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Lung Cancer |

Pulmonary Epithelioid Hemangioendothelioma: One Cause of Nodular Parenchymal Lung Disease FREE TO VIEW

Shireen Mirza, MD; Aaron Glucksman, MD; John McArdle, MD
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University of Connecticut School of Medicine, Farmington, CT


Chest. 2015;148(4_MeetingAbstracts):572A. doi:10.1378/chest.2280618
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Abstract

SESSION TITLE: Lung Cancer Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary Epithelioid Hemangioendothelioma (PEH) is a rare low-grade angiosarcoma that may be discovered incidentally on imaging or present with signs and symptoms related to tumor burden, location and constitutional complaints. On imaging, PEH typically presents with bilateral multiple nodular opacities of varying sizes and distribution, and should be considered in the differential diagnosis of nodular lung disease.

CASE PRESENTATION: A previously healthy 34 year-old Peruvian male presented with 3 months of non-productive cough, increasing dyspnea, left-sided chest discomfort, and 12 pounds of weight loss over a month. He denied known sick contacts, illicit drug use, and significant tobacco or occupational exposures. Family history was significant for gastric, ovarian, and prostate cancer. Preliminary testing revealed a left lower lobe airspace opacity. Chest CT showed a 6.2 x 4.4 x 7.1 cm left lower lobe mass, bilateral non-calcified pulmonary nodules, a small left pleural effusion, and no lymphadenopathy (Fig 1). Infectious disease work-up was negative. Histopathologic evaluation of CT-guided left lung biopsy confirmed a diagnosis of PEH and staging PET/CT demonstrated FDG avidity of left lower lobe mass and two mediastinal lymph nodes.

DISCUSSION: First described in 1975, the etiology of PEH remains elusive but chronic infection with Bartonella species is being investigated as a cause. Existing literature is mostly in the form of case reports or case series, and a female and non-smoker preponderance exists. The prognosis, disease course and life expectancy is unpredictable. On initial presentation and radiologic evaluation, misdiagnosis as primary or metastatic lung infection or malignancy occurs frequently and obtaining tissue for definitive diagnosis is paramount given the broad differential diagnosis. Pathologic diagnostic criteria are not clear-cut but PEH is distinguished by vascular endothelial cell marker positivity, and tumor cells suggestive of vascular origin (Fig 2). There is no established standard of care for treatment given rarity of the disease. Both surgical resection and chemotherapy have both been utilized with lack of long term follow-up data. Recent data support use of vascular endothelial growth factor (VEGF) antagonists. Our patient tolerated a chemotherapy regimen of bevacizumab, paclitaxel, and gemcitabine well with radiologic and symptomatic improvement (Fig 1).

CONCLUSIONS: PEH is likely an underdiagnosed disease with unclear etiology and prognosis. Further investigation is required to elucidate a causative pathway and investigate therapeutic options for patients with epithelioid hemangioendotheliomas.

Reference #1: Shao et al. Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature. Oncol Lett. 2014

Reference #2: Bagan et al. Prognostic factors and surgical indications of pulmonary epithelioid hemangioendothelioma: A review of the literature. Ann Thorac Surg. 2006

DISCLOSURE: The following authors have nothing to disclose: Shireen Mirza, Aaron Glucksman, John McArdle

No Product/Research Disclosure Information


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