Metastatic cutaneous epithelioid hemangioendothelioma involving right atrium - a case report (original) (raw)
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An unusual case of aggressive malignant spread of epithelioid hemangioendothelioma
Rare Tumors, 2021
Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm which typically originates from liver, lung, or bone. Due to the low incidence of disease, the most effective treatment is not easily studied and much of the information known about EHE has been learned through case reports and case series. In this case, we will present an uncommon form of primary soft tissue EHE with local recurrence, bone metastasis, and lymphangitic spread to the lungs leading to respiratory failure. Imaging of the chest was atypical for EHE with intraseptal thickening and hilar lymphadenopathy. Respiratory failure was progressive despite aggressive multimodal treatment. This case highlights an unusually aggressive recurrence and metastasis of primary soft tissue EHE with atypical pulmonary imaging findings.
Cardiac epithelioid hemangioendothelioma with 8-year follow-up
Cardiovascular Pathology, 2007
An incidentally discovered right atrial mass excised from the heart of a 51-year-old woman was found on pathological examination to be an epithelioid hemangioendothelioma. No further therapy was undertaken, and the patient has remained free of recurrence for 8 years. To our knowledge, this is the first report on the behavior of this distinctive vascular neoplasm occurring as an isolated cardiac tumor.
Cutaneous Epitheloid Hemangioendothelioma: A Rare Tumor
International Journal of Advanced Research, 2020
Epithelioid hemangioendothelioma is a rare, slowly unfavorable vascular tumor. It is considered to be a malignant tumor. Localization in the skin is very rare and often part of a multi-systemic disorder. It has the potential for local recurrence, lymph node metastasis and at a distance and it's not frequently accompanied by death. We report the observation of a 57-years-old patient, who is presented with an epithelioid hemangioendothelioma of the left buttock with a 1-year follow-up without recurrence.
Aggressive multifocal form of epithelioid hemangioendothelioma--case report
Collegium antropologicum, 2011
Epithelioid hemangioendothelioma (EHE) is a rare tumor of the vascular origin. It was first described in its pulmonary form by Dail and Leibow in 1975 and named "intravascular bronchioalveolar tumor" (IVBAT). Since then, reports of occurrences of the tumor have been made for number of locations, but most often tumor can be found in soft tissues, liver, lungs, bone and skin. It is considered to be a low or borderline malignant tumor with, usually, slow progression, but aggressive forms have been described. We here report a case of a 46-year old female patient with multifocal malignant tumor spreading to lungs, liver, spleen and with synchronous involvement of lumbal vertebrae, illiac bones and central nervous system dissemination. To the best of the authors knowledge, no case of malignant EHE with multiorgan involvement of this proportions and synchronous central nervous system and bone involvement in one patient has been reported to this date in English-speaking literature.
Epithelioid hemangioendothelioma – A rare pulmonary tumor
Revista Portuguesa de Pneumologia (English Edition), 2008
Caso Clínico Clinical Case Resumo Os autores descrevem um caso de uma neoplasia pulmonar rara-hemangioendotelioma epitelióide-numa doente do sexo feminino, de 39 anos, assintomática até Dezembro de 2003, altura em que apresentou toracalgia direita de características pleuríticas. A doente era portadora de uma radiografia torácica antiga, efectuada há 13 anos, que revelava múltiplos pequenos nódulos pulmonares bilaterais, atribuídos a sequelas de tuberculose pulmonar. O diagnóstico histológico definitivo foi efectuado através de biópsia pulmonar por toracotomia. Dado
Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma
Oman Medical Journal, 2015
pithelioid hemangioendothelioma (EHE) is a rare vascular tumor with an estimated prevalence of less than one in one million. It has an intermediate malignant potential and clinically behaves in an intermediate way between a benign hemangioma and a highly malignant angiosarcoma. It usually involves the liver, bone, lungs, skin, and other soft tissue. 1,2 Single organ involvement is more common than multi-organ involvement (64%). 2 Together the liver and lung (51%) were the most commonly involved organs in those who presented with multi-organ disease, as reported in a series of 264 patients of EHE. 2 The distribution of EHE involving soft tissue as described in a series of 49 cases are extremities (n=32), head and neck (n=6), mediastinum and trunk (n=4, each), genitals (n=2), and the retroperitoneum (n=1).
ESMO Open, 2021
Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.