Ovarian Stromal Tumors Research Articles

Sertoli–Leydig Cell Tumour with Heterologous Element with Rare Mitotic Figure Associated with Multinodular Goitre: A Rare Case of Sex Cord Stromal Tumour of Ovary with Rare Association

Sertoli–Leydig cell tumours are uncommon sex cord stromal tumours of the ovary that often present diagnostic confusion due to the various morphological and histopathological patterns that may be assumed by the tumour. A 28-year-old female presented with abnormal uterine bleeding and thyromegaly is found to have right adnexal mass with elevated serum testosterone. The mass was diagnosed as a Sertoli–Leydig cell tumour, intermediate differentiation with heterologous elements and focal anaplasia. She was treated with surgical resection followed by adjuvant chemotherapy. Definitive diagnosis is made by histopathological study with immunohistochemistry. Prognosis is related to the degree of cellular differentiation and to the presence of heterologous elements with stage of disease at the time of diagnosis.

Gynecologic Malignancies in Pregnancy: Balancing Fetal Risks With Oncologic Safety.

Cancer occurs in 0.05% to 0.1% of all pregnancies. Despite literature reporting good oncologic and fetal outcomes in women treated for cancer during pregnancy, as many as 44% of gynecologists would offer termination, and 37% would not administer chemotherapy or radiotherapy in pregnancy. The aims of this study were to summarize current recommendations for the treatment of cervical and ovarian cancers in pregnancy and to review updates on existing knowledge regarding the safety of surgical and chemotherapeutic treatments in pregnancy, including both oncologic and fetal outcomes. A detailed literature review was performed on PubMed. The treatment of gynecologic malignancies during pregnancy mirrors that outside pregnancy, with a balance between maternal versus fetal health. Fertility-sparing surgery can be offered to stage IA2 and low-risk IB1 cervical, stage I epithelial ovarian, germ cell ovarian, or sex-cord stromal ovarian tumors. Delayed treatment can be offered for stage IB1 cervical cancer. Neoadjuvant and/or adjuvant chemotherapy can be given for advanced gynecologic cancers with good disease-free survival without significant adverse neonatal outcomes. A multidisciplinary approach and improved education of providers regarding the surgical and chemotherapeutic treatments in pregnancy are needed in order to fully inform patients regarding treatment options. Further research in women who are pregnant is needed to determine the safety of diagnostic and therapeutic procedures used in the nonpregnant woman. This article reviews and supports treatment of gynecologic cancer during pregnancy, calls for additional study and long-term follow-up, and justifies improved education of patients and providers regarding treatment options. Obstetricians and gynecologists, family physicians. After completing this activity, the learner should be better able to (1) review general principles in the management and treatment of gynecologic cancers in pregnancy, (2) review the diagnosis and treatment of cervical cancer in pregnancy, and (3) review the diagnosis and treatment of ovarian cancer in pregnancy.

Sclerosing Stromal Tumor of Ovary: A Case Report and Review of the Literatures

Sclerosing stromal tumor (SST) is a rare benign ovarian neoplasm classified as a type of sex cord-stromal tumor, accounting for approximately 2% of sex cord-stromal tumors. We present a 23 year old virgin female in this article as an SST case that came with complaint of menstrual abnormalities and right lower quadrant abdominal pain then the US, MRI and pathological findings will be described.

Ovarian tumors in children: 10-year experience from a tertiary care center in South India.

Ovarian tumors are uncommon in childhood and constitute around 1% of childhood malignancies. Two thirds of pediatric ovarian tumors are germ cell tumors. Epithelial ovarian tumors and stromal tumors are less frequent. We share our experience in childhood ovarian cancers, analyzing a series of cases with respect to the clinical profile, treatment and survival. All newly diagnosed ovarian tumors in children up to 14 years of age, registered in our Pediatric Oncology Division between January 2000 and December 2009 were retrospectively reviewed. There were 47 patients with newly diagnosed ovarian tumors. The mean age at presentation was 10.0 ± 3.4 years. The most common symptoms at presentation were acute abdominal pain (48.9%) and abdominal mass (40.4%). Precocious puberty was uncommon (6.3%). Histology was germ cell tumors in 44 cases and nongerm cell tumors in three cases. The benign teratomas (mature and immature grade 1 and 2; n=9) underwent complete surgical resection alone; none had recurrence on follow up. Of the remaining 35 GCTs, 31 patients were given chemotherapy and 4 refused treatment.26 out of the 31 patients completed chemotherapy with BEP (bleomycin, etoposide, cisplatin) regimen with acceptable toxicities. 5 children i.e.; (19.2%) developed recurrence. At a median follow up of 80 months, 10 year disease free survival was 80.8 ± 7.7% and 10 year overall survival was 92.7 ± 4.9%. Germ cell tumors are the most common ovarian malignancy in children. With surgery and chemotherapy using BEP, good outcome can be expected in these patients.

A phase II study of paclitaxel for the treatment of ovarian stromal tumors: An NRG Oncology/ Gynecologic Oncology Group Study

A phase II study of paclitaxel for the treatment of ovarian stromal tumors: An NRG Oncology/ Gynecologic Oncology Group Study

A phase II study of paclitaxel for the treatment of ovarian stromal tumors: An NRG Oncology/ Gynecologic Oncology Group Study

ObjectiveTo estimate the probability of complete clinical response and toxicity of paclitaxel as second-line chemotherapy in measurable disease patients with malignant tumors of the ovarian stroma, and to evaluate the value of inhibin for predicting response. MethodsThirty-one patients with histologically confirmed ovarian stromal tumor were enrolled from 2000 to 2013. Patients were required to have measurable recurrent disease, and to have received only one prior chemotherapy regimen. Paclitaxel 175mg/m2 was administered over a 3hour infusion, cycling every 21days. Inhibin levels were drawn within two weeks of initiation of treatment. ResultsOf 31 women enrolled, there was only one complete response (3.2%), and partial response in eight of 31 cases (25.8%). The pretreatment inhibin level for the single patient who had complete response was 88pg/mL. Median progression-free survival was 10.0months and overall survival was 73.6months. Myelosuppression was common with 12 of 31 patients (38.7%) suffering grade 3 or 4 neutropenia, leukopenia, or anemia. ConclusionThere were too few complete responses to warrant continued evaluation of paclitaxel as a single agent treatment for women with recurrent malignant ovarian stromal tumors with measurable disease according to the primary objective of the study. Toxicity of the regimen was acceptable. Pretreatment inhibin is not a reliable tumor marker as it was not elevated in the majority of patients.

Pathologic Risk Factors in Pediatric and Adolescent Patients With Clinical Stage I Testicular Stromal Tumors.

Testicular stromal tumors (TSTs) are rare. In adult men with TSTs, various pathologic risk factors have been identified in patients with clinically localized disease that increase the risk of occult metastatic disease (OMD). We systematically reviewed existing literature to analyze the impact of these risk factors on OMD in prepubertal (0 to 12 y) and postpubertal (13 to 21 y) patients. A literature search was conducted using the combination of terms: "testicular stromal tumors," "testicular leydig cell tumors," "testicular sertoli tumors," "testicular interstitial tumors," "testicular granulosa tumor," and "testicular sex cord tumors." Studies of patients 0 to 21 years with clinical stage I TSTs were included. A total of 100 patients from 31 publications were included with a median age at diagnosis of 5.7 years (range, 1.2 mo to 21 y). Seventy-nine patients were 12 years and below (median 7.2 mo) and 21 patients were 13 to 21 years (median 16 y). No patients in either group were identified to have OMD at retroperitoneal lymph node dissection or during follow-up surveillance (median follow-up 45.6 y; range, 4 to 360 mo). 99% of those 12 years and below versus 95% of those above 12 years had 0 to 1 pathologic risk factors, and 1% versus 5% had 2+ pathologic risk factors (P=0.38). Clinical stage I TSTs in adolescent, postpubertal patients appear to behave in a benign manner with few pathologic risk factors, similar to prepubertal patients. Given the low risk of relapse in this population, low-impact surveillance strategies are paramount. Prospective study of these patients is needed, and entry into a tumor registry such as the International Ovarian and Testicular Stromal Tumor Registry is important to learning more about this rare disease.

Lats1 Deletion Causes Increased Germ Cell Apoptosis and Follicular Cysts in Mouse Ovaries.

The Hippo signaling pathway is essential for regulating proliferation and apoptosis in mammalian cells. The LATS1 kinase is a core member of the Hippo signaling pathway that phosphorylates and inactivates the transcriptional co-activators YAP1 and WWTR1. Deletion of Lats1 results in low neonate survival and ovarian stromal tumors in surviving adults, but the effects of Lats1 on early follicular development are not understood. Here, the expression of Hippo pathway components including Wwtr1, Stk4, Stk3, Lats2, and Yap1 transcripts were decreased by 50% in mouse ovaries between 2 and 8 days of age while expression was maintained from 8 days to 21 days and after priming with eCG. LATS1, LATS2, and MOB1B were localized to both germ and somatic cells of primordial to antral follicles. Interestingly, YAP1 was predominantly cytoplasmic, whereas WWTR1 was nuclear in oocytes and somatic cells. Deletion of Lats1 caused an increase in germ cell apoptosis from 1.7% in control ovaries to 3.6% in Lats1 mutant ovaries and a 58% and 32% decrease in primordial and activated follicle numbers in cultured mutant ovaries. Surprisingly, there was an increase in Bmp15 but not Gdf9, Figla, Nobox transcripts or the somatic-specific transcripts Amh and Wnt4 in cultured Lats1 mutant ovaries. Last, Lats1 mutant ovaries developed ovarian cysts at a higher frequency (43%) than heterozygous (24%) and control ovaries (8%). Results showed that the Hippo pathway is active in ovarian follicles and that LATS1 is required to maintain the pool of germ cells and primordial follicles.

17β-estradiol inhibits spreading of metastatic cells from granulosa cell tumors through a non-genomic mechanism involving GPER1.

Granulosa cell tumor (GCT) is a rare and severe form of sex-cord stromal ovarian tumor that is characterized by its long natural history and tendency to recur years after surgical ablation. Because there is no efficient curative treatment beyond surgery, ~20% of patients die of the consequences of their tumor. However, very little is known of the molecular etiology of this pathology. About 70% of GCT patients present with elevated circulating estradiol (E2). Because this hormone is known to increase tumor growth and progression in a number of cancers, we investigated the possible role of E2 in GCTs. Cell-based studies with human GCT metastases and primary tumor-derived cells, ie KGN and COV434 cells, respectively, aimed at evaluating E2 effect on cell growth, migration and invasion. Importantly, we found that E2 did not affect GCT cell growth, but that it significantly decreased the migration and matrix invasion of metastatic GCT cells. Noteworthy, our molecular studies revealed that this effect was accompanied by the inhibition through non-genomic mechanisms of extracellular signal-regulated kinase 1/2 (ERK1/2), which is constitutively activated in GCTs. By using pharmacological and RNA silencing approaches, we found that E2 action was mediated by G protein-coupled estrogen receptor 1 (GPER1) signaling pathway. Analyses of GPER1 expression on tissue microarrays from human GCTs confirmed its expression in ~90% of GCTs. Overall, our study reveals that E2 would act via non-classical pathways to prevent metastasis spreading in GCTs and also reveals GPER1 as a possible target in this disease.

Sclerosing Sromal Tumor of the Ovary in Postmenopausal Women: A Report of Two Cases

Sclerosing stromal tumor (SST) was first delineated as a distinct ovarian sex cord stromal tumor in 1973 by Chalvardjian and Scully. It is a benign neoplasm, distinguished from other ovarian stromal tumors by the production of collagen and a pseudolobular pattern, and it tends to occur in the second and third decades of life in diagnosed patients. We discovered two rare cases of SST in post-menopausal women which are the topic of this report. These case studies are accompanied by a brief review of the literature.

Bilateral Sclerosing Stromal Ovarian Tumor in an Adolescent.

Sclerosing stromal tumor of the ovary is a rare, benign, sex cord stromal tumor occurring predominantly in younger women in the 2nd and 3rd decades of life. It typically presents unilaterally with only 2 previously reported cases of bilateral presentation. Common clinical presentations include pelvic or abdominal pain, a mass, or menstrual changes. Although occasionally presenting with hormonal manifestations, virilization as a result of androgen production by the tumor is rare. Here we present an extremely rare case of a sclerosing stromal ovarian tumor in a 14-year-old patient with bilateral presentation and with clinical and biochemical evidence of hyperandrogenemia.

Perspectives on signet ring stromal cell tumor and related signet ring cell lesions of the gonads.

In this article, we discuss advances in our knowledge of the pathology of signet ring stromal cell tumor and related signet ring cell lesions of the ovary and a single case of signet ring stromal cell tumor of the testis. We divide ovarian signet ring cell lesions into 3 categories that reflect differences in their pathogenesis and histologic appearance. With 1 exception, all authentic cases of signet ring stromal cell tumor have been unilateral. Cases of ovarian signet ring stromal cell tumor from the literature can arise in 2 ways. The majority of cases arise multifocally from fibroma, whereas the remainder likely arise directly from the ovarian stroma. In difficult cases, immunocytochemistry provides improved diagnostic accuracy in distinguishing signet ring stromal cell tumor and its mimics from Krukenberg tumor. The most useful antibodies in this regard are epithelial membrane antigen and vimentin.

CT diagnosis in the thecoma-fibroma group of the ovarian stromal tumors.

The aim of this study was to investigate characteristic CT manifestations of the group of ovarian thecoma-fibroma. 24 patients (26 lesions) presenting with the ovarian thecoma-fibroma were analyzed retrospectively, and the diagnosis were confirmed by pathology after surgery. Our findings included: 22 patients were unilateral, while 2 were bilateral; 12 lesions were located in the right side of ovary, while 14 lesions were in the left side. Of the 26 lesions, there were ovarian thecoma (16 lesions), fibrothecoma (6 lesions), and fibroma (4 lesions). The largest diameters of tumor ranged from 37 to 231mmwith the mean value of 100±44.29mm. 14 patients were accompanied by ascites. All the tumors had well-defined borders.The shape of 22 lesions appeared round or oval, and 4 lesions were irregular. The tumors were solid in 19 lesions, cystic in 2 lesions, and mixed in 5 lesions. Most of the tumors were of heterogeneous density. There were no (20 lesions) or slight enhancement (6 lesions) after injection of the contrast medium. CT values of plain scan, arterial phase and venous among three groups had no significant difference. The enhancement were in the range of 0-5HU in 10 lesions, and 6-17HU in 16 lesions. In conclusion, the characteristic CT manifestations of the group of ovarian thecoma-fibroma were: often unilateral solid mass with the shape of oval and well defined border; no enhancement or slight enhancement; accompanied by small amount of ascites.

Distinct Immunophenotypic Features of Ovarian Microcystic Stromal Tumor

Distinct Immunophenotypic Features of Ovarian Microcystic Stromal Tumor

On the pathogenesis of sclerosing stromal tumor of the ovary: a neoplasm in transition.

Sclerosing stromal tumor (SST) is a distinctive benign ovarian stromal neoplasm first reported in 1973. Although its initial description supports its characterization as an ovarian stromal tumor, its exact pathogenesis remains uncertain. It is usually hormonally inactive, but occasional tumors are estrogenic or androgenic, and virilization can occur during pregnancy. We report 11 cases of SST, 6 of which were associated with another type or other types of ovarian stromal tumor. In 4 of these, a transition from thecoma of either typical or luteinized type to SST was observed. Our index case was that of a 16-yr-old girl who had a typical thecoma that underwent involutional changes in an extensive subserosal portion of the tumor with conversion to SST. In our series, 3 cases of SST underwent transformation to ovarian myxoma, one of which also contained a component of thecoma. The active SST components stained for inhibin, steroidogenic factor 1, and α-smooth muscle actin, but were negative or occasionally weakly positive for desmin.

Clinical and genetic aspects of ovarian stromal tumors: A report from the International Ovarian and Testicular Stromal Tumor Registry.

5520 Background: Ovarian sex cord-stromal tumors are rare tumors that tend to occur in children, adolescents and young adults. The International Ovarian and Testicular Stromal Tumor (OTST) Registry...

Ovarian Microcystic Stromal Tumor: Report of A New Entity with Immunohistochemical and Ultrastructural Studies

Microcystic stromal tumor is a recently described rare subtype of ovarian tumor for which there has been no previously reported ultrastructural study. We report a case with the characteristic histological and immunohistochemical features and the first ultrastructural study. The immunohistochemical findings of strong and diffuse nuclear staining for beta catenin and P27 are suggestive of dysregulation of more than one genetic pathway. The ultrastructural findings are supportive of the previous postulation of an ovarian stromal origin of the neoplastic cells.

Ovarian Myxoma Mimicking Mucinous Cystadenocarcinoma in a Prepubertal Girl

Ovarian myxoma is a benign, hormonally inactive ovarian stromal tumor typically reported in reproductive aged women. While histologically similar to fibroma-thecomas, myxomas demonstrate unique oval to stellate cells with bland unclear nuclear features and rare mitotic activity. These tumors may be pure myxoma or associated with other sex-cord stromal tumors.

Sclerosing stromal tumor of the ovary: A rare entity with distinctive features

Sclerosing stromal tumor (SST) is an extremely rare ovarian sex cord-stromal tumor with distinctive pathological featuresand benign nature. Awareness of such entity is crucial because of its histopathologic similarity with other neoplastic andnon-neoplastic lesions of the ovary. In this report, a 29-year-old woman was presented with menstrual irregularities andpelvic pain. She underwent unilateral oophorectomy after detecting a right ovarian mass by ultrasonography. The case wasdiagnosed as ovarian sclerosing stromal tumor based on clinicopathologic and immunohistochemical features. Differentialdiagnosis is discussed with review of the literature data.

Involuting Luteinized Thecoma of the Ovary

Spontaneous tumor involution in ovarian stromal tumors is a poorly understood phenomenon. In this report, we describe a rare case of luteinized thecoma that showed extensive involutional changes, such that cellular elements diagnostic of luteinized thecoma were sparse. The convoluted contour of the tumor resembled that observed in a corpus albicans; however, the neoplasm was considerably larger, and the contents of the nodule differed from that of a corpus albicans. The diagnosis of luteinized thecoma was established by the identification of residual aggregates of neoplastic theca cells and a nodule of lutein cells that were positive for inhibin and steroidogenic factor-1. Features of involution within the tumor included a few theca and lutein cells with pyknotic nuclei and abundant cytoplasmic lipid, occasional large adipocytes among the lutein cells, extensive hyalinization, dystrophic calcification, a myxohyaline nodule, and adipose metaplasia. It is likely that some of the aforementioned changes are the result of accompanying ischemia. Cleaved caspase-3 staining patterns were negative within residual lutein and theca cells; thus, we were unable to establish the occurrence of apoptotic bodies.