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.

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。.

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本文报道1例罕见的原发灶未明的肺上皮样滋养细胞肿瘤，子宫等生殖道部位未见病变。患者女，33岁。因胆囊结石手术发现肺部巨大占位行肺叶切除。镜下肿瘤细胞呈单一的嗜酸性上皮样形态，巢团状或条索状排列，免疫组织化学：广谱细胞角蛋白、细胞角蛋白（CK）7、CK18、p63、p40、GATA3弥漫阳性，β-人绒毛膜促性腺激素、CD10部分阳性，CK5/6、CD146、抑制素、人胎盘泌乳素、胎盘碱性磷酸酶局灶阳性，波形蛋白、SALL4、甲状腺转录因子1均阴性，Ki-67阳性指数约60%，病理诊断为转移性上皮样滋养细胞肿瘤。该肿瘤属于比较罕见的妊娠滋养细胞肿瘤，且对化疗不敏感，因此首选外科手术完整切除病变。因其罕见性且容易误诊为肺鳞状细胞癌，临床需要加强对其认识。.

Temozolomide combined with whole-brain radiotherapy has good near-term efficacy and safety in the treatment of brain metastases from nonsmall cell lung cancer. In this study, we analyzed the risk factors for treatment and prognosis of brain metastases in gestational trophoblastic neoplasm (GTN) during pregnancy. Thirty-one patients with brain metastases were included in the study. All patients had a pathological diagnosis of primary lesions, including 23 adenocarcinomas, 7 squamous carcinomas, and 1 adenosquamous carcinoma, and had ≥3 intracranial metastases, controlled primary lesions (including resected primary lesions or unresectable primary lesions in partial remission (PR)/complete remission (CR) for ≥2 months) by cranial enhancement MRI and no extracranial metastases. Presence or control of extracranial metastases was for ≥2 months. The common adverse toxic effects were nausea, vomiting, neutropenia, and thrombocytopenia, but most patients tolerated them with symptomatic management.

BACKGROUND: Mixed gestational trophoblastic neoplasms are extremely rare and comprise a group of fetal trophoblastic tumors including choriocarcinomas, epithelioid trophoblastic tumors, and placental site trophoblastic tumors. We present a case of a patient with extrauterine mixed gestational trophoblastic neoplasm adjacent to the abdominal wall cesarean scar. On the basis of a literature review, this type of case has never been reported before due to the unique lesion location and low incidence.
METHODS: Our patient was a 39-year-old Chinese woman who had a history of two cesarean sections and one miscarriage. She had a recurrent anterior abdominal wall mass around her cesarean scar, and the mass was initially suspected of being choriocarcinoma of unknown origin. The patient had concomitant negative or mildly increased serum β-human chorionic gonadotropin at follow-up and no abnormal vaginal bleeding or abdominal pain. However, she underwent local excision twice and had two courses of chemotherapy with an etoposide and cisplatin regimen. She finally opted for exploratory laparotomy with abdominal wall lesion removal, subtotal hysterectomy, bilateral salpingectomy, and left ovarian cyst resection, which showed the abdominal wall lesion, whose components were revealed by microscopy and immunohistochemical staining to be approximately 90% epithelioid trophoblastic tumors and 10% choriocarcinomas from a solely extrauterine mixed gestational trophoblastic neoplasm around an abdominal wall cesarean scar.
CONCLUSIONS: It is worth noting whether epithelioid trophoblastic tumor exists in the setting of persistent positive low-level β-human chorionic gonadotropin. More studies are required to provide mechanistic insights into these mixed gestational trophoblastic neoplasms.

Epithelioid trophoblastic tumor (ETT) derived from intermediate trophoblasts is one type of gestational trophoblastic neoplasia (GTN), and it accounts for less than 2% of all gestational trophoblastic diseases (GTD). Extrauterine ETT is extremely rare, and there is currently no consistent strategy for its treatment and management. Therefore, the aim of the study is to analyze and summarize the clinicopathologic features of extrauterine ETT with or without metastasis.
The Web of Knowledge, Google Scholar, EMbase, congress of library, and PubMed were searched for extrauterine ETT without primary uterine lesions. All available data were extracted from published case reports or serial case reports, and then, the clinical and pathological characteristics were analyzed.
Twenty-two clinical studies consisting of 27 patients diagnosed with extrauterine ETT, according to the given inclusion and exclusion criteria, were included in the study. A total of 27 cases of extrauterine ETT were identified. Of these cases, four (14.81%) were located in the lungs, three (11.11%) in the ovaries, two (7.41%) in the vagina, and eight (29.63%) patients had other primary lesions. The patients originated from different continents, with 59% located in Asia and 26% in North America. Among 23 patients, the antecedent pregnancy prior to the diagnosis was full-term in 12 cases, abortion in 6 cases, hydatidiform mole in 3 cases, and invasive mole in 1 case. From the available antecedent information on pregnancy, the median interval from pregnancy to diagnosis of extrauterine ETT was 4 years. Additionally, the median gravidity and para of the patients was three times and two times, respectively. The median hCG titer was 14,374 mIU/mL in 5 patients, and the mean β-HCG titer was 3,724,805 mIU/mL in 14 patients. For all patients, the disease was confined to extrauterine ETT at diagnosis. From the available information, 20 cases were successfully treated by extraction of local lesions, and 12 cases received chemotherapy. Diagnosis was confirmed by histological tests. The Ki-67 staining ranged from 8.7 to 80%, and tumors were positive for hCG, PLAP, EMA, and p63.
In this study, we observed that abnormal levels of serum hCG titers and the local presentation of lesions with varying intervals after antecedent term pregnancy were the most common presenting features of extrauterine ETT. In addition, we found that the extraction of extrauterine lesions was needed for the treatment of extrauterine ETT. Of course, the follow-up was also important.

Objective: To elucidate the clinicopathologic characteristics of atypical epithelioid trophoblastic lesions with cyst and fistula formation after cesarean section. Methods: The clinical and pathological data of 4 cases of post-cesarean atypical epithelioid trophoblastic lesions with cyst and fistula formation diagnosed at Women\'s Hospital, School of Medicine, Zhejiang University during April 2007 to June 2018 were evaluated by hematoxylin and eosin stain and EnVision two-step immunohistochemical staining technique. Results: The age of the 4 patients ranged from 32 to 41 years, with a mean age of 36.5 years. Three patients recieved cystectomy and one underwent subtotal hysterectomy. Histologically, the lesions were well circumscribed and consisted of uniform cells of medium size, irregularly enlarged with hyperchromatic nuclei and 1 to 2 inconspicuous nucleoli embedded in abundant hyalinized matrix with fibrinoid material in the center. The cells exhibited immunohistochemical feature of chorionic-type intermediate trophoblastic cells (CK18+, p63+ and CD146-). All patients were alive without recurrence during follow-up of 1 to 40 months (mean=22 months). Conclusion: Atypical epithelioid trophoblastic lesion with cyst and fistula formation after cesarean section has unique histological features, and its biological behavior and prognosis are still unclear, which need further exploration.
目的： 探讨剖宫产术后不典型上皮样滋养细胞病变伴囊肿或瘘管形成的临床病理学特征，以进一步认识此类病变。 方法： 收集2007年4月至2018年6月浙江大学医学院附属妇产科医院4例子宫下段瘢痕处的剖宫产术后不典型上皮样滋养细胞病变伴囊肿或瘘管形成的患者临床病理学资料，免疫组织化学染色采用EnVision二步法，观察病变的组织学特征并总结临床病理学特点。 结果： 4例患者均为女性，发病年龄为32~41岁（平均36.5岁）。4例患者中，3例为包块切除标本，另1例为子宫次切标本。4例病变边界清楚，囊肿壁内衬4~10层单核滋养细胞，细胞膜清楚，核染色质略增粗，可见1~2个不明显的核仁，具有丰富的透明或嗜酸性细胞质，细胞周围可见纤维素样或透明变性物质沉积。免疫组织化学证实其具有典型的绒毛膜型中间型滋养细胞特点（CK18+，p63+，CD146-）。4例患者均有随访资料，随访时间1~40个月（平均22个月），均无复发。 结论： 剖宫产术后不典型上皮样滋养细胞病变伴囊肿或瘘管形成具有独特的组织学特征，其生物学行为及预后尚不明确，有待进一步探索。.

The aim is to analyze the clinical characteristics of intermediate trophoblastic tumor (ITT).
12 cases diagnosed at Qilu Hospital of Shandong University from January 2005 to December 2016 were investigated. Additionally, 50 cases were selected from MEDLINE and CBM databases between January 2010 and December 2016. The clinical data extracted from those aforementioned 62 cases were analyzed.
There were 42 cases with placental site trophoblastic tumor (PSTT), 19 cases with epithelioid trophoblastic tumor (ETT), and 1 case with mixed type (PSTT and ETT). No significant differences were found between PSTT and ETT in terms of age, type of antecedent pregnancy, main complaints, serum β-hCG peak, FIGO stage or prognosis. However, the interval between antecedent pregnancy and the onset was longer in ETT than in PSTT (P = 0.01). FIGO stage was irrelevant to serum β-hCG (P = 0.263). All 62 cases underwent surgeries and seven cases preserved fertility. Fifteen cases with high risk factors were not treated with adjuvant chemotherapy. Univariate analysis results showed that age ≧ 40 years, serum β-hCG peak ≧ 1000 IU/L and nonstandard treatment were associated with poor survival, but only age remained significant on multivariate analysis for ITT (P = 0.018).
PSTT and ETT have similar clinical characteristics generally. Serum β-hCG can not reflect the progress of ITT. Age ≧ 40 years is the independent high risk factor for ITT.