A vaginal yolk sac tumor (YST) is a rare malignant germ cell tumor for infants and children, and it has been >50 years since the first case was reported. The treatment strategy has changed markedly in the past 50 years, from radical surgical treatment to conservative surgery combined with chemotherapy, and then to combined chemotherapy alone. The present study reports the case of a primary vaginal YST in a 13-month-old girl that was successfully treated by tumor resection combined with chemotherapy. The clinical symptoms, imaging features and treatment characteristics are described in detail, as well as the postoperative treatment. There was no local recurrence or metastasis for the 2 years of follow-up to date. A literature review was also conducted to investigate the clinicopathological features, treatment and prognosis of this tumor. Overall, surgery combined with bleomycin, etoposide and carboplatin combination chemotherapy can be an effective option for vaginal YST.

In vitro systems capable of reconstituting the process of mouse oogenesis are now being established to help develop further understanding of the mechanisms underlying oocyte/follicle development and differentiation. These systems could also help increase the production of useful livestock or genetically modified animals, and aid in identifying the causes of infertility in humans. Recently, we revealed, using an in vitro system for recapitulating oogenesis, that the activation of the estrogen signaling pathway induces abnormal follicle formation, that blocking estrogen-induced expression of anti-Müllerian hormone is crucial for normal follicle formation, and that the production of α-fetoprotein in fetal liver tissue is involved in normal in vivo follicle formation. In mouse fetuses, follicle formation is not carried out by factors within the ovaries but is instead orchestrated by distal endocrine factors. This review outlines findings from genetics, endocrinology, and in vitro studies regarding the factors that can affect the formation of primordial follicles in mammals.

Large cell neuroendocrine carcinoma (LCNEC) is a rare and aggressive cancer that typically presents in the lung. The current case report describes a 56 year old male who presented to Strong Memorial Hospital with progressive dyspnea and was revealed to have a large anterior mediastinal tumor with metastases to axillary, hilar and mediastinal lymph nodes. Tumor marker results revealed an elevated plasma level of α-fetoprotein (AFP), which initially pointed towards a diagnosis of teratoma, but the tumor stained positive for neuroendocrine markers CD56, chromogranin, and synaptophysin on biopsy, consistent with LCNEC. AFP-positive tumor cells were identified, and no alternate cause for the elevated AFP was identified. The patient underwent genetic testing revealing the tumor to be ALK, ROS1, KRAS, BRAF and EGFR wild type. The patient received 6 cycles of chemotherapy with cisplatin (80 mg/m2) and etoposide (100 mg/m2) and then radiation with an initial minor response. The patients course was complicated by the development of superior vena cava syndrome requiring emergency stenting. The results of the current case suggest that AFP may be worthy of further exploration as a potential tumor marker in LCNEC.

A 60-year-old male patient presented with a serum α-fetoprotein (AFP) level of 2940.5 ng/mL accompanied by a significant increase in serum globulin. Hepatitis B virus (HBV) DNA was 2.85 × 103 (normal value <1.0 × 103). B-mode ultrasound and magnetic resonance imaging showed characteristic manifestations and he was clinically diagnosed with hepatocellular carcinoma in January 2015. He received radiofrequency ablation and tenofovir disoproxil anti-HBV therapy and his serum AFP and globulin levels were significantly reduced. In March 2018, he presented at our Hematology Department with fatigue and a pale complexion. At that time, his serum AFP level was normal, with hemoglobin 61 g/L and globulin 64.7 g/L. He was diagnosed with multiple myeloma (MM) by bone marrow examination, and immunofixation electrophoresis. The patient received PCD chemotherapy (bortezomib 2.0 g/dL on days 1, 4, 8, and 11 plus cyclophosphamide 0.3 g/dL on days 1-4 plus dexamethasone 20 mg/dL on days 1-2, 4-5, 8-9, and 11-12). The patient finally died of MM complicated by disseminated intravascular coagulation.

Liver cancer is a common malignant disease in China, while the primary hepatic neuroendocrine tumor (PHNET) is extremely rare presented with various manifestations. We herein describe an interesting PHNET case, which was clinically diagnosed as hepatocellular carcinoma (HCC) based on strong clinical evidence and the national guideline, but confirmed to be PHNET by pathology. A42-year-old Chinese male was admitted for persistent upper abdominal pain, and CT scan revealed a huge liver tumor in the left lobe. The tumor presented attributes of tumor rupture, portal vein tumor thrombus, elevated serum AFP level, background hepatitis B virus infection history, and radiological features mimicking typical HCC. After left semi-hepatectomy was performed for curative treatment of the primary \"HCC\", the pathology demonstrated the correct diagnosis be poorly differentiated neuroendocrine carcinoma (NEC). The immunohistochemistry assays showed positive neuroendocrine markers of CgA and Syn and negative HCC markers of Hep Par 1 and GPC3, ruling out concurrent HCC. This case and literature review suggest that in spite of rare incidence, PHNET should be considered as a possible diagnosis when lacking a confirmative pathology result, even when sufficient evidence of typical presentation exist to establish the clinical diagnosis of HCC.

Yolk sac tumor (YST) is a malignant tumor derived from germ cells and usually occurs in the gonads. Extra-gonadal YST is most commonly seen in the vagina of children, but rarely in the cervix, vulva and endometrium. Primary YST of endometrium was extremely rare, standard treatment was still controversial and no guideline was established so far. The aim of the present study was to provide a comprehensive understanding and systematic thought for the management of primary YST of endometrium.
A systematic research of the literature was conducted in Scopus, PubMed database and Cochrane Library, including case reports and case series. We summarized clinical characteristics, treatments and prognosis of all collected cases. We collected data regarding patients, serum AFP level, initial symptoms, surgical information, postoperative chemotherapy and radiotherapy. A new case was also discussed.
We found only 26 cases have been reported previously. We reported a new case of primary endometrial YST in a 27-year-old woman, and in this case, we creatively performed bilaterally ovarian preservation and used DC (docetaxel and carboplatin) regimen of postoperative chemotherapy, we achieved a relatively good prognosis during the follow-up period of 14 months.
Primary YST of endometrium, kind of highly malignant germ cell tumors, was extremely rare, of which initial symptom is usually abnormal vaginal bleeding. Standard treatment was still controversial and no guideline was established so far. Surgery combining with postoperative chemotherapy was considered effective for treatment of primary endometrial YST. Decision on whether to preserve ovaries in young patient with early stage needs careful consideration, comprehensive preoperative assessment and full communication. Intraoperative biopsy and strict postoperative follow-up are recommended. However, standard chemotherapy regimen and feasibility of postoperative radiotherapy remains to be discussed.

Laminin (Ln)-332 consists of α3, β3, and γ2 chains, which mediate epithelial cell adhesion to the basement membrane. Ln-γ2, a component of Ln-332, is frequently expressed as a monomer in the invasion front of several types of malignant tissues without simultaneous expression of Ln-α3 and/or Ln-β3 chains. Moreover, monomeric Ln-γ2 induces tumor cell proliferation and migration in vitro. These unique biological activities indicate that monomeric Ln-γ2 could be a candidate biomarker for early cancer surveillance. However, the present immune method for monomeric Ln-γ2 detection can only predict its expression, since no antibody that specifically reacts with monomeric γ2, but not with heterotrimeric γ2 chain, is commercially available. We have, therefore, developed monoclonal antibodies to specifically detect monomeric Ln-γ2, and devised a highly sensitive method to measure serum monomeric Ln-γ2 levels using a fully automated chemiluminescent immunoassay (CLIA). We evaluated its diagnostic value in sera from patients with several digestive cancers, including hepatocellular carcinoma (HCC), and found serum monomeric Ln-γ2 to be a clinically available biomarker for HCC surveillance. The combination of monomeric Ln-γ2 and prothrombin induced by Vitamin K Absence II (PIVKA-II) may be more sensitive for clinical diagnosis of HCC than any currently used combination.

Hepatoid adenocarcinoma (HAC) is a rare and aggressive cancer subtype with a poor prognosis under metastatic conditions. Currently, there is no specific chemotherapy treatment protocol for advanced stages of the disease. This review evaluates two cases of HAC of gastric cardia with synchronous liver metastasis, which were successfully treated by chemotherapy with cisplatin (25 mg/m2 each day) (day 1 to day 3) and etoposide (100 mg/m2) (day 1 to day 3), every three weeks. A structured literary evaluation and reviewed pertinent articles are additionally presented to analyse the different approaches for the treatment of metastatic HAC (mHAC). The two described case reports demonstrated good partial responses to treatment and one of the two patients exhibited a good prognosis after a 9-year follow-up. A total of 20 case reports concerning the use of chemotherapy in mHAC were presented in the literature, 11 of which were regarding gastric HACs. The two aforementioned cases result in a total of 22 reports, 11 of which exhibited objective responses to chemotherapy, 8 patients demonstrated a partial response and 3 a complete response. The cisplatin-based regimen concerned 55% (12/22) patients and enabled 9 (75%) to exhibit a partial or complete response. A total of three patients exhibited a good prognosis in the long-term follow-up, all of them treated with a cisplatin-based regimen. It was demonstrated that the usual digestive regimens were not efficient in the treatment of HAC. In the absence of prospective trials, it may be hypothesized that cisplatin-based chemotherapy may be the most efficient first-line treatment in mHAC, with a 75% patient response, in accordance with the literature and follow-up cases.

Caroli\'s disease is a very rare congenital malformation, currently included in cystic diseases of the biliary tract, and is characterized by ectasia and dilatation of the intrahepatic bile ducts. Two clinical entities can be distinguished, Caroli\'s disease in which congenital hepatic impairment is limited to cystic dilatation and Caroli\'s syndrome in which congenital hepatic fibrosis coexists. We present two cases of atypical presentations of Caroli\'s disease. Case one was a 76-year-old man who was referred to our hospital for chronic non-remitting epigastric pain prior to diagnosis. Magnetic resonance cholangiopancreatography (MRCP) was performed, which revealed findings consistent with Caroli\'s disease. Laboratory investigation disclosed a raised α-fetoprotein. Left hepatectomy was performed due to suspected cholangiocarcinoma. Morphological findings were compatible with Caroli\'s disease and no evidence of malignancy was found. Case two was a 47-year-old man who presented with chronic epigastric pain and generalized abdominal discomfort. MRCP revealed findings compatible with Caroli\'s disease. The patient was discharged with ursodeoxycholic acid treatment and was later admitted twice due to inaugural episodes of cholangitis that were medically managed. Bisegmentectomies II and III were performed for suspected neoplasia after a gradual rise in α-fetoprotein and CA19-9 values were noted during follow-up. The surgical specimen confirmed Caroli\'s disease and there was no evidence of malignancy. Postoperative periods for both patients were favorable, and they remain asymptomatic and well to date.

BACKGROUND: Oxaliplatin-based chemotherapy is an alternative systemic treatment for patients with metastatic hepatocellular carcinoma (HCC) who were refractory or intolerant to sorafenib. To date, there have been no biomarkers reported to monitor the therapeutic efficacy and to predict the outcomes of HCC patients receiving oxaliplatin-based chemotherapy.
METHODS: Eighty-one HCC patients with elevated baseline α-fetoprotein (AFP) levels and extrahepatic spreading who received oxaliplatin-based chemotherapy between 2012 and 2014 were retrospectively enrolled in this study. Two AFP tests were performed, at baseline and 2-4 weeks after the initiation of chemotherapy. The change in AFP levels was calculated for survival analysis.
RESULTS: In the AFP decline group (decreased compared to baseline), the median progression-free survival (PFS) and overall survival (OS) were 7.0 months and 12.3 months, respectively. In the AFP nondecline group, the median PFS and OS were 2.3 months and 3.0 months, respectively. The difference in OS between the two groups was significant (p < 0.005). In the multivariate analysis for disease progression, the best response to chemotherapy and AFP decline were independent factors, with p values of 0.004 and 0.009, respectively. In the multivariate analysis for OS, the baseline Child-Pugh score, best response to chemotherapy, and AFP decline were independent prognostic factors, with p values of 0.01, 0.001, and 0.008, respectively. Additionally, the unit change in AFP level was predictive of PFS and OS with p values of 0.007 and 0.001, respectively.
CONCLUSIONS: The change in AFP levels 2-4 weeks after initiating oxaliplatin-based chemotherapy is useful to predict treatment response and survival.