Is the presence of endometriosis associated with a survival benefit in pure ovarian clear cell carcinoma?

BACKGROUND: The purpose of this study was to compare the prognoses of women with pure ovarian clear cell carcinoma (OCCC) arising from endometriosis to those of women with pure OCCC not arising from endometriosis treated in the same manner. METHODS: A dual-institutional, retrospective database review was performed to identify patients with pure OCCC who were treated with maximal or optimal cytoreductive surgery (CRS) followed by paclitaxel/carboplatin chemotherapy between January 2006 and December 2016. Patients were divided into two groups according to the detection of cancer arising in endometriosis or not, on the basis of pathological findings. Demographic, clinicopathological, and survival data were collected, and prognosis was compared between the two groups. RESULTS: Ninety-three women who met the inclusion criteria were included. Of these patients, 48 (51.6%) were diagnosed with OCCC arising in endometriosis, while 45 (48.4%) had no concomitant endometriosis. OCCC arising in endometriosis was found more frequently in younger women and had a higher incidence of early stage disease when compared to OCCC patients without endometriosis. The 5-year overall survival (OS) rate of the patients with OCCC arising in endometriosis was found to be significantly longer than that of women who had OCCC without endometriosis (74.1 vs. 46.4%; p = 0.003). Although univariate analysis revealed the absence of endometriosis (p = 0.003) as a prognostic factor for decreased OS, the extent of CRS was identified as an independent prognostic factor for both recurrence-free survival (hazard ratio (HR) 8.7, 95% confidence interval (CI) 3.15-24.38; p < 0.001) and OS (HR 11.7, 95% CI 3.68-33.71; p < 0.001) on multivariate analysis. CONCLUSION: Our results suggest that endometriosis per se does not seem to affect the prognosis of pure OCCC.

A rare case of leiomyosarcoma originating from the left round ligament of the uterus.

Uterine leiomyosarcomas (LMS) are rare malignancies with a poor prognosis. The incidence is reported to be 3-7/100.000 per year. Preoperative and intraoperative differentiation between LMS and large leiomyoma is always challenging. Therefore, LMS are often diagnosed during postoperative histologic evaluation of hysterectomy or myomectomy specimens. LMS of the round ligament of the uterus which can represent as an inguinal or pelvic mass is extremely rare. To our knowledge, there is only one case report of LMS arising from the round ligament available in the literature. Herein, we aimed to present the second case of LMS originating from the left round ligament of the uterus in a premenopausal woman initially misdiagnosed as an ovarian tumor.

Diagnostic value of CA 19-9 in pregnancies complicated by spinal neural tube defects: a preliminary study.

OBJECTIVES: Various physiological and pathological conditions can induce significant variations in plasma concentrations of tumor markers, such as CA 19-9, which is present in the serum and amniotic fluid of pregnant women. Herein, we aimed to determine the clinical importance of maternal serum CA 19-9 levels in the diagnosis of neural tube defects (NTDs). MATERIAL AND METHODS: A total of 100 women were included in this controlled cross-sectional study. Thirty-three patients whose pregnancies were complicated by isolated meningocele or meningomyelocele constituted the study group, whereas 33 normal, healthy pregnant women constituted the control group, and 34 age- and body mass index (BMI)-matched non-pregnant women were chosen for the validation group. RESULTS: The mean maternal serum CA 19-9 levels were 17.2 ± 17.0 IU/mL, 7.1 ± 5.9 IU/mL, and 4.7 ± 3.6 IU/mL in the study, control, and validation groups, respectively (p < 0.001). ROC analyses showed that elevated CA 19-9 values may predict NTDs (p < 0.001). The cut-off value for CA 19-9 was found to be 9.6 IU/mL at 70% (51%-84%, 95% CI) sensitivity and 84% (74%-92%, 95% CI) specificity. CONCLUSIONS: CA 19-9 may be a promising noninvasive marker for NTDs. Further studies are needed to reveal the clinical applicability and diagnostic potential of maternal serum CA 19-9 levels in the identification of NTDs.

Retrospective evaluation of borderline ovarian tumors: single center experience of 183 cases.

PURPOSE: Borderline ovarian tumors (BOTs) constitute about a quarter of epithelial ovarian malignancies and require different treatment approaches. The present study aims to document the experience of a single center on the treatment outcome of women who had conservative or comprehensive surgery for BOTs. METHODS: One hundred eighty-three patients with BOTs, diagnosed and/or treated in our center between January of 2000 and March of 2013, were reviewed retrospectively. RESULTS: The mean age at diagnosis was 40.6 years old (range 17-78). Ninety-five patients (51 %) were ≤40 years. Comprehensive surgical staging and fertility sparing surgery were performed in 49 % (n = 91) and 48 % of patients (n = 89) respectively. A hundred and forty-seven patients had stage IA disease (80 %). The most common type of BOT was serous in histology with 18 % bilateralism. CA-125 and CA-199 levels were increased in 29 (19 %) and 15 (10 %) patients with stage IA disease. Non-invasive tumor implants were diagnosed in 9 patients (4 %) and uterine involvement was 2 % among BOT patients that underwent hysterectomies. The mean post-operative follow-up period was 20.4 months (range 6-78 months). Disease recurrence was seen in 5 patients indicating overall recurrence rate of 2.7 %. CONCLUSIONS: In our study, we evaluated a large data pool of 183 patients diagnosed with borderline epithelial ovarian tumors. BOTs have a relatively better prognosis than invasive epithelial ovarian cancer. Surgery with proper staging is the cornerstone of treatment. Patients with BOTs at the early stage can undergo fertility sparing surgery with close follow-up.

Treatment of recurrent vulvar Paget disease with imiquimod cream: a case report and review of the literature.

INTRODUCTION: Extra-mammary Paget disease is one of the rare neoplastic conditions of the skin. The most common site of involvement is the vulva and presents itself with erythematous plaques. Surgery is the most important treatment option. In the recent years, there are publications of the topical use of imiquimod cream in extra-mammary cutaneous Paget disease. We report the case of a woman with recurrent vulvar Paget disease, who underwent successful treatment with imiquimod cream. We also review the reports of other patients with vulvar Paget disease who were treated with topical imiquimod cream. CASE REPORT: A 65-year-old woman presented to the Oncology Outpatient Clinic with an itchy lesion in her vulva for 2 years. In the gynecologic examination, a hyperkeratotic erythematous lesion was found starting from the right labium to involve clitoris, with a size of 4 × 3 cm. Pathology result was reported as Paget disease. She was operated and wide vulvar excision was performed with a safety margin of 2 cm. Then recurrence two times occurred. Because she refused surgery, imiquimod cream 5% was applied for treatment. CONCLUSION: Imiquimod cream is an effective and safe therapeutic agent for both primary and recurring vulvar Paget disease.

A randomised controlled trial on melatonin and rosiglitazone for prevention of adhesion formation in a rat uterine horn model.

PURPOSE: To investigate the effectiveness of melatonin and rosiglitazone in reducing postoperative adhesion formation in a rat uterine horn model. METHODS: Thirty non-pregnant female Wistar albino rats, weighing 180-220 g, were used as a model for postoperative adhesion formation. The rats were randomised into three groups after seven standard lesions were inflicted in a 2-cm segment of each uterine horn and lower abdominal sidewall using bipolar cauterisation. The rats were treated with 10 mg/kg, intraperitoneal melatonin, and 1 mg/kg per day peroral rosiglitazone. No medication was given to the control group. As much as 20 uterine horns of 10 rats were evaluated in each group. Extent, severity, and degree of the adhesions to the uterine horns and, inflammation and fibrosis scores (histopathologically) were evaluated after 2 weeks of the treatment. RESULTS: There was no mortality in the groups and all of the rats recovered without incident after operation. Rosiglitazone group had lower adhesion scores [median (min-max ranges)] regarding extent, severity, and degree of the adhesions [0 (0-3), 0 (0-3) and 0 (0-3), respectively], which were significantly different (P < 0.001, P < 0.05 and P < 0.01, respectively) from those of the controls [1 (0-3), 2 (0-2) and 2 (0-3), respectively]; however, there were no statistically significant differences between rosiglitazone versus melatonin groups [1 (0-4), 2 (0-3) and 1 (0-3), respectively] and melatonin versus control groups. Moreover, no significant differences were determined between groups regarding histopathologic findings. CONCLUSION: Rosiglitazone, but not melatonin, is effective in prevention of adhesion formation in a rat uterine horn model.

Visceral bullae: A new finding in Bart's syndrome.

Congenital localized absence of the skin (CLAS) has been observed in various subsets of inherited epidermolysis bullosa (EB). Pyloric atresia (PA) is a rare disorder that is associated with EB. Here, we describe three preterm male infants with PA, CLAS, and EB at birth, one of whom had multiple bullae and erosions on the stomach and pericardium. To the best of our knowledge, this is the first report of findings regarding a PA-EB-CLAS association.

Metformin and atorvastatin reduce adhesion formation in a rat uterine horn model.

The aim of the present study was to determine whether atorvastatin and metformin are effective in preventing adhesions in a rat uterine horn model. A total of 40 non-pregnant, female Wistar albino rats, weighing 180-210 g, were used as a model for post-operative adhesion formation. The rats were randomized into four groups after seven standard lesions were inflicted in each uterine horn and lower abdominal sidewall using bipolar cauterization. The rats were given atorvastatin 2.5 mg/kg/day, p.o. (10 rats), atorvastatin 30 mg/kg/day, p.o. (10 rats), metformin 50 mg/kg/day, p.o. (10 rats) and no treatment was applied in the control group (10 rats). The animals were killed 2 weeks later and adhesions were scored both clinically and pathologically by authors blinded to groups. One rat in the control group died before the end of the 2 week period. Total clinical adhesion scores regarding extent, severity and degree of adhesions and histopathological findings including inflammation and fibrosis were significantly lower in the metformin (P < 0.001 and P < 0.01, respectively) and atorvastatin 30 mg/kg/day (P < 0.001 and P < 0.01, respectively) groups when compared with control group. Metformin and atorvastatin are both effective for prevention of adhesion formation in a rat uterine horn model.

Recurrence patterns and prognostic factors in lymphovascular space invasion-positive endometrioid endometrial cancer surgically confined to the uterus.

OBJECTIVE: The purpose of this study was to determine the patterns of failure and prognostic factors for lymphovascular space invasion (LVSI)-positive endometrioid endometrial cancer (EC) patients in the setting of negative lymph nodes (LNs). MATERIALS AND METHODS: A multicenter, retrospective department database review was performed to identify LVSI-positive patients with disease surgically confined to the uterus at two gynecologic oncology centers in Turkey. Demographic, clinicopathological and survival data were collected. RESULTS: We identified 185 LVSI-positivewomen with negative LNs during the study period. Fifty-five (29.7%) were classified as Stage IA, 94 (50.8%) as Stage IB, and 36 (19.5%) as Stage II. The median age at diagnosis was 59 years and the median duration of follow-up was 44 months. The total number of the recurrences was 12 (6.5%). We observed 5 (2.9%) loco-regional recurrences, 3 (1.5%) retroperitoneal failures, and 4 (2.0%) distant relapses. The 5-year progression-free survival (PFS) was 86.1% while the 5-year overall survival (OS) rate was 87.7%. Grade 3 histology (Hazard Ratio [HR] 2.9, 95% Confidence Interval [CI] 1.02-8.50; p = 0.04), cervical stromal invasion (HR 4.5, 95% CI 1.61-12.79; p = 0.004) and age ≥ 60 years (HR 5.8, 95% CI 1.62-21.32; p = 0.007) were found to be independent prognostic factors for decreased OS. Adjuvant treatment did not appear as a prognostic factor for OS even in univariate analysis. CONCLUSION: The recurrence rate among LVSI-positive endometrioid EC patients is low in the setting of negative LNs. However, one out of three patients with a recurrence experiences distant relapses which usually portend worse outcomes.

Vanishing endometrial carcinoma in hysterectomy specimens: probable implications for fertility sparing management

Background/aim: The vanishing cancer phenomenon was first reported in radical prostatectomy specimens in the absence of neo-adjuvant treatment. Reported cases are mostly well-differentiated and low-volume tumors. A similar entity was described for hysterectomy specimens of patients with biopsy proven endometrial cancer (EC). In this study, we discuss the probable reasons for vanishing EC and long-term follow-up results of EC patients without residual tumors in hysterectomy specimens. Materials and methods: This study was carried at two institutions in Ankara, Turkey, in a retrospective design. The computerized databases of both institutions were searched for endometrioid type EC patients whose final pathological specimens failed to show any residual tumor. Results: We evaluated 38 endometrial biopsy confirmed EC patients with no residual tumor detected in the hysterectomy specimens among a total of 224 women (17%) with the disease confined to the endometrium. During the follow-up period, no recurrences were noted among the patients. Conclusion: It can be suggested that premenopausal women with FIGO grade 1 endometrioid type EC with MRI proven "absent myometrial invasion" would have a significant probability of having no residual tumor after endometrial biopsy without any further medical treatment.

Clinical outcomes of cases with cervical dysplasia absent in cold knife conization specimens.

BACKGROUND: Cold knife conization is a surgical procedure that allows both diagnosis and treatment of cervical lesions at the same time. It is mainly performed for indications of high-grade cervical intraepithelial neoplasia (CIN). In this study, we aimed to investigate the clinical outcome of cases without CIN in cold knife conization specimen, following a high-grade lesion (CIN2/3) in cervical biopsy. MATERIALS AND METHODS: We performed a retrospective cohort study at a tertiary referral hospital between January 1st 2008 and August 1st 2012. Cases that underwent cold knife conization for CIN2/3 within the study period were included. Cone-negative (Group 1) and cone-positive (Group 2) cases were analyzed for various clinical parameters, and were compared in the 1-year post-conization period for histological recurrence and human papillomavirus (HPV) DNA status. RESULTS: A total of 173 women underwent cold knife conization for CIN2/3 within the study period. Twenty-two cases (12.7%) were included in Group 1 and 151 cases (87.3%) in Group 2. There were no significant differences between the two groups in terms of age, gravidity, parity, menopausal status and HPV-DNA status (pre-conization and 1 year post-conization) (p>0.05). Recurrence rates were also similar between the groups (9.1% vs 9.9%, p>0.05). CONCLUSIONS: Clinical outcomes were similar in terms of histological recurrence and HPV persistence after 1 year of follow-up between cone-negative and cone-positive cases. Clinical follow-up of cone-negative cases should therefore be performed similar to cone-positive cases.

Roles of sonography and hysteroscopy in the detection of premalignant and malignant polyps in women presenting with postmenopausal bleeding and thickened endometrium.

BACKGROUND: To assess the role of sonographic endometrial thickness and hysteroscopic polyp size in predicting premalignant and malignant polyps in postmenopausal women. MATERIALS AND METHODS: A total of 328 postmenopausal women with abnormal uterine bleeding and thickened endometrium underwent operative hysteroscopy due to detection of endometrial polyps were included in this retrospective study. Preoperative endometrial thickness measured by transvaginal ultrasonography and polyp size on hysteroscopy were noted. Hysteroscopic resection with histology was performed for endometrial polyps. Endometrial thickness and polyp size were evaluated on the basis of final diagnosis established by histologic examination. Receiver operator characteristic curves were calculated to assess the sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy of endometrial thickness and polyp size for detecting premalignant and malignant polyps. RESULTS: Premalignant and malignant polyps were identified in 26 (7.9%) of cases. Sonographic measurement showed a greater endometrial thickness in cases of premalignant and malignant polyps when compared to benign polyps. On surgical hysteroscopy, premalignant and malignant polyps were also larger. Endometrial thickness demonstrated a sensitivity of 53.8%, specificity of 85.8%, PPV of 24.6% and NPV of 95.6% at a cut-off limit of 11.5 mm with diagnostic accuracy of 83.2%. Polyp size has a diagnostic accuracy of 94.8% with a sensitivity of 92.3%, specificity of 95.0%, PPV of 61.5% and NPV of 99.3% at a cut-off point of 19.5mm. CONCLUSIONS: Endometrial thickness measured by transvaginal ultrasonography is not sufficient in predicting premalignant and malignant endometrial polyps in postmenopausal women with abnormal uterine bleeding and thickened endometrium. Polyp size on hysteroscopy is a more accurate parameter, because of better sensitivity and specificity. However, while polyp size ≥ 19.5mm seems to have a great accuracy for predicting premalignancy and malignancy, histologic evaluation is still necessary to exclude premalignant and malignant polyps.

Diagnosis and management of female adnexal tumor of probable Wolffian origin (FATWO) arising from ovary: a case report.

Female adnexal tumor of probable Wolffian origin (FATWO) is a rare neoplasm which is usually considered as benign, although in some cases metastasis or recurrences have been reported even after a long interval following the initial diagnosis. Preoperative diagnosis of FATWO is very difficult because of the rarity of the disease and the limited literature available. In this case report, we present a case of FATWO arising from the ovary and review the literature based on the clinical characteristics and management of this rare condition. A 51- year- old postmenopausal woman was referred to our clinic for evaluation of an adnexal mass. After diagnostic evaluation, the patient underwent explorative laparotomy. Intra-operatively, a solid- cystic mass was found in the right ovary, the rest of the abdomen and the pelvis were normal. The ovarian mass was removed and examined with frozen-section (FS). When the frozen section proved negative for malignancy, total abdominal hysterectomy and bilateral adnexectomy were performed. The anatomic study revealed a well-capsulated mass which was 3.5×1.5 cm in diameter. Based on pathological and immunohistochemical results, the final diagnosis was concluded to be FATWO. Adjuvant therapy was not administered. Te patient was followed up after discharge from the hospital. One year after surgery she was asymptomatic. No evidences of recurrence were observed throughout this period. Although FATWOs are rare tumors, they should be kept in mind in women with an abdominal mass. They can present diagnostic difficulties and the diagnosis is based on the exclusion of other neoplasms. FATWO has malignant potential, after the initial surgical treatment patients should be appropriately followed up for possible recurrence and metastasis.

Female Adnexial Tumor of Probable Wolffian Origin (FATWO) without Ki-67 Expression Reflecting Low Malignant Potential in a 55-Year-Old Woman.

Female adnexial tumors of probable Wolffian origin (FATWO) are rare tumors derived from the remnants of the mesonephric duct. These tumors generally exhibit a low malignant potential, but recurrence and or metastasis is possible during the course of the disease according to the tumors' possible malignant potential. We report a case of FATWO without estrogen and progesterone receptors and with negative immunostaining for Ki-67 (a proliferation marker) as a probable low-malignant-potential tumor. A 55-year-old woman presented with a complaint of heavy menstrual bleeding and pelvic pain. Pre-operative ultrasonographic evaluation revealed an intramural uterine leiomyoma of 4 cm in diameter and a right adnexial solid mass measuring 5 cm in diameter. Following total abdominal hysterectomy and bilateral salpingo-oophorectomy, immunostaining based on the labeled streptavidin-biotin method was performed on sections from representative blocks of paraffin-embedded tissues sampled from the mass, revealing a low mitotic index with negative Ki-67 immunostaining. Immunohistochemical staining with promising new markers and pathological investigation of the entire tumor are needed to determine the malignant behavior of an individual FATWO. Ki-67 is a helpful marker for determining Wolffian duct tumors' potential malignant behavior.

Prevention of adhesion formation in Wistar-albino rats by increased bowel movements achieved with oral Ricinus oil use for 8 days postoperatively: an experimental study.

OBJECTIVE: Adhesion formation frequently occurs after abdominopelvic surgery and can cause significant morbidity for patients. Meticulous hemostasis, minimal access surgery and utilization of surgical adjuvants intraoperatively are clinically useful measures to minimize adhesion formation. We investigated the clinical efficiency of oral Ricinus oil treatment for 8 days postoperatively to decrease adhesion formation in this case-control study in a rat model. STUDY DESIGN: Following computer-generated randomization, 24 female Wistar-albino rats were operated on, with 10 standard cautery lesions on the right uterine horn and two simple suture lesions on left uterine horn generated with absorbable material. Half (n=12) the rats received 0.13 g (0.2 ml) Ricinus oil emulsion (40 g/60 ml) via the oral route during the first 8 days postoperatively, and the remaining rats (n=11) were considered as controls. The extent, severity, degree, total adhesion scores and histopathological features of the adhesions were the main outcome measures. RESULTS: The degree and total adhesion formation scores in the Ricinus oil group and control group revealed significant differences in adhesion extent and severity. The total adhesion scores of the Ricinus oil and control groups were 3.00 ± 2.21 and 5.18 ± 2.78 respectively (P<0.05). Differences in type of inflammation, extent of inflammation and vascularization were statistically insignificant for suture and cautery lesions individually (P>0.05). CONCLUSIONS: Ricinus oil treatment following abdominopelvic surgery for the 8-day period that covers the completion of tissue healing process may be a promising, cheap and cost-effective treatment strategy for patients.

Squamous cell carcinoma of the vulva in a virgin patient with Turner syndrome.

Two types of gynecologic tumors are commonly described in the Turner syndrome, the first one is gonadoblastoma, which occurs in patients with Y chromosome abnormalities, and the second one is endometrial carcinoma which is mostly related with exogenous estrogen usage. Here, we describe an extremely rare case of squamous cell carcinoma of the vulva in a virgin woman with Turner syndrome. A 35-years old single, virgin woman referred to our Oncology Department with warty, necrotized, exophytic 6-7 cm vulvar mass. She had a history of primary amenorrhea and mosaic Turner syndrome was determined in her karyotype analysis. Biopsy specimen of the vulvar mass revealed squamous cell carcinoma of the vulva, and total vulvectomy with inguinal femoral lymphadenectomy was performed. The postoperative course was uneventful and there has been no recurrence of the disease up to date. Women with Turner syndrome have streak ovaries that produce very low estrogen and the squamous cell carcinoma of the vulva may have developed at an early age with Turner syndrome because of this low estrogen value similar to postmenopausal women. The current case is a special case due to its age of occurrence, virgin and Turner syndrome status.

Coexistence of borderline ovarian epithelial tumor, primary pelvic hydatid cyst, and lymphoepithelioma-like gastric carcinoma.

OBJECTIVES: Borderline ovarian tumors (BOTs) represent a heterogeneous group of ovarian epithelial neoplasms. Despite a favorable prognosis, 10-20% of BOTs exhibit progressively worsening clinic. Primary involvement of pelvic organs with echinococcus is very rare. Lymphoepithelioma-like gastric carcinoma is a rare neoplasm of the stomach. CASE REPORT: A 58-year-old woman referred with abdominal swelling and gastric complaints. Imaging studies revealed a huge cystic mass with multiple septations and solid component, another cystic mass with an appearance of cyst hydatid in the pelvis, and thickening of the small curvature of stomach. Gastroscopy revealed an ulcer with a suspicious malignant appearance, and histology of the endoscopic specimen showed severe chronic inflammation and lymphocytic infiltration. No other involvement of hydatid cyst was detected. In the exploration, there was a 25cm cystic lesion with solid components arising from right ovary, another 6cm cyst over the former, 7cm cystic lesion arising from left ovary, and 10cm mass near the small curvature of the stomach. Excision of the masses; total gastrectomy with esophagojejunal anastomosis; total abdominal hysterectomy; bilateral salpingo-oophorectomy; omentectomy; appendectomy; splenectomy; and pelvic, paraaortic, and coeliac lympadenectomy were performed. Final pathology revealed lymphoepithelioma-like gastric carcinoma, bilateral serous BOT, and hydatid cyst. DISCUSSION: Hydatid cyst should always be considered in the differential diagnosis of abdominopelvic masses in endemic regions of the world. Preoperative diagnosis of primary pelvic hydatid disease is difficult and awareness of its possibility is very important especially in patients residing in or coming from endemic areas.

Malignant struma ovarii with a focus of papillary thyroid cancer: a case report.

Struma ovarii is a rare form of ovarian neoplasm and its malignant transformation is even rarer. Because of its rarity, there is no consensus about its diagnosis and management in the literature. A 53-year-old woman with left adnexial mass underwent total abdominal hysterectomy with bilateral salpingo-oopherectomy. Postoperatively, she was diagnosed with a malignant struma ovarii. Postoperative thyroid ultrasonography revealed a 0.5-cm solid nodule in the thyroid gland and total thyroidectomy was done. Pathology report was nodular hyperplasia of benign character. She is currently disease-free for 1 year but long-term follow-up with thyroglobulin levels is necessary due to reports of increasing recurrence rates.