Lung Cancer: Student/Resident Case Report Poster - Lung Cancer I |

A Rare Case of Lung Entrapment Secondary to Pulmonary Epithelioid Hemangioendothelioma FREE TO VIEW

Christina Kwan, MD; Laura Chia, MD; Steven Wong, MD
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Scripps Mercy Hospital, San Diego, CA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):754A. doi:10.1016/j.chest.2016.08.849
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SESSION TITLE: Student/Resident Case Report Poster - Lung Cancer I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is a rare low-intermediate grade vascular tumor with an estimated prevalence of less than one in 1 million. It can arise from multiple organ systems, but only 19% of cases are isolated to the lungs. We present a case of pulmonary epithelioid hemangioendothelioma (PEH) manifesting with rapid recurrent pleural effusions and lung entrapment, which has not previously been reported in literature.

CASE PRESENTATION: A healthy 34 year-old woman presented with progressive dyspnea and right shoulder and back pain for 3 weeks. A moderate right pleural effusion was noted on chest radiography and a solitary pulmonary nodule in the right upper lobe was observed on computed tomography (CT). Initially, pleural fluid analysis was thought to be non-diagnostic. Subsequent CT-guided needle biopsy of the nodule resulted in a diagnosis of PEH; this was verified on re-analysis of the pleural fluid. In the following month, two additional pleural drainage procedures were performed for recurrent symptomatic effusions, with significant lung entrapment noted after the third pleural drainage. She underwent VATS decortication and pleurodesis with partial success due to noted extensive pleural involvement. She is currently receiving chemotherapy with carboplatin, paclitaxel, and bevacizumab with treatment response.

DISCUSSION: PEH is a rare lung tumor of vascular origin. It more commonly affects women with an average age at diagnosis of 40 years. Symptoms at presentation are often minimal, making diagnosis challenging. Imaging most commonly reveals multiple bilateral pulmonary nodules, less often as a unilateral solitary nodule, and only rarely as pleural effusions. In one study, only 11% of PEH cases presented with pleural effusions, while 20.4% had distant metastases to pleura. Clinical course varies with survival ranging from 5 months to 24 years with an average 5-year survival of 60% (47-71%) and spontaneous regression reported in 3 cases. Increased mortality is noted in those with pulmonary symptoms and hemorrhagic pleural effusions. At this time, there is no standard treatment. Due to its borderline malignancy potential, watchful waiting, surgical resection for localized tumor activity, and chemotherapy for more widespread disease have all been used with variable results.

CONCLUSIONS: While EHE is considered a low-intermediate grade malignancy, it can behave aggressively as evidenced by our case of rapidly recurrent malignant effusion and lung entrapment from extensive pleural involvement.

Reference #1: Bagan P, Mohammed H, et al. Prognostic Factors and Surgical Indications of Pulmonary Epithelioid Hemangioendothelioma: A Review of the Literature. Ann Thorac Surg. 2006;82: 2010-2013.

Reference #2: Amin R, Kenzo H, et al. Risk factors and independent predictors of survival in patients with pulmonary epithelioid hemangioendothelioma. Review of the literature and a case report. Respirology. 2006;11:818-825.

DISCLOSURE: The following authors have nothing to disclose: Christina Kwan, Laura Chia, Steven Wong

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