BACKGROUND: Epithelioid trophoblastic tumor (ETT) is an extremely rare variant of gestational trophoblastic neoplasms (GTNs). The biological behavior and therapeutic schedule of ETT remains to be defined which frequently poses diagnostic and therapeutic challenges. Although ETT is a relatively indolent malignancy tumor, the therapeutic efficacy and survival rate decrease significantly when presented with metastases. The lung is the most common site of ETT metastasis.
METHODS: A 39-year-old female patient presented with irregular vaginal bleeding and slight distention pain in lower abdomen.
METHODS: The patient was diagnosed ETT with lung metastasis after surgery and immunohistochemical staining.
METHODS: A total abdominal hysterectomy plus bilateral salpingectomy and histopathology were performed. The patient received 3 cycles of etoposide, methotrexate, actinomycin-D/etoposide, cisplatin (EMA/EP) regimen chemotherapy after surgery. Due to the presence of lung metastasis, she received pulmonary lesion resection and another cycle of postoperative chemotherapy.
RESULTS: The patients showed a good response to treatment initially. However, the patient did not complete the full initial treatment for family reasons and had signs of recurrence after 2.5 months. The serum β-hCG level gradually elevated and the lung imaging showed that the lesion area gradually expanded. After 15 months of follow-up, the patient declined further treatment due to a lack of presenting symptoms.
CONCLUSIONS: The diagnosis of ETT should be taken into consideration in patients with abnormal vaginal bleeding and low levels of β-hCG. Patients with metastatic disease should be treated with complete surgical resection and intensive combination chemotherapy to maximize the opportunity for cure. Targeted biological agents might be potential therapeutic strategies for chemotherapy-resistant or recurrent patients.

UNASSIGNED: Primary epithelioid trophoblastic tumor (ETT) of the lung is exceedingly rare. Only about three cases have been reported in Pubmed and worldwide literature. We presented a case of multiple primary ETT of the lung occurring in a 33-year-old Chinese female patient. Comprehensive physical examinations revealed no evidence of a primary lesion on the uterus or cervix uteri. Microscopic examination of the tumor demonstrated ETT of the lung, which was confirmed by immunohistochemical staining and declining level of beta-human choriogonadotropin ( β -HCG) after the operation. Our case revealed that the ETT can occur in the lung and should be considered when a female had a tumor of lung with increasing β -HCG.

BACKGROUND: Epithelioid Trophoblastic Tumor (ETT) and Placental Site Trophoblastic Tumor (PSTT) are two of the rarest GTNs that share certain features at diagnosis and management. Atypical Placental Site Nodule (APSN) is a relatively new entity considered as a premalignant lesion.
OBJECTIVE: The aim of this review was to summarize the main characteristics of each of these entities, their diagnostic features, and their treatment\'s standard of care including fertility-sparing treatments.
RESULTS: This study provides a thorough review of ETT, PSTT, and APSN.
CONCLUSIONS: The reader will gain an insight view of these rare tumors arising from the intermediate trophoblast.

We previously described a series of cases which characterize a distinct group of primary ovarian placental site trophoblastic tumor (PSTT) and epithelioid trophoblastic tumor (ETT) as a non-gestational set consistent with germ cell type/origin. Here we report a new case of ovarian non-gestational PSTT. The patient was a 13 year-old young female admitted for a spontaneous pneumothorax of the left lung. The pathology of lung wedge excision specimen demonstrated metastatic PSTT and ovarian biopsy showed atypical intermediate trophoblastic proliferation which was found to be PSTT in the subsequent salpingo-oophorectomy specimen. In the ovary, the tumor was composed of singly dispersed or small clusters of predominantly mononuclear cells and rare multinucleated cells extensively infiltrating the ovarian parenchyma, tubal mucosa, and paraovarian/paratubal soft tissue. A minor component of mature cystic teratoma (less than 5% of total tumor volume) was present. Immunohistochemically, the neoplastic cells of main tumor were diffusely immunoreactive for hPL, Gata3 and AE1/AE3, and had only rare hCG-positive or p63-positive cells. The morphology and immunohistochemical results support a PSTT. Molecular genotyping revealed an identical genotype pattern between the normal lung tissue and the metastatic PSTT, indicating its non-gestational nature of germ cell type/origin. This case represents the first case of such tumor with distant (lung) metastasis. This case also provides further evidence to support our recommendation that primary ovarian non-gestational intermediate trophoblastic tumors of germ cell type/origin, including PSTT and ETT, should be formally recognized in classification systems.

Choriocarcinoma is a highly vascular and invasive tumor of anaplastic trophoblast, predominantly made up of cytotrophoblasts and syncytiotrophoblasts without villi. Based on its origin, choriocarcinoma can be either gestational or non-gestational. Non-gestational choriocarcinoma can be of germ cell origin, or can be seen in association with a somatic high-grade malignancy. It is difficult to differentiate gestational from non-gestational choriocarcinoma, especially in the reproductive age group. It is important to distinguish between the two, for accurate staging and prognostication, deciding the primary treatment modality, (ie, surgery or chemotherapy), and tailoring follow-up timeframes after diagnosis. An extensive literature search was performed regarding all cases of non-gestational choriocarcinoma, published before March 2023. A note was made of whether the origin of choriocarcinoma was ascertained and how gestational choriocarcinoma was differentiated from non-gestational choriocarcinoma. The keywords used for literature search were \"non-gestational choriocarcinoma\", \"primary choriocarcinoma\", \"ovarian choriocarcinoma\", \"ovarian germ cell tumors\", or \"choriocarcinomatous differentiation\". This review aims to summarize the similarities and differences in the epidemiology, pathogenesis, clinical presentation, and management guidelines between gestational and non-gestational choriocarcinoma, which can form an important educational resource for clinicians and laboratory physicians dealing with such cases.

BACKGROUND: Gestational trophoblastic disease (GTD) is an uncommon but highly treatable condition. There is limited local evidence to guide therapy.
OBJECTIVE: To report the experience of a statewide registry in the treatment of low-risk gestational trophoblastic neoplasia (GTN) over a 20-year period.
METHODS: A retrospective review of the prospectively maintained GTD registry database was conducted. There were 144 patients identified with low-risk GTN, of which 115 were analysed. Patient demographics, treatment details and outcomes, including development of resistance, toxicity or relapse were reviewed.
RESULTS: The incidence of GTD was 2.6/1000 live births. There was 100% survival. The mean time from diagnosis to commencing treatment was 1.9 days (range 0-29 days). Seventy-seven percent of patients treated with methotrexate achieved complete response. Thirteen patients (11.3%) required multi-agent chemotherapy, for the treatment of resistant or relapsed disease. There was a higher rate of treatment resistance in those with World Health Organization (WHO) risk scores 5-6 (odds ratio (OR) 6.56, 95% CI 1.73-24.27, P = 0.005) and those with pre-treatment human chorionic gonadotropin >10 000 (OR 4.00 95% CI 1.73-24.27 P = 0.007). Four patients (3.5%) were diagnosed with choriocarcinoma after commencing treatment. Nine patients (7.8%) had successful surgical treatment for GTN, both alone and in combination with chemotherapy. The relapse rate was 4.3%; all were treated successfully with a combination of chemotherapy and surgery, and 93.9% of patients completed follow up through the registry.
CONCLUSIONS: Methotrexate is a highly effective treatment for low-risk GTN, especially with WHO risk score ≤4. The optimal treatment for those with risk scores of 5-6 requires further investigation.

上皮样滋养细胞肿瘤（epithelioid trophoblastic tumor）是一种罕见的恶性妊娠滋养细胞肿瘤，绝大多数发生于子宫体。本文报道1例原发于卵巢的上皮样滋养细胞肿瘤。镜下肿瘤细胞呈巢状或条索状排列，肿瘤巢之间有嗜酸性玻璃样物质沉积；细胞大小较一致，胞质嗜酸性至透明，胞膜清晰，核圆或椭圆形，可见小核仁，部分核有异型，散在多核细胞，未见核分裂象。免疫组织化学示：肿瘤细胞表达p63、细胞角蛋白（CK）8/18，PTEN弱表达，散在弱表达人绒毛膜促性腺激素和胎盘碱性磷酸酶，Ki-67阳性指数约30%。测序PIK3CA基因第9、20号外显子未检出突变。短串联重复序列（short tandem repeats，STR）基因分型示肿瘤组织在大多数STR基因位点显示有过量的非母源性（即父源性）等位基因。通过复习相关文献及最新进展，总结该病的临床病理特征，提高对本病的认识，以减少误诊和漏诊。.

For gestational trophoblastic neoplasia (GTN) affecting women of reproductive age, the chemotherapy-first approach is often preferred over the surgery-first approach. Low-risk GTN is treated with a chemotherapy-first approach, but the number of courses required can affect fertility. A surgery-first approach may decrease the number of chemotherapy courses, but its efficacy and safety compared to a chemotherapy-first approach are unclear. Thus, we investigated the efficacy and safety of the surgery-first approach compared to the chemotherapy-first approach in treating low-risk GTN. We searched the MEDLINE, Embase, Cochrane Central Register of Controlled Trials, ClinicalTrials.gov, and World Health Organization International Clinical Trials Registry Platform databases for relevant articles in July 2023. A systematic review and meta-analysis of outcome measures were conducted using a random-effects model. The primary outcomes were remission, the mean number of chemotherapy courses required to cure, and adverse events. The certainty of the evidence was evaluated using the Grading of Recommendations, Assessment, Development, and Evaluation approach. This study protocol was registered in the Open Science Framework (https://osf.io/kysvn/). Studies for low-risk GTN included a qualitative synthesis (with 2,192 participants and ten studies, eight of which were about second uterine curettage and two about hysterectomy) and a meta-analysis (with 138 participants and two randomized controlled trials (RCTs) that compared first-line treatments of second uterine curettage and chemotherapy). Second uterine curettage may result in little to no difference in remission (risk ratio: 1.00, 95% confidence interval: 0.96-1.05; low certainty) and a slight reduction in adverse events (risk ratio: 0.87, 95% confidence interval: 0.47-1.60; low certainty). The evidence is very uncertain on the mean number of chemotherapy courses (mean difference: 2.84 lower, 95% confidence interval: 7.31 lower to 1.63 higher; very low certainty). Based on clinical outcomes, second uterine curettage can be comparable to the chemotherapy-first approach as a first-line treatment option for low-risk GTN; however, the overall certainty of the evidence was low or very low.

To evaluate outcomes of European cross-border multidisciplinary tumor boards in terms of participation, adherence to treatment recommendations, and access to novel treatment strategies.
The European reference network for rare gynecological tumors (EURACAN G2 domain) aims to improve the diagnosis, management, and treatment of patients with these cancers. Cross-border multidisciplinary tumor boards were initiated to facilitate intercollegiate clinical discussions across Europe and increase patients\' access to specialist treatment recommendations and clinical trials. All G2 healthcare providers were invited to participate in monthly multidisciplinary meetings. Patient data were collected using a standardized form and case summaries were distributed before each meeting. After each tumor board, a meeting summary with treatment recommendations was sent to all participants and the project manager at the coordinating center. The multidisciplinary tumor board format and outcomes were regularly discussed at G2 domain meetings. Anonymized clinical data and treatment recommendations were registered in a prospective database. For this report, clinical data were collected between November 2017 and December 2020 and follow-up data retrieved until May 2021.
During the 3-year period, 31 multidisciplinary tumor boards were held with participants from 10 countries and 20 centers. 91 individual patients were discussed between one and six times for a total of 109 case discussions. Follow-up data were retrieved from 64 patients and 80 case discussions. Adherence to treatment recommendations was 99%. Multidisciplinary tumor board recommendations resulted in 11 patients getting access to off-label treatment and one patient being enrolled in a clinical trial in another European country. 14/91 patients were recommended for surveillance only when additional treatment had been considered locally.
Cross-border multidisciplinary tumor boards enable networking and clinical collaboration between healthcare professionals in different countries. Surveillance strategies, off-label drug use, and increased participation in clinical trials are possible benefits to patients with rare gynecological tumors.

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