MR imaging of primary benign cardiac tumors in the pediatric population

Primary cardiac tumors are rare in all ages, especially in children, with a reported prevalence range of 0.0017–0.28% in autopsy series. Due to their rarity, the diagnostic and therapeutic pathways reserved to them are usually described by single case reports, leading to the point where a common diagnostic protocol is imperative to obtain a differential diagnosis. The first diagnostic approach is done with transthoracic echocardiogram (TTE), due to its wide availability, low cost, absence of ionizing radiations and non-invasiveness. Several tumors are discovered incidentally and, in many cases, TTE is helpful to determine location, size and anatomical features, playing a key role in the differential diagnosis. In the last few years, cardiac magnetic resonance imaging (CMR) has had an increased role in the diagnostic pathway of pediatric cardiac masses, due to its high accuracy in characterizing mass tissue properties (especially for soft tissue), and in detecting tumor size, extent, pericardial/pleural effusion, leading to the correct diagnosis, treatment and follow-up. Therefore, nowadays, several consensus statements consider CMR as a leading imaging technique, thanks to its non-invasive tissue characterization, without the use of ionizing radiation, in an unrestricted field of view. As suggested by the most recent literature, the pediatric protocol is not so different from the adult one, adapted to the size and cardiac frequency of the patient, sometimes requiring special conditions such as free-breathing sequences and/or sedation or general anesthesia in non-cooperating patients.</p

Supersolar Ni/Fe production in the Type IIP SN 2012ec

SN 2012ec is a Type IIP supernova (SN) with a progenitor detection and comprehensive photospheric-phase observational coverage. Here, we present Very Large Telescope and PESSTO observations of this SN in the nebular phase. We model the nebular [O I] 6300, 6364 lines and find their strength to suggest a progenitor main-sequence mass of 13-15 Msun. SN 2012ec is unique among hydrogen-rich SNe in showing a distinct and unblended line of stable nickel [Ni II] 7378. This line is produced by 58Ni, a nuclear burning ash whose abundance is a sensitive tracer of explosive burning conditions. Using spectral synthesis modelling, we use the relative strengths of [Ni II] 7378 and [Fe II] 7155 (the progenitor of which is 56Ni) to derive a Ni/Fe production ratio of 0.20pm0.07 (by mass), which is a factor 3.4pm1.2 times the solar value. High production of stable nickel is confirmed by a strong [Ni II] 1.939 micron line. This is the third reported case of a core-collapse supernova producing a Ni/Fe ratio far above the solar value, which has implications for core-collapse explosion theory and galactic chemical evolution models.Comment: Published versio

Anomalous venoatrial connections – CT and MRI assessment

Abnormal venous atrial (VA) connections present a congenital heart disease (CHD) challenge for pediatric cardiologists. Fully anatomical evaluation is very difficult in prenatal and perinatal follow-up, but it has a profound impact on surgical correction and outcome. The echocardiogram is first-line imaging and represents the gold standard tool for simple abnormal VA connection. CT and MRI are mandatory for more complex heart disease and “nightmare cases”. 3D post-processing of volumetric CT and MRI acquisition helps to clarify anatomical relationships and allows for the creation of 3D printing models that can become crucial in customizing surgical strategy. Our article describes a ten-year (2013–2022) tertiary referral CHD center of abnormal AV connections investigated with CT and MRI, illustrating most of these complex diseases with the help of volume rendering (VR) or multiplanar reconstructions (MPR). The nightmarish cases will also be addressed due to the complex cardiovascular arrangement that requires a challenging surgical solution for correction along with the post-surgical complications.</p

Fatality rate and predictors of mortality in an Italian cohort of hospitalized COVID-19 patients

Clinical features and natural history of coronavirus disease 2019 (COVID-19) differ widely among different countries and during different phases of the pandemia. Here, we aimed to evaluate the case fatality rate (CFR) and to identify predictors of mortality in a cohort of COVID-19 patients admitted to three hospitals of Northern Italy between March 1 and April 28, 2020. All these patients had a confirmed diagnosis of SARS-CoV-2 infection by molecular methods. During the study period 504/1697 patients died; thus, overall CFR was 29.7%. We looked for predictors of mortality in a subgroup of 486 patients (239 males, 59%; median age 71 years) for whom sufficient clinical data were available at data cut-off. Among the demographic and clinical variables considered, age, a diagnosis of cancer, obesity and current smoking independently predicted mortality. When laboratory data were added to the model in a further subgroup of patients, age, the diagnosis of cancer, and the baseline PaO2/FiO2 ratio were identified as independent predictors of mortality. In conclusion, the CFR of hospitalized patients in Northern Italy during the ascending phase of the COVID-19 pandemic approached 30%. The identification of mortality predictors might contribute to better stratification of individual patient risk

SN 2009ip à la PESSTO: no evidence for core collapse yet★

We present ultraviolet, optical and near-infrared observations of the interacting transient SN 2009ip, covering the period from the start of the outburst in 2012 October until the end of the 2012 observing season. The transient reached a peak magnitude o

Intraoperative Neuromonitoring for Thyroid Surgery in Children and Adolescents: A Single Center Experience

Intraoperative neuromonitoring (IONM) of the recurrent laryngeal nerve (RLN) has been shown in adults to minimize nerve palsy after thyroid surgery, but only few studies on its efficacy in a pediatric population have been reported. We conducted a retrospective study on patients operated for thyroid lesions from 2016 to 2022. The analyzed population was divided in two groups: patients treated from 2016 to 2020, when the identification of the RLN was performed without IONM (Group A); and patients treated since 2021, when IONM was implemented in every surgical procedure on the thyroid (Group B). Intraoperative Neurophysiological Monitoring was performed by using corticobulbar motor-evoked potentials and continuous electromyography. Twentyfive children underwent thyroid resection, 19 (76%) of which due to thyroid carcinoma. Each patient’s recurrent nerve was identified; IONM was used in 13 patients. In Group A, one temporary nerve palsy was identified postoperatively (8.3%), while in group B one nerve dysfunction occurred (7.7%). No statistically significant difference was found between the two groups in terms of post-operative RLN palsy. No surgical complication due to the use of IONM was reported. In children and teenagers, intraoperative neuromonitoring of the recurrent laryngeal nerve is a safe and accurate method, minimizing the risk of nerve damage

Malignant pancreatic endocrine tumor in a child with tuberous sclerosis

Tuberous sclerosis complex (TSC) is an autosomal dominant condition whose signs and symptoms may vary from a few hypopigmented skin spots to epilepsy, severe mental retardation, and renal failure. The disease is caused by mutations in either TSC1 or TSC2 gene, at chromosome 9q34 and 16p 13.3. Inactivation of both alleles at TSC1 or TSC2 loci is associated with the development of hamartomas in different organs, and only rarely with malignant neoplasms. In this study we present a 6-year-old boy with TSC and with a malignant islet cell tumor of the pancreas. Mutation analysis of DNA extracted from peripheral blood cells of the patient identified an R1459X de novo mutation in exon 33 of the TSC2 gene. Immunohistochemical analysis with anti-tuberin antibodies on paraffin-embedded tissue sections showed loss of tuberin immunostaining in tumor cells but normal expression in residual normal pancreas. DNA analysis of tumor and normal cells showed chromosome 16p13 loss of heterozygosity in malignant pancreatic islet cell tumor but not in normal pancreas. These findings suggest a role for tuberin, the TSC2 gene product, in the pathogenesis of malignant pancreatic endocrine tumor

Chemotherapy and Surgery in Children With Cystic Echinococcosis

[No abstract available

Vascular Access in Pediatric Oncology and Hematology: State of the Art

Management and successful use of vascular access are critical issues in pediatric patients affected by malignancies. Prolonged course of disease, complex and various treatment protocols require long-lasting vascular access providing adequate tools to administrate those therapies and to collect routine blood sampling without painful and repeated venipuncture. For these reasons, central venous catheters are currently an important component in pediatric onco-hematological care, with a direct influence on outcome. Indeed, there are peculiar issues (techniques of insertion, management, complications etc.) which must be well-known in order to improve the outcome and the quality of life of children with cancer

Gastric cancer, inflammatory bowel disease and polyautoimmunity in a 17-year-old boy: CTLA-4 deficiency successfully treated with Abatacept

Gut involvement is frequent in immunologic disorders, especially with inflammatory manifestations but also with cancer. In the last years, advances in functional and genetic testing have improved the diagnostic and therapeutic approach to immune dysregulation syndromes. CTLA-4 deficiency is a rare disease with variable phenotype, ranging from absence of symptoms to severe multisystem manifestations and complications. We describe a rare case of CTLA-4 deficiency in a boy with gastric cancer, very early onset inflammatory bowel disease and polyautoimmunity, the second-ever reported in the literature with the same characteristics. A 17-year-old boy was referred to Bambino Gesu Children's Hospital of Rome, a tertiary care center, for a gastric mass and a long-term history of very early onset inflammatory bowel disease, diabetes mellitus type 1, polyarthritis and psoriasis. Histology of gastric biopsies revealed the presence of neoplastic signet ring cells. Imaging staging showed localized cancer; therefore, the patient underwent subtotal gastrectomy with termino-lateral gastro-jejunal anastomosis. Immunological work up and genetic testing by next-generation sequencing panels for primary immunodeficiencies led to the diagnosis of CTLA-4 deficiency. Good disease control was obtained with the administration of Abatacept. The patient experienced an asymptomatic SARS-CoV-2 infection without any concern. Eighteen months after treatment initiation, the patient is alive and well. Immunologic and genetic testing, such as next-generation sequencing, should always be part of the diagnostic approach to patients with complex immune dysregulation syndrome, severe clinical course, poor response to treatments or cancer. The early recognition of the monogenic disease is the key for disease management and targeted therapy. Copyright (C) 2021 Wolters Kluwer Health, Inc. All rights reserved