Lung Pathology: Chest Tumors |

Epithelioid Hemangioendothelioma of the Pericardium Presenting With Hemoptysis and Dyspnea: A Case Report and Literature Review FREE TO VIEW

Shaoqiang Li, MA; Chunli Liu, PhD; Cheng Hong, PhD; Nuofu Zhang, PhD
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Guangzhou Institute of Respiratory, Guangzhou, China

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

Chest. 2016;149(4_S):A341. doi:10.1016/j.chest.2016.02.355
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SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is a rare malignant cancer of vascular origin that can mainly affect multiple tissue sites in one or multiple organs. It is hard for us to judge where are the primary lesion. A subtype of EHE, pulmonary epithelioid hemangioendothelioma (PHE), is more unusual with about 210 reported cases. Of these, no cases have been classified as heart or pericardium in origin. Here we add to this knowledge-base with a report of an unusual case of pericardium EHE and tumor metastasis to the left lower lobe with the hemoptysis and dyspnea, pericardial effusion. He was repeatedly treated for fungal infection and tuberculosis with symptoms aggravated gradually. A high index of suspicion is needed to reduce the risk of missing or delaying the diagnosis.

CASE PRESENTATION: A 69-year -old Chinese-born man was admitted to our hospital in May 2015. Due to the progressive symptoms of palpitate, tachypnea and dyspnea on exertion 6 months and a hemoptysis 2 months ago. With the pericardial effusion, any lesion couldn't be found in his lungs on serial chest radiographs or computed tomography (CTs) in October 2014. Before admission, he was diagnosed as “The pericardium infection”, “The tuberculosis” one after another in the local hospital and with a serial treatment while his symptoms still persist. He had began to be hemoptysis in March 2015. At the same time, bilateral multiple nodular opacities in left lower lobes have been found on CT at local hospital. After a series of the treatment, the symptoms of dyspnea and hemoptysis aggravated gradually. In our hospital, To confirm our dignosis, (18F-FDG-PET)/CT revealed intense fluorodeoxy glucose uptake around the pericadium and around the lesion in left lower lobe.the patients had underwent the wedge resection of the left lower lobe with the video-assisted thoracoscopic surgery. In pathology, characteristic clusters of atypical epithelioid endothelial cells in a myxoid background. Tumor cells show strong positive staining in immunohistochemical studies with both CD31 and CD34

DISCUSSION: In this case, at the beginning of the disease, with pericardial effusion, any lesion could not be found in lungs, Local hospital inferred that the primary lesion was pericardium. As time goes on, the lesion had been found in left lower lobes of lung in CT. At the same time, patient had started to suffered the hemoptysis, tachypnea on exertion. But at local hosptal, Doctor still focus on the heart disease and complete the relativity examination without the further examination of pulmonary so that the paitent had missed the best dignosis chance.

CONCLUSIONS: Whilst EHE is an extremely rare malignant cancer, and its pericardium subtype is even rarer, it is important for us to continue to raise our vigilance to the diagnosis of the EHE.

Reference #1: Cazzuffi R, Calia N, Ravenna F, Primary Pulmonary Epithelioid Hemangioendothelioma: A Rare Cause of PET-Negative Pulmonary Nodules. Case Reports in Medicine. 2011;2011:262674.

DISCLOSURE: The following authors have nothing to disclose: Shaoqiang LI, Chunli Liu, Cheng Hong, Nuofu Zhang

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