Rare Pulmonary Neoplasm: A Case Report on Epithelioid Hemangioendothelioma

Document Type

Conference Proceeding

Publication Date

10-2025

Publication Title

Chest

Abstract

INTRODUCTION: Epithelioid Hemangioendothelioma (EHE) is a rare vascular neoplasm arising from vascular endothelial cells and their precursors [1]. It represents 1% of all vascular tumors, and primary involvement of lungs is a rarer occurrence with involvement only in 12% of patients. It is mostly incidentally diagnosed because of its slow growing and indolent nature. CASE PRESENTATION: A 50-year-old female presented to pulmonology office for evaluation of a lung mass incidentally diagnosed while being worked up for organ donation. She had no active pulmonary symptoms, and physical examination was completely unremarkable. Computed Tomography (CT) of the Chest, Abdomen and Pelvis demonstrated 2.9 x 2 cm lobulated soft tissue nodule containing eccentric coarse calcification in right lung base (Figure 1). Her prior chest radiograph from 15 years before presentation also showed mass in similar area which was 1.2 cm in size (Figure 2). A fluorodeoxyglucose positron emission tomography (FDG PET) was done, which demonstrated mildly increased uptake in the lesion with maximum standardized uptake value of 2.7, but did not show uptake anywhere else (Figure 3).CT guided biopsy of the nodule was done, which demonstrated findings consistent with EHE with immunohistochemistry showing CAMTA 1 and ERG positivity. Video-assisted thoracoscopic wedge resection of right lower lobe was done, histopathology was consistent with EHE with lympho-vascular invasion and 0.1 cm margin free of tumor. Follow up imaging have not shown new nodules or lymph node enlargement at 12 months. DISCUSSION: Due to the rarity of the neoplasm, there is no standardized treatment protocol for EHE, although wedge resection of tumor with free margins is sought for localized lesions [1] and has a promising outcome. For unresectable, pleural or metastatic tumor, combination of Carboplatin and Etoposide is a potential systemic therapy and has been reported to have led to complete remission [2]. As the origin is from endothelial and pre-endothelial cells, anti-vascular endothelial growth factor antibodies like Bevacizumab could have a potential role in metastatic EHE, especially when combined with chemotherapy [3]. There is a potential association between Bartonella infection and EHE, but it is not clear if treatment of the infection has a role in management [4]. Personalized medicine with targeted therapies towards WWTR1-CAMTA1 fusion and TAP1 rearrangements could be useful in management as the role of these genetic abnormalities is established [5].Prognostic factors for having poorer prognosis include location of tumor with pulmonary involvement, male sex, presence of symptoms, pleural involvement, metastasis and specific histologic findings like spindle tumor cells and fibrinous pleuritic lesions [1]. CONCLUSIONS: EHE is a rare vascular neoplasm which is mostly incidentally diagnosed. Wedge resection is an established management strategy for pulmonary EHE with anatomically limited lesion, but the guidelines regarding follow up is not established. While a handful of management strategies are reported, more studies are needed for establishing guidelines for management. Due to the rarity of EHE, establishing and maintaining cancer registries is necessary to understand the neoplasm and formulate evidence-based management strategies.

Volume

168

Issue

4S

First Page

5107A

Comments

American College of Chest Physicians CHEST Annual Meeting, October 19-22, 2025, Chicago, IL

Last Page

5108A

DOI

10.1016/j.chest.2025.07.2865

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