Unexpectedly large myoepithelioma-like tumor of the vulvar region with two local recurrences: A rare case report and review of the literature.

Myoepithelioma-like tumors of the vulvar region (MELTVR) are rare. Only a limited number of MELTVRs are reported in the literature, and various aspects of this lesion still need to be clarified. In this study, we reported a case of MELTVR in a 46-year-old female. The uniqueness of the present case was its large size (12 cm) compared to the MELTVRs reported in earlier studies, the presence of two separate but attached lesions (one in labia majora and one in the mons pubis), and two recurrences within ten years after wide local excision. The second recurrence was managed with wide excision, and the patient remained disease-free during the two-year follow-up. This case highlights the local aggressiveness of MELTVR and the necessity of resection with an adequate margin. It also urges more awareness regarding the differential diagnosis of MELTVR with other lesions of the vulva, particularly when located in the labia majora and mons pubis.

Epithelioid Inflammatory Myofibroblastic Sarcoma With Poor Response to Crizotinib: A Case Report.

Introduction: Epithelioid type inflammatory myofibroblastic sarcoma (EIMS) is a subtype of inflammatory myofibroblastic tumor (IMT). It consists of round or epithelioid cells, and almost all types of EIMS contain rearrangements of the anaplastic lymphoma kinase (ALK) gene. Case presentation: We describe a 20-year-old female presenting with abdominal pain and a rapidly growing intraabdominal mass who underwent surgical tumor resection. She was diagnosed with EIMS. ALK and ki-67 expressions were detected in immunohistochemistry assessment. She was started with Crizotinib 200 mg twice a day, and chemotherapy was also initiated due to the recurrence of the disease 4 months after the initial treatment. She was unresponsive to all the medical regimens and died in 8 months. Conclusion: Approach to patients with EIMS is really challenging in terms of both diagnosis and treatment. Patients with combined surgical and non-surgical treatment regimen were seen to have a more favorable outcome in some EIMS cases. Therefore, it is essential to implement a multidisciplinary approach to diagnose and treat patients suspicious of EIMS.

Second recurrence of aggressive vulvar angiomyxoma: a case report.

Aggressive angiomyxoma is a rare benign tumor with diverse clinical and radiologic presentations, making its differential diagnosis challenging. Here we report the second recurrence of an aggressive angiomyxoma of the vulva in a 33-year-old woman who presented with pain and swelling. Clinical examination revealed a well-circumscribed large palpable mass extending from the inferior part of the right labia major into the right side of the gluteus. A second recurrence of aggressive angiomyxoma was suspected based on the patient's history, clinical examination, and magnetic resonance imaging report, and the patient underwent surgical resection with a negative margin. Histological evaluation of the extracted lesion confirmed the diagnosis of aggressive angiomyxoma. At the 1-year follow-up, the patient was recurrence-free. The present report urges more awareness regarding the aggressiveness of angiomyxoma of the vulva. Closer attention should be given to margin-free removal of such tumors, and patients should be routinely followed up for at least 2 years postoperatively for early diagnosis of recurrence, thereby reducing the risk of morbidity.

PTEN promoter methylation and expression in endometrial cancer tissues.

Introduction: Some gene expression regulation in cancers can be controlled by epigenetic change like methylation. PTEN promoter methylation and expression were evaluated in endometrial cancer. Methods: The study was run on 39 tumor tissues of endometrial cancer patients and 41 normal endometrial tissues. After total RNA extraction, cDNA synthesis was done by reverse transcription of the total (real-time PCR) using SYBER Green master mix. DNA extraction and bisulfite treatment were conducted and methylation was semiquantified by the methylation-sensitive high-resolution melting method. Finally, promoter methylation quantification of the total number of 25 tumors and 22 non-neoplastic tissues was done. Results: PTEN gene expression showed a significant decrease in endometrial cancer tissues. Promoter methylation was significantly lower in the non-neoplastic group (7.2; p < 0.001). In addition, PTEN promoter methylation was observed in 52.0% of tumor tissues compared with 13.6% in the non-neoplastic group (p = 0.06). There were no significant correlations between PTEN expression and methylation and clinicopathological features in endometrial cancer patients (p > 0.05). Conclusion: PTEN gene expression in endometrial cancer tissues decreased because of its promoter hypermethylation.

PEComa-A Rare Uterine Neoplasm: A Case Report.

Background: Perivascular Epithelioid Cell Tumors (PEComas) are rare mesenchymal tumors originating from perivascular epithelioid cells. The second common affected organ is uterine. Most of PEComas are benign and patients have good prognosis. At the present time, surgery is the main treatment and adjuvant chemotherapy is used in malignant cases, although the best diagnostic and management method is yet to be discovered considering the rarity of this neoplasm. Case Presentation: The patient was a 53 year old lady with a history of two vaginal deliveries and no previous surgery. She had severe pelvic pain and underwent MRI with the primary impression of sarcoma. In MRI, she had a 7 cm mass in lower segment of uterus. The patient underwent laparoscopic hysterectomy, bilateral oophorectomy, lymphadenectomy, and omental biopsy in Jam Hospital. Pathologic report of the patient revealed malignant PEComa without lymph node and omentum involvement. Conclusion: Diagnosis of PEComa before surgery is difficult and its differential diagnoses form uterine leiomyoma or leiomyosarcoma. Final diagnosis can be made after surgical biopsy and immunohistochemistry evaluation. Surgery is still the main treatment and adjuvant therapy is used in high risk patients.

Evaluation of the diagnostic accuracy of the cervical biopsy under colposcopic vision.

This study was conducted to evaluate the diagnostic accuracy of the cervical biopsy under colposcopic vision. This retrospective study was performed on 190 women, who were selected from a total of 412 cases referring for colposcopy in one year. All patients underwent colposcopy and loop electrosurgical excision procedure (LEEP). After the investigation of demographic characteristics and data confirmation, colposcopic characteristics were examined. Then, the diagnostic indicators and diagnostic accuracy of the cervical biopsy under colposcopic vision were determined. The mean age of patients was 35.51± 5.91 years. In smokers, the percentage of cancer and CIN3 cases was higher than in normal individuals, and this difference was statistically significant in terms of the frequency of cancerous lesions (P = 0.2). A comparison of colposcopic biopsy with LEEP has shown that the frequency of advanced cases in LEEP has been detected more, and the correlation coefficient (kappa) indicated the weak agreement between the findings of colposcopically directed biopsy (CDB) and LEEP methods. (k = 0.23). The diagnostic accuracy of the cervical biopsy under colposcopic vision for cervical cancer is effectively high. It is recommended that this procedure be performed to diagnose cancerous lesions; however, contrary to what is seen in colposcopy, malignant cases may be spreading and follow-up of patients can affect therapeutic performance.

Using Bakri balloon as a visceral replacement for occupying pelvic cavity in pelvic exenteration, a case report.

INTRODUCTION AND IMPORTANCE: Total pelvic exenteration is the choice treatment for locally advanced or recurrent cervical cancers. However, the procedure is usually associated with serious complications. One of the most common complications is "empty pelvic syndrome". In this case report, we described a novel method to investigate its efficacy in prevention of empty pelvic syndrome. CASE PRESENTATION: A 51-year-old woman presented with recurrent cervical cancer underwent TPE after chemoradiotherapy. After removing the organs of the pelvic cavity, a silicone-made Bakri balloon was placed in there through the laparotomy incision. The balloon was removed 5 days later through the vaginal canal. She was followed for 6 months after the surgery and did not experience neither complications nor the recurrence of the cervical cancer. CLINICAL DISCUSSION: We intended to use a novel technique by placing a Bakri balloon in the pelvic cavity after the total pelvic exenteration. The silicone-made balloon creates an appropriate physical barrier to support colon and small intestine loops and other pelvic contents. CONCLUSION: Bakri balloon, which has been used to control the post-partum hemorrhage, can be a useful tool to provide a physical barrier to prevent the descending of intestinal loops and a breeding ground for reconstruction of the pelvic floor.

Primary large B-cell lymphoma of the cervix: A case report and review of literature.

The primary diffuse large B-cell lymphoma (DLBCL) of the uterine cervix is extremely rare. In the present study, we described two cases of DLBCL of the uterine cervix in reproductive-aged women complaining of postcoital bleeding, recurrent vaginal discharge, and abnormal uterine bleeding.

Diagnostic accuracy for alternative cervical cancer screening strategies: A systematic review and meta-analysis.

We performed a systematic review and meta-analysis to evaluate the accuracy of screening cervical cancer tests as alternative standalone methods. The combined estimates of sensitivity of visual inspection with acetic acid, visual inspection with lugol's iodine, conventional pap smear, liquid-based cytology, High risk HPV testing by clinician, High risk HPV testing by self- sampling, cervicography were 64%, 80%, 55%, 70%, 70% and 67% respectively; the combine values of specificity of these screening strategies were 88%, 88%, 96%, 59%, 94%, and 95% respectively. Our findings draw attention to an attractive opinion to facilitate the collection of specimens for DNA HPV by patients in settings where they don't have access to a regular screening programs.

Efficacy of daily rectal micronized progesterone for prevention of preterm delivery: a randomized clinical trial.

INTRODUCTION: Preterm delivery is the leading cause of neonatal morbidity and mortality and its prevention is always under serious concern. OBJECTIVE: The aim of the present study was to determine the efficacy of rectal progesterone as a maintenance tocolytic after arresting preterm labor, for increasing the duration of pregnancy, and postponing preterm birth. METHOD: The study was performed as a double blind randomized clinical trial on women with preterm labor in whom contractions have been stopped. The eligible women were randomly divided into two groups. In the intervention group (progesterone group), progesterone was administered rectally as a dose of 200 mg daily until 36+6 weeks or spontaneous delivery before that time, whichever came first; and in the placebo group, placebo was administered in a similar manner. Primary outcomes were number of deliveries before 37 weeks of gestation and time to delivery interval in two groups. Secondary outcomes were neonatal Apgar score and weight, and need for NICU admission. RESULTS: 160 women finished the study (80 women in each group). The women of the two groups did not have significant difference according to the baseline characteristics. Frequency of preterm labor (earlier than 37 weeks) and mean gestational age at the time of delivery did not show significant difference in two groups. Also, neonatal outcome including Apgar score, birth weight, NICU admission and neonatal complications were not different between the two groups. The pregnancy length was longer in progesterone group (28.84 ± 3.36 VS 21.19 ± 4.62 days), [p = .001, CI 95%: 3.71-4.83]. The time-to-event (delivery) analysis showed a hazard ratio of 1.02 (95% CI 0.36-2.77). CONCLUSION: Rectal progesterone at a daily dose of 200 mg as a maintenance tocolytic agent, cannot lower the frequency of preterm delivery but was suggested to prolong pregnancy length.

Rhabdomyosarcoma of the uterus in an adult patient with osteopetrosis: a case report.

BACKGROUND: Uterine sarcoma accounts for 3-7% of uterine malignant neoplasms. It is more aggressive than epithelial neoplasms, and patients have a poor prognosis. Rhabdomyosarcoma is classified as a heterologous uterine sarcoma. It is the most common soft tissue malignancy in children while rare in adults. In young patients, the majority of genital tract rhabdomyosarcomas occur in vagina; however, the most common site of gynecologic rhabdomyosarcoma is cervix followed by uterine corpus, in adults. Uterine corpus rhabdomyosarcoma is rare in adults. Diagnosis of pure rhabdomyosarcoma in uterus involves widespread and perfect sampling as well as precise histopathological evaluation to uncover any epithelial component. CASE PRESENTATION: Here we report a case of pure rhabdomyosarcoma of uterine corpus in a 60-year-old Iranian postmenopausal female who had osteopetrosis, presenting with 8-month heavy vaginal bleeding and a protruding cervical mass. She is alive on 18-month follow-up after treatment. CONCLUSIONS: Rhabdomyosarcoma of uterine corpus is rare in adults. Diagnosis of pure rhabdomyosarcoma in uterus involves widespread and perfect sampling as well as precise histopathological evaluation to uncover any epithelial component. Treatment options in adult gynecological rhabdomyosarcoma are based on studies in younger patients, and more studies may help us choose the best approach for improving outcome.

Investigation of TGF-ß1 gene variant and expression in a group of Iranian women with endometriosis.

PURPOSE: Endometriosis is defined as a common gynecologic and inflammatory disease. Transforming growth factor-beta 1 (TGF-ß1) gene and its protein level might play a role in the pathogenesis of endometriosis. The present study aimed for the first time to assess the associations between endometriosis risk and - 509 C/T (rs1800469) variant of the TGF-ß1 gene as well as TGF-ß1 mRNA expression in eutopic endometrium tissue of patients with and without endometriosis among a group of Iranian women. METHODS: Genotyping was carried out in 100 endometriosis patients (cases) with confirmed histological diagnosis of endometriosis and 197 non-endometriosis subjects (controls). The expression level of TGF-ß1 mRNA was determined using Real-Time PCR assay in 15 eutopic endometrium tissue of women with endometriosis and 15 healthy controls. RESULTS: There was a significant association for allele and genotype frequencies of rs1800469 variant and endometriosis. No significant difference for TGF-ß1 expression was observed between eutopic endometrium of patients and healthy group. Also, evaluation of TGF-ß1expression across the menstrual cycle showed the same level of TGF-ß1 among case and control subjects. CONCLUSION: Our investigations indicated enough evidence for the effect of TGF-ß1 genetic variant on endometriosis risk in an Iranian population. Furthermore, we could not find any relations between TGF-ß1 mRNA expression and susceptibility to endometriosis.

Prevalence and Genotype Distribution of Human Papillomavirus Infection among 12 076 Iranian Women.

INTRODUCTION: Human papillomavirus (HPV) infection is one of the major health concerns of women in developing countries. This study gives an insight into the prevalence and genotype distribution of HPV infection and compares it with Pap smear results among Iranian women. METHODS: In this study, 12 076 Iranian women underwent routine examination from November 2016 to November 2018 using HPV Direct Flow CHIP System for HPV DNA typing. Cytology was undertaken for 5138 samples. RESULTS: Overall HPV prevalence was calculated at 38.68%. The most frequent HPV types were HPV 6, 16, 11, 62/81, 52 and 54. The most high-risk HPV (HR-HPV) types were HPV 16, 52, 18, 39, 31 and 51. These 2 groups represent approximately half of all HPV types detected, 47% and 55%, respectively. Among individuals who underwent cytological tests, 135 individuals (2.63%) were cytologically positive. In this group, 81 individuals (60%) were HPV positive, 62 (76%) of whom were HR-HPV positive, most frequently with HPV 16 (34%). CONCLUSION: This study highlights the urgent need for public education and early diagnosis using HPV screening tests to prevent cervical cancer.

The Effect of Metformin on Expression of Long Non-coding RNA H19 in Endometrial Cancer.

Background: Endometrial cancer is the fourth most widespread cancer among females, with a growing prevalence in recent years. Management by combined therapies along with surgery, radiotherapy, and chemotherapy have improved patients' prognoses. Besides, the development of new therapies helps preserve fertility and prognosis in aggressive tumors. The purpose of this research was to identify the efficacy of metformin on the H19 long non-coding RNA expression in endometrial cancer to provide further insight into the pathogenesis and treatment of the disease. Methods: A total of 23 patients with endometrial cancer, diagnosed by biopsy or diagnostic curettage, were recruited and divided into three groups, before and after metformin treatment and placebo. Real-time PCR was used to evaluate the H19 expression in cancer tissue in all patients. Results: : It has been observed that in endometrial tissue of the "after-metformin" treatment group, the H19 expression level was significantly reduced, compared with the "before-metformin" treatment group, but not in comparison with the placebo. These findings indicate that metformin reduced the H19 expression in endometrial cancer. Conclusion: Anti-diabetic drugs, such as metformin, may be beneficial by reducing the H19 expression in endometrial cancer due to the H19 relation to cancer progression.

Prophylactic Chemotherapy with Methotrexate Leucovorin in High-Risk Hydatidiform Mole.

AIM: Gestational Trophoblastic Neoplasia (GTN) is used to describe a group of malignant gestational tumors originating from the placenta. The chance of having malignant GTN is high in a high-risk molar pregnancy. The main aim of this study is to investigate the effectiveness of using prophylactic chemotherapy in high-risk molar pregnancy to prevent malignant GTN. METHOD: In this case-control retrospective study, all patients with high-risk mole referred to Firoozgar and Akbarabadi Hospitals affiliated with Iran University of Medical Sciences (IUMS) from 2003 to 2013 were divided into two groups of recipient and non-recipient of methotrexate prophylactic chemotherapy.Demographic information including age, parity, weight, serum ßHCG before and after the intervention, level of liver function tests (LFT) and GTN were analyzed. RESULTS: There were 102 patients with a mean age of 27.13 years (SD= 0.37), and 51 patients (50 %) received prophylactic Methotrexate (MTX), and others were the non-receivers. Finally, 23 patients (22.5%) were inflicted with GTN, and 79 (77.5 %) did not. The average time of ßHCG spontaneous remission between the groups were 2.5 (SD=1.33) and 3.2 (SD=1.21), for the recipient and non-recipient, respectively, which showed a significant difference (p). CONCLUSION: This study concludes that prophylactic chemotherapy with MTX and leucovorin may be capable of reducing GTN, which supports the prescription of MTX in high-risk mole, especially in countries with limited resources. The toxicity of methotrexate can be reduced with the addition of leucovorin.
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Association of vascular endothelial growth factor (VEGF) Gene polymorphisms and expression with the risk of endometriosis: a case-control study.

Endometriosis is a polygenic and multifactorial gynecology situation which might be associated with angiogenesis. In the current study we assess the role of vascular endothelial growth factor (VEGF) - 2578 A/C, and + 936 C/T polymorphisms in susceptibility to endometriosis and checking the expression of VEGF mRNA in eutopic tissue of endometrium with and without endometriosis. The study was comprised of 300 patients who underwent laparascopic or laparotomy surgery with 100 cases who had confirmed histological diagnosis of endometriosis, and 200 controls with no histological diagnosis of disease. The genotyping of VEGF polymorphisms was done by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique and the gene expression in tissue was determined using Real-Time PCR assay. There was no important difference of allele distribution of the - 2578 A/C (P = 0.7) and + 936 C/T (P = 0.5) polymorphisms among endometriosis cases and controls. Study of VEGF expression during the menstrual cycle, showed that endometrial tissue in cases group expressed more VEGF mRNA at the secretory phase compared to the proliferative phase (P = 0.03). Our results suggest that - 2578 A/C and + 936 C/T polymorphisms of VEGF did not seem to have impact on endometriosis predisposition in our study population. Also we did not find any link between VEGF mRNA expression and risk of endometriosis.

Outcome of treatment with EMA/EP (etoposide methotrexate and actinomycin-D/ etoposide and cisplatin) regimen in gestational trophoblastic neoplasia.

Background: Gestational trophoblastic neoplasia (GTN) originates from placental trophoblast and is a highly chemosensitive and curable gynecologic malignancy. The present study was conducted to evaluate the effectiveness and safety of EMA/EP (etoposide, methotrexate, actinomycin-D, etoposide, and cisplatin) regimen in the treatment of high-risk GTN as well as patients' outcome. Methods: Hospital charts of all patients with confirmed diagnosis of high-risk GTN who received EMA/EP regimen treatment during a 12-year period (2001-2012) at the tertiary center of comprehensive women's hospital in Tehran, Iran, were reviewed from 2012 to 2013, retrospectively. Results: In this study, 25 patients with GTN who were treated with EMA/EP regimen during the study were identified. Complete remission rate in GTN patients with failure of single agent chemotherapy who were treated with EMA/EP regimen, as the first- line treatment, was 100%, while it was 81% in those with primary high-risk GTN. Overall remission rate in high-risk GTN patients treated with EMA/EP regimen was 88%. Anemia (92%) and leucopenia (72%) were the most common adverse effects of EMA/EP chemotherapy regimen. Acute myeloid leukemia (AML) and mortality, as the most severe adverse effects of EMA/EP regimen, were seen only in 1 patient. Conclusion: According to the results, EMA/EP regimen could induce complete remission in 88% of patients with high-risk GTN. Application of EMA/EP is recommended as the first- line therapy in patients with failure of single agent chemotherapy. However, proper care should be considered to prevent and reduce EMA/EP hematologic toxicity.

Electrosurgery and clinical applications of electrosurgical devices in gynecologic procedures.

Background: Electrosurgery is widely used in reproductive related surgeries and technological advancements to improve efficacy and reduce potential complications. However, some reports have indicated lack of sufficient knowledge and training about basic principles and technical aspects of electrosurgery among obstetricians and gynecologists. Methods: In this paper we present a summary on basic concepts and principles of electrosurgery and review the recent evidence on the use of electrosurgical devices in gynecologic procedures including endometrial ablation, gynecologic malignancies, loop electrode excision procedure (LEEP), and infertility. Result: Considering the extensive use of these technologies in reproductive related surgeries, procedures including laparoscopy, hysteroscopy, and loop procedures further highlights the importance of more detailed training in this field. Gynecologists must learn the basics in more detail and update their knowledge on the growing body of evidence regarding the advancements of these technologies to reduce potential complications and select the most cost-effective treatment options for each patient. Conclusion: Try to understanding the underlying biophysical principles and more in-depth familiarity with various electrosurgical devices could lead to less complications and optimize evidence-based gynecological practice.

Unusual Presentation of Invasive Mole: A Case Report.

BACKGROUND: Invasive mole is responsible for most cases of localized gestational trophoblastic neoplasia. Gestational trophoblastic disease describes a number of gynecologic tumors that originate in trophoblastic layer including hydatidiform mole (complete or partial), invasive mole, choriocarcinoma, placental site trophoblastic tumor and epitheloid trophoblastic tumor. Invasive mole may arise from any pregnancy event although in most cases is diagnosed after molar pregnancy. Overall cure rate in low risk patients is nearly 100% and in high-risk patient 90%. In rare cases, molar tissue traverses thickness of myometrium and leads to perforation and acute abdomen and invasive mole infrequently metastasis. The best treatment option is chemotherapy (according to stage and score with single or multiple agent) and in patients that fertility is not the matter, hysterectomy can be done. CASE PRESENTATION: A 41 years old G3P2ab1 woman referred to Firouzgar hospital 2 months after curettage of molar pregnancy with vaginal bleeding and acute abdomen. In workup, HCG 224000 mIU/ml and evidence of metastasis was detected. Chemotherapy due to stage 3 and score 9 and surgery due to acute abdomen was done. This case was reported for its rarity. DISCUSSION: This case reported about ovarian metastasis and uterine rupture with acute abdomen and involvement of omentum in metastatic invasive mole. Lack of surveillance led to extensive morbidity. Management of this patient was successful. In follow up, she was free of disease without sequel of any kind for five years now.