Diagnosis and management of primary vulvar adenocarcinoma of mammary gland type: report of two distinct cases.

Adenocarcinoma of mammary gland type (AMGT) of the vulva is extremely rare and its aetiopathogenesis is not fully understood. Low incidence is partly responsible for the lack of guidelines for patient management. Here, two cases of postmenopausal patients diagnosed with AMGT with different therapeutical approaches are reported. Histopathological patterns are considered essential for diagnosis. The triple-negative immunohistochemical (IHC) profile of one of the cases represented a diagnostic challenge. Interestingly, it presented an immunophenotypical profile similar to triple-negative breast cancers, supporting the molecular similarities between vulvar AMGT and breast carcinomas. Surgical procedures include radical vulvectomy or radical local excision. Lymphatic involvement may be assessed by sentinel lymph node biopsy or lymphadenectomy. Adjuvant treatment was dependent on the IHC profile and disease staging. Although both cases had similar features on clinical examination, pathological and molecular characteristics and treatment approach were distinct. That illustrates the diagnostic and therapeutical challenge of this rare entity.

Induction of labour vs. spontaneous vaginal delivery in twin pregnancy after 36 weeks of gestation.

Time for delivery and delivery pathway in twin pregnancies are still in great debate. Our study goal was to compare the characteristics of delivery and maternal-foetal outcome in uncomplicated near-term twin pregnancies undergoing labour induction and those with spontaneous labour. We found no statistical differences in patients with twin pregnancies who underwent labour induction and those with spontaneous labour regarding the history of previous caesarean delivery, parity, pregnancy achieved by assisted reproductive techniques (ART), chorionicity and cervical dilation at the admission as well as maternal and neonatal morbidity, and admission to the neonatal intensive care unit. There were significant differences in the caesarean section rate (60.6 vs. 33.3%, p < .05) and the time interval between delivery of the first and second foetus (9.8 vs. 11.7 min, p = .024). There was an increased incidence of caesarean section after the induction of labour. However, it appears to be a safe option.

Monoamniotic twins discordant for body stalk anomaly.

Body stalk anomaly is a rare malformation. This anomaly in monozygotic twins is extremely unusual. We describe a case of monoamniotic pregnancy discordant for body stalk anomaly diagnosed at 11 weeks. Ultrasound showed a fetus with a large anterior abdominal wall defect, anomaly of the spine and no evidence of lower extremities and other with a normal morphology. As far as our concern, only three monoamniotic pregnancies discordant for this malformation were reported. Our case represents the fourth reported monoamniotic pregnancy discordant for body stalk anomaly with diagnosis made by ultrasound and the second diagnosed in the first trimester.

Antenatal manifestations of mitochondrial disorders.

Mitochondrial respiratory chain diseases are a heterogeneous group of pathologies caused by genetic alterations affecting mitochondrial energy production. Theoretically, this deficiency may lead to any symptoms, in any organ or tissue, at any age even before birth. The aim of our study was to identify the frequency and characterize antenatal manifestations identifying possible associations between mitochondrial disease and more specific and earlier manifestation. We retrospectively review the files of 44 paediatric subjects with genetic and biochemical alterations of respiratory chain identified in the first decade of life and compare data with a control group (n = 88). Our results show that maternal age was similar in both groups. The female gender was predominant in patients group. Gestational age at delivery was similar in both groups. Concerning birth weight, it was significantly lower (p = 0.001) in patients (2899.9 ± 538.3 vs. 3246.6 ± 460.2 g). Fifteen pregnancies of the patients group were considered abnormal. Our findings show that intrauterine growth restriction was the most frequent antenatal feature observed. Neonatal morbidity was significantly higher (fivefold) in patients (p < 0.001). The clinical findings are independent of the molecular defect type. Our results are preliminary and more studies are needed, in order to learn more about mitochondrial physiology and activity in embryological development for the assessment of mitochondrial disease progress in fetal life. However, the present work is a significant contribution, given the scarcity of information in this field.

[Vaccines and pregnancy]. / Vacinas e gravidez.

Routine vaccination is part of the pediatrics universe. In adulthood and particularly when women voluntarily access to medical care, immunization should be reviewed and updated. There are many doubts that generate in all health professionals anxiety and concern about the vaccination of a pregnant woman. This article aims to describe the immunological changes in pregnant women, to clarify the purpose of immunization during pregnancy, and to enumerate indications, contraindications and risks of vaccines of the Portuguese National Vaccine Plane and other vaccines against diseases with prevalence in other countries. Due to the medical and social impact of vaccination against seasonal influenza and influenza A (H1N1) in the winter of 2009, during an influenza (H1N1) pandemic flu, we make reference to the indications and vaccination against these infections in pregnancy.