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Primary pulmonary synovial sarcoma is a rare type of soft tissue tumor. Exceptionally it can occur during pregnancy, representing a challenge in management and treatment given its notable aggressiveness and the not infrequent incidence of maternal death. We report our case of metastatic recurrence of pulmonary synovial sarcoma during pregnancy, with the aim to emphasize the decision-making, diagnostic, and therapeutic multidisciplinary processes and the evolution of the pathology. Besides, we focused on the analysis of the limited literature data available on the topic.
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(1) Background: The number of adnexal masses detected during pregnancy has increased due to the use of first-trimester screening and increasingly advanced maternal age. Despite their low risk of malignancy, other risks associated with these masses include torsion, rupture and labor obstruction. Correct diagnosis and management are needed to guarantee both maternal and fetal safety. Adnexal masses may be troublesome to classify during pregnancy due to the increased volume of the uterus and pregnancy-related hormonal changes. Management should be based on ultrasound examination to provide the best treatment. The aim of this study was to describe the ultrasound features of ovarian masses detected during pregnancy and to optimize and personalize their management with the expertise of gynecologists, oncologists and sonographers. (2) Methods: Clinical, ultrasound, histological parameters and type of management (surveillance vs. surgery) were retrospectively retrieved. Patient management, perinatal outcomes and follow-up were also evaluated. (3) Results: according to the literature, these masses are most frequently benign, ultrasound follow-up is the best management, and obstetric outcomes are not considerably influenced by the presence of adnexal masses. (4) Conclusions: the management of patients with ovarian masses detected during pregnancy should be based on ultrasound examination, and a centralization in referral centers for ovarian masses should be considered.
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Placental site trophoblastic tumor (PSTT) is a very rare form of gestational trophoblastic disease (GTD) that occurs mainly in women who have a history of termination of pregnancy. It has different characteristics from other gestational trophoblastic tumors: it grows slowly, secretes low levels of beta-human chorionic gonadotropin (ß-hCG), with low metastatic potential. We report a case of PSTT of a 32-year-old patient. Seven months after delivery, the patient presented at our Center with persistence of menorrhagia for at least 1 month. A slightly high level of beta-human chorionic gonadotropin (ß-hCG) was observed. TVUS and MRI, an operative hysteroscopy and a laparoscopy were performed. The histological and immunohistochemical findings demonstrated PSTT. Diagnosis of juvenile cystic adenomyoma (JCA) was also added. A total body CT scan was negative for metastases. A total hysterectomy with salpingectomy was performed. We performed a search of relevant studies about PSTT of the last years. A systematic search of Pubmed databases was conducted. Appropriate search terms were constructed by reviewing abstracts, titles and keywords relating to PSTT known to the authors. All articles known to the authors useful to the review were included, comparing with our clinical case. Stages and treatment are related to survival rates, with long term survival expected for stage I low-risk disease after hysterectomy. Our case is a stage I disease with good prognostic factors (patient's age and absence of metastases) and, as described in the literature, a total hysterectomy with salpingectomy was performed.
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Doença Trofoblástica Gestacional , Tumor Trofoblástico de Localização Placentária , Neoplasias Uterinas , Feminino , Humanos , Gravidez , Adulto , Tumor Trofoblástico de Localização Placentária/diagnóstico , Neoplasias Uterinas/cirurgia , Placenta/patologia , Gonadotropina Coriônica Humana Subunidade betaRESUMO
INTRODUCTION: The aim of this study is to evaluate short- and long-term consequences in children born to women after different bariatric surgery (BS) procedures. METHODS: A questionnaire survey was given to the mothers referred from 1994 to 2019 to our center for pregnancy and delivery management after BS procedures: (a) malabsorptive surgery, (b) restrictive procedures, and (c) combined restrictive-malabsorptive procedures. RESULTS: Data from 74 children born after BS, aged 0 month to 12 years, were analyzed. The prevalence of children with underweight was 5.4%, normal weight 59.5%, overweight 16.2%, and obesity 18.9%. The prevalence of obesity was higher in children pre-school aged than that in school-aged ones. Neurodevelopmental disorders were more frequent if maternal BMI before bariatric surgery was ≥ 41 kg/m2 (p = 0.008), as well as if the pregnancy occurred less than 18 months after BS (p = 0.028). In school-aged children conceived within 18 months after BS, the highest risk of neurodevelopmental disorders (p = 0.028) and overweight (p = 0.018) was observed. The prevalence of neurodevelopmental disorders was much higher for small for gestational age babies (p = 0.048). Children born after biliopancreatic diversion (BPD) showed less maternal breastfeeding, shorter breastfeeding duration, more overweight, and more occurrence of atopic dermatitis in comparison with children born after other bariatric procedures. CONCLUSIONS: Postnatal health in children born to women after BS was impaired by long-term consequences and by other diseases later in life. Children born after BPD were particularly at higher risk for short and long term consequences when compared to children born after other BS procedures.
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Cirurgia Bariátrica , Desvio Biliopancreático , Obesidade Mórbida , Cirurgia Bariátrica/efeitos adversos , Criança , Pré-Escolar , Feminino , Humanos , Recém-Nascido , Obesidade/cirurgia , Obesidade Mórbida/cirurgia , Sobrepeso , GravidezRESUMO
INTRODUCTION: Myasthaenia gravis (MG) is the most common disease of the neuromuscular junction; clinical presentation of the disease includes a variety of symptoms, the most frequent beign the only ocular muscles involvement, to the generalized myasthenic crisis with diaphragmatic impairment and respiratory insufficiency. It is most common in women between 20 ad 40 years. EVIDENCE ACQUISITION: We performed a comprehensive search of relevant studies from January1990 to Dicember 2019 to ensure all possible studies were captured. A systematic search of Pubmed databases was conducted. EVIDENCE SYNTHESIS: Pregnancy has an unpredictable and variable effect on the clinical course of MG; however, a stable disease before is likely not to relapse during pregnancy. exacerbations can still occur more often during the first trimester and the post partum period. The transplacental passage of antibodies results in a neonatal transient disease, whereas the major concern is related to foetal malformations such as fetal arthrogryposis and polyhydramnios. The overall neonatal outcome described in literature is variable, perinatal mortality in women with MG is generally the same as non affected patients, although in one study the risk of premature rupture of the membranes was higher. Treatment of MG in pregnangncy includes pyridostigmine and corticosteroids, although the latter have been associated with higher risk of cleft palate, premature rupture of the membranes and preterm delivery. These drugs appear also to be safe in breastfeeding. In MG patients spontaneous vaginal delivery should be encouraged, for surgery could cause acute worsening of myasthenic symptoms; also an accurate anesthesiological evaluation must be performed prior to both general and local anesthesia due to increased risk of complications. CONCLUSIONS: Most of the myasthenic women could have uneventful pregnancy with good obstetrical outcomes, both for mother and neonate. However, a careful planning of pregnancy and multidisciplinary team approach, composed by neurologists, obstetricians, neonatologists and anesthesiologists, is required to manage these pregnancies.
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Parto Obstétrico , Miastenia Gravis/tratamento farmacológico , Complicações na Gravidez/tratamento farmacológico , Corticosteroides/efeitos adversos , Corticosteroides/uso terapêutico , Artrogripose/etiologia , Aleitamento Materno , Inibidores da Colinesterase/uso terapêutico , Fissura Palatina/induzido quimicamente , Anormalidades Congênitas/etiologia , Anormalidades Congênitas/imunologia , Progressão da Doença , Feminino , Ruptura Prematura de Membranas Fetais/etiologia , Humanos , Recém-Nascido , Miastenia Gravis/complicações , Equipe de Assistência ao Paciente , Mortalidade Perinatal , Poli-Hidrâmnios/etiologia , Gravidez , Primeiro Trimestre da Gravidez , Brometo de Piridostigmina/uso terapêutico , RecidivaRESUMO
Autoimmune Congenital Heart Block (CHB) is an immune-mediated disease due to transplacental passage of circulating anti-Ro/SSA and anti-La/SSB autoantibodies. It occurs in 2% of anti-Ro/SSA-exposed pregnancies, and recurrence rate is nine times higher in subsequent pregnancies. Aim of this review is to identify biomarkers of CHB and treatment strategies. The Ro-system is constituted by two polypeptides targeted by the anti-Ro52 and anti-Ro60 autoantibodies. The central portion of Ro52 (p200), more than the full amino-acid sequence of Ro-52, is recognized to be the fine specificity of anti-Ro associated to the highest risk of cardiac damage. If anti-p200 antibody should be tested, as biomarker of CHB, over standard commercial ELISAs is still debated. Recent studies indicate that type I-Interferon (IFN) can activate fibroblasts in fetal heart. In the mother the anti-Ro/La antibodies activate the type I IFN-signature, and maternal IFN-regulated genes correlate with a similar neonatal IFN-gene expression. Evaluation of maternal IFN-signature could be used as novel biomarker of CHB. The measurement of "mechanical" PR interval with weekly fetal echocardiogram (ECHO) from 16 to at least 24 weeks of gestation is strongly recommended for CHB prenatal diagnosis. However, ECHO screening presents some limitations due to difficult identification of first-degree block and possible occurrence of a complete block from a normal rhythm in few days. Maternal administration of Hydroxychloroquine from the tenth week of gestation, modulating toll-like receptor and autoantibody-dependent type I IFN activation on the fetus, has an important role in preventing CHB in pregnant women with high risk for recurrent CHB.