ABSTRACT
Pleuropulmonary blastoma (PPB) is a rare embrionary mesenchymal neoplasm but is recognized as the most common pulmonary malignancy of childhood. It may present metastasis to the brain and also be indicative of other neoplasms in affected individuals or in their relatives. Being such a rare disease, it is considered a difficult diagnosis to be made. A 3-year-old female presented with fever and respiratory distress. At first, she had been treated for pneumonia with antibiotics for 14 days in another hospital with no response. Computed tomography (CT) scan showed a right lung/mediastinal mass. The patient was referred to our institution and a new CT-scan evidenced a complex mass of irregular borders, cystic areas and solid projections, along with a right pneumotorax. The mass was biopsied and hematoxylin-and-eosin (HE) stained histological sections showed a neoplasm composed of small and round cells with hyperchromatic nuclei and scant cytoplasm. The immunohistochemical profile demonstrated positivity for desmin, myogenin and Myo-D1, suggesting the diagnosis of rhabdomyosarcoma. After two weeks of hospitalization, the patient was clinically stable and initiated the first chemotherapy cycle. Surgical resection of the mass was performed and the HE slides demonstrated a neoplasm composed of anaplastic and condrossarcomatous cells with extensive necrosis. The correlation of clinical data, radiological and morphological features were conclusive of a PPB type II. The recognition and diagnosis of this entity is of great importance due to its clinical and prognostic particularities.
Subject(s)
Humans , Pulmonary Blastoma/diagnosis , Pleural Neoplasms/diagnosis , Lung Neoplasms/diagnosis , ImmunohistochemistryABSTRACT
Análise clínico-epidemiológica de crianças com Constipação Intestinal (CI). Método: estudo transversal de prevalência das crianças com CI atendidas no Ambulatório de Gastroenterologia Pediátrica da Fundação Santa Casa de Misericórdia do Pará (FSCMPA), entre agosto de 2006 a março de 2008. Resultados: houve predomínio de CI nos pacientes do sexo feminino; em 53,7% dos pacientes, os sintomas iniciaram nos primeiros 2 anos de vida e 65,9% tinham mais de 4 anos à primeira consulta especializada; 90,2% tinham fezes com calibre aumentado; 63,4% tinham fezes endurecidas e 56,1% tinham escape fecal; distensão abdominal foi encontrado em 82,9% dos pacientes e dor abdominal em 24,4%; 73,2% receberam tratamento prévio à consulta especializada e a Constipação Funcional (CF) correspondeu a 90,2% das causas de CI. Conclusão: as características clínicas e epidemiológicas das crianças pesquisadas aproximaram-se das encontradas na literatura, com predomínio da CF.
To analyze the clinical Epidemiologic profile on patients with Constipation Intestinal(CI).Methods: cross-sectional study of the children with CI assisted in the ambulatory of Pediatric Gastroenterology of the Santa Casa de Misericórdia do Pará Foundation (FSCMPA), between August 2006 and March of 2008. Results: there was a most prevalence of CI in femalepatients. In 53,7% of patients, the symptoms started before two year old age and 65,9 % of patients had 4 years old up in the first visit to the ambulatory; About 90, 2% of patients had large diameter stools; 63.4% had hard stools and 56.1% had fecal incontinence; abdominal distension was found in 82.9% of patients and abdominal pain in 24.4%; 73.2% of patients received treatment prior to specialized visit and Functional Constipation (CF) accounted in 90.2% of the causes of CI. Conclusion: the study showed similar results to those observed at other studies, with predominance of the CF.