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
OBJETIVO: Analisar as características clínico-patológicas do tecido pancreático heterotópico em órgãos digestivos abdominais. MÉTODOS: Realizamos estudo retrospectivo analisando 18 portadores de pâncreas heterotópico diagnosticados histologicamente. Seus dados clínicos e histopatológicos foram revistos. O tecido pancreático heterotópico foi classificado em três modelos histológicos: tipo I constituído por três componentes do pâncreas normal (ácinos, ductos e ilhotas), tipo II com dois componentes e tipo três com somente um componente. RESULTADOS: A média de idade foi de 52,7 anos, variando de 34 a 73 anos, com nove homens e nove mulheres. Sintomas foram observados em somente quatro doentes, sendo suas lesôes diagnosticadas por gastroscopia. Os 14 restantes eram assintomáticos e suas anomalias descobertas acidentalmente. A maioria das lesões situava-se no trato superior: sete (38,9%) no estômago, seis (33,3%) no duodeno e três (16,6%) no jejuno. A heterotopia localizou-se preferencialmente na submucosa (83,3%), mas também foi observada na muscular própria e na sub-serosa. Em sete (38,9%) espécimes todos os componentes pancreáticos foram constatados (tipo I), em oito (44,4%) estavam presentes glândulas exócrinas e ductos excretores (tipo II) e em três (16,7%) somente o tecido exócrino foi observado (tipo III). CONCLUSÃO: A heterotopia pancreática é rara. Doentes com ectopia pancreática diagnosticadas pelo estudo patológico, assintomáticos ou com sintomas discretos devem permanecer em observação. As lesões detectadas acidentalmente durante procedimentos cirúrgicos necessitam ser removidas por procedimentos conservadores.
OBJECTIVE: To analyze the clinical and pathological features of heterotopic pancreatic tissue in abdominal digestive organs. METHODS: We retrospectively studied 18 patients with histologically diagnosed heterotopic pancreas. Clinical and histopathologic data were reviewed. Heterotopic pancreatic tissues were classified in three histological models: Type I consists of three components of normal pancreas (acini, ducts and islets), type II with two components and type three with only one component. RESULTS: The mean age was 52.7 years, ranging from 34 to 73 years, nine of them men and nine women. Symptoms were observed in only four patients, and their lesions were diagnosed by gastroscopy. The remaining 14 were asymptomatic and their anomalies were discovered accidentally. Most of the lesions were located in the upper gastrointestinal tract: seven (38.9%) in the stomach, six (33.3%) in the duodenum and three (16.6%) in the jejunum. Heterotopia was mostly located in the submucosa (83.3%) but was also observed in the muscularis propria and in the sub-serosa. In seven specimens (38.9%) all pancreatic components were found (type I), in eight (44.4%) exocrine glands and excretory ducts were present (type II) and in three (16.7%) only exocrine tissue was observed (type III). CONCLUSION: Pancreatic heterotopia is rare. Patients with pancreatic ectopia diagnosed by pathological study, whether asymptomatic or with mild symptoms, should be observed. Lesions incidentally detected during surgeries need to be removed by conservative procedures.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Choristoma/diagnosis , Digestive System Diseases/pathology , Pancreas , Abdomen , Digestive System Diseases/surgery , Retrospective StudiesABSTRACT
We present two cases of juvenile form of paracoccidioidomycosis (PCM), a systemic mycosis frequently found in rural areas, whose prognosis is poor in children and young adults. They are a 14-year-old boy and a 25-year-old woman, both residents in an urban area in São Paulo - Brazil, without any history of travelling to an endemic area. They have been admitted to the hospital due to fever, weight loss and lymphadenopathy. The diagnosis was confirmed by serologic and histopathologic study. Patients have recovered after therapy with oral itraconazole and were discharged from hospital, maintaining outpatient visits. In this article, the authors discuss the unusual presentation of PCM in an urban area.
Subject(s)
Adolescent , Adult , Female , Humans , Male , Paracoccidioides/isolation & purification , Paracoccidioidomycosis/diagnosis , Antifungal Agents/therapeutic use , Itraconazole/therapeutic use , Paracoccidioidomycosis/drug therapy , Urban PopulationABSTRACT
Pancreatic heterotopia in the gall bladder is a rare entity, with approximately 31 cases reported in English language literature until 2006; there are no Brazilian studies on the topic. As it is usually asymptomatic, the definitive diagnosis is made by the anatomopathological material excised due to common diseases of the organ, such as lithiasis, inflammatory processes, polyps and tumors. A case is reported of a 40-year-old female patient submitted to surgery with a diagnosis of lithiasic cholecystopathy. Ectopy was evidenced by the histological study, due to the presence of acinar and ductal components of the pancreas and gall bladder corpus. The authors used their own pathological classification for this ectopy, and recommended that this entity be included more frequently in the differential diagnosis of alithiasic cholecystopathy and intramural and exophytic lesions of the gall bladder.
A heterotopia pancreática na vesícula biliar é entidade rara, com cerca de 31 casos relatados na literatura inglesa até 2006, inexistindo trabalhos nacionais a respeito. Quase sempre assintomática, seu diagnóstico definitivo é realizado pelo estudo anatomopatológico do material extirpado em virtude de doenças comuns do órgão, como a litíase, processos inflamatórios, pólipos e tumores. Relata-se o caso de uma paciente de 40 anos, operada com diagnóstico de colecistopatia litiásica. A ectopia foi evidenciada pelo estudo histológico, devido à presença dos componentes acinar e ductal do pâncreas no corpo vesicular. Os autores utilizaram a classificação patológica própria para essa ectopia e recomendam a inclusão mais frequente desta entidade no diagnóstico diferencial das colecistopatias alitiásicas e das lesões intramurais e exofíticas da vesícula biliar.