Childhood carcinoid tumors: description of a case series in a Brazilian cancer center.

CONTEXT AND OBJECTIVE: Carcinoid tumors are very rare both in children and adults. About 85% of these tumors develop in the gastrointestinal tract. The objective of the present study was to describe our experience with children treated of carcinoid tumors, and investigate the frequency morphological findings and results. DESIGN AND SETTING: Report on case series, at the Department of Pediatrics of Centro de Tratamento e Pesquisa Hospital do Câncer, São Paulo. METHODS: This was a retrospective analysis of clinical pathological data and outcomes among children (< 18 years old) with carcinoid tumors admitted from January 1, 1990, to December 31, 2001. RESULTS: Nine patients (mean age 12.2 years) were included: six girls and three boys (2:1), all of them Caucasian. In eight cases (89%), the primary tumor site was the appendix and in one (11%) it was the left bronchus. For those with primary tumor in the appendix, the main complaint was abdominal pain, which led to appendectomy. Only one patient underwent right hemicolectomy due to tumor extension into the serosa. The patient with bronchial tumor underwent left pneumonectomy. All patients had localized disease and are alive and free of disease. They have had follow-ups lasting from 1 to 11 years (mean of 3.5 years). CONCLUSION: Although the majority of carcinoid tumors arise from the appendix, these tumors can also occur in other primary sites. Surgical resection at an early stage allows for good prognosis without the need for any adjuvant treatment.

Frequency of copy number abnormalities in common genes associated with B-cell precursor acute lymphoblastic leukemia cytogenetic subtypes in Brazilian children.

Copy number alterations (CNAs) in genes committed to B-cell precursors have been associated with poor survival in subgroups of patients with B-cell precursor acute lymphoblastic leukemia (BCP-ALL). We investigated submicroscopic alterations in a series of 274 Brazilian children with BCP-ALL by multiplex ligation-dependent probe amplification and evaluated their correlation with clinical and laboratory features. The relevance of overlapping CNA abnormalities was also explored. Deletions/amplifications in at least one gene were identified in 83% of the total series. In children older than 2 years, there was a predominance of CNAs involving deletions in IKZF1, CDKN2A, and CDKN2B, whereas the pseudoautosomal region 1 (PAR1) had deletions that were found more frequently in infants (P <0.05). Based on the cytogenetic subgroups, favorable cytogenetic subgroups showed more deletions than other subgroups that occurred simultaneously, specifically ETV6 deletions (P <0.05). TCF3-PBX1 was frequently deleted in RB1, and an absence of deletions was observed in IKZF1 and genes localized to the PAR1 region. The results corroborate with previous genome-wide studies and aggregate new markers for risk stratification of BCP-ALL in Brazil.

Childhood carcinoid tumors: description of a case series in a Brazilian cancer center

CONTEXTO E OBJETIVO: Tumores carcinóides são bastante raros, tanto em crianças quanto em adultos. Cerca de 85% deles desenvolvem-se no trato gastrintestinal. O objetivo do presente estudo foi descrever a experiência de nossa instituição com crianças tratadas de tumores carcinóides, investigando sua freqüência, achados morfológicos e resultados. TIPO DE ESTUDO E LOCAL: Relato de série de casos, no Departmento de Pediatria do Centro de Tratamento e Pesquisa Hospital do Câncer, São Paulo, Brasil. MÉTODOS: Análise retrospectiva de dados clinicopatológicos e resultados de crianças menores de 18 anos de idade com tumores carcinóides, admitidas entre 1º de janeiro de 1990 e 31 de dezembro de 2001. RESULTADOS: O estudo inclui nove pacientes, todos brancos, com idade média de 12,2 anos, sendo seis meninas e três meninos (2:1). O apêndice foi o sítio primário em oito casos (89%) e o brônquio esquerdo em um caso (11%). Nos primeiros, o sintoma principal foi dor abdominal que levou a apendicectomia. Apenas um paciente foi submetido a hemicolectomia direita em razão da extensão do tumor na serosa. O paciente que tinha o tumor primário no brônquio foi submetido a pneumectomia à esquerda. Todos os pacientes tinham doença localizada e estão vivos sem doença, com acompanhamento de 1 a 11 anos (média de 3,5 anos). CONCLUSÃO: Embora a maioria dos tumores carcinóides tenha sua origem no apêndice, eles podem ocorrer em outros sítios. A ressecção cirúrgica em estágios iniciais permite um bom prognóstico sem necessidade de tratamento adjuvante.

Avaliaçäo de amigdalite estreptocócica em crianças / Streptococci tonsillitis evaluation in children

Os autores estudaram 51 crianças de ambos os sexos, com idade entre 18 meses a 13 anos que apresentavam diagnóstico clínico de amigdalite estreptocócica, e sugerem que os sintomas clínicos e dados de exame físico säo insuficientes para o diagnóstico preciso de amigdalite bacteriana, levando a erros na conduta terapêutica.