We retrospectively reviewed two fine-needle aspiration biopsy (FNAB) specimens from two patients with histologically confirmed epithelioid hemangioendothelioma (EH). Both patients were men, ages 79 and 39 years; their primary tumors arose in the soft tissues of the mediastinum and within the proximal tibia, respectively. The former patient had symptoms of superior vena cava syndrome; multicentric intraosseous lesions involved the proximal tibia of the latter patient. All cytologic smears were hypercellular and composed of mostly disassociated single cells and small aggregates of ovoid to polygonal-shaped epithelioid cells. Nuclei were variable, ranging from ovoid and reniform to round and polylobated and surrounded by an abundant amount of dense cytoplasm. Binucleated epithelioid neoplastic cells were frequent. Nuclear pleomorphism ranged from slight to moderate, and small solitary to multiple nucleoli were identified within the majority of tumor cells. Rare neoplastic cells with a single, sharply demarcated intracytoplasmic vacuole and intranuclear cytoplasmic pseudoinclusions were observed in the smears of one tumor. Metachromatic stromal fragments, probably representing hyalinized chondromyxoid stroma, were seen in the other tumor. Neither case was recognized initially on FNAB as EH. Immunohistochemically, sections from the surgical biopsy specimens of both cases showed diffuse and strong immunopositivity for the endothelial marker CD31. Although the cytomorphology of EH appears distinct, clinicoradiologic correlation is essential, and immunohistochemistry may be helpful to avoid misdiagnoses.
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http://dx.doi.org/10.1002/(sici)1097-0339(199807)19:1<38::aid-dc8>3.0.co;2-k | DOI Listing |
J 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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