INTRODUCTION: Epitheloid hemangioendothelioma (EHE) is a rare tumor with varied presentations depending on the organ of involvement. Spontaneous hemothorax is one of the presentations and we report a case of bilateral hemothorax due to EHE.
CASE PRESENTATION: A 53-year old female was referred for evaluation of an abnormal chest radiograph and progressive dyspnea fro 3–4 weeks.. She denied any cough, hemoptysis, weight loss, fevers or prior history of chest trauma. Her past medical history was significant for Hepatitis C, osteoarthritis and hypertension.She was obese, afebrile and tachypneic at rest. Other vital signs were: pulse 100/min regular, blood pressure 115/68 and oxygen saturation 90% on room air. A dull percussion note was present on the left hemithorax up to the mid zone with decreased breath sounds. The cardiac exam was normal with no jugular venous distension. There was no palpable lymphadenopathy and no pedal edema. Rest of her examination was unremarkable. A chest radiograph revealed a large left pleural effusion and a small right effusion.Her hematological profile showed a white cell count of 16,000 /cu.mm and a hemoglobin of 11 g/dl (Hct 32). Her coagulation profile and serum chemistry was normal. 1.2 liters of free flowing bloody exudative effusion was removed by left thoracentesis.. The fluid analysis revealed: Hematocrit 29, fluid protein 6.1 g/dl (serum 7.4 g/dl), fluid LDH 464 IU/ml (serum 244), pH 7.24, RBC 3,610,000/mm3, WBC 3900–63% neutrophils. The fluid did not clot and cytology was negative. A chest tube was placed. A CT chest done after the tap revealed left sided residual effusion, a right pleural effusion and multiple pleural based nodules and 8–9mm sized parenchymal nodules. (Fig1) A thoracotomy was done and biopsies of right middle lobe and pleura were obtained. (Fig2). The pathology from both lung and pleura revealed atypical epitheloid cell clusters with sinusoidal channels positive for CD31, CD34 and Factor VIII confirming a diagnosis of high grade hemangioendothelioma.
DISCUSSIONS: Epitheloid hemangioendothelioma (EHE) is a rare tumor of endothelial origin, which has been described primarily in the liver and in the lung. Pulmonary EHE has been described as “intravascular bronchioalveolar tumor”. It has a remarkable propensity to invade pulmonary vessels and small airways. Less than 50 cases have been described in the literature worldwide. EHE usually tends to follow an indolent course. Pulmonary EHE may present with nonspecific symptoms, such as chest pain, cough or sputum, or it may remain asymptomatic. It usually affects middle-aged patients, with a female predominance. Multifocal nodular densities are the most common feature on chest radiography. These are often mistaken for metastatic disease but they show little or no growth on serial imaging. An unfavorable prognosis is associated with extensive lymphangitic spread, pleural invasion and peripheral lymphadenopathy . Treatment can vary from observation in asymptomatic patients or surgery in patients with resectable disease. Chemotherapy in widely disseminated disease with carboplatin, etoposide and interferon IIA has been reported.
CONCLUSION: A high index of suspicion needs to be maintained for vascular tumors particularly in middle aged females with above suggestive symptoms and radiology. Also spontaneous hemothorax is a rare entity particularly without any anticoagulation. The differential includes bleeding disorders , vascular malformations , exostoses and rare tumors like EHE and angiosarcomas.
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