Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary epithelioid haemangioendothelioma (EHE) of the pleura is a rare vascular tumour that occurs mainly in men. Pleural effusion and thickening are the most common clinical presentations. A 58 year old female, nonsmoking patient presented to us with dry cough, dyspnoea and left chest pain for several weeks (no asbestos exposure). Standard chest X-ray and contrast enhanced multislice computed tomography revealed a large-size lobulated mass originating from the pleura which was diagnosed as primary pleural haemangioendothelioma (PHE) by histology and immunohistochemistry (reactivity for vimentin, CD31, CD34, Factor VIII and ulex europeaus). No metastases were detected. The patient refused treatment and died three months later due to the onset of acute and progressive respiratory failure. Despite the lack of high-grade malignancy, primary PHE displays a poor prognosis while curative therapies are actually not available. To our knowledge, this is the first case of primary PHE in a female patient occurring in Italy and the third one to have been reported in English literature. Difficulties in diagnosis and treatment management are discussed below.
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PMID:Primary pleural haemangioendothelioma in an Italian female patient: a case report and review of the literature. 2121 44

In this report we describe a case of pulmonary epithelioid hemangioendothelioma (PEH) in a young woman. The neoplasm manifested with dry cough, chest pain, finger clubbing, and multiple bilateral pulmonary nodules on chest x-ray and computed tomographic (CT) scan. She underwent thoracoscopy, and the histological features of the lung biopsies were initially interpreted as consistent with a not-well-defined interstitial lung disease. Our patient was clinically and radiologically stable over a period of four years, after which the disease progressed to involve not only the lung but also mediastinal lymph nodes, liver and bone. Fiberoptic bronchoscopy showed subtotal occlusion of the right middle and lower lobe bronchi. The histologic examination of bronchial biopsies revealed a poorly differentiated neoplasm immunohistochemically positive for vimentin and vascular markers CD31, CD34 and Factor VIII. A diagnosis of malignant hemangioendothelioma was made. Positron emission tomography (PET) is more sensitive than CT scan and bone scintigraphy in detecting PEH metastases. Furthermore, 18-fluorodeoxyglucose (FDG) uptake seems to be related to the grade of malignancy of PEH lesions. Therefore, we suggest that FDG-PET should be included in the staging system and follow-up of PEH.
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PMID:Role of FDG-PET scan in staging of pulmonary epithelioid hemangioendothelioma. 2835 86