报道1例卵巢黏液瘤，局部合并硬化性间质瘤，复习相关文献，总结其临床病理特征，以提高病理医师对该病的认识。.