Surgical management of complex atypical endometrial hyperplasia in a woman with rare genitourinary anomalies: unicornuate uterus with rudimentary horn, ipsilateral ectopic ovary and pelvic kidney.

AIM: According to the so far published literature, only one case of endometrial cancer in a patient with unicornuate uterus has been reported. This is a case report study, presenting a rare case of complex atypical endometrial hyperplasia in a woman with unicornuate uterus and multiple genitourinary anomalies. CASE REPORT: A 43-year old G1P1 woman presented with episodes of menometrorrhagia and anemia. She had previous surgical history of laparoscopy due to infertility, in which she was diagnosed with unicornuate uterus with a rudimentary left uterine horn and ipsilateral ectopic ovary in the anatomic place of the left kidney. Dilatation and curettage was performed. Histology showed complex atypical endometrial hyperplasia. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, in an extremely interesting operation due to the multiple genitourinary anomalies. The uterus with a 6-centimeter uterine myoma and the adnexae were removed en block. Great effort was put into dissecting the left fallopian tube which arised from the cervix and via the rudimentary horn led to the left ectopic ovary that was located at the left kidneys' anatomic space. The patient recovered well and final histology was negative for malignancy. DISCUSSION: All necessary imaging examinations have to be scheduled prior to surgical intervention in order to give valuable anatomic information in cases of women diagnosed with Mullerian abnormalities.

Bilateral primary ovarian non-Hodgkin's lymphoma and fertility preservation: 5-year follow-up.

AIM: Primary ovarian non-Hodgkin's lymphoma is a very rare disease. Median age at diagnosis is estimated at 42 years, something that leads to fertility preservation issues in many cases. This was a case report study, presenting a rare case of bilateral primary ovarian non-Hodgkin's lymphoma. CASE REPORT: A 38-year old nulliparous woman, underwent exploratory laparotomy because of bilateral ovarian masses. Left salpingooophorectomy, partial omentectomy and excision of an ovarian mass of the right ovary was performed. Great effort in order to preserve healthy ovarian tissue of the right ovary as well as the right fallopian tube was given, due to fertility reasons. Final histology showed bilateral diffuse large B-cell primary ovarian non-Hodgkin's lymphoma. Postoperatively, the patient underwent chemotherapy with the CHOP regimen in combination with rituximab. Five years after initial diagnosis, the patient remains well with normal menstrual cycle, without evidence of recurrence. DISCUSSION: Fertility preservation issues in some cases of rare gynecological malignancies could be managed via minimally invasive oncological approach.

Increased Maternal Serum Interleukin-6 Concentrations at 11 to 14 Weeks of Gestation in Low Risk Pregnancies Complicated with Gestational Diabetes Mellitus: Development of a Prediction Model.

The aim of the study was to examine interleukin-6 (IL-6) maternal serum concentration at 11 to 14 gestational weeks in normal pregnancies and pregnancies complicated by gestational diabetes mellitus (GDM) and to create first trimester prediction models for GDM. Case-control study conducted in a Fetal Medicine Unit. Study population included 40 GDM cases and 94 controls. Maternal characteristics, first trimester ultrasound markers, biochemical indices, and IL-6 levels were used for our analysis. IL-6 was related to maternal weight among the maternal characteristics, (R(2)=0.0679, p=0.01). IL-6 was increased (p=0.001) in the GDM group (median=2 pg/ml) compared to the control group (median=1.5 pg/ml) even after adjustment for maternal weight. IL-6 was inversely related to birth weight adjusted for gestational age at delivery (r=-0.3382, p<0.001) and glucose levels at oral glucose test. Maternal weight and age were the only predictors of GDM among the maternal characteristics [Detection Rate (DR)=59.4%; for 25% False Positive Rate (FPR); Area Under the Curve (AUC)=0.7291; Model R(2)=0.1096, p<0.001]. IL-6 alone was a significant predictor of GDM (DR=51.3%; for 25% FPR; AUC=0.6731; Model R(2)=0.0616, p<0.001). Combination of maternal characteristics with IL-6 yielded an improved prediction (DR=67.5%; for 25% FPR; AUC=0.7586; Model R(2)=0.1521, p<0.001). IL-6 concentrations are increased at 11-14 weeks in pregnancies with GDM. Combination of maternal characteristics and maternal serum IL-6 levels may provide effective first trimester screening for GDM.

Maternal hyperthyroidism after intrauterine insemination due to hypertrophic action of human chorionic gonadotropin: a case report.

PURPOSE OF INVESTIGATION: To report a rare case of maternal hyperthyroidism after intrauterine insemination due to hypertrophic action of hCG. MATERIALS AND METHODS: A 36-year-old woman after successful intrauterine insemination and triplet pregnancy, developed hyperthyroidism with resistance to medical treatment. RESULTS: All signs of hyperthyroidism resolved and the results of thyroid function tests returned to normal without any medication after embryo meiosis. CONCLUSIONS: De novo maternal hyperthyroidism may develop during pregnancy as a result of pathological stimulation of the thyroid gland from the high levels of hCG hormone that can be seen in multiple pregnancies. The risk of hyperthyroidism is related to the number of fetuses. Reversibility of symptomatology can be seen after fetal reduction of multiple pregnancies.

Secondary hypoparathyroidism during pregnancy--a case report and review of the literature.

Secondary hypoparathyroidism is seldom seen during pregnancy. Usually, it presents with hypocalcemia. Even if there is no established therapeutic treatment, vitamin D or its analogues are required. In the present case, a 36-year-old, second gravida, with known hypoparathyroidism for the last ten years, was admitted in the prenatal clinic of "Aretaieion" University Hospital in Athens at her 39 weeks of pregnancy. She was treated with calcitriol and calcium and she was monitored monthly. She had a cesarian section and delivered a healthy female baby of 3,380 gr at 39 weeks and 1 day.

Ovarian cancer diagnosed during pregnancy: clinicopathological characteristics and management.

The aim of this study was to discuss the diagnostic and therapeutic dilemmas in cases of pregnant women with adnexal masses, reporting an interesting case with synchronous literature review. The patient, a gravida 2, para 1, 37 year-old woman was diagnosed with a large unilateral adnexal lesion during a scheduled third trimester ultrasound assessment. A large papillary papule with a network of blood vessels showing decreased resistance in blood flow was noticed as well. Surgical intervention revealed ascitic fluid and a large cystic mass arising from the right ovary. Cesarean section and right salpingooophorectomy, including the mass, were performed. Frozen section biopsy was positive for malignancy. Total hysterectomy and left salpingo-oophorectomy, total omentectomy, biopsies from the pelvic peritoneum, pelvic/para-aortic lymphadenectomy and appendicectomy followed. Histology showed mucinous ovarian adenocarcinoma Grade I Stage Ic according to FIGO classification. Surgical intervention, in cases of persisting adnexal lesions, is often necessary, even during pregnancy.

Recurrent short rib polydactyly syndrome.

We present three consecutive cases of skeletal dysplasias of a non-consanguineous couple with five pregnancies. The diagnosis of short-rib polydactyly syndrome (SRPS) was feasible by ultrasound during the 1st trimester of pregnancy. SRPS represents a heterogeneous group of lethal skeletal dysplasias. It is characterised by short limb dwarfism complicated by thoracic hypoplasia, polydactyly and different anomalies of major organs such as congenital heart defects and renal dysplasia. Four major types of the SRPS have been described: type I (Saldino-Noonan); type II (Majewski); type III (Verma-Naumoff) and type IV (Beemar-Langer). However, there is phenotypic overlapping between four types and with those of non-lethal skeletal dysplasias (i.e. Ellis-van Creveld syndrome and Jeune syndrome). Our cases show the importance of the nuchal translucency (NT) scan that offers the opportunity to examine fetal anatomy in the 1st trimester and diagnose rare skeletal abnormalities early in pregnancy.

Second-trimester miscarriage and umbilical cord knot. Case report and review of the literature.

PURPOSE OF INVESTIGATION: The present study presents a case with an umbilical cord knot along with extensive literature review. MATERIALS AND METHODS: Presentation of a rare case of second-trimester abortion which was attributed to a tight umbilical cord knot. Furthermore the authors reviewed the literature from 1952 to 2012 in order to compare impact of knots on intrapartum and perinatal outcomes. RESULTS: Four large retrospective studies assessed several predisposing factors. Long umbilical cords, male embryos, and multiparity were correlated with knots in three of these studies. Data regarding perinatal effects of true knots from three studies were summarized and compared. CONCLUSION: Umbilical cord true knots during the second trimester could be a very rare cause of abortion. The presence of knots during the third trimester and labor do not seem to be associated with increased perinatal and intrapartum morbidity and mortality, although there is still some controversy in the literature on this topic.

Immunohistochemical study of inhibin A and B expression in placentas from normal and pathological gestations.

OBJECTIVE: The aim of the study was to examine, by an immunohistochemical method, the distribution of Inhibin-A and -B, in placentas from normal and pathological gestations. MATERIALS AND METHODS: Sixty-two specimens of placental tissue were examined: i) ten cases from early gestations, ii) 28 cases from mature placentas, iii) six cases associated with intrauterine growth restriction, iv) four cases associated with diabetes mellitus and v) 14 placentas from gestations with fetal chromosome abnormalities. The expression of Inhibin A and B was studied by automatic Ventana method. RESULTS: i) Early gestation specimens: Inhibin A (+) immunoreaction was observed in the syncytiotrophoblast (8/10 cases) and in the intermediate trophoblast (6/10 cases). Inhibin B (+) immunoreaction was observed in the syncytiotrophoblast (10/10 cases) and in the intermediate trophoblast (4/10 cases), ii) Normal mature placentas: Inhibin A (+) immunostain was observed in 2/28 cases in the syncytiotrophoblast and in 7/28 cases in the intermediate trophoblast. Inhibin B (+) immunostain was observed in 28/28 cases in the syncytiotrophoblast and in 18/28 cases in the intermediate trophoblast. iii) Placentas associated with intrauterine growth restriction: Inhibin A (+) immunostain was observed in the intermediate trophoblast in 2/6 cases. Inhibin B (+) immunostain was observed in 5/6 cases in the syncytiotrophoblast and in 4/6 cases in the intermediate trophoblast. iv) Placentas associated with gestational diabetes mellitus: Inhibin A (+) immunostain was observed in 2/4 cases in the intermediate trophoblast. Inhibin B (+) immunostain was observed in 2/4 cases in the syncytiotrophoblast. v) Placentas from gestations with fetal chromosome abnormalities: no Inhibin A immunoreaction was observed. Inhibin B (+) immunostain was observed in 13/14 cases in the syncytiotrophoblast and in 9/14 cases in the intermediate trophoblast. The cytotrophoblast, the umbilical cord, and the membranes do not participate in the production of Inhibins. DISCUSSION: Inhibin A and B are located in the syncytiotrophoblast and the intermediate trophoblast of the placenta, during early pregnancy (Inhibin A) and present throughout pregnancy (Inhibin B). No remarkable findings in placentas of pathological gestations support the evidence that Inhibins do not participate in processes that affect the development of the placenta or the fetus, but may participate in,the mechanism of labor.

Prenatal diagnosis of fetal left ventricular non-compaction cardiomyopathy.

Isolated left ventricular non-compaction (LVNC) is a rare disorder, classified as a primary genetic cardiomyopathy by the American Heart Association or as an unclassified cardiomyopathy by the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. The key features are the prominent trabeculae and deep intratrabecular recesses resulting in thickened myocardium with the two layers consisting of compacted and non-compacted myocardium. These recesses are in continuity with the left ventricular cavity and are filled with blood without evidence of communication to the epicardial coronary artery system. We present a case of LVNC detected prenatally at 25 + 4 weeks of gestation.

The implication of second-trimester amniotic fluid TNF-alpha, cytochrome C and cell death nucleosomes in the prediction of preterm labor and/or premature rupture of membranes.

AIM: The multifactorial pathway leading to preterm labor possibly includes the implication of apoptosis. This study aimed to clarify the role of amniotic fluid apoptotic molecules (TNF-alpha, cytochrome C and cell death nucleosomes) at midtrimester as possible predictors of preterm labor (PTL) and/or premature rupture of membranes (PROM). METHOD: In this case-control study, comprising 360 women undergoing genetic amniocentesis and out of whom 38 delivered preterm and 18 out of the latter after PROM, the above apoptotic molecules were determined by ELISA. The 38 cases with PTL and 18 cases with PROM were matched for age with 38 and 18 respective controls delivering at term, and the levels of apoptotic molecules were compared. RESULTS: Cell death nucleosome levels were found to be significantly associated with preterm delivery. Specifically, for every unit increase in nucleosomes, women were on average 0.2% more likely to deliver preterm (OR: 1.002, CI: 1.0-1.003, p = 0.018). In contrast, such an association was not found concerning the other two apoptotic molecules (TNF-a and Cytochrome C). CONCLUSION: Second-trimester amniotic fluid cell death nucleosomes' levels are significantly associated with preterm delivery and could possibly serve as predicting markers.

Clinicopathological study of 112 cases of benign, pre-invasive and invasive lesions of the vagina: a 15-year review.

OBJECTIVE: Benign vaginal lesions are mainly asymptomatic and often diagnosed during routine screening gynecological examinations. Additionally, vaginal intraepithelial lesions are asymptomatic and diagnosis is often confirmed after vaginal biopsy under colposcopic evaluation in cases of abnormal cytological Papanicolaou examination or synchronous cervical intraepithelial neoplasia. On the other hand, primary vaginal cancer is rare representing approximately 1-2% of all gynecological cancers. Metastatic invasion of the vagina is common especially in cases of advanced stage cervical cancer. The aim of this study was to examine the diagnostic approach, the management strategy, and the pathological findings in cases of benign, pre-invasive and invasive vaginal lesions that were diagnosed and treated in our Department. MATERIALS AND METHODS: This was a 15-year retrospective study. Cases of benign, pre-invasive, and invasive vaginal lesions diagnosed during the last fifteen years at Aretaieion Hospital of the University of Athens, were analyzed. RESULTS: During this study period 40 cases of vaginal cysts (35.7% of all vaginal lesions) were diagnosed. Surgical excision of the lesions was decided in all cases and histology showed that the most frequent cyst type was mucus-secreting Mullerian (30%). During the study period, 23 cases of vaginal intraepithelial neoplasia (VAIN, 20.5% of all vaginal lesions) were detected. In 43.5% of the cases, histological diagnosis revealed low grade VAIN, while the remaining cases were classified as high grade VAIN. Furthermore, 11 cases of primary vaginal cancer (9.8% of all vaginal lesions) were diagnosed. The vast majority of them (91%) were squamous cell carcinomas. Additionally, histology confirmed the diagnosis of metastatic invasion of the vaginal wall in 38 cases (34% of all vaginal lesions). In the majority of these cases (55.2%), primary cancer was located in the cervix. DISCUSSION: Benign, pre-invasive and invasive vaginal lesions are relatively uncommon and usually accompany lesions in other sites of the lower genital tract. Their diagnosis is based on gynecological or colposcopical examination. Treatment depends on the type of the lesion and the progression of the disease.

Clinicopathological characteristics of adnexal lesions diagnosed during pregnancy or cesarean section.

OBJECTIVE: The diagnosis of an incidental adnexal lesion during pregnancy has become more common after the widespread use of routine ultrasonography (US). The aim of this study was to examine the diagnostic approach, management strategy and the pathological findings in cases of adnexal lesions that were diagnosed and treated during pregnancy in our department. MATERIALS AND METHODS: This was a 15-year retrospective study. Cases of adnexal lesions detected during routine prenatal care by US or while performing cesarean section, between January 1996 and December 2010 at Aretaieion Hospital of the National University of Athens, were analyzed. RESULTS: In this study period 39 cases of adnexal lesions were diagnosed during pregnancy or cesarean section. The age of the women was between 21 and 40 years (mean age 32.4). Surgical excision of the lesions was decided in 32 cases and conservative treatment was followed in the remaining seven cases. Surgical removal of the lesions was performed during cesarean section in 13 cases of term gestations and in four cases of preterm gestations in which pregnancy termination was considered necessary. Laparotomy during the antepartum period led to excision of adnexal lesions in 15 cases. Histology revealed benign ovarian lesions in 25 cases (78.1%), borderline ovarian tumors in two cases (6.3%), malignant ovarian tumors in four cases (12.5%) and adenocarcinoma of the appendix in one case (3.1%) presenting as an ovarian mass. DISCUSSION: The management of cases diagnosed with adnexal lesions during pregnancy remains controversial. According to the literature, the estimated risk of malignancy for adnexal masses during pregnancy is low (2-3%) and complications of these lesions are extremely rare. These data suggest that adnexal masses could be managed conservatively if possible with US follow-up. On the other hand, the results of this study showed a higher incidence of malignancy among adnexal lesions that were surgically treated (15.6%). CONCLUSION: Surgical intervention and histological examination in cases suspicious for malignancy at US and clinical findings remain the treatment of choice even during pregnancy.

Clinicopathological changes of uterine leiomyomas after GnRH agonist therapy.

OBJECTIVE: Gonadotrophin-releasing hormone agonist (GnRHa) has been commonly used for the medical treatment of prostate cancer, precocious puberty, endometriosis, adenomyosis and uterine leiomyomas. GnRHa therapy in cases of symptomatic uterine leiomyomas aims for the reduction of their size and remission of symptoms such as menometrorrhagia, causing a state of hypoestrogenemia. This is considered to be a helpful preoperative strategy in cases of large myomas, or anemia because of abnormal vaginal bleeding. The aim of this retrospective study was to examine the clinicopathological changes in uterine leiomyomas exposed to preoperative GnRHa therapy for two up to six months. MATERIALS AND METHODS: The study group consisted of 10 premenopausal patients who were treated with GnRHa prior to surgery. RESULTS: In all cases the size of leiomyomas was reduced after GnRHa therapy. A microscopic review of the surgical specimens showed increased cellularity and ischemic type of necrosis. CONCLUSION: Morphological changes of uterine leiomyomas are often associated with preoperative GnRH agonist therapy. The differential diagnosis from uterine leiomyosarcomas includes absence of mitotic activity.

Isolated sigmoid colon metastasis from a primary fallopian tube carcinoma: a case report.

Isolated metastasis of primary fallopian tube carcinoma (PFTC) is extremely rare. We describe a case of a 41-year-old asymptomatic woman who was referred three years after the initial treatment for PFTC due to elevated sertum CA-125 levels. The abdominal and pelvic CT scans revealed a pelvic mass near the top of the vaginal vault. On surgery, a sigmoid colon tumour was found and a sigmoidectomy was performed. On histopathology the tumour involved the bowel wall from serosa to submucosa, without involvement of the underlying mucosa. Immunohistochemical staining was positive for cytokeratin 7 and negative for cytokeratin 20, and the tumour was determined to be a metastatic müllerian neoplasm, consistent with the initial PFTC. Although this is the first reported case of colon metastasis of PFTC, the possibility of such an unusual site of metastasis should be kept in mind, as PFTC may recur as isolated bowel lesions even in the absence of peritoneal disease.

Obstetric hysterectomy: a review of cases over 16 years.

Emergency peripartum hysterectomy (EPH), is performed when life-threatening obstetric conditions occur. The authors attempt to assess the incidence of EPH as well as to investigate risk factors and patients' characteristics. A retrospective study of all cases of EPH performed at the 2nd Department of Obstetrics and Gynecology, Medical School of Athens University, from 1994 to 2009 has been conducted. Data were abstracted from individual medical charts and laboratory records. Among 16,182 deliveries, 15 EPH were performed (0.92 per 1,000 deliveries). Indication was uncontrollable haemorrhage due to placenta accreta (73.3%) or uterine atony (26.6%). Incidence of 1.54 EPHs per 1,000 caesarean sections and 0.51 per 1,000 vaginal deliveries, were noted. Morbidity rate was 46.6%. One (6.6%) mother died because of pulmonary embolism. In conclusion, peripartum hysterectomy is a severe but life-saving procedure. Caesarean section increases the risk of EPH. Obstetricians should always be prepared to confront this emergency situation.

Single umbilical artery: fetal and placental histopathological analysis of 24 cases.

AIM: 24 cases of single umbilical artery (SUA) are presented and the related histopathological findings of the fetuses and placentas examined. SUA is the most common congenital anomaly of the umbilical cord, resulting in the absence of one of the two umbilical arteries. It has an incidence of approximately 2.1% in autopsy material and there is evidence that is associated with anomalies of the fetus and placenta. MATERIAL-METHOD: The files were reviewed of 24 cases with SUA, out of 1,570 autopsies of fetuses and placentas performed in the Pathology Laboratory of Aretaieion Hospital, due to spontaneous or induced abortions after written parental consent. RESULTS: The incidence of SUA was 1.6%. Gestational age ranged between the 15th and 33rd week and mother's age ranged from 17-44 years. Three of 24 cases were twin pregnancies; 17/24 fetuses were male. In 21/24 cases complex congenital anomalies were observed and in five of 24 cases chromosomal anomalies were detected. In eight of 24 placentas extensive infarcts were observed; 7/24 dysmaturity, 5/24 severe chorioamnionitis, 3/24 extensive fibrin accumulation and 1/24 chorioangiosis. CONCLUSION: SUA is an umbilical congenital anomaly associated with severe fetal congenital anomalies and once detected with ultrasound techniques, further and more detailed control of the fetus is considered mandatory.

The role of human beta defensins 2 and 3 in the second trimester amniotic fluid in predicting preterm labor and premature rupture of membranes.

AIM: Human beta defensins 2 (HBD2) and 3 (HBD3) are peptides expressed in the amnion and chorion. This is a matched case control study conducted in our Department to determine whether second trimester amniotic fluid HBD2 and HBD3 concentrations measured at the time of genetic amniocentesis could be potential markers of preterm labor prediction. METHODS: Amniotic fluid HBD2 and HBD3 were determined by an enzyme-linked immunosorbent assay (ELISA) Women with preterm labor were defined as cases (N=41) while for each case a woman matched for age delivering at term served as control (N=41). Subgroup analysis was conducted to examine possible associations of HBD2 and HBD3 in cases of premature rupture of membranes. Nineteen women with preterm labor and premature rupture of membranes were defined as cases while for every case a woman matched for maternal age delivering at term served as control (N1=19). Results were presented as odds ratios (OR) and 95% confidence intervals. Statistical analysis used STATA 8.2 and SPSS 11.5 edition. A P-value of <0.05 was considered statistically significant. RESULTS: Amniotic fluid concentrations of HBD2 at the time of genetic amniocentesis were positively associated with preterm premature rupture of membranes (P=0.028), but not with preterm labour. No association of HBD3 and preterm birth was documented. CONCLUSION: Second trimester amniotic fluid HBD2 might be a predictor of premature rupture of membranes.

A rare case of congenital pulmonary lymphangiectasia, hydrothorax and ascites in a male embryo aborted at 20 weeks of gestation.

A case of a male embryo aborted at the 20th week of gestation with extensive ascites, hydrothorax, pulmonary lymphangiectasia and pulmonary hypoplasia is presented together with the pathological findings, the etiology, differential diagnosis, course and therapy of this pathologic entity. Also a short review of the literature is discussed.

Adrenomedullin concentration in second trimester amniotic fluid cannot be used as a predictor of preterm delivery.

BACKGROUND: Adrenomedullin, secreted by decidua and trophoblast cells, is considered to participate in regulating uterine and placental blood flow, leading to control of placental hormonal secretion. Furthermore, adrenomedullin has an antimicrobial activity. The objective of this study was to determine whether adrenomedullin concentrations in midtrimester amniotic fluid can be used as a predictor of preterm delivery. PATIENTS AND METHODS: Amniotic fluid samples were collected in a retrospective cross-matched study that included 362 women with singleton pregnancies who presented for genetic amniocentesis. Adrenomedullin concentrations were determined by ELISA in amniotic fluid taken from women with spontaneous preterm delivery (n=41) and maternal age-matched controls who had normal pregnancy at term (n=41). RESULTS: No difference was found in adrenomedullin concentrations between women with spontaneous preterm delivery (median: 1.33 ng/ml, range: 0.36-8.53 ng/ml) and controls (median: 1.32 ng/ml, range: 0.33-4.07 ng/ml), nor between a subset of cases of preterm premature rupture of membranes (n=19) and their controls (n=19). CONCLUSION: Adrenomedullin concentration in amniotic fluid cannot serve as a predictor of preterm delivery.