INTRODUCTION: Pulmonary epithelioid hemangioendothelioma (EHE) is a rare and malignant vascular neoplasm with varied presentation. We describe a case of EHE that was discovered following a traumatic accident.
CASE PRESENTATION: A 26 yr old nonsmoking, African-American male, without significant past medical history, presented to the emergency department at a large inner-city teaching hospital following of a fall from a third story apartment window. There was no obvious serious injury or loss of consciousness. His physical examination was relatively unremarkable except for contusions along the left hip and pelvis. He did not complain of shortness of breath, cough, or chest pain. There was no prior history of chronic respiratory symptoms or of childhood disease. Multiple radiographs were performed as part of his trauma evaluation. His chest x- ray was abnormal showing bilateral lung nodules and there were no comparison images. Computerized tomography of the chest confirmed innumerable, sub centimeter, non calcified, bilateral lung nodules, of varying size, with no mediastinal or hilar lymphadenopathy. The liver and other abdominal and pelvic organs appeared normal. A fiberoptic bronchoscopy with transbronchial biopsy was diagnostic with pathology revealing vacuolated spindle shaped cells in a myxoid parenchyma and recent hemorrhage, consistent with the diagnosis of EHE. Further examination with PET/CT scanning again revealed the lesions, but there was no evidence of increased metabolic activity within the nodules, contrary to other published reports.
DISCUSSIONS: Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor of borderline or low-grade malignancy mainly affecting the liver and more rarely the lung. EHE typically occurs among young patients with a mean age of onset of 35 years. It is four times more common in women than in men. It is mostly an incidental finding as the majority of patients are asymptomatic. If present symptoms typically include cough, dyspnea or chest pain. The characteristic features of EHE on radiographs or CT is the presence of multiple perivascular nodules ranging in size up to 2cm but usually <1cm. There have been a few case reports showing positive PET scans in EHE lesions but our patient did not show any increased FDG uptake. Atypical radiologic appearance of EHE includes ground glass opacities and interlobular septal thickening. Considering the rarity of this disease there is no clear standard for treatment. Patients with symptoms of vascular endothelial cell proliferation have a significantly worsened survival. Case reports of partial spontaneous regression in three patients have been described. Chemotherapy regimen of carboplatin and etoposide or interferon have been tried. Lung transplantation is considered in patients with aggressive vascular manifestations of hemorrhagic pleural effusion and anemia. The prognosis of EHE is unpredictable with life expectancies ranging from 1 to 15 years.
CONCLUSION: EHE is a very rare tumor and is mostly an incidental finding in young patients. There is no single effective treatment in cases of bilateral pulmonary disease. PET scanning is not a reliable predictor of the extent of disease.
DISCLOSURE: Fatima Ajaz, No Financial Disclosure Information; No Product/Research Disclosure Information