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The endodermal sinus tumor or Yolk sac tumor is a rare ovarian tumor that classically occurs in adolescents and young women, it is a histological type rarely found in clinical practice. We report the case of a 24-year-old woman presenting with an ovarian tumor of the endodermal sinus with hepatic metastasis revealed by a painful abdominal mass in the right hypochondrium associated with a deterioration of the general condition. The blood Alpha-Fetoprotein (AFP) level was 71,300 ng / ml. Abdominal magnetic resonance imaging revealed multiple liver nodules and masses, associated with a magma of secondary lymphadenopathy. The immunohistological study of the hepatic puncture biopsy allowed the diagnosis of a hepatic localization of an ovarian endodermal sinus tumor (Yolk-Sac-Tumor). The tumor was classified stage IV-B of the FIGO 2014 classification, which does not allow a curative approach. Chemotherapy treatment (BEP protocol) was started.

Objective : This article objective is to describe cytology diagnosis difficulties of yolk sac tumors of the ovary. Method : Case reports and literature review. Case : The author reports the case of a 24 year old woman who complained of an enlarged stomach. Serum AFP increased to 16,519.7 U/mL. Ultrasound examination revealed solid and irregular mass of ovarian, so the conclusion was suspect ovarian carcinoma. Conclusion of CT scan examination was a solid ovarian tumor. The working diagnosis was suspect ovarian carcinoma. Optimal debulking was performed, accompanied by taking a sample from the peritoneal rinse fluid. Microscopic examination of peritoneal fluid showed the distribution and group of cells with pleomorphic nuclei, partly hyperchromatic, partly vesicular with coarse chromatin and prominent nucleoli. There were also cells with polygonal nuclei, small nuclei, basophilic and vacuole cytoplasm with a mucoid background. These cells formed a solid arrangement. Conclusion from these features was carcinoma metastases to the peritoneal fluid. Microscopic examination from tumor tissue sample showed an ovarian yolk sac tumor appearance. Conclusion : Cytologic examination of peritoneal fluid in cases of ovarian yolk sac tumor is quite difficult to determine the diagnosis. This is due to the microscopic appearance of tumor cells which often looks like a carcinoma and limited literature about this tumors in the peritoneal fluid. Keywords: Yolk sac tumor, ovary

The lack of appropriate preclinical models of ovarian yolk sac tumor (OYST) is currently hindering the pursuit of new methods of treatment and investigation of the pathogenesis of the disease. We developed and characterized an OYST patient-derived xenograft (PDX) model in this study. Tumor fragments from a patient with an OYST were implanted subcutaneously into BALB/c Nude mice. Engrafted xenografts were compared with the original tumor according to histology, immunohistochemistry, humanized identified, and drug efficacy testing with in vivo treatment programs. There was a high degree of histologic and immunohistochemical (IHC) resemblance between the established PDX model and its corresponding human tumors. Bleomycin, etoposide, and cisplatin (JEB) chemotherapy regimens were effective in clinical patients and were effective in the OYST PDX model; therefore, the effect of PDX intervention was consistent with clinical outcomes of OYSTs. We have successfully established an OYST PDX model. This OYST model preserves the basic molecular features of the primary human tumor, thereby providing a valuable method to preclinically evaluate new treatments and explore disease pathogenesis.

Yolk sac tumor (YST), also called endodermal sinus tumor, is a rare malignant tumor accounting for less than 1% of all ovarian malignancies and is the second most common malignant ovarian germ cell tumor (MOGCT) (1,2). Disorder of sexual differentiation (DSD), once known as hermaphroditism, is also a rare cause of intersexuality disorders, where an individual has both male and female gonads (3,4). Due to the rarity of the condition, most articles about DSD are case reports and are limited in number (3,5). To the best of our knowledge, the YST of the ovary in adult DSD has rarely been reported in the literature. Here, we report a case of an adult DSD patient with an ovarian YST and their follow-ups after surgical treatment and chemotherapy.

Background Yolk sac tumors (YSTs) are rare malignancies that originate from germ cells and rarely present with endocrine symptoms. We report a case of a 13-year-old girl with a YST manifesting as virilization. Case A 13-year-old girl was diagnosed with a YST with endocrine symptoms because of Leydig cells in the stroma, which were identified using hematoxylin and eosin staining and immunohistochemistry. Summary and Conclusion This case suggests that clinicians should consider the possibility of YST when encountering patients with symptoms of virilization. When a YST is found to have endocrine function, a functioning stroma could present. Immunohistochemistry is useful for identifying stromal cells and performing a differential diagnosis.

Most patients with malignant ovarian germ cell tumors (MOGTCs) have a very good prognosis and chemotherapy provides curative treatment, however, patients with yolk sac tumors (OYSTs) have a significantly worse prognosis. OYSTs are rare tumors and promising results are expected with the use of specific therapeutic strategies after the failure of platinum-based first-line and salvage regimens. We initiated a project in collaboration with EORTC SPECTA, to explore the molecular characteristics of OYSTs. The pilot project used retrospective samples from ten OYST relapsed and disease-free patients. Each patient had a molecular analysis performed with FoundationOne CDx describing the following variables according to the Foundation Medicine Incorporation (FMI): alteration type (SNV, deletion), actionable gene alteration, therapies approved in EU (for patient&rsquo, s tumor type and other tumor types), tumor mutational burden (TMB), and microsatellite instability (MSI) status. A total of 10 patients with OYST diagnosed between 2007 and 2017 had a molecular analysis. A molecular alteration was identified in four patients (40%). A subset of three patients (33.3% of all patients) harbored targetable oncogenic mutations in KRAS, KIT, ARID1A. Two patients at relapse harbored a targetable mutation. This retrospective study identifies clinically relevant molecular alterations for all relapsed patients with molecular analysis. Dedicated studies are needed to demonstrate the efficacy of specific therapeutic strategies after the failure of platinum-based first-line and salvage regimens and to explore the potential relationship of a molecular alteration and patient outcome.

Ovarian germ cell tumors derive from ovarian germ cells and account for approximately 20% of all ovarian neoplasms. Only 1–2% of ovarian germ cell tumors are called malignant ovarian germ cell tumors and constitute approximately 3–5% of all malignant ovarian neoplasms.[1][1] Yolk sac tumor

Germ cell tumors account for 2–3% of ovarian malignancies. They typically occur in young females and have been rarely documented in postmenopausal women. Their rarity in postmenopausal women along with the histologic variability of yolk sac tumors, sometimes mimicking somatic malignancies, may pose a diagnostic challenge. We report a case of ovarian yolk sac tumor with associated mucinous carcinoma in a postmenopausal woman. They have been reported to behave aggressively and are less responsive to chemotherapy.

ObjectiveThe aim of this study was to evaluate the clinicopathologic characteristics of patients with ovarian yolk sac tumor and the benefit of omentectomy in patients with clinical early-stage disease.MethodsThe medical records of 66 patients with ovarian yolk sac tumor were reviewed retrospectively.ResultsThere were 37, 8, 14, and 7 patients with stages I, II, III, and IV disease, respectively. Sixty-five patients received surgery and adjuvant chemotherapy, and 1 had chemotherapy only. The median follow-up was 78 months. The overall 5-year survival rate was 86.0%. Univariate analysis revealed that stage (P = 0 .022), age (P = 0.001), residual tumor (P = 0.036), and satisfactory α-fetoprotein (AFP) decline (defined as normalization of AFP after the first or second cycles of postsurgery chemotherapy, P = 0.006) were significant prognostic factors. Multivariate analysis revealed that satisfactory AFP decline was an independent significant prognostic factor for overall survival (P = 0.028). The postoperative pathology showed that only 1 (2.7%) of 37 patients who received omentectomy without gross spread had omentum metastasis microscopically. The 5-year survival rates were 89.2% and 100.0% for stage I-II patients with (36 cases) or without (9 cases) omentectomy, respectively (P > 0.05). Three of the 7 patients with recurrence were successfully salvaged and lived 38.0, 102.6, and 45.2 months after initial diagnosis.ConclusionsPostsurgery satisfactory AFP decline was an independent significant prognostic factor for patient survival. Omentectomy might not be of therapeutic significance for clinical stage I-II patients.

Ovarian yolk sac tumors are germ cell tumors that usually present in children and young women. Rarely, these tumors can arise in older women, usually in conjunction with surface epithelial tumors, suggesting divergent differentiation from the latter. The combination of mixed ovarian yolk sac tumor and high-grade serous carcinoma is rare, with only one case documented in the literature. We present a case of mixed ovarian yolk sac tumor and high-grade serous carcinoma in a postmenopausal woman, including a brief discussion of the immunohistochemical findings and differential diagnosis. Despite the rarity of mixed ovarian yolk sac tumor and surface epithelial tumors, it is important to recognize the biphasic nature of the tumor, which should prompt a thorough immunohistochemical evaluation. The therapeutic and prognostic implications of proper diagnosis cannot be overemphasized.

Background: Ovarian Yolk sac tumor (OYST) is a rare entity of malignant ovarian germ cell tumors (MOGCT). Abdominal pain, a rapidly growing distending mass or irregular vaginal bleeding is the main presentation. Serum AFP is elevated in nearly all cases. The standard management is fertility preserving surgery with adjuvant chemotherapy. Aim of Work: To report and analyze retrospectively recorded cases that were either treated at National Cancer Institute/Egypt or referred there for advice about therapy. Materials and Methods: This is a retrospective single-institutional analysis of 18 cases of OYST treated at National Cancer Institute-Cairo University from January 2011 till December 2015. The clinical and pathological characteristics, treatment, and outcomes of these patients were analyzed. Results: Data from eighteen patients were obtained. The median age was 18 years (range: 15 - 22). Abdominal pain was the most common presentation (89%). The mean tumor size was 21cm (range: 8 - 30 cm). Eleven of our cases (61%) were stage I, seven cases and (39%) were stage IV at presentation. Fifteen cases (83%) underwent fertility preserving procedure & the standard surgical staging. Panhysterectomy & formal staging procedure was done only in two cases (11%). One case (6%) underwent bilateral salpingo-oophorectomy. 2 cases (11.1%) only underwent lymph node biopsy. 11 patient (61.1%) showed pure type YST while mixed type was present in the remaining 7 cases (38.8%): Dysgerminoma (one case, 5.6%), Dysgerminoma + immature teratoma (one case, 5.6%), Immature teratoma (2 cases, 11.1%) and Teratoma (3 cases, 16.7%). AFP was extremely elevated in all cases at presentation (median 4191 ng/mL; ranging: 725 ng/mL - 402,908 ng/mL). It showed decreased level after surgery (median 145 ng/ mL; ranging: 2 ng/mL - 38,000 ng/mL) & normalized after chemotherapy except for progressive disease. All cases started BEP regimen after surgery with complete remission in twelve cases. In follow up period (median 17 months; ranging: 2 - 48 months) two patients relapsed; the mean overall survival time was 34.2 and progression free survival was 33.84 months respectively. Conclusion: Ovarian yolk sac tumors are rare neoplasms. Surgery with adjuvant chemotherapy is the standard management. AFP is important for diagnosis, monitoring response to treatment and predicting relapse.

BackgroundWhereas among pediatric oncologists, ovarian yolk sac tumor (O-YST) is considered a chemosensitive tumor, it is often cited as an adverse prognostic factor in adult women with ovarian germ cell tumors.MethodsThe Malignant Germ Cell International Consortium data set included 6 pediatric clinical trials (United States, United Kingdom, and France) and 2 adult gynecology clinical trials (United States). Any patient with an O-YST that was International Federation of Gynecology and Obstetrics stage IC or higher and treated with a platinum-based chemotherapy was eligible. Age was modeled as a continuous and a categorical variable (children, 0-10 years; adolescents, 11–17 years; and adults, ≥18 years). In addition, analyses to establish the optimal cut point for age were conducted. Tumors were coded as pure YST (YST +/− teratoma), mixed YST (YST + other malignant germ cell component), or putative YST (“mixed” germ cell tumor + alpha-fetoprotein >1000 ng/mL). Histology, stage (II/III vs IV), preoperative alpha-fetoprotein levels (10,000 ng/mL), and chemotherapeutic regimen (carboplatin vs cisplatin) were analyzed as covariates.ResultsTwo hundred fifty-one patients (median age, 13 years; range, 0–38 years) were identified (78 children, 139 adolescents, and 34 adults). Histology was pure, mixed, and putative in 129, 56, and 66 cases, respectively. Twenty-six patients had stage IV disease, similarly distributed in the 3 age groups. Median follow-up was 5.8 years. The overall 5-year event-free survival and overall survival was 91% (95% confidence interval, 87%–94%) and 96% (92%–98%), respectively. Age did not affect risk of event or death, modeled either as a categorical or continuous variable. Analysis failed to identify an age cut point that affected risk. None of the other covariates investigated had a prognostic impact on event-free survival or overall survival.ConclusionsOvarian yolk sac tumors have an excellent outcome across all age-groups. Age has no apparent impact on the probability of event or death, allowing pediatric and gynecologic oncologists to enroll patients onto joint pediatric and adult trials.

An 8-year-old Japanese girl was admitted with an ovarian yolk sac tumor. Regarding birth history, the patient had been delivered by cesarean section at 25 weeks of gestation with a birthweight of 711g. She had required neonatal intensive care including oxygenation, various medications, and tests. After surgery and chemotherapy, there was no recurrence for 2 years, at the time of writing. Yolk sac tumor, which is a malignant germ cell tumor, is rare in children. Although the cause and risk factors are unclear, it has been reported that malignant germ cell tumors in childhood have been associated with pathophysiology at birth. Given that premature infants are more likely to survive due to advances in perinatal care, it is expected that such cases will increase in the near future. We suggest that children born prematurely require careful follow up.

A total of 3 to 5% of all ovarian malignancies include malignant ovarian germ cell tumors (MOGCTs). They are subdivided into germinomatous and non-germinomatous tumors. Common types of non-germinomatous tumors include yolk sac and immature teratoma. Ovarian yolk sac tumors (YST) are the second most frequent histological subtype of MOGCTs, after ovarian dysgerminoma. They account for 20% of MOGCTs and are frequent especially in childhood and in early adulthood. We report the case of a yolk sac tumor of the ovary in a 13yrs years old female.How to cite this articleSreehari A, Rupakala BM, Sarojamma C. Ovarian Yolk Sac Tumor: A Case Report with Review of Literature. J Med Sci 2016;2(1):15-17.

This study was conducted to determine the prognostic value of serum α-fetoprotein (AFP) levels in patients with ovarian yolk sac tumor (OYST). We performed a systematic review and meta-analysis to assess the associations between serum AFP level and prognosis in OYST. A total of 12 quantitative studies met the inclusion criteria. Preoperative AFP was not found to be associated with overall survival (OS) [odds ratio (OR)=0.84, 95% confidence interval (CI): 0.43–1.62] in OYST. However, a high postoperative AFP level was associated with worse OS (OR=0.16, 95% CI: 0.05–0.48) and relapse-free survival (RFS) (OR=0.18, 95% CI: 0.08–0.43) compared to a low postoperative AFP level in patients with OYST. In addition, a postoperative AFP level of >1,000 ng/ml was associated with a decrease in OS (OR=0.16, 95% CI: 0.05–0.50) and RFS (OR=0.21, 95% CI: 0.08–0.57). In conclusion, the postoperative, but not the preoperative, AFP level was found to be a prognostic factor in patients with OYST. In particular, a postoperative AFP level of >1,000 ng/ml was an indicator of poor prognosis in patients with OYST.

Ovarian yolk sac tumor (YST) is characterized by endodermal differentiation, and represents approximately 20% of malignant germ cell neoplasms. The age distribution of patients reported with YST ranges from 16 months to 46 years, but most patients are under 30 years of age (Talerman and Vang, 2011). YST is often found admixed with other types of germ cell neoplasm, but YST associated with epithelial ovarian carcinoma is extremely rare (Talerman and Vang, 2011). Unlike pure YST, YST associated with epithelial ovarian carcinoma is reported in elderly patients, and has a poor response to chemotherapy. Here we describe a case of YST associated with endometrioid adenocarcinoma in a postmenopausal woman. Our patient responded favorably to treatment with Docetaxel and Carboplatin combination chemotherapy, and has survived without evidence of relapse for 48 months postoperatively including a long term follow-up program. To the best of our knowledge, this is the first report of YST associated with endometrioid adenocarcinoma with longterm (> 48 months) successful therapy treatment, according to a Medline search of English publications.

The ovarian yolk sac tumor (OYST) is a very rare malignancy arising in young women. Our objective was to determine whether an early decline in serum alpha-fetoprotein (AFP) during chemotherapy has a prognostic impact.This retrospective study is based on prospectively recorded OYST cases at Gustave Roussy (Cancer Treatment Center). Survival curves were estimated using the Kaplan-Meier method. The serum AFP decline was calculated with the formula previously developed and validated in male patients with poor prognosis non-seminomatous germ cell tumors. Univariate and multivariate analyses were performed using the log-rank test and logistic regression, respectively.Data on AFP were available to calculate an early AFP decline in 57 patients. All patients had undergone surgery followed by chemotherapy. The 5-year overall survival (OS) and event-free survival (EFS) rates were 86% (95% CI: 74%-93%) and 84% (95% CI: 73%-91%), respectively. The disease stage, presence of ascites at presentation, use of the BEP regimen, serum AFP half-life and an early AFP decline were significantly predictive factors for OS and EFS in the univariate analysis. The OS rate was 100% and 49% (95% CI: 26%-72%) in patients with a favorable AFP decline and in those with an unfavorable decline, respectively (p0.001). In the multivariate analysis, only the presence of ascites at diagnosis (RR=7.3, p=0.03) and an unfavorable early AFP decline (RR=16.9, p0.01) were significant negative predictive factors for OS.An early AFP decline during chemotherapy is an independent prognostic factor in patients with OYSTs.No conflict of interest.

Ovarian yolk sac tumor (YST) is a malignant germ cell tumor generally arising in young females and occurs very rarely after menopause. We describe a case of ovarian YST in a 60-year-old postmenopausal woman. The patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and retroperitoneal lymphadenectomy for a right ovarian tumor 10 cm in diameter. Microscopic examination revealed that the tumor had the polyvesicular vitelline pattern and the glandular pattern of YST. There were no other types of germ cell tumor or any components of surface epithelial tumors, excluding “hetero-differentiation” of these. Immunohistochemically, tumor cells were positive for α-fetoprotein (AFP), SALL4, and only occasionally positive for cytokeratin 7, and negative for CD15 (leu-M1), and hepatocyte nuclear factor-1 beta. After surgery, the patient received 10 cycles of cisplatin-based chemotherapy. Preoperatively elevated serum AFP decreased to within the normal range after chemotherapy; however, the disease recurred and progressed rapidly, and she died of the disease 21 months after the operation. This case implies that the histogenesis of postmenopausal YST is quite different from that of a germ cell neoplasm in young females and this different histogenesis may cause the resistance to chemotherapy and poor prognosis.

Highlights • Tumor lysis syndrome is an oncologic emergency with profound metabolic derangements. • Germ cell tumors with large disease burden increase the risk for tumor lysis syndrome. • Herein we present a case of tumor lysis syndrome prior to initiation of cytotoxic chemotherapy for ovarian yolk sac tumor.

Background: Cisplatin is used as a key drug for ovarian yolk sac tumor (YST), but relapse may occur. Details of the molecular mechanism responsible for cisplatin resistance remain unclear. Methods: We established cisplatin-resistant ovarian YST cells (NOY1-CR) from parent NOY1. To characterize these cells, we examined cross-resistance to other anticancer drugs. Then, cDNA microarray analysis was performed to quantify gene expression in NOY1 and NOY1-CR cells. The expression of several potential genes related to drug resistance was compared with parent cells by real-time PCR and Western blotting. Knockdown experiments using small interfering RNA (siRNA) were also performed to confirm the genetic association with drug resistance. Results: The IC50 for cisplatin of NOY1-CR was 22.3-fold higher than that for parent cells. NOY1-CR cells showed cross-resistance to some drugs, but not to VP-16 and bleomycin. Microarray analysis identified 315 up-regulated and 412 down-regulated genes in NOY1-CR cells. Knockdown of GSTA1, which was up-regulated in resistant cells, by GSTA1 siRNA restored cisplatin sensitivity in NOY1-CR cells. Conclusions: Our data suggest the molecular mechanisms of cisplatin resistance and show the potential for GSTA1 to become a novel therapeutic target for cisplatin-resistant ovarian YST.

Rationale Ovarian yolk sac tumors (YSTs) are the second most common histologic type of ovarian germ cell tumors. Most patients are adolescent and young women, while cases in postmenopausal women were rarely reported. Due to its rarity, we know little about the treatment and prognosis of postmenopausal patients with ovarian YSTs. We reported 3 cases of mixed ovarian YST in postmenopausal females reviewed the related current English literature. Patient concerns The ages of the three patients were 61, 58 and 77 respectively. The three patients came to the hospital because of the abdominal discomfort or tenderness, and the third patient also has vaginal bleeding. Diagnoses Imaging examination revealed pelvic mass with cystic and solid components. The elevated serum AFP level and pathologcial examination confirmed mixed ovarian YST. Interventions All patients received surgery and chemotherapy. Two patients received PEB (cisplatin, etoposide, and bleomycin) chemotherapy initially and one patient received TC (paclitaxel carboplatin) chemotherapy. Outcomes One patient relapsed 8 months after diagnosis and underwent re-cytoreductive surgery. The three patients all survived at last follow-up. Lessons The diagnosis of postmenopausal ovarian YST is relatively difficult and it can coexist with other germ cell or epithelial tumors. Postmenopausal ovarian YSTs are aggressive, and may have a worse prognosis compared with those in young patients. More aggressive treatment is needed. When YST mixed with epithelial cancer components, adjuvant chemotherapy regimen should include platinum-based chemotherapy aiming at both epithelial ovarian cancer and germ cell tumors.

We are presenting a case of a 13-year-old girl with a chief complaint of lower abdominal pain. A computed tomographic scan showed a 10-cm right ovarian mass. The serologic testing showed high alpha-fetoprotein level. The patient underwent a right adnexectomy and an omentectomy. The final diagnosis was a yolk sac tumor with a major (95%) fibrosarcoma component, based on morphology and further confirmed by an extensive panel of immunohistochemistry. Postoperatively, the alpha-fetoprotein level dropped to normal. The patient completed 7 cycles of multiagent chemotherapy with cisplatin without major complications. At her 7-month follow-up, the patient still remains disease free. To our knowledge, our case is the first of its kind in the literature to document yolk sac tumor associated with fibrosarcoma in a teenage female. Owing to the rarity of this entity, the prognosis of this patient is widely unknown and it is yet still to be seen.

In the uterus, most alpha-fetoprotein (AFP) producing neoplasms belong to the categories of malignant mixed muellerian tumor, hepatoid carcinoma, and yolk sac tumor. We describe the case of a 44-year-old woman who presented with vaginal bleeding, pelvic mass, and preoperative elevated AFP serum level, clinically suggestive of a primary ovarian yolk sac tumor. However, histological examination revealed a uterine AFP-producing papillary serous carcinoma, which has metastasized to the ovaries. Upon review of the literature on primary endometrial neoplasms with AFP production, 2 categories with possibly different histogenesis and biological behavior become evident: the primary yolk sac tumor of the uterus in young patients (range, 24-49 years; mean, 34 years) and the common high grade endometrial carcinoma with yolk sac dedifferentiation or aberrant AFP production in elderly patients (range, 55-69 years; mean, 63.7 years). In addition to being only the second case of uterine AFP-producing papillary serous carcinoma, this case is unusual for its clinical and radiological presentation as well as the relatively young age of the patient.

Flagellate linear hyperpigmentation can rarely be caused by the chemotherapy agent, bleomycin. Herein, we describe the case of a 20-year-old woman treated with bleomycin for an ovarian yolk sac tumor and review the prominent features of this form of dermatitis.

Ovarian yolk sac tumors (YSTs) are rare neoplasms. No radiological study has been done to compare the imaging findings between this type of tumor and other ovarian tumors. Here we analyzed the CT findings of 11 pathologically proven ovarian YSTs and compared their imaging findings with 18 other types of ovarian tumors in the same age range. Patient age, tumor size, tumor shape, ascites and metastasis of two groups did not differ significantly (P > 0.05). A mixed solid-cystic nature, intratumoral hemorrhage, marked enhancement and dilated intratumoral vessel of two groups differed significantly (P

Aims Yolk sac tumours of the ovary (YST), also called endodermal sinus tumours (EST) are rare and highly malignant tumours of utmost importance occurring in children and young adults. In the past, outcome was very poor and the disease was almost always fatal. With the refinement of chemotherapeutic regimens in the last several decades, survival rates have improved dramatically and fertility preserving surgery has become possible. The aim of this review is to provide the reader with an analysis of the available literature and a rational approach to patient management. Methods We performed a literature search in the PubMed database and the reference lists of relevant articles concerning yolk sac tumours of the ovary. Findings and conclusion There are no randomised studies relating to the management of YST of the ovary. The available literature is composed of retrospective reviews and case reports that span several decades. Prognosis nowadays is good in stage I and II but still comparable to that of ovarian epithelial cancer in stage III and IV. The overall good prognosis is due to the fact that most of ovarian YST are diagnosed at an early stage where 5 years survival reaches 95%. Appropriate surgical treatment for patients where fertility needs to be preserved consists in laparotomy with unilateral salpingo-oophorectomy, peritoneal cytologic studies, omentectomy, multiple peritoneal and abdominal biopsies and resection of all visible disease. Three courses of BEP (bleomycin, etoposide, cisplatin) is the current standard therapy and four courses is recommended in case of bulky residual disease after surgery. Serum alpha-feto-protein (AFP) is a useful marker for the diagnosis and management of YST.

BACKGROUND: Functioning stromal cells are sometimes seen in primary and metastatic ovarian neoplasms. However, the cytologic features of functioning stromal cells have been described only rarely. CASE: A 19-year-old woman had an alpha-fetoprotein-producing ovarian yolk sac tumor with functioning stroma. Her preoperative serum testosterone level was elevated. Imprint cytology showed that the functioning stromal cells had centrally located nuclei with low nuclear/cytoplasmic ratios. Occasionally these cells had vacuolated cytoplasm, suggesting the presence of lipids. In sharp contrast, the yolk sac tumor cells had more pleomorphic and hyperchromatic nuclei. We were able to distinguish between neoplastic and functioning stromal cells on the basis of these findings. In addition, immunostaining for inhibin on imprint cytologic slides was of great help in identifying functioning stromal cells. CONCLUSION: Because functioning stromal cells may unexpectedly induce hormonal effects in a variety of ovarian tumors, it is important to identify such cells in cytologic specimens.

The co-existence of an endometrioid adenocarcinoma with an ovarian yolk sac tumor is very rare. Only eight cases have been reported in the English language literature. A 54-year-old postmenopausal woman with a 6-month history of progressive abdominal distension was seen at our hospital. MR imaging revealed a large cyst with a solid intramural node. Serum alpha-fetoprotein and CA125 levels were 13143 ng/ml and 170 U/ml, respectively. At laparotomy, a large tumor approximately 20 cm in diameter was found to occupy the abdominal cavity, adhering to the swollen appendix and part of the omentum. Microscopically, foci of endometrioid adenocarcinoma together with a yolk sac tumor component were observed within a large endometriotic cyst. Since the tumor was clinically staged 1c, the patient was given 500 mg of intraperitoneal carboplatin postoperatively, followed by five courses of combination chemotherapy consisting of cisplatin, etoposide and peplomycin at 4-week intervals. The levels of both serum alpha-fetoprotein and CA 125 decreased gradually to normal ranges and remained normal at the most recent follow-up on 29 December, 2001. In contrast to a very poor prognosis of this tumor in previously reported cases, our patient showed no sign of recurrence during a 21-month follow-up period.

5570 Background: Due to the highly aggressive biological behavior and early intra-abdominal spread potential of ovarian yolk sac tumor (YST), a considerable proportion of patients were inoperalbe at initial diagnosis. The aim of this study was to investigate the role of neoadjuvant chemotherapy (NACT) in this cohort of patients. Methods: Between July 1982 and December 2015, 58 patients diagnosed as YSTs were initially treated at Cancer Hospital of China Academy of Medical Science (CAMS), among which 18 were evaluated to be inoperable and received NACT. They were either too debilitated by the disease (ECOG ps≥2) to undergo a major surgery, or were with too extensively disseminated lesions to be optimally debulked. Massive ascites, pleural effusion, dyspnea, neoplastic fever, hypoproteinemia, or electrolyte disturbance were also common in these 18 patients. This cohort of patients was retrospectively reviewed. Results: One or 2 cycles of BEP regimens were prescribed to the majority of patients preoperatively. At the completion of NACT, all the 18 patients had ECOG ps≤1 . Seventeen of them (94.4%) exhibited clinical partial tumor regression and 1(5.6%) had clinical stable disease. Pathological complete tumor regression was observed in 2 (11.1%) patients, whereas the remaining 16(88.9%) had nearly complete pathological response. All these 18 patients were rendered operable at the completion of NACT, yielding a resection rate of 100%. Seventeen patients (94.4%) were cytoreduced to no macroscopic residual disease, 1 (5.6%) patient was cyto-reduced to macroscopic residual disease ≤2 cm. No major surgical complications occurred in our series. After a median follow-up of 83.5 months, 17 patients were free of recurrence. Five-year DFS and OS were both 94.4%. Fertility-sparing surgery was carried out in all the 17 patients with fertility desire, and 5 infants were delivered in 6 patients who attempted conception. Conclusions: One or 2 cycles of NACT followed by early cyto-reductive surgery offers a chance for cure in patients with extensively advanced YSTs. It allows for a more through and safe cyto-reductive surgery, improves survival outcomes, and helps pave the way for fertility-sparing surgery.

Yolk sac tumors (endodermal sinus tumors) are an important subgroup of germ cell tumors of the ovary. They are seen in young females and are highly aggressive, but respond well to treatment. Although different morphological patterns can be seen on light microscopy, granulomatous reaction and giant cells are a very rare occurrence. We report the case of a young female with ovarian yolk-sac tumor in which a widespread granulomatous reaction was seen, causing a diagnostic dilemma.

Objective of this article is to appraise diagnostic aspects and treatment modalities in childhood ovarian tumor in background of available evidence. Literature search on Pubmed revealed various aspects of epidemiology, histopathological diagnosis, and treatment of pediatric ovarian tumor. 85 % of childhood tumors are germ cell tumors. The varied histopathological picture in germ cell tumors poses a diagnostic and therapeutic challenge. Immunohistochemistry and newer genetic markers like SALL4 and karyopherin-2 (KPNA2) have been helpful in differentiating ovarian yolk sac tumor from dysgerminoma, teratomas, and other pictures of hepatoid, endometrioid, clear cell carcinomatous, and adenocarcinomatous tissues with varied malignant potential. Before platinum therapy, these tumors were almost fatal in children. Fertility-conserving surgery with bleomycin, etoposide, and cisplatin has dramatically changed the survival rates in these patients. This modality gives cancer cure with healthy offspring to female patients with childhood ovarian tumor. Evidence also supports this protocol resulting in successful pregnancy rates and safety of cytotoxic drugs in children born to these patients.

Objective The aim of the study was to identify the role of neoadjuvant chemotherapy (NACT) in the treatment of the patients with advanced stage ovarian yolk sac tumor (OYST). Methods The comparative study was based on 53 cases with advanced stage OYST registered at Peking Union Medical College Hospital from 1995 to 2010. Twenty one cases were treated with NACT followed by interval debulking surgery (IDS). Thirty two cases were treated with primary debulking surgery (PDS). Data on patient characteristics, treatment and survival were analyzed and compared between two groups to assess the outcome of NACT. Results After NACT, the overall status of the patients was improved significantly. Patients in NACT had better optimal cytoreduction rate and less peri-operative morbidities. Seven patients (13.2%) suffered from relapse. There was a significantly better PFS for patients with ovarian tumor size >20cm in the NACT than those underwent PDS. Residual disease >2cm was the independent risk factor of relapse. Conclusions NACT is the better treatment option for some patients with advanced stage OYST, especially for those with unresectable tumors and poor general condition.

A case of a yolk sac tumor (YST) with an ovarian endometrioid adenocarcinoma arising from endometriosis in a postmenopausal woman is described. Clinically, the case showed an aggressive course and did not respond to chemotherapy; the patient died of her disease 6 months after the operation. Histologically, the tumor consisted predominantly of an endometrioid adenocarcinoma, but it also showed microscopic features characteristic of YST. The tumor also contained benign endometriotic lesions with direct transition to the endometrioid adenocarcinoma. Immunohistochemical study revealed that not only the YST, but also the endometrioid adenocarcinoma was partly positive for alpha-fetoprotein. There was an inverse relationship between the endometriosis and the endometrioid adenocarcinoma in terms of the expression of sex steroid receptors and p53: adenocarcinoma cells were positive for p53 but negative for sex steroid receptors, whereas endometriotic epithelial cells were positive for sex steroid receptors but negative for p53.

Chen R-J, Huang S-C, Chow S-N, Hsieh C-Y, Lin M-C, Hsu H-C. Influence of chemotherapy or liver metastasis on the immunoelectrophoretic discrimination of alpha-fetoprotein from yolk sac tumor. Int J Gynecol Cancer 1997;7: 486– 489. The main purpose of this study was to determine whether chemotherapy or liver metastasis can interfere with the discrimination of yolk sac tumor alpha-fetoprotein (AFP) from that of hepatocellular carcinoma. The AFP pattern in six cases of yolk sac tumor with or without liver metastasis was studied both during and after chemotherapy using affinity immunoelectrophoresis. Six cases of hepatocellular carcinoma which werematched by AFP level with yolk sac tumors were used for comparison. The pattern produced by yolk sac tumor consists of a prominent L2 band and a light L3 band. Hepatocellular carcinoma produced a prominent L1 band and a light L3 band. The presence or absence of liver metastasis did not affect the pattern from ovarian yolk sac tumor, nor did chemotherapy interfere with the ability to discriminate between patterns. Our findings indicate that, using affinity immunoelectrophoresis, we are able to discriminate yolk sac tumor with or without liver metastasis from hepatocellular carcinoma. This discrimination is not affected by the administration of chemotherapy.

Ovarian yolk sac tumors are highly malignant germ cell tumors that commonly occur in young women. The hepatoid yolk sac tumor is a variant form of yolk sac tumor in which there has been extensive tumor differentiation to early liver tissue. Hepatoid differentiation is traditionally considered to signify a poor prognosis. This review focuses on diagnostic criteria and establishes the optimal treatment for patients with hepatoid yolk sac tumor. Immunohistochemical stains are useful for distinguishing hepatoid yolk sac tumor from the other hepatoid-appearing tumors. With a multidisciplinary treatment approach using platinum-based regimens, the outcome is similar to those of any yolk sac tumor.