Lymphoepithelioma-like carcinoma of the uterine cervix: absence of Epstein-Barr virus and high-risk human papilloma virus infection

Objective Lymphoepithelioma-like carcinoma (LELC) is a rare variant of squamous cell carcinoma of the uterine cervix. In contrast to Asian women, Caucasian women have a low prevalence of cervical LELC with no infection of the Epstein-Barr virus (EBV) and sporadic appearances of human papilloma viruses (HPV) in Caucasian women. Case We report a 36-year-old female Caucasian patient with LELC of the cervix. Result In contrast to the present studies, our case of LELC of the cervix is not associated with EBV or high-risk HPV appearances. Conclusion This is the first study, that showed no relationship between LELC of the uterine cervix with EBV and high-risk HPV infections in a Caucasian woman.     

Giant placental angioma with polyhydramnios, high level of alpha-fetoprotein and neonatal congenital lactic acidosis

Placental tumors are rare in pregnancy. They cause nonspecific complications such as polyhydramnios, fetal anemia, fetal thrombocytopenia and cardiac decompensation with non-immunological hydrops fetalis. In the presented case a very large placental hemangioma was connected with polyhydramnios, premature delivery, fetal anemia and thrombocytopenia, maternal serum alpha fetoprotein elevation and congenital lactic acidosis. After delivery a severe state of the newborn was caused by oligovolemic shock. In the course of the disease the neonatal state steadily deteriorated mainly because of sepsis, cerebral hemorrhage and metabolic acidosis despite adequate therapy. The organic acids assessment in the blood serum of a newborn child showed a very strong signal of lactic acid and an increase in parahydroksyfenylolactic acid. Postmortal examination confirmed prematurity, respiratory distress syndrome, sepsis and cerebral hemorrhage. These symptoms probably resulted from the presence of a placental tumor of considerable size of 12 cm and congenital lactic acidosis, which to our knowledge, have not been described in the available literature until now. In conclusion it should be underlined, that there exists difficult to explain relationship between the presence of a placental haemangioma and severe metabolic changes resulting in high mortality and morbidity of the newborn.     

Urologic and genital anomalies in patients with congenital absence of the vagina.

Forty-three patients with primary amenorrhea secondary to congenital absence of the vagina have been seen at this institution over the past 25 years. Associated urologic anomalies were present in 47% of patients in whom evaluation of the urinary tract was performed. Two patients recently presented with the rare combination of vaginal agenesis and solitary pelvic kidney, and one other such patient was found in a retrospective study of these records. A review of the literature concerning the associations of these conditions is presented with reference to frequency, embryology, pathophysiology, diagnostic methods, and various surgical procedures for correction of vaginal agenesis. The results of surgical correction using simple techniques in this institution have been comparable to those of other authors. The need for thorough preoperative evaluation of patients with genital malformations to include complete urologic studies is stressed.     

Woman with virilizing congenital adrenal hyperplasia and leydig cell tumor of the ovary

We report the case of a 36-year-old woman with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, and corticosteroid replacement therapy since birth. She manifested persistent virilization and high testosterone levels that were attributed to nonadherence to medical treatment. The patient was referred to our gender unit for genitoplastic surgery. We recommended the patient for left oophorectomy after detecting an ovarian mass. Pathologic findings confirmed an ovarian hilus cell tumor. Testosterone levels fell back to normal and masculinization disappeared but ACTH remained elevated. This case represents a very rare type of primary ovarian tumor that must be considered in persistent virilizing symptoms in women with CAH.     

Diverse maternal and fetal pathology associated with absent diastolic flow in the umbilical artery of high-risk fetuses

Twenty-two of approximately 450 high-risk pregnancies referred to a regional center for a level II sonographic examination after 20 weeks' gestation were characterized by absent or reversed diastolic flow in the umbilical artery. Ten fetuses had congenital malformations or were aneuploid. Ten were growth-retarded in association with other problems: maternal hypertension, preeclampsia, cyanotic heart disease, elevated maternal serum alpha-fetoprotein levels, or twin gestation. In two cases, no etiology could be identified. Knowledge of the fetal karyotype, fetal anatomy, gestational age, maternal disease, and fetal status as determined by other tests of fetal well-being was required to optimize outcome in each case. In view of the heterogeneous etiologies of absent or reversed diastolic flow, management of such cases must be individualized.     

Lymphoepithelioma-like carcinoma of the uterine cervix: absence of Epstein-Barr virus, but presence of a multiple human papillomavirus infection

BACKGROUND: Cervical lymphoepithelioma-like carcinoma (LELC) is a rare variant of squamous cell carcinoma of the cervix. Association with Epstein-Barr virus (EBV) is still controversial. EBV has been demonstrated in Asian women with cervical LELC. In Western women, human papillomavirus (HPV) might play a role in the etiology. CASE: We describe a 44-year-old Caucasian woman with a lymphoepithelioma-like carcinoma of the cervix without EBV, but in the presence of multiple HPV infection. CONCLUSION: Our case supports a possible different pathway of development of cervical LELC in Western women as compared to Asian women.     

Elevated maternal serum alpha-fetoprotein: association with placental sonolucencies, fetomaternal hemorrhage, vaginal bleeding, and pregnancy outcome in the absence of fetal anomalies.

Elevated maternal serum alpha-fetoprotein (MSAFP) levels have been associated with an increased incidence of both placental sonolucencies and pregnancy complications. We designed a prospective study to test the hypothesis that the presence of these sonolucencies or a positive maternal Kleihauer-Betke stain would be associated with an elevated risk of obstetric complications. We enrolled 95 women with singleton pregnancies, elevated MSAFP, and no evidence of fetal anomalies on second-trimester ultrasound evaluation. Placental sonolucencies were documented at the time of ultrasound examination, and a maternal Kleihauer-Betke stain for fetal cells was obtained on the same day. Complications of pregnancy included fetal growth retardation, preterm delivery, late vaginal bleeding (at or after the 20th week of gestation), and fetal death. Women with elevated MSAFP had an increased incidence of placental sonolucencies, positive maternal Kleihauer-Betke stains, first-trimester vaginal bleeding, late vaginal bleeding, preterm delivery, fetal growth retardation, and fetal death compared with controls. Thirty-nine of 95 women with elevated MSAFP (41.1%) had at least one complication. In women with elevated levels, neither the presence of placental sonolucencies nor a positive Kleihauer-Betke stain correlated with first-trimester vaginal bleeding, the MSAFP level, or an increased risk of pregnancy complications. First-trimester vaginal bleeding was associated with an increased risk of preterm delivery in subjects with elevated MSAFP.     

The relationship between an aortic isthmus blood flow velocity index and the postnatal neurodevelopmental status of fetuses with placental circulatory insufficiency

OBJECTIVE: The purpose of this study was to evaluate the association between an abnormal aortic isthmus blood flow index and postnatal neurodevelopmental outcome in fetuses with placental circulatory insufficiency. STUDY DESIGN Forty-eight children who were born between 1991 and 1999 were included in this study on the basis of abnormal umbilical artery Doppler velocimetry. Prenatal isthmus blood flow index was obtained by dividing the sum of the systolic and diastolic Doppler blood flow velocity integrals by the systolic blood flow integrals. Neurodevelopmental outcome between 2 and 5 years was classified as optimal, when neurologic assessment and developmental quotient were within normal limits and as nonoptimal when abnormal neurologic findings and/or a nonoptimal developmental quotient was present. Neurodevelopmental outcome was analyzed in relation to isthmus flow index and pulsatility indices in the umbilical artery. RESULTS: The mean gestational age at delivery was 33.0 +/- 2 weeks. Nonoptimal neurodevelopmental outcome was found in 60.4% of the children (29/48). An inverse correlation was found between the isthmus blood flow index and postnatal neurodevelopmental outcome. All 13 children with an isthmus blood flow index of <0.5 were in the nonoptimal group. All 19 children with an optimal outcome had an isthmus blood flow index of >0.5, but this was also the case for 16 other children with nonoptimal neurodevelopmental outcome. An isthmus blood flow index cut-off value of 0.70 was associated with the highest overall positive and negative predictive values. The pulsatility index in the umbilical artery did not provide any significant contribution in the explanation of the outcome. CONCLUSION: The isthmic blood flow index can help to identify a subgroup of fetuses with placental circulatory insufficiency that might benefit from early delivery.     

A fibrin matrix modulates the proliferation, hormone secretion and morphologic differentiation of cultured human placental trophoblast.

Term placental trophoblast epithelialize fibrin deposits attached to villi in vitro and trophoblast cultured on a fibrin matrix form an epithelial bilayer typical of the trophoblast layer on term villi. We compared the morphology of cells grown on fibrin with cells grown on substrates of type IV collagen, laminin, type I collagen, or Matrigel. We also used autoradiography, hormone assays, electron microscopy, and immunofluorescence to determine what functional activities were influenced by trophoblast-fibrin interactions. Cultured cellular trophoblast from term placentae differentiated to form syncytial trophoblast and to secrete estrogen, progesterone, and hCG in the presence or absence of matrices. Trophoblast proliferation was lower in cells grown on matrices and was inversely related to cell height after 24 h in culture. Cells grown on fibrin remained the tallest and had the lowest labelling index. Cells grown for 72 h on fibrin had the most dilated rough endoplasmic reticulum but the lowest media hormone levels. Only cells grown on a fibrin matrix formed a basal lamina-like structure at the trophoblast-substrate interface, and only a fibrin matrix facilitated trophoblast to form an epithelial bilayer in culture. However, this histology was not accompanied by a change in the amount of syncytial trophoblast formed by the cells grown on fibrin. The results suggest that a fibrin matrix uniquely modulates the trophoblast phenotype, away from the secretion of placental specific products like hCG in favour of a repair-oriented phenotype that forms basement membrane and a trophoblast bilayer.     

Vaginal microbicides to prevent human immunodeficiency virus infection in women: perspectives on the female genital tract, sexual maturity and mucosal inflammation

Topically applied vaginal microbicides to protect against human immunodeficiency (HIV) virus infection offer an important female-controlled prevention strategy. Microbicides have been in development for more than 2 decades, and have included various agents that disrupt cellular and microbial membranes (surfactants), restore the natural acidic protective pH of the vagina (acid buffers), and those that interfere with interactions between HIV envelope proteins and cellular receptors (anionic polymers). Although none of these candidate microbicides have shown significant protection against HIV in clinical trials, a topical gel, including the antiretroviral drug tenofovir (TFV) 1% was the first microbicide to be tested to show some protection against HIV infection. This review explores the effect of female genital tract biology and anatomy, mucosal inflammation, and age on the effectiveness of microbicides to prevent HIV infection.