RESUMO
BACKGROUND AND PURPOSE: Doctor's delay in diagnosing Ewing sarcoma is often long. We examined the initial symptoms of Ewing sarcoma of the rib to find characteristics that could help to shorten the delay in diagnosis and hopefully influence the prognosis. PATIENTS AND METHODS: We examined the records from the first visit for symptoms related to Ewing sarcoma of the rib in 26 consecutive patients, all of whom were <30 years of age and had been diagnosed between 1981 and 2000. All patients had a minimum of 5 years follow-up. RESULTS: A tumor was suspected at the first visit in only 5 of the 26 patients; pleurisy was the most common misdiagnosis. 23 patients complained of pain, localized or pleuritic, and 11 had a palpable mass. 15 of the 26 patients had chest radiographs taken at the first visit, but a tumor was suspected in only 4 cases. The most common radiographic finding was pleural effusion--diagnosed in 10 of the 15 patients. 23 patients were operated. It is noteworthy that in 6 patients no tumor was suspected before surgery. 12 patients died from the Ewing sarcoma. Median survival was 2.4 years. Doctor's delay was median 3.0 (0-10) months, but it exceeded 7 months in 4 patients. Patient's delay was 2.5 (0-10) months. INTERPRETATION: The most important clinical finding in Ewing sarcoma of the rib was a palpable mass and pleural effusion. Both findings are rare in this age group and should be thoroughly investigated.
Assuntos
Neoplasias Ósseas/diagnóstico , Costelas , Sarcoma de Ewing/diagnóstico , Adolescente , Adulto , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/cirurgia , Criança , Erros de Diagnóstico , Feminino , Seguimentos , Humanos , Masculino , Derrame Pleural/diagnóstico , Pleurisia/diagnóstico , Prognóstico , Radiografia , Costelas/diagnóstico por imagem , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/mortalidade , Sarcoma de Ewing/cirurgia , Taxa de Sobrevida , Fatores de TempoRESUMO
A probable new type of osteopenic bone disease in two sisters and one female cousin is described. In infancy, the radiological findings were osteopenia, coxa vara, periosteal cloaking, bowing of the long bones, and flaring of the metaphyses. During growth, spinal pathology developed with compression of the vertebral bodies and scoliosis in one girl and kyphosis in another. All three children had genu valgum and two developed severe S-shaped bowing of the tibiae. Growth was stunted. Inheritance of this disorder is probably recessive. Type I and III collagen biosynthesis was normal. This condition is probably a hitherto undescribed form of osteogenesis imperfecta type III or a new bone disease.
