Objectives: Epithelioid hemangioendothelioma (EHE) is a vascular tumor of intermediate malignant potential, which presents as infiltrative lesions involving multiple organs. We reviewed our institutional experience with the cytologic diagnosis of this neoplasm including the performance of rapid on-site evaluation (ROSE).
Material And Methods: From our institutional database, we identified 29 cytology specimens, obtained between 2012 and 2020, from 21 patients with biopsy confirmation of EHE. ROSE and final diagnosis were compared. All cytology slides were reviewed, and selected cytologic features were recorded.
Results: The cohort included 29 specimens comprising 17 (59%) from liver, 6 (21%) from lung, 2 (7%) from lymph node, and 4 (14%) from other sites. At ROSE, 8/27 (30%) were reported inadequate, yet on review, all cases contained scattered cells typical of EHE in the touch imprint air-dried slides including two cases reported with a final diagnosis of non-diagnostic. All cases contained epithelioid and plasmacytoid cells with ovoid nuclei, fine chromatin, delicate (or biphasic) cytoplasm, and scattered cells with delicate, elongated cytoplasmic tails. The majority 26/29 (90%) of cases had multi-nucleated and multi-lobated nuclei. Intracytoplasmic lumens/blister cells were in 17/29 (59%), and a subset had erythrocytes therein (4/29, 14%). Metachromatic fibromyxoid or fibrotic stroma fragments were commonly seen (23/29, 79%). Mitoses and necrosis were absent in all cases. Of 11 tested cases, and fusions were detected in nine and two cases, respectively.
Conclusion: EHE has distinctive cytologic features which are often under-recognized during ROSE.
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http://dx.doi.org/10.25259/Cytojournal_57_2022 | DOI Listing |
Cureus
January 2025
Oral and Maxillofacial Surgery, Queen Elizabeth Hospital Birmingham, Birmingham, GBR.
Epithelioid haemangioendothelioma (EHE) is a rare vascular neoplasm characterised by proliferation of vascular endothelial and pre-endothelial cells. The prevalence is less than one in a million people. It is principally observed in the soft tissues of the extremities but can also occur in the bone, brain, liver, lung and lymph nodes.
View Article and Find Full Text PDFJ Cardiothorac Surg
December 2024
Thoracic and Vascular Surgery Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Background: Epithelioid hemangioendothelioma (EHE) is an uncommon vascular malignancy characterized by an unpredictable clinical course and a high potential for recurrence and metastasis. The lack of standard treatment guidelines, coupled with the tumor's inconsistent response to available treatments, complicates the management of EHE and leads to widely varying patient prognoses.
Case Presentation: We report two cases of EHE with distinct presentations reflecting the site of involvement.
J Int Med Res
December 2024
Department of Hepatobiliary Surgery, Affiliated Hangzhou First People's Hospital, Westlake University, School of Medicine, Hangzhou, China.
Epithelioid hemangioendothelioma is a low-grade malignant tumor of vascular origin. The rarity of hepatic epithelioid hemangioendothelioma (HEHE) makes the diagnosis and treatment of this entity challenging. We report a case of a 69-year-old female patient who suffered from HEHE and complained of abdominal distension pain with dizziness and appetite loss for more than half a month.
View Article and Find Full Text PDFNeurology
January 2025
Department of Neurology, National Center for Neurological Disorders, Huashan Rare Disease Center, Huashan Hospital, Affiliated to Fudan University, Shanghai, China.
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