Pulmonary Epithelioid Hemangioendothelioma: Two Case Reports of a Rare Neoplasm with an Unpredictable Prognosis
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Pulmonary Epithelioid Hemangioendothelioma: Two Case Reports of a Rare Neoplasm with an Unpredictable Prognosis T. P. F. Da Silva 1 & M. de Oliveira Taveira 1 & H. O. Guzman 1 & M. D. Guimaraes 1 Accepted: 23 September 2020 # Springer Nature Switzerland AG 2020
Abstract This is a comparative analysis of two patients diagnosed with pulmonary epithelioid hemangioendothelioma (PEH). The objective is to educate readers to PEH, a rare disease with a wide range of clinical manifestations that may be mistaken for lung adenocarcinoma or pulmonary metastases. This study is a retrospective analysis of electronic medical records and PACS diagnostic imaging files followed by literature review using PubMed. Case 1 is a male diagnosed incidentally with multiple lung nodules during a job admission exam. Case 2 is a female, symptomatic, found to have multiple lung nodules on investigation. Both patients had a CT-guided biopsy that established their diagnoses. Case 1 remains asymptomatic in his ninth year of follow-up, whereas case 2 died less than 9 months after development of symptoms despite treatment with radiotherapy, endobronchial prosthesis, and an oral multikinase inhibitor with anti-angiogenics properties. In this study, we aimed to illustrate the heterogeneity of PEH. Because of PEH’s rarity, its natural history and prognostic factors remain obscure. Medical literature is primarily composed of case studies with inconsistent methodology and diagnostic criteria. Keywords Epithelioid hemangioendothelioma . Vascular neoplasms . Lung neoplasms . Hemangioendothelioma . Vascular tissue neoplasms
Introduction Epithelioid hemangioendothelioma (EHE) is a type of primary vascular sarcoma of the small vessel neoplasm subgroup [1]. A primarily histopathological diagnosis, EHEs are mainly composed of epithelioid and endothelial cells associated with other less prominent vascular components. On a pathology slide, EHE has intermediate degrees of differentiation, which has led to it being described as indolent form of angiosarcoma, high degree neoplasia, or aggressive variety of hemangioma. It can arise in virtually any vascularized organ, with the liver
being the most frequent site involved (63%) followed by lungs (10%), bone (8%), and skin (6%) [1]. The disease that we currently recognize as EHE was first described in the lungs as an atypical presentation of lung carcinoma by Dail and Liebow in 1975 [2]. They coined the term “intravascular bronchioloalveolar carcinoma” to describe a special variety of lung carcinoma with extensive local vasculature invasion. Later, technological advances in pathology, especially immunohistochemistry (IHC), uncovered the mesenchymal origin of this unusual subset of lung tumors. There were only 248 cases of EHE reported in the literature up to 2014, and the true prevalence of EHE in the community is unknown [3].
This article is part of the Topical Collection on Imaging T. P. F. Da Silva and M. de O. Taveira are co-first authors Electronic supplementary material The online version of this arti
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