Differential diagnosis of cystic lymphangioma of the pancreas based on imaging features.

Lymphangioma is a benign tumor, which is a consequence of lymphatic malformation with blockage of lymphatic flow. Most lymphangiomas occur in the neck and axillary region, and < 1% occur in the mesentery or retroperitoneum. Lymphangiomas arising from the pancreas are extremely rare. We report the case of a 34-year-old woman with cystic lymphangioma of the pancreas without major symptoms or signs. A 6 x 6 cm intra-abdominal cystic mass was incidentally revealed by sonography during a health examination. It is always a challenge to differentiate the lesion from other possible cystic-like pancreatic neoplasms. Differential diagnosis of cystic lymphangioma from other cystic-like tumors of the pancreas can be performed based on their imaging characteristics, including presence of septa, cystic or wall calcification, soft tissue, wall thickness, single or multiple loculation, and dilatation of the pancreatic duct. Post-gadolinium magnetic resonance imaging is excellent in defining the origin of intra-abdominal cystic mass and intracystic septa.     

Prenatal diagnosis and outcome of lymphangiomas and its relationship with fetal chromosomal abnormalities

Objectives: Our aim was to evaluate ultrasound findings and perinatal outcome after prenatal diagnosis of lymphangioma.

Methods: This was a retrospective case series study. We searched the archives of our ultrasound database at our center for cases with the prenatal diagnosis of the lymphangioma in the period between January 2008 and November 2014. We described maternal, fetal and perinatal variables for all cases.

Results: Nine fetuses with lymphangioma were identified. All cases were diagnosed during the second and third trimesters with the average gestational age of 22.6?±?3.9 weeks. The average diameter of lymphangioma was 55.4?±?20.1?mm at the time of diagnosis. Five fetuses (55.6%) had lymphangioma on the neck, and four fetuses (44.4%) had lymphangioma on other localizations. Normal fetal karyotype was detected in all cases. There were a total of six live births, one intrauterine death and two medical terminations of pregnancy following the diagnosis of lymphangioma. No abnormal Doppler finding or hydrops were detected in the antenatal follow-up of remaining six cases.

Conclusion: The risk of chromosomal abnormalities is very low in pregnancies with isolated lymphangioma. The outcome of pregnancies with lymphangioma is generally favorable and prognosis depends on their locations and size.     

Bilateral primary breast lymphoma--case report

Primary lymphoma of the breast is an uncommon malignant breast tumor which is seldom distinguished preoperatively from other more common forms of breast cancer. Bilateral breast lymphoma affects younger women especially during pregnancy or postpartum. We report a case of a 55-year-old woman who was admitted to our hospital with painless bilateral breast enlargement. A bilateral radical mastectomy with bilateral axillary lymph node dissection was performed. The histology of the surgical specimen was non-Hodgkin's malignant lymphoma of the diffused large B cell type. Most of the neoplastic cells resembled large centrocytes and sometimes blast cells showing some degree of plasmacytoid differentiation. Foci with a sufficient number of immunoblasts were also noted. The patient was also found to have a bilateral axillary lymph node metastasis. After additional clinical and laboratory screening, there was no other evidence of lymphatic disease at other sites. The patient was submitted to the anticancer hospital for further treatment. She was free of recurrence two years after surgery. The rarity of the disease, lack of uniform classification and variable treatment modalities make prognostic predictions of breast lymphoma difficult.     

Galactocele as a changing axillary lump in a pregnant woman

Materials and methods 36-year-old woman visited to our hospital for her axillary lump. The diagnosis could be made under ultrasound examination and her recent pregnancy history. However, sonography-guided aspiration was done for confirmation. Result We could confirm the galactocele arising in axillary accessory breast tissue. Conclusion Clinical information indicating that such a palpable mass is associated with pregnancy as well as its sonography features along with adjunctive diagnostic aspiration, can help to correctly diagnose this rare entity.     

Pregnancy-induced changes in insulin-like growth factor I (IGF-I), insulin-like growth factor binding protein 3 (IGFBP-3), and acid-labile subunit (ALS) in patients with growth hormone (GH) deficiency and excess

BACKGROUND: Under most circumstances with altered growth hormone (GH) secretion, the changes of insulin-like growth factor I (IGF-I), insulin-like growth factor binding protein 3 (IGFBP-3), and acid-labile subunit (ALS) are in parallel. The aim of the present study was to compare the effects of pregnancy in a hypopituitary patient with those of pregnancy in an acromegalic patient on IGF-I, IGFBP-3, and ALS. METHODS AND RESULTS: IGF-I and ALS were low before pregnancy in the hypopituitary patient under glucocorticoid and thyroxine treatment. Gonadotropin treatment allowed her to become pregnant; IGF-I and ALS levels rose in the second half of pregnancy and fell again after delivery. IGF-I concentrations were elevated in the patient with persistent acromegaly before and dropped into the normal range during the first half of pregnancy. In the second half of pregnancy and following delivery, IGF-I levels increased again. IGFBP-3 levels (as assessed by immunoblot analysis as well as by 125I-IGF II ligand blotting) decreased markedly during pregnancy in both patients, suggesting that the placenta rather than pituitary GH regulates IGFBP-3 proteolysis in human pregnancy. The increase of IGF-I (and ALS) during the second half of pregnancy in the individual with pituitary GH deficiency may be attributed to placental GH. The fall of IGF-I (and ALS) into the normal range in the acromegalic patient during the first trimester of pregnancy may be related to decreased production or decreased half-life of these proteins. CONCLUSION: Our data suggest that measures to continuously replace GH or to suppress GH secretion during pregnancy in patients with GH deficiency or excess, respectively, may not be warranted.     

Breast cancer and pregnancy. Review of the literature

INTRODUCTION: The pregnancy-associated breast cancer seems to have become increasingly common with a high frequency of advanced breast cancer with axillary node metastases and so associated with poor prognosis. MATERIALS AND METHODS: This review examines the diagnosis, prognosis, and management of cancer during pregnancy; both in terms of the cancer's effect on the pregnancy, and the pregnancy's effect on the cancer. RESULTS: Diagnostic procedures (breast sonography) and excisional biopsies are necessary to reduce the delay of several months or more after discovery of a mass and before treatment. No histological difference, between patients with pregnancy-associated breast cancer and patients with non-pregnancy-associated breast cancer, was diagnosed. CONCLUSION: The treatment is linked to the effects of adjuvant therapy on the fetus.     

Undiagnosed maternal phenylketonuria: own clinical experience and literature review

Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (Phe) metabolism resulting from deficiency of phenylalanine hydroxylase (PAH). Most forms of PKU are caused by mutations in the PAH gene. Untreated PKU is associated with an abnormal phenotype, which includes growth failure, seizures, global developmental delay and severe intellectual impairment. The maternal PKU (MPKU) syndrome is caused by high blood Phe concentrations during pregnancy and presents with serious foetal anomalies, especially microcephaly, congenital heart disease and mental retardation. However, since the introduction of newborn screening programs and with early dietary intervention, children born with PKU can now expect to lead relatively normal lives. We present the case of a 33-year-old woman who had been diagnosed as having PKU only after a pregnancy with MPKU embryopathy, to emphasize that undiagnosed maternal phenylketonuria still exists. On that ground, we reviewed updated literature on the pathogenesis of this syndrome, possibility of prophylaxis and treatment.     

Increase in maternal placental growth hormone during pregnancy and disappearance during parturition in normal and growth hormone-deficient pregnancies

OBJECTIVE: The purpose of this study was to evaluate placental growth hormone levels in maternal circulation throughout pregnancy in normal and growth hormone-deficient women with the use of a specific assay and to determine the clearance of placental growth hormone from maternal circulation after birth. STUDY DESIGN: Seventeen healthy pregnant women and 1 patient with growth hormone deficiency substituted with recombinant growth hormone during pregnancy participated in a longitudinal study from early pregnancy until birth with repetitive blood sampling and measurement of placental growth hormone levels throughout pregnancy. Furthermore, serial blood samples were drawn before, during, and after elective caesarean deliveries in 5 healthy women to calculate the half-life of placental growth hormone. Placental growth hormone was measured with the use of two monoclonal antibodies in a commercially available solid-phase iodine 125-labeled immunoradiometric assay (Biocode, Liège, Belgium). RESULTS: Placental growth hormone levels were detectable from as early as 8 weeks of gestation in some of the women and increased throughout gestation, with a maximum at approximately 35 to 36 weeks of gestation (13.7 ng/mL; range, 5.9-24.4 ng/mL) and large interindividual variations. Placental growth hormone levels did not correlate with birth weight or placental weight. In the patient with isolated growth hormone deficiency, placental growth hormone levels were detectable from 11 weeks of gestation (3.4 ng/mL) and increased throughout pregnancy to 13.9 ng/mL, which is similar to values that are obtained in the healthy pregnant women. Substitution therapy with recombinant human growth hormone did not suppress the increase in placental growth hormone. We found a mean half-life of placental growth hormone of 13.8 minutes (range, 11.5-15.2 minutes) in healthy pregnant women and an apparently similar half-life of placental growth hormone (15.8 minutes) in the growth hormone-deficient patient, assuming a monoexponential disappearance of placental growth hormone during the first 30 minutes after the delivery. After the initial 30 minutes, approximately 75% (range, 65%-89%) of the placental growth hormone had been cleared from the maternal circulation. CONCLUSION: Levels of placental growth hormone in maternal circulation increase throughout pregnancy from as early as 8 weeks of pregnancy, with maximum levels around the week 35 of gestation. The pregnancy-induced rise in placental growth hormone levels in the growth hormone-deficient patient was comparable to the rise seen during normal pregnancies and was not suppressed by the concurrent human growth hormone treatment. We speculate that maternal serum levels of placental growth hormone reflect placental function and fetal growth. However, further studies are needed to evaluate the potential clinical use of placental growth hormone determinations.     

Preeclampsia--abnormal uterine artery Doppler is related to recurrence of symptoms during the next pregnancy

BACKGROUND: Impaired trophoblast invasion is suggested as the main cause of reduced placental perfusion, which results in fetal growth restriction and preeclampsia. Immunological response against the invading tissue has been given as the explanation. Preeclampsia frequently recurs during the next pregnancy. Doppler ultrasound can predict increased vascular impedance in the uteroplacental circulation. Whether signs of increased vascular resistance in pregnancies complicated by preeclampsia are predictive of recurrence during the next pregnancy is unknown. METHODS AND MATERIAL: Uterine artery Doppler was performed in 570 pregnant women with preeclampsia. Of these, 139 became pregnant again. The uterine artery Doppler results during the first pregnancy were related to symptoms of preeclampsia in the succeeding pregnancy. RESULTS: Preeclampsia developed again in 43 of the 139 women. Pregnancies with signs of increased uterine artery vascular impedance during the first pregnancy were 3.4 times more likely to develop preeclampsia again (CI 1.58-7.6). Similar results for a small for gestational age newborn were 9.7 (CI 1.1-90). CONCLUSION: Increased uterine artery vascular impedance in pregnancies complicated by preeclampsia increases the likelihood of recurrence and growth restriction during the next pregnancy. The Doppler information gathered during the first pregnancy might thus select cases for special surveillance and possibly prophylactic anti-platelet treatment in the next pregnancy.     

Herceptin (trastuzumab) therapy during pregnancy: association with reversible anhydramnios

BACKGROUND: Herceptin (trastuzumab), a new a chemotherapeutic agent, is a monoclonal antibody that blocks the human epidermal growth factor receptor 2 protein. There is no reported experience with use of this agent during pregnancy and possible effects on the fetus. CASE: A patient with breast cancer was treated with Herceptin during pregnancy. This treatment was associated with anhydramnios, which resolved slowly after the drug was discontinued. CONCLUSION: Although listed as a category B drug, experience with Herceptin in human pregnancy is limited, and it should be used with caution. Investigation of the role of human epidermal growth factor receptor 2 protein in the embryonic kidney may further our understanding of amniotic fluid dynamics.     

Evaluation of factor V Leiden, prothrombin and methylenetetrahydrofolate reductase gene mutations in patients with severe pregnancy complications in northern Finland

BACKGROUND: Thrombosis in placenta may lead to severe pregnancy complications. Most important inherited thrombophilias are factor V Leiden mutation, prothrombin mutation, and methylenetetrahydrofolate reductase mutation. The aim of our research was to evaluate the prevalence of inherited thrombophilias in severe pregnancy complications and in normal pregnancies. MATERIAL AND METHODS: The study subjects with severe preeclampsia, intrauterine growth restriction, placental abruption or fetal death were collected during the period 1999-2004 from Oulu University Hospital. We also collected during the same period voluntary parturients with normal pregnancy outcome as the control group. FVL, FII, and MTHFR gene mutations of the patients and controls were analyzed. RESULTS: We found a significant difference in the prevalence of FVL mutation between the groups. There were 9.5% FVL mutations in the study group compared to 1.8% in the control group; the observed difference between prevalences was 7.7% (95% CI 2.0-13.4). No statistical difference was found in the FII or MTHFR mutations between the groups. All FV and FII mutations were heterozygous and all the MTHFR mutations homozygous. CONCLUSION: Women with thrombophilia have a risk for severe pregnancy complications. Randomized controlled trials are needed to assess the influence of low-molecular-weight heparin in pregnant women with thrombophilia.     

The risk of complications of diagnostic and therapeutic cordocentesis

OBJECTIVES: Cordocentesis is one of the wide range of invasive diagnostic and therapeutic procedures used during pregnancy. Most common indications for is are suspicion of chromosomal abnormalities, Rh-immunisation or assessment of fetal renal function. Authors report the frequency of complications of this procedure as high as 1.5-5% with pregnancy loss rate up to 3%. DESIGN: The aim of our study was to determine the frequency of complications after cordocentesis. MATERIAL AND METHODS: We analyzed 145 pregnancies, during which 199 cordocentesis were performed. 124 of them were qualified as diagnostic with obtaining fetal blood (during 4 of them furosemidum was administered to the fetus), 22 diagnostic with furosemidum administration only and 53 therapeutic with blood transfusion (52) or pentaglobin administration (1). RESULTS: Complications of the cordocentesis were diagnosed in three cases--it was 1.5% of all of the procedures. Fetal bradycardia was reported in our material--in two cases it was isolated complication, in one case it appeared with retroplacental haematoma. In all three cases cesarean section was performed and alive babies were born. Data about the end of the pregnancy were obtained from 69 patients (47.5%). CONCLUSIONS: Cordocentesis in many case allows to establish proper diagnosis, make up decisions about treatment or termination of pregnancy and introduce management according to the needs and decisions of the patient. Complications are not very frequent and only in 1.5% were the reason of immediate caesarean section. Cordocentesis is safe and very useful tool of diagnosis in management of high risk pregnancies.     

Chemotherapy for gynecologic cancers occurring during pregnancy

Chemotherapy treatment of gynecologic malignancies during pregnancy should provide maternal benefit without undue fetal harm. We review the treatment regimens for gynecologic cancers occurring during pregnancy and the effects of chemotherapy on fetal development, parturition, and lactation. Women diagnosed with a gynecologic cancer during pregnancy require individualized treatment plans from a multidisciplinary team. TARGET AUDIENCE: Obstetricians & gynecologists, family physicians. LEARNING OBJECTIVES: After completing this CME activity, physicians should be better able to specify the indications for chemotherapy in gynecologic cancers during pregnancy and postpartum periods, discuss the risks and benefits of chemotherapy for gynecologic cancer during pregnancy and postpartum periods. In addition, they should also be able to distinguish the mechanism of action of various chemotherapy agents to choose the best treatment options for patients and monitor for impacts of chemotherapy on fetal growth to determine the best treatment and management strategies.     

Epidemiologie der fetalen Wachstumsretardierung

The duration of pregnancy and growth of the fetus are closely related. The variance in fetal weight is determined by genetic factors as well as maternal and fetal illness during pregnancy. Fetal growth is defined as retarded when it lies under the 10th percentile. Below this limit, the mortality of the fetus is increased 2–3 times both during pregnancy and during the neonatal period. Symmetrical growth retardation is caused by chromosomal abnormalities and infections in the early phase of pregnancy while asymmetrical retardation is caused by factors effecting the later phase of pregnancy. There are significant relationships between retarded growth and socio-economic factors, genetic influences and illnesses during pregnancy. A low growth rate in utero represents a danger to the fetus and makes directed intervention necessary.     

Therapeutic strategies for the prevention and treatment of pre-eclampsia and intrauterine growth restriction

Intrauterine growth restriction and pre-eclampsia are common pregnancy complications that contribute significantly to maternal and perinatal morbidity and mortality, and long term health outcomes. The underlying aetiology of these conditions involves placental under perfusion and placental ischaemia. Most prophylactic and treatment measures for these conditions are hypothesised to have effect through improved placental perfusion, or reduced oxidative stress and subsequent placental damage. However, while many therapies have biologic plausibility, there is a lack of high quality evidence that they substantially improve important outcomes such as birth weight, prematurity, mortality or serious morbidity. This review will describe and evaluate therapies currently available in clinical practice for the prevention and treatment of pre-eclampsia and intrauterine growth restriction, and outline some promising new therapies, which may change the way these conditions are managed in the future.     

Solid pseudopapillary pancreatic tumor in pregnancy. A case report

BACKGROUND: Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm, occurring predominantly in young African American women. Tumor growth, which is typically slow, may be accelerated during pregnancy secondary to the influence of progesterone. CASE: We report a rare case of an 8-cm SPT in the head of the pancreas presenting as hyperemesis gravidarum during pregnancy. In contrast to previous case reports, surgical resection of the tumor at 16 weeks' gestation, although successful, did not ameliorate the patient's abdominal pain, nausea or vomiting. With intravenous nutritional support, she delivered a healthy infant near term. CONCLUSION: SPT may present as hyperemesis gravidarum. Patients with refractory hyperemesis gravidarum should be evaluated for thyroid disease, gastroesophageal reflux, cholestasis and pancreatitis. If these more common etiologies are excluded, then one may consider SPT in the differential diagnosis, particularly in African American patients. SPT may grow during pregnancy due to progesterone responsiveness. Surgical resection during pregnancy is possible without harmful fetal effects but may not correct gastrointestinal dysfunction during pregnancy.     

Pregnancy and sarcoma

Six cases of pregnancy associated with sarcoma are presented. Among these six cases is one patient with vulvar sarcoma which was diagnosed during the 34th week of pregnancy. The literature was reviewed with reference to the effect which pregnancy may have on the growth of malignant tumors. Most authors believe that pregnancy has no deleterious effect on the course of cancer, however, this is not well supported through adequate data. Several reports to the contrary contain evidence that pregnancy may have a deleterious effect on the course of cancer. These discrepancies could be explained on the basis of differences in the natural history and biological behavior of different malignant tumors in relation to the pregnancy.     

Fetal abdomino-perineal lymphangioma: differential diagnosis and management

Fetal lymphangiomas are rare congenital anomalies of the lymphatic system most commonly presenting in the head and neck. Cystic abdominal lymphangiomas are more rare with only a few cases reported prenatally. We report a case of a prenatally detected abdomino-perineal lymphangioma that mimicked the more fatal prenatally detected sacrococcygeal teratoma (SCT), which resulted in one caregiver suggesting termination of the pregnancy. This case demonstrates the importance of carefully considering the differential diagnosis of fetal abdomino-perineal masses when counseling parents.     

Prenatal marijuana use: epidemiology, methodologic issues, and infant outcome

What do we know about marijuana use among women of reproductive age and about the use of marijuana during pregnancy? Marijuana is the most commonly used illicit substance, and after alcohol and tobacco, the most commonly used drug during pregnancy. Women who use marijuana are more likely to be white, younger, and to use other substances. The characteristics of women who use marijuana during early pregnancy are similar, although women who continue to use marijuana throughout pregnancy are somewhat different. These women are less-well educated, of lower social class, much more likely to use other substances, and more likely to be black. We do not know why some women use marijuana while others do not, and why some women discontinue their use during pregnancy while others do not. What do we know about the effects of marijuana use during pregnancy? A number of studies have investigated the relationship between prenatal marijuana exposure and outcome at birth. The results, unfortunately, are equivocal. Prospective studies that have examined women at regular and frequent intervals during pregnancy, in general, have not found a relationship between marijuana use and birthweight (Day NL, Sambamoorthi U, Taylor P, et al: unpublished data, 1990) although some have reported a small effect of marijuana use on birth length (Day NL, Sambamoorthi U, Taylor P, et al: unpublished data, 1990). Other studies, some prospective and some retrospective, have reported correlations between marijuana use during pregnancy and smaller size at birth. Several of these studies, however, failed to control adequately for other illicit drug use while one used marijuana only as a dichotomous variable in the analysis. Therefore, we do not yet know whether there is or is not an effect of marijuana use during pregnancy on intrauterine growth retardation. Only a few studies have reported on growth outside the neonatal period, and these studies have not found a consistent effect of prenatal marijuana exposure. There are, however, too few reports to assume that this is definitive. Several studies reported a relationship between prenatal marijuana use and the gestational age of the infant. As with growth, however, other studies have not corroborated these findings. Similarly, two studies have noted an increase in morphologic abnormalities, although one of these did not have a control group for comparison. Most studies have reported finding no relationship with either minor or major morphologic abnormalities. At birth, investigators have assessed the relationship between prenatal marijuana exposure and neurobehavioral outcome. Again, the results are contradictory.(ABSTRACT TRUNCATED AT 400 WORDS)