Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma (original) (raw)

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.

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.

Epithelioid Hemangioendothelioma of the Liver—Case Report

Journal of Pharmacy and Pharmacology 6 (2018) 153-156

EH (Epithelioid hemangioendothelioma) is a rare vascular malignant neoplasm. Only 434 cases had been reported until 2006. Symptoms may be nonspecific, but it also can involve hepatic veins resulting in Budd-Chiari syndrome. The main differential diagnosis is cholangiocarcinoma, sometimes only distinguishable through immunohistochemistry study. We report a case of a young woman diagnosed with hepatic EH and listed for liver transplantation.

[Multifocal epithelioid hemangioendothelioma treated by liver transplantation--case report]

PubMed, 2011

Multifocal epithelioid hemangioendothelioma of the liver is a rare primary tumor with a variable course of disease. A case is presented of a 27-year-old female patient with multiple hepatic lesions on ultrasonography, suspect of metastatic tumor of the liver. Serum tumor markers were not elevated, while clinical examination of the lungs, gastrointestinal and gynecologic systems did not confirm the presence of a primary tumor process. Metastatic tumor and primary hepatocellular tumor were ruled out by fine needle aspiration cytology. Along with a characteristic immunophenotype of the vascular cell endothelium (positive for CD31 and CD34), high proliferation demonstrated by the analysis of argyrophilic nucleolar organization regions (AgNOR) and DNA aneuploidy, cytomorphological pattern suggested the diagnosis of angiosarcoma. Histopathologic finding corresponded to epithelioid hemangioendothelioma. Ten years after orthotopic liver transplantation, the patient is free from disease relapse, with regular follow up testing. Hemangioendothelioma of the liver is characterized by multifocality, which excludes resection; thus, liver transplantation is the method of choice. Therefore, preoperative diagnostic workup is of utmost importance to differentiate it from other primary and metastatic tumors of the liver.

Hepatic epithelioid hemangioendothelioma: a report of six patients

2010

Epithelioid hemangioendothelioma (EH) was first reported by Weiss and Enzinger in 1982 1 and was defined as a unique borderline tumor characterized by the composition of an epithelioid, endothelial or dendritic cell. 2 EH is not specific to soft tissue and has been reported in other organs such as the lung, 3,4 liver, 2 bone, 5,6 brain, 7,8 heart, 9 salivary gland, 10 vein 11,12 and pleura. 13 Hepatic EH (HEH) is a rare tumor that was reported first by Ishak et al. in 1984. 2 Here, we report six clinical cases of epithelioid hemangioendothelioma of the liver. CASE PRESENTATIONc dd_447 254..258 General conditions and clinical manifestations From 2003 to 2008, six HEH patients confirmed by pathology were treated in our hospital. Their general information, including the site and size, symptoms and signs of HEH are listed in Table 1.

Epithelioid hemangioendothelioma of the liver

Journal of Hepato-biliary-pancreatic Surgery, 2006

Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin, which occurs at sites such as soft tissues, liver, or lung, and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. We present two cases of this rare disease in which the tumor was detected fortuitously and the definitive diagnosis was based on histological evidence. Both our cases are highly illustrative of the two ways in which hepatic EH can present (nodular or diffuse) and of its diagnostic and therapeutic management. Neoplastic cells expressed the factor VIII-related antigen, CD31 or CD34. Treatment was surgical resection in one patient and liver transplant in the other. Although EH of the liver has a better prognosis than other hepatic neoplasms, conservative treatment is not recommended. Our cases highlight the importance of a histological diagnosis to avoid it being mistaken for another entity.

Epitheloid hemangioendothelioma in patient with liver transplantation

Collegium antropologicum, 2010

Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown aetiology and a variable natural course. At the time of diagnosis, most patients present with multifocal tumours lesions that involve both liver lobes. From the therapeutic aspect, liver resection (LRx), liver transplantation (LTx), chemotherapy, radiotherapy, and/or immunotherapy have been used in the treatment of patients with HEH. However, because of the rarity of this tumor and its unpredictable natural history, it is impossible to assess the effectiveness of these respective therapies. In this report, our objective was to present clinical aspects, diagnostic options, therapeutic modalities, and the clinical outcome of single patient with LTx because of this rare tumor.

Epithelioid Hemangioma of Bone and Soft Tissue: A Reappraisal of a Controversial Entity

Clinical Orthopaedics and Related Research®, 2012

The controversy surrounding diagnosis of an epithelioid hemangioma (EH), particularly when arising in skeletal locations, stems not only from its overlapping features with other malignant vascular neoplasms, but also from its somewhat aggressive clinical characteristics, including multifocal presentation and occasional lymph node involvement. Specifically, the distinction from epithelioid hemangioendothelioma (EHE) has been controversial. The recurrent t(1;3)(p36;q25) chromosomal translocation, resulting in WWTR1-CAMTA1 fusion, recently identified in EHE of various anatomic sites, but not in EH or other epithelioid vascular neoplasms, suggests distinct pathogeneses. We investigated the clinicopathologic and radiologic characteristics of bone and soft tissue EHs in patients treated at our institution with available tissue for molecular testing. Seventeen patients were selected after confirming the pathologic diagnosis and fluorescence in situ hybridization analysis for the WWTR1 and/or CAMTA1 rearrangements. Four patients had multifocal presentation. Most patients with EH of bone were treated by intralesional curettage. None of the patients died of disease and only four patients had a local recurrence. Our results, using molecular testing to support the pathologic diagnosis of EH, reinforce prior data that EH is a benign lesion characterized by an indolent clinical course with an occasional multifocal presentation and rare metastatic potential to locoregional lymph nodes. These findings highlight the importance of distinguishing EH from other malignant epithelioid vascular tumors as a result of differences in their management and clinical outcome. Level IV, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.