Bronchiolitis obliterans organising pneumonia: a clinical and radiological review.

BACKGROUND: The clinical presentation, course, and radiological spectrum of bronchiolitis obliterans organising pneumonia (BOOP) is still being characterised to aid differentiation from other causes of organising pneumonia. AIMS: To define the clinical presentation, response to therapy, and radiological spectrum of BOOP. METHODS: Fifteen cases of BOOP were retrospectively reviewed. The clinical presenting features, treatment and outcome of each patient were determined. Three independent readers and chest X-rays (CXRs) were blinded. CXRs were scored by a semi-quantitative method. Modal scores were calculated for type and profusion of opacification of each CXR. RESULTS: The mean age of presentation was 64 years and the median duration of follow-up was 12.5 months. Thirteen patients received corticosteroid therapy. Outcome was varied. One patient had progressive loss of lung function, five had persisting symptoms with stable abnormal lung function, and nine were asymptomatic with near normal lung function. Five patients had a disease relapse. Symptoms length prior to presentation, duration and intensity of treatment were not associated with outcome (p = 0.23-0.9). Radiological opacities were alveolar in 73%, large localised infiltrates in 13%, nodular in 20% and mobile in 33% of CXR series. There was no relationship between overall profusion, type of CXR opacities and patient outcome, treatment duration or treatment intensity (p = 0.42-1.0). CONCLUSIONS: The clinical spectrum of BOOP includes mild subacute, chronic progressive, and acute life threatening illness. Prognosis and response to treatment is variable. The diversity of radiological findings and clinical presentations should prompt consideration of the diagnosis in patients with undiagnosed respiratory tract symptoms and persisting or varying radiological abnormalities.

Cerebral metastasis of an atrial myxoma mimicking an epithelioid hemangioendothelioma.

A 60-year-old woman presenting with a 15-month history of Jacksonian seizures was found on computed tomography (CT) scan to have an enhancing subcortical lesion high in the left anterior parietal lobe. The excised tumor had light microscopic features similar to those of an epithelioid hemangioendothelioma, and immunohistochemical stains confirmed its vascular nature. At surgery, the patient had no clinical or radiological (including CT body scan) evidence of any other lesion. However, 22 months after symptoms first appeared, she presented with dyspnea and bilateral pleural effusions, and a left atrial myxoma was detected echocardiographically. The histologic features of the resected myxoma were identical to those of the previously excised cerebral lesion, and it became apparent that the intracranial tumor was a metastasis of the atrial myxoma. This case illustrates that before a diagnosis of visceral epithelioid hemangioendothelioma is rendered, the possibility of metastatic occult cardiac myxoma should be considered. Echocardiography is warranted for such patients.