Epithelioid Hemangioendothelioma Presenting With Recurrent Pleural Effusion: When the Pleura is on Fire

Document Type

Conference Proceeding

Publication Date

5-2024

Publication Title

American Journal of Respiratory and Critical Care Medicine

Abstract

Introduction Epithelioid hemangioendothelioma (EHE) is an extremely rare sarcoma of vascular origin. EHE has an incidence of less than 0.1 per 100,000 patients per year. It can involve multiple organs; pleural involvement is relatively rare and under-reported in the medical literature. We present a unique case of pleural EHE presenting with recurrent pleural effusion, severe pleuritis, scarring, and trapped lung syndrome. Case presentation A 54-year-old Caucasian female was referred to the pulmonary clinic fort wo months of progressive dyspnea, fatigue, and right pleuritic chest pain. Initial chest x-ray showed a large right pleural effusion. Thoracentesis identified exudative lymphocyte predominant pleural fluid with normal pH, high protein, high lactate dehydrogenase, normal glucose, negative infectious workup, and fluid cytology negative for malignancy. Chest computed tomography scan showed pleural calcifications, nodular thickening of the pleura, and a 1.8 cm right paraoesophageal lymph node. She underwent esophagogastroduodenoscopy (EGD) with endoscopic ultrasound-guided lymph node biopsy that was unrevealing. In two months, the patient underwent five thoracenteses with negative cytology. To address the recurrent pleural effusions, she underwent video-assisted thoracoscopic surgery, which showed chronically inflamed right parietal pleura with pleural thickening and fibrosis (Figure). A thick fibrous scar partially entrapped the right lower lobe. Pleural biopsies revealed calmodulin binding transcription activator 1 (CAMTA-1) positive epithelioid cells with prominent intracytoplasmic vacuoles resembling signet ring cells, confirming the diagnosis of EHE. Discussion The pleura is an infrequent primary site of EHE, with roughly 10% of cases having primary pleural involvement. Its delayed presentation, difficulty establishing a tissue diagnosis, and aggressive nature make pleural EHE associated with the poorest prognosis compared to EHE involving other sites. In addition to recurrent pleural effusions, it can present with pleural thickening, hemoptysis if lung involvement exists, or lung entrapment from severe pleural inflammation and scarring, as illustrated in our patient. Surgical resection of pleural EHE is often not feasible due to metastasis at presentation and tumor friability. Symptomatic management with pleurodesis versus indwelling pleural catheter therapy is often warranted to prevent recurrent pleural effusion. There is a lack of consensus on system therapy for metastatic disease. However, agents like bevacizumab, anthracyclines, or ifosfamide are commonly used. Molecular medicine targeting the CAMTA-1 downstream signaling pathway is currently under investigation.

Volume

209

Issue

Suppl

First Page

A7943

Comments

International Conference of the American Thoracic Society, May 17-22, 2024, San Diego, CA

DOI

10.1164/ajrccm-conference.2024.209.1_MeetingAbstracts.A7493

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