Incidental diagnosis of cardiac fibroma in an infant with acute viral bronchiolitis / Diagnóstico incidental de fibroma cardíaco em um lactente com bronquiolite viral aguda

ABSTRACT Objective: Pediatric cardiac tumors are rare and, among them, 90% are benign. Cardiac fibroma is the second most frequent tumor, after rhabdomyoma. The objective of this study is to report a case of cardiac fibroma diagnosed incidentally in a patient admitted with acute viral bronchiolitis. Case description: A 5-month-old male infant was admitted to the pediatric emergency department with acute viral bronchiolitis requiring hospitalization. He presented a detectable respiratory syncytial virus in oropharyngeal swab, blood test with lymphocytosis and a chest radiography revealed cardiomegaly. Further cardiologic testing was performed detecting elevation of cardiac biomarkers, an electrocardiogram with alteration of left ventricular repolarization and echocardiogram with a heterogeneous mass in the left ventricular, with areas of calcification. A chest angiotomography suggested rhabdomyosarcoma or cardiac fibroma and a magnetic resonance showed a mass, with characteristics suggesting fibroma. The final diagnosis was made after two cardiac catheterizations for biopsy of the lesion, confirming cardiac fibroma by anatomopathological examination. Because the patient had moderate to severe systolic dysfunction, he was submitted to heart transplant. Comments: One third of cardiac fibromas are asymptomatic, generally diagnosed late through tests ordered for other reasons. The gold-standard test for definitive diagnosis is biopsy. Cardiac fibroma usually does not present spontaneous regression and, in most cases, partial or total surgical resection is necessary. When tumors are unresectable, heart transplantation should be indicated. It is essential to have detailed characterization of the cardiac mass to establish the most appropriate therapeutic approach for each patient.

RESUMO Objetivo: Tumores cardíacos pediátricos são raros e, entre eles, 90% são benignos. O fibroma cardíaco é o segundo mais frequente, após o rabdomioma. O objetivo deste trabalho é relatar um caso de fibroma cardíaco, cujo diagnóstico foi incidental em um paciente internado por bronquiolite viral aguda. Descrição do caso: Lactente do sexo masculino com cinco meses foi atendido em um pronto atendimento pediátrico com quadro de bronquiolite viral aguda, necessitando de hospitalização. Em exames complementares, apresentou vírus sincicial respiratório detectável, hemograma com linfocitose, além de radiografia de tórax com cardiomegalia. Em investigação cardiológica, as enzimas cardíacas encontravam-se elevadas, o eletrocardiograma apresentava alteração de repolarização de parede lateral de ventrículo esquerdo e o ecocardiograma demonstrou massa heterogênea em ventrículo esquerdo, com áreas de calcificação. A angiotomografia de tórax sugeriu rabdomiossarcoma ou fibroma cardíaco e a ressonância magnética demonstrou massa cuja localização e características sugeriram fibroma. O diagnóstico foi conclusivo após dois cateterismos para biópsia da lesão, confirmando fibroma cardíaco pelo anatomopatológico. Em razão da disfunção sistólica moderada a grave, o paciente foi submetido a transplante cardíaco. Comentários: Um terço dos fibromas cardíacos é assintomático, geralmente diagnosticado tardiamente por meio de exames solicitados por outro motivo. O padrão-ouro para o diagnóstico definitivo é a biópsia. O fibroma cardíaco não costuma apresentar regressão espontânea, sendo submetido, na maioria dos casos, a ressecção cirúrgica parcial ou total. Nos tumores irressecáveis, o transplante cardíaco deve ser indicado. A caracterização detalhada da massa cardíaca é fundamental para traçar a conduta terapêutica mais adequada para cada paciente.

Intra- and interobserver agreement of histopathological findings in pediatric patients with eosinophilic esophagitis

Abstract Objective: To assess intra- and interobserver agreement among non-expert pathologists in identifying features of the eosinophilic esophagitis histologic scoring system (EoEHSS) in pediatric patients. Patients and methods: The authors used 50 slides from patients (aged 1-15 years; 72% male) with EoE. EoEHSS evaluates eosinophilic inflammation and other features including epithelial basal zone hyperplasia, eosinophilic abscesses, eosinophil surface layering, dilated intercellular spaces, surface epithelial alteration, dyskeratotic epithelial cells, and lamina propria fibrosis. Grade and stage of abnormalities are scored using a 4-point scale (0 normal; 3 maximum change). Four pathologists determined EoEHSS findings on two occasions. Intra- and interobserver agreement was assessed using Kappa (κ) statistics and intra-class correlation coefficients. Results: Intra- and interobserver agreement for the identification of eosinophil counts ≥ 15/high power field (HPF) was excellent, however varied when assessing additional features of the EoEHSS. For the more experienced pathologist, agreement for most EoEHSS items and the composite scores was substantial to excellent. For the less experienced pathologists, intraobserver agreement ranged from absent to substantial for individual features and ranged from moderate to substantial for the composite scores. Conclusion: Most items of the EoEHSS had substantial to excellent reliability when assessed by a pathologist experienced in the diagnosis of EoE but presented lower repeatability among less experienced pathologists. These findings suggest that specific training of pathologists is required for the identification of EoEHSS characteristics beyond eosinophil count, as these features are considered useful in the evaluation of response to treatment and correlation with clinical manifestations and endoscopic findings.

Endoscopic and histological characteristics in patients with eosinophilic esophagitis responsive and non-responsive to proton pump inhibitors / Características endoscópicas e histológicas em pacientes com esofagite eosinofílica responsiva e não responsiva aos inibidores de bomba de prótons

Abstract Objective: To compare endoscopic and histologic features of pediatric patients with eosinophilic esophagitis (EoE) responding to proton pump inhibitor (PPI) to those not responding to PPI. Methods: Endoscopic reports and photographs of patients with symptoms of esophageal dysfunction and ≥15 eosinophils per high-powered field (eos/hpf) in esophageal biopsies prior to PPI trial were reviewed. Patients were classified as responsive to PPI (PPIREoE) or non-responsive to PPI (PPINREoE) according to response totreatment (<15 eos/hpf) at second endoscopy after 8 weeks. Results: Of the 231 patients (72.3% male), 64 (27.7%) were responsive to the proton pump inhibitors. Edema (77.3% vs. 62.5%, p = 0.031) and vertical lines (69.5% vs. 51.6%, p = 0.014) were more frequent in PPINREoE patients. An eosinophil count in the mid-esophagus ≥ 35 eos/HPF (25.1% vs. 12.5%) was more frequent in these patients (p = 0.001). Those with eosinophil count < 15 eos/HPF in the mid-esophagus at the first endoscopy were more likely to respond to treatment with proton pump inhibitors compared to patients with 15-34 eos/HPF (p = 0.004, OR: 3.26, 95% CI: 1.46-7.24) and to patients with ≥ 35 eos/HPF (p = 0.006, OR: 3.20, 95% CI: 1.39-7.41). Conclusion: Edema and vertical lines at the endoscopy and a higher eosinophil count in the mid-esophagus were more frequent in patients who were non-responsive to proton pump inhibitors. As there were no significant differences in the other findings between the groups, it cannot be affirmed that these characteristics are sufficient to differentiate between PPINREoE and PPIREoE patients.

Resumo Objetivo: Comparar características endoscópicas e histológicas entre pacientes com esofagite eosinofílica responsiva (EoERIBP) e não responsiva (EoENRIBP) ao tratamento com inibidores de bomba de prótons. Métodos: Avaliados laudos e imagens endoscópicas de pacientes com sintomas de disfunção esofágica associados a contagem ≥ 15 eosinófilos por campo de grande aumento (eos/CGA) em biópsia do esôfago. Os pacientes foram classificados em responsivos (EoERIBP) ou não responsivos (EoENRIBP) aos inibidores de bomba de prótons conforme resposta ao tratamento na segunda endoscopia (< 15 eos/CGA) após 8 semanas. Resultados: Dos 231 pacientes (72,3% masculino), 64 (27,7%) foram responsivos aos inibidores de bomba de prótons. Edema (77,3% vs. 62,5%, p = 0,031) e linhas verticais (69,5% vs. 51,6%, p = 0,014) foram mais frequentes nos EoENRIBP. A contagem de eosinófilos em esôfago médio ≥ 35 eos/CGA (25,1% vs. 12,5%) foi mais frequente nesses pacientes (p = 0,001). Os que apresentaram contagem de eosinófilos < 15 eos/CGA no esôfago médio à primeira endoscopia apresentaram maior chance de responder ao tratamento com inibidores de bomba de prótons em comparação aos pacientes com 15-34 eos/CGA (p = 0,004; OR: 3,26; IC95%: 1,46-7,24) e aos pacientes com ≥ 35 eos/CGA (p = 0,006; OR: 3,20; IC95%: 1,39-7,41). Conclusão: Edema e linhas verticais à endoscopia e maior contagem de eosinófilos em esôfago médio foram mais frequentes nos pacientes não responsivos aos inibidores de bomba de prótons. Uma vez que não houve diferenças significativas nos outros achados entre os grupos, não se pode afirmar que essas características sejam suficientes para distinguir pacientes com EoENRIBP dos pacientes com EoERIBP.