ArticleThe American journal of surgical pathology2015
Thoracic epithelioid malignant vascular tumors: a clinicopathologic study of 52 cases with emphasis on pathologic grading and molecular studies of WWTR1-CAMTA1 fusions.
Article in The American journal of surgical pathology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
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Who cites it
41 citing papers in PubMed, 138 citations in OpenAlex.
- Pathological complete remission of pulmonary epithelioid hemangioendothelioma: a case report and literature review.Journal of cardiothoracic surgery · 2026Review
- Advances in the Basic Sciences in Thoracic Oncology in the Last 20 Years and Their Translational Impact.Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer · 2026Review
- Auricular epithelioid hemangioendothelioma: Two cases first diagnosed as auricular pseudocyst.Oncology letters · 2025Article
- Epithelioid hemangioendothelioma prognosis and outcome: report of two challenging cases.Journal of cardiothoracic surgery · 2024Article
- Proposed diagnostic and prognostic markers of primary malignant hepatic vascular neoplasms.Diagnostic pathology · 2024Article
- A case of primary pleural epithelioid hemangioendothelioma achieving stable disease with paclitaxel treatment: A case report and literature review.Respirology case reports · 2024Article
- Imaging features and deep learning for prediction of pulmonary epithelioid hemangioendothelioma in CT images.Journal of thoracic disease · 2024Article
- Atypical epithelioid cells in pleural effusion as foreign second population: A diagnostic cytopathology dilemma.CytoJournal · 2024Article
- Article
- Mediastinal Epithelioid Angiosarcoma, New Insights into an Uncommon Diagnosis: A Case Report and Literature Review.Hawai'i journal of health & social welfare · 2023Review
- NOTCH3 missense mutations as predictor of long-term response to gemcitabine in a patient with epithelioid hemangioendothelioma.Journal of cancer research and clinical oncology · 2023Review
- Epithelioid haemangioendothelioma-a rare cause of right pleural effusion and multiple primary nodules: Case report & review of the literature.JRSM open · 2023Article
- The Utility of Fine Needle Aspiration (FNA) Biopsy in the Diagnosis of Mediastinal Lesions.Diagnostics (Basel, Switzerland) · 2023Review
- Genetic Alterations and Deregulation of Hippo Pathway as a Pathogenetic Mechanism in Bone and Soft Tissue Sarcoma.Cancers · 2022Review
- Subcutaneous axillary primary epithelioid hemangioendothelioma: report of a rare case.Surgical case reports · 2022Article
- Primary malignant epithelioid hemangioendothelioma of the pleura: A review and report of a novel case.Clinical case reports · 2022Article
- Primary pulmonary epithelioid angiosarcoma with thyroid tumor history: A case report and literature review.Experimental and therapeutic medicine · 2022Article
- Epithelioid Hemangioendothelioma withCase reports in pathology · 2022Article
- A rare case of primary angiosarcoma of the anterior mediastinum.General thoracic and cardiovascular surgery · 2021Article
- Sarcomas of the mediastinum with epithelioid morphology.Mediastinum (Hong Kong, China) · 2021Review
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Authors and funding
6 authors at 1 institution in 1 country.
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Abstract
Malignant thoracic epithelioid vascular tumors are an uncommon and heterogenous group of tumors that include low-grade to intermediate-grade epithelioid hemangioendothelioma (EHE) and high-grade epithelioid angiosarcoma (EAS). We examine the morphologic and immunohistochemical features of 52 malignant epithelioid vascular tumors (10 low-grade EHE, 29 intermediate-grade EHE, and 13 EAS) involving the thorax (lung, pleura, mediastinum, heart, great vessels) including cases with exclusively thoracic disease (35) and with multiorgan disease including the thorax (17). Intermediate-grade EHE differs from low-grade EHE by the presence of necrosis, increased mitotic activity, and increased atypia. Morphologic features such as intranuclear inclusions, intracytoplasmic vacuoles, and stromal changes (chondroid, myxoid, or hyalinized stroma) are seen more frequently in EHE, whereas blood lakes, proliferation of slit-like vessels, and prominent nucleoli favor EAS. Fluorescence in situ hybridization analysis showed CAMTA1-WWTR1 fusions in 4/7 low-grade and 23/23 intermediate-grade EHE (P<0.001). In EAS, CAMTA1 rearrangement was negative in all cases, whereas a WWTR1 complex abnormality was found in 1/5 cases (P<0.001). This offers an objective means of differentiating intermediate-grade EHE from EAS, especially on limited biopsies. All cases show expression of at least 1 vascular marker, which allows differentiation from primary thoracic epithelial malignancies, although keratin expression is a potential pitfall with 29% of EHE and 25% of EAS showing keratin expression. Survival analysis shows that higher tumor grade for all tumors (P=0.026) as well as lung and pleural tumors only (P=0.010) and the presence of pleural involvement in lung and/or pleural tumors (P=0.042) correlate with poor prognosis.
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