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Common Presentation, Uncommon Diagnosis - A Case of Pulmonary Epithelioid Hemangioendothelioma FREE TO VIEW

Tathagat Narula, MD; Amer Raza, MD; Jeffrey Robertson, BS; Shane Guillory, MD; Arthur Zieske, MD; Betty Lo-Blais, MD; Bennett deBoisblanc, MD
Chest. 2011;140(4_MeetingAbstracts):78A. doi:10.1378/chest.1119770
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INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is an uncommon neoplasm of vascular origin. Typically occurring in young women, it is considered a tumor of borderline malignant potential. We present a rare case of a young man with pulmonary malignant EHE. The tumor involved the mediastinum, endobronchial mucosa, pulmonary parenchyma and pleural surfaces. To our knowledge, this is the first case of pulmonary EHE with such diffuse pattern of involvement in a single patient.

CASE PRESENTATION: 31-year-old man presented with chest pain accompanied by intermittent episodes of hemoptysis for the previous 3 months. Inpatient evaluation at an outlying facility 2 months prior had revealed multifocal bilateral pulmonary nodular opacities on chest imaging. However, a definitive diagnosis had not been obtained. On current presentation, the patient reported significant weight loss, increased chest pain, and worsening hemoptysis. Social history was significant for heavy marijuana use as well as 10 pack-year history of cigarette smoking. Physical exam was significant for coarse breath sounds bilaterally. Initial laboratory data showed iron deficiency anemia. Chest x-ray showed worsening bilateral opacities when compared to previous studies. Chest CT scan was remarkable for bilateral airspace opacities along with mediastinal and hilar lymphadenopathy. Bronchoscopy revealed cobblestoning with extensive erythema of tracheal as well as bronchial mucosa. Microbiologic and histologic evaluation of endobronchial and transbronchial lung biopsy specimens was non-specific. An extensive work-up for infectious, non-infectious and rheumatological etiologies was unrevealing. Video assisted thoracoscopic (VATS) examination of the right lung revealed multiple hemorrhagic nodular masses both on the pulmonary and pleural surfaces that were biopsied. Histologic examination of the pulmonary wedge-biopsy specimen and mediastinal lymph nodes was consistent with malignant epithelioid hemangioendothelioma. The immunohistochemical evaluation was also consistent with the diagnosis and showed immunoreactivity for vascular markers CD31, CD34 and CD68. Patient had a progressively worsening hospital course with increased respiratory insufficiency and hemoptysis. Patient succumbed to his illness 3 weeks after his second hospitalization and before any therapy could be initiated.

DISCUSSION: Initially known as intravascular bronchioloalveolar tumor, pulmonary EHE is a rare vascular tumor with less than 100 cases described in literature. Primarily occurring in younger individuals, the median age of diagnosis is 36 years; females account for 80% of cases. Patients are often asymptomatic, or present with dyspnea, cough or pleuritic chest pain. Less commonly, alveolar hemorrhages and hemorrhagic pleural effusions may occur that portend a poor prognosis. Multiple, bilateral infiltrates are the most common radiological finding. Radiological features of many infectious or inflammatory lung diseases can mimic those of pulmonary EHE. Diagnosis therefore requires histological correlation and ancillary immunohistochemical studies. There is no clear standard of treatment. Surgical resection is an option in patients with a solitary lesion. Chemotherapy, radiotherapy and interferon have all been tried with variable and conflicting results. Recent discovery of vascular endothelial growth factor (VEGF) and VEGF receptor on EHE tumor cells raises the possibility of utilizing targeted therapies. Overall, the tumor has an unpredictable behavior. Spontaneous regression with long-term survival, even with metastatic disease, has been reported.

CONCLUSIONS: Pulmonary EHE is an extremely rare illness that can mimic many rather common pulmonary disorders. Awareness of this entity and a thorough work-up is the key to diagnosis.

Reference #1 Weissferdt A, Moran CA. Primary vascular tumors of the lungs: a review. Ann Diagn Pathol. 2010 Aug; 14(4): 296-308.

Reference #2 Bagan P et al. Prognostic factors and surgical indications of pulmonary epithelioid hemangioendothelioma: a review of the literature. Ann Thorac Surg. 2006 Dec; 82(6):2010-3.

Reference #3 Cronin P, Arenberg D. Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature. Chest. 2004 Feb; 125(2):789-93.

DISCLOSURE: The following authors have nothing to disclose: Tathagat Narula, Amer Raza, Jeffrey Robertson, Shane Guillory, Arthur Zieske, Betty Lo-Blais, Bennett deBoisblanc

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