Search

Clear cell carcinomas of the uterine corpus and cervix are rare gynecological cancers with limited information regarding the pathogenesis and biology. At present, the approach to management is the same as for patients with the more common histological subtypes of endometrioid endometrial cancer and adenocarcinoma of the cervix. Surgical resection is the standard treatment for patients with early-stage disease, but there is no evidence-based approach to direct the management of patients with more advanced-stage disease at presentation or with recurrent disease. We review the epidemiology, pathology, and what is known about both uterine corpus and cervical clear cell cancers and make management recommendations. [ABSTRACT FROM AUTHOR]

Sex cord stromal tumors (SCST) are rare cancers of the ovarian area in adults. They constitute a heterogeneous group of tumors that develop from the sex cords and the ovarian stroma. These tumors are detected typically at an early stage, and they may recur as late as 30 years after the initial treatment. Because 70% of the patients present with stage I tumors, surgery represents the most important therapeutic arm. There are no data to support any kind of postoperative adjuvant treatment for patients with stage IA or IB SCSTs, given the indolent nature of these neoplasms and the overall good prognosis. The long natural history of the disease may lead to repeated surgical procedure should a relapse occurs. Platinum-based chemotherapy is currently used for patients with advanced stage SCSTs or recurrent disease, with an overall response rate of 63% to 80%. The indolent nature of SCSTs with the tendency for late recurrence requires long-term follow-up. [ABSTRACT FROM AUTHOR]

Clear cell carcinoma of the ovary (CCC) is a histologic subtype of epithelial ovarian cancer with a distinct clinical behavior. There are marked geographic differences in the prevalence of CCC. The CCC is more likely to be detected at an early stage than high-grade serous cancers, and when confined within the ovary, the prognosis is good. However, advanced disease is associated with a very poor prognosis and resistance to standard treatment. Cytoreductive surgery should be performed for patients with stage II, III, or IV disease. An international phase III study to compare irinotecan/cisplatin and paclitaxel/carboplatin as adjuvant chemotherapy for stage IIV CCC has completed enrollment (GCIG/JGOG3017). Considering the frequent PIK3CA mutation in CCC, dual inhibitors targeting PI3K, AKT in the mTOR pathway, are promising. Performing these trials and generating the evidence will require considerable international collaboration. [ABSTRACT FROM AUTHOR]

Uterine serous carcinoma (USC) represents a rare and aggressive histologic subtype of endometrial cancer, associated with a poor prognosis. This article critically reviews the literature pertinent to the epidemiology, pathology, molecular biology, diagnosis, management, and perspectives of patients with USC.As one of a series of The Gynecologic Cancer InterGroup (GCIG) Rare Tumor Working Group in London, November 2013, we discussed about USC many times with various experts among international GCIG groups.Both USC and approximately 25% of high-grade endometrioid tumors represent extensive copy number alterations, few DNA methylation changes, low estrogen and progesterone levels, and frequent P53mutations. Uterine serous carcinoma shares molecular characteristics with ovarian serous and basal-like breast carcinomas. In addition to optimal surgery, platinum- and taxane-based chemotherapy should be considered in the treatment of both early- and advanced-stage disease. The combination of radiation and chemotherapy appears to be associated with the highest survival rates. The role of radiation therapy in the management of this disease, with a high propensity for distant failures, remains elusive.Uterine serous carcinoma is a unique and biologically aggressive subtype of endometrial cancer and should be studied as a distinct entity. Futures studies should identify the optimized chemotherapy and radiation regimens, sequence of therapy and schedule, and the role of targeted biologic therapy. [ABSTRACT FROM AUTHOR]

Mullerian adenosarcomas of the female genital tract are rare malignancies, originally described in the uterus, the most common site of origin, but they may also arise in extrauterine locations. Uterine adenosarcomas make up 5% of uterine sarcomas and tend to occur in postmenopausal women. They are usually low-grade tumors and are characterized by a benign epithelial component with a malignant mesenchymal component, which is typically a low-grade endometrial stromal sarcoma but can also be a high-grade sarcoma. Tumors that exhibit a high-grade sarcomatous overgrowth have a worse outcome. Adenosarcomas have been described as being midway along the spectrum between benign adenofibromas and carcinosarcomas. They generally have a good prognosis with the exception of deeply invasive tumors or those with high-grade sarcomatous overgrowth. Extrauterine adenosarcomas also have a higher risk for recurrence. In view of their rarity, there have not been any clinical trials in mullerian adenosarcomas and relatively little research. This article reviews the current knowledge and provides recommendation for the management of mullerian adenosarcomas. [ABSTRACT FROM AUTHOR]

Carcinosarcomas (also known as malignant mixed müllerian tumors) are rare and highly aggressive epithelial malignancies that contain both malignant sarcomatous and carcinomatous elements. Uterine carcinosarcomas (UCs) are uncommon with approximately more than 35% presenting with extra uterine disease at diagnosis. Up to 90% ovarian carcinosarcomas (OCs) will have disease that has spread beyond the ovary. Prognosis for localized stage disease is poor with a high risk of recurrences, both local and distant, occurring within 1 year. The survival of women with advanced UC or OC is worse than survival of endometrioid or high-grade serous histologies. No improvement in survival rates has been observed in the past few decades with an overall median survival of less than 2 years. Currently, there is no clear evidence to establish consensus guidelines for therapeutic management of carcinosarcomas. Until recently, gynecological carcinosarcomas were considered as a subtype of sarcoma and treated as such. However, carcinosarcomas are now known to be metaplastic carcinomas and so should be treated as endometrial or ovarian high-risk carcinomas, despite the lack of specific data. For UCs, a comprehensive approach to management is recommended with complete surgical staging followed by systemic chemotherapy in patients with both early and advanced stage disease. Active agents include paraplatin, cisplatin, ifosfamide, and paclitaxel. The combination of carboplatin-paclitaxel is the most commonly used regimen in the adjuvant and advanced setting. Adjuvant radiotherapy (external beam irradiation and/or vaginal brachytherapy) has not shown any overall survival benefit but has been reported to decrease local recurrences. For OCs and for other ovarian epithelial cancer, the mainstay of treatment remains cytoreductive surgical effort followed, even in early stage, by platinum-based chemotherapy, usually carboplatin-paclitaxel. [ABSTRACT FROM AUTHOR]

Squamous cell carcinoma of the ovary is a rare complication of mature cystic teratoma. The epidemiology, pathology, diagnosis, and management of this rare tumor are reviewed. Clinical characteristics, preoperative imaging, and tumor markers may help to predict malignancy preoperatively. Complete cytoreduction should be the aim of surgery. The prognosis for stage 1A disease is good, but for women with advanced or recurrent disease, it is very poor and has not improved in recent years. At present, there are insufficient data to provide clear guidance on the optimal management strategy for advanced disease, and there is a need to gain an understanding of the biology and to develop novel effective therapies. This will require coordinated international collaboration. [ABSTRACT FROM AUTHOR]

Low-grade serous ovarian cancer is a recently described histological subtype of ovarian cancer that is clinically and molecularly distinct from the 4 other main histological subtypes (high-grade serous, clear cell, endometrioid, and mucinous). In particular, it differs from high-grade serous ovarian cancer in that it presents at a much younger age, is more indolent, and is relatively chemoresistant. Very few clinical trials have been performed exclusively in this tumor type; and as such, specific data guiding optimal management are limited. [ABSTRACT FROM AUTHOR]

Introduction The majority of patients who receive parenteral nutrition (PN) at the Beatson West of Scotland Cancer Centre (BWoSCC) have ovarian cancer and bowel obstruction. A previous study at the BWoSCC only included 10 ovarian cancer patients with bowel obstruction receiving PN. We now have the opportunity to review outcomes in a larger group of patients. Methods Using the nutritional team records patients who received PN with solid tumours were identified. A retrospective review of 63 individual case notes of patients who received PN from 2013 to 2017 was completed. Results During the above period 63 patients received PN of which 39 had ovarian cancer. 19 patients had platinum sensitive (PS) ovarian cancer (median age 61, age range 38-73) and 17 had platinum resistant (PR) ovarian cancer (median age 60, age range 43-77). 3 patients could not be classified by platinum sensitivity as they did not survive beyond the first cycle of treatment. In 38 patients with ovarian cancer the indication for PN was bowel obstruction. Mean and median hospital stays in ovarian cancer patients were 52.4 (PS: 51.2, PR: 52.4) and 40 (PS: 40, PR 51) days respectively. Inpatient hospital stays ranged from 18-116 days in PS patients and 5-104 days in PR patients. 65% of PS patients who received systemic anticancer treatment (SACT) during obstruction responded to this versus 38% of PR patients. Patients were most likely to respond to SACT during the 1st line of chemotherapy. Both PS and PR patients were on average receiving 3rd line chemotherapy during PN. 15 ovarian cancer patients experienced complications associated with PN which included line sepsis (11 patients), electrolyte disturbance (4 patients) and blocked line (1 patient). The mean and median survival in patients with PS ovarian cancer was 1068 and 1128 days respectively (range 80-2245 days). The mean and median survival in patients with PR ovarian cancer was 694 and 690 days respectively (range 198-1164). The mean survival in all ovarian cancer patients after PN was 110 days (133 days in PS, 104 days in PR). Conclusion The majority of PN patients had ovarian cancer and were being treated for bowel obstruction. These patients experience long hospital admissions. A greater proportion of PS ovarian cancer patients responded to SACT during bowel obstruction. Patients receiving 1st line chemotherapy are most likely to respond to SACT. Most patients do not experience complications associated with PN. Patients with PS ovarian cancer survived longer on average. Although the number of patients in this study is much larger than the previous study, numbers are still small. These results do however give an indication of outcomes for patients with ovarian cancer and bowel obstruction who receive PN. Although PS patients do better generally than PR patients it would seem that some PR patients do benefit from PN and SACT for bowel obstruction. Future work should focus on identifying the correct patients for such treatments. [ABSTRACT FROM AUTHOR]

Introduction We sought to identify the genomic abnormalities in squamous cell carcinomas (SCC) arising in ovarian mature cystic teratoma (MCT, also known as dermoid cyst), a rare gynaecological malignancy of poor prognosis. Materials /Patients and methods We performed copy number, mutational state and zygosity analysis of 151 genes in SCC arising in MCT (n=25) using next-generation sequencing. The presence of high/intermediate risk HPV genotypes was assessed by quantitative PCR. Whole genome sequencing (WGS) was completed on 2 MCT cases and all genomic events were correlated with clinical features and outcome. Results MCT had a low mutation burden with a mean of only 1 mutation per case. WGS on 2 MCT cases revealed no driver mutations or rearrangements. Zygosity analyses of MCT indicated four separate patterns, suggesting that MCT can arise from errors at various stages of oogenesis. A total of 244 abnormalities were identified in 79 genes in MCTassociated SCC, and the overall mutational burden was high (mean 10.2 mutations per megabase). No SCC was positive for HPV. The most frequently altered genes in SCC were TP53 (20/25 cases, 80%), PIK3CA (13/25 cases, 52%) and CDKN2A (11/25 cases, 44%). Mutation in TP53 was associated with improved overall survival. In 8/20 cases with TP53 mutations, two or more variants were identified, which were bi-allelic. Conclusions Ovarian MCT has low mutation burden. By contrast, ovarian SCC arising in MCT has a high mutational burden with TP53 mutation the most common abnormality. The presence TP53 mutation is a good prognostic factor. SCC arising in MCT share similar mutation profiles to other SCC. Given their rarity, they should be included in basket studies that recruit patients with SCC of other organs. [ABSTRACT FROM AUTHOR]