These tumors are so named because their histologic appearance resembles a proliferation of epithelioid cells, with polygonal shape and eosinophilic cytoplasm. The entity was first described in the soft tissues in 1982 by weiss and enzinger who evaluated 41 cases of solid tumors of vascular origin with epithelioid or histiocytoid. Pseudomyogenic hemangioendothelioma mdedge dermatology. Most tumors are diagnosed in an advanced unresectable phase and when clinically aggressive, require systemic. Epithelioid hemangioendothelioma ehe is a rare tumor usually presenting in soft tissue. Hepatic epithelioid hemangioendothelioma radiology. Hepatic epithelioid hemangioendothelioma and adult liver. May 03, 2020 epithelioid hemangioendothelioma is also known by several other terms, which include angioendothelioma, cellular hemangioma, lowgrade anaplastic angiosarcoma and histiocytoid hemangioma. Jul 03, 2017 hepatic epithelioid haemangioendothelioma hehe represents a very rare vascular tumor of the liver of epithelioid origin. Thirtynine tumors, from 24 females and 15 males with an age range 985 years.
An alternate yap1tfe3 fusion has been detected in a small and distinct subset of cases. Epitheliod hemangioendothelioma of liver is a rare lowgrade malignancy that can also occurs in other organs as a primary malignancy. Mri of hepatic epithelioid hemangioendothelioma heh. Sep 10, 2018 an epithelioid hemangioendothelioma ehe is a tumor involving the blood vessels and surrounding epithelioid cells. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum. Hepatic epithelioid hemangioendothelioma heh is a rare malignant liver tumor first described by weiss and enzinger in 1982 1. But they are slowgrowing and do not usually spread. Epithelioid hemangioendothelioma ehae is a rare tumor, first characterized by sharon weiss and franz enzinger that both clinically and histologically is intermediate between angiosarcoma and hemangioma. Pseudomyogenic hemangioendothelioma epithelioid sarcoma. Pathology of epithelioid hemangioendothelioma dr sampurna. The four subgroups of he are epithelioid ehe, spindle cell, kaposiform, and malignant endovascular papillary angioendothelioma dabska tumor. Hemangioendothelioma genetic and rare diseases information.
Epithelioid hemangioendothelioma is a malignant vascular tumour. My wife was recently diaognised with epithelioid hemangioendothelioma ehe after she underwent a surgery for excision of an aggressive sebaceous cyst at the back of her skull on the scalp. Our case involved a 49yearold female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing heh. Epithelioid hemangioendothelioma is a rare connective tissue tumor of vascular origin. May involve venous structures as intravascular proliferations or fibrothrombotic occlusions. Pseudomyogenic hemangioendothelioma is a rare lesion, with marked male predominance male to female ratio of 4. Epithelioid hemangioendothelioma ehe is a rare vascular neoplasm of endothelial origin with clinical behavior intermediate between hemangioma and angiosarcoma. Ehe is a vascular malignancy of intermediate clinical behavior, with a histologic appearance of endothelial cells growing in nests or cords. Epithelioid and retiform variants are most commonly observed, but foci of spindlecell. Cravat hopes the following information will give you a starting point and basis for understanding ehe so that you can better cope with the diagnosis.
Epidemiology there may be a greater female incidence with reported maletofemale ratio, 3. Epithelioid and retiform variants are most commonly observed, but. Even when the appropriate diagnosis is achieved, the best treatment for hehe has not been defined or standardized, further complicating the care of these patients. Jul 10, 20 likewise, for individuals with retiform hemangioendothelioma, local recurrence is common but metastatic disease is rare. Epithelioid hemangioendothelioma of the liver ehl is a rare vascular tumor of unknown etiology and unpredictable biological outcome. Although the value of liver transplantation lt is well established, its place in the management of hehe is still unclear. Epithelioid hemangioendothelioma ehe is a very rare vascular tumor or anomaly that arises from the cells lining the blood vessels.
On the other hand, a followup study of 30 patients with cutaneous epithelioid hemangioendothelioma showed that 21% of the affected individuals developed systemic metastases, and 17% of these patients died as a consequence. Unlike its microscopic mimicker epithelioid sarcoma, pmhe rarely metastasizes and longterm. The biopsy of the cyst which was large in size which had grown in a samall benign form over last ten to twelve years reveleade it to be ehe. Epithelioid haemangioendothelioma ehe is a malignant vascular neoplasm. Its peak incidence is in young adults mean age, 30 years, with only about 20% of patients being older than 40 years. Heh has a vascular origin like hemangioma and angiosarcoma. A case of an epithelioid hemangioendothelioma arising from. In some patients with more aggressive disease, ehe behaves like a lowgrade slowgrowing cancer. Pulmonary epithelioid hemangioendothelioma misdiagnosed as. Hepatic epithelioid haemangioendothelioma hehe represents a very rare vascular tumor of the liver of epithelioid origin. Soft tissue ehe equally affects both sexes, but liver and.
Pseudomyogenic hemangioendothelioma epithelioid sarcomalike. Benign endothelial cell neoplasms hemangiomas are very common while malignant endothelial cell cancers hemangioendotheliomas and. Histopathologically, heh consists of epithelioid and dendritic cells with central fibrotic stroma. Epithelioid hemangioendothelioma tumors most commonly affect the soft tissues, liver, lungs and bones. Epithelioid hemangioendothelioma is also known by several other terms, which include angioendothelioma, cellular hemangioma, lowgrade anaplastic angiosarcoma and histiocytoid hemangioma. Skin tumor nonmelanocytic hemangioendothelioma and. The tumor has variable biology, ranged from benign haemangioma to highly malignant angiosarcoma, thus it is very difficult to prognose the outcome. Hepatic epithelioid hemangioendothelioma hehe is a rare, often misdiagnosed vascular neoplasm with clinical behaviors that range from indolent to highly aggressive. Forbes takes privacy seriously and is committed to transparency. The aim of this study is to confirm, based on a very large patient cohort, the value of lt in the management of hehe and to identify risk.
Although ehe often originates from a vessel, it is relatively rare for a primary vascular ehe to originate from a large vein or artery. Epithelioid hemangioendothelioma is an uncommon vascular tumor of intermediate malignancy that was first described by steven billings, andrew folpe, and sharon weiss in 2003. Pulmonary epithelioid hemangioendothelioma peh is a rare vascular tumor of borderline malignancy that originates from endothelial cells. Epithelioid hemangioendothelioma ehe boston childrens. Subsets have been characterized previously by translocations resulting in either wwtr1 camta1 or yap1 tfe3 fusion. Primary malignant hepatic epithelioid hemangioendothelioma. Epithelioid hemangioendothelioma is a malignant angiocentric vascular neoplasm figure 4 that commonly arises in the super. Most of the cases of oral epithelioid hemangioendothelioma are asymptomatic, and diagnosis is hampered by the fact that the histological features are somewhat between. Hepatic epithelioid hemangioendothelioma a rare tumor. Cytologic features of epithelioid hemangioendothelioma. Epithelioid hemangioendothelioma of the liver makhlouf. We present the case of a young man, 24yearold, with ehe of the forearm with secondary involvement of the distal radius. Cords of epithelioid cells in a myxohyaline background.
Epithelioid hemangioendothelioma of the liver ehl is a rare vascular tumor of epithelioid origin. Most tumors are diagnosed in an advanced unresectable phase and when clinically aggressive, require systemic cytotoxic. The tumor occurs among people from 1986 years of age, and is twice as more frequent in male than in female patients. Epithelioid hemangioendothelioma eh is a rare neoplasm of vascular origin that involves soft tissue and visceral organs.
It is most commonly found in young to middle aged women, and its clinical behavior is remakably variable from an indolent metastatic tumor to an aggressive rapidly growing neoplasm. However, a distinct, diseasedefining genetic alteration recently described for ehe indicates that it is an entirely separate entity from both angiosarcoma and hemangioma. Hepatic epithelioid hemangioendothelioma archives of. Sep 23, 2014 the term epithelioid hemangioendothelioma was introduced in 1982 by weiss and enzinger to describe a vascular tumor of bone and soft tissue showing features between hemangioma and angiosarcoma. Bone ehe comprises less than 1% of the bone neoplasms. Oral epithelioid hemangioendothelioma unusual location of a. No other sites of disease were found on metastatic survey. Epithelioid hemangioendothelioma ehe is a rare, welldifferentiated endothelial tumor with intermediate malignancy which develops more frequently from the peripheral veins, generally in the lower limb.
It was first described by dail and liebow in 1975 as an epithelial lesion of the lung, which they named an intravascular bronchioloalveolar tumor. Oral epithelioid hemangioendothelioma unusual location. Webpathology is a free educational resource with 10328 high quality pathology images of benign and malignant neoplasms and related entities. Epithelioid hemangioendothelioma ehe is a rare vascular endothelial cell sarcoma that is composed of mostly round epithelioid cells that can arise in soft tissue, bone, and organs such as liver and lung. Soft tissue, lung formerly known as intravascular bronchioalveolar tumour, pleura, liver, peritoneum, bone, lymph nodes and skin. Hepatic epithelioid hemangioendothelioma hehe is a rare vascular tumor which has an aggressiveness graded between hemangioma and hepatic hemangiosarcoma hhs. Crazy sexy lifes kris carr on living a peaceful life with cancer. Epithelioid hemangioendothelioma of the livercase report. End of skin nonmelanocytic tumors vascular tumors hemangioendothelioma and subtypes. Epithelioid hemangioendothelioma ehe is a rare vascular tumor composed of dendritic and endothelial cells with an epithelioid morphology. However, a distinct, diseasedefining genetic alteration recently described for ehe indicates that it is an entirely separate entity from.
Histopathology images of epithelioid hemangioendothelioma. Imaging findings in epithelioid hemangioendothelioma. The biological behaviour of this malignant tumor is uncertain and usually falls between that of benign haemangioma and that of malignant angiosarcoma. Crazy sexy lifes kris carr on living a peaceful life with. Background hepatic epithelioid hemangioendothelioma hehe is a rare vascular tumor which has an intermediate aggressive behavior. Combination capecitabine and bevacizumab in the treatment. Epithelioid hemangioendothelioma ehe is a rare neoplasm of vascular origin that occurs in the liver and other organs. This study uses an internet registry to identify clinical patterns with prognostic significance in. These tumors have a unique clinical presentation and microscopic appearance as compared to other vascular tumors in the differential diagnosis. Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patients clinical data using reasonable medical judgment. An epithelioid hemangioendothelioma ehe is a tumor involving the blood vessels and surrounding epithelioid cells. Apr 23, 2015 epithelioid hemangioendothelioma is a rare connective tissue tumor of vascular origin.
Hepatic epithelioid hemangioendothelioma a rare tumor and. We describe the case of a 59yearold caucasian male patient admitted to university hospital merkur for diagnostic workup and liver transplantation lt. Epithelioid hemangioendothelioma of the forearm with. Epithelioid hemangioendothelioma epithelioid hemangioendothelioma, liver. Eight years tumor control with pazopanib for a metastatic. Epithelioid hemangioendothelioma ehe is a vascular tumor of intermediate malignancy that may occur in a variety of sites, including soft tissues, 1,2 bone, 3,4 lung, 5 liver, 6 pleura and peritoneum, 7,8 skin, 2,9 lymph nodes, 10,11 stomach, 12 and brain. Epithelioid hemangioendothelioma definition of epithelioid. In patients with stable tumors, or tumors that shrink without treatment, ehe behaves more like a benign non. Hepatic epithelioid hemangioendothelioma and adult liver tra. Composite hemangioendothelioma american journal of clinical. This page is intended for people who have just been diagnosed with hemangioendothelioma he and particularly epithelioid hemangioendothelioma ehe the disease this author knows most about. Chest computed tomography ct performed during a routine cancer screening revealed multiple small pulmonary nodules in a 50yearold man who had previously undergone endoscopic submucosal dissection of early gastric cancer. Treatment of hepatic epithelioid hemangioendothelioma. Most of the cases of oral epithelioid hemangioendothelioma are asymptomatic, and diagnosis is hampered by the fact that the.
Hepatic epithelioid hemangioendothelioma hehe is a rare, low to intermediate grade malignant hepatic vascular tumor. The entity of composite hemangioendothelioma che has been recognized as a low to intermediategrade malignant vascular tumor displaying varying combinations of benign and malignant vascular components. Epithelioid hemangioendothelioma of the suprasellar area pdf file primary pleural epithelioid haemangioendothelioma with metastases to the skin. The entity was first described in the soft tissues in 1982 by weiss and enzinger who evaluated 41 cases of solid tumors of vascular origin with epithelioid or histiocytoid endothelial cells. Thirtynine tumors, from 24 females and 15 males with an age. Pathology outlines epithelioid hemangioendothelioma. Epithelioid hemangioendothelioma ehe is a rare vascular malignancy with intermediate behavior between that of benign hemangioma and malignant angiosarcoma 1,2. The authors analyzed the clinicopathologic and immunohistochemical features of 7 patients with ehe of the liver in an attempt to identify features that might predict tumor behavior. The tumour occupies an intermediate position in the spectrum of epithelioid vascular tumours lying between the benign epithelioid hemangioma and the more aggressive epithelioid angiosarcoma.
After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized. Hemangioendothelioma he describes a group of vascular tumors of intermediate malignancy that usually involve soft tissue but can involve the liver, lungs, skin, lymph nodes, and bones. The final pathologic diagnosis was an epithelioid hemangioendothelioma, or grade 2 hemangioendothelioma, of bone origin. Hepatic epithelioid hemangioendothelioma heh is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Epithelioid hemangioendothelioma is a rare, locally aggressive vascular tumor, originating from soft tissue, bone, skin, and organs such as the liver or lung, exceptionally located in the oral cavity. Epithelioid hemangioendothelioma with extensive cystic change and camta1 rearrangement. Pseudomyogenic hemangioendothelioma pmhe is a rare vascular tumor that was added to the world health organization classification of soft tissue tumors. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head. Epithelioid hemangioendothelioma is a rare type of vascular tumor that affects the epithelial cells, which line the inside of blood vessels.
Pseudomyogenic hemangioendothelioma phe, also known as epithelioid sarcomalike hemangioendothelioma or fibromalike variant of an epithelioid sarcoma, is an uncommon soft tissue tumor characterized with its predominance in males, extremity sites, multifocality. Likewise, for individuals with retiform hemangioendothelioma, local recurrence is common but metastatic disease is rare. Multiple 80%, tan gray, firm, circumscribed and focally confluent nodules up to 12 cm with infiltrative borders. Epithelioid hemangioendothelioma is the term applied to a vascular tumor first described in 1981. Epithelioid hemangioendothelioma presenting in the skin. The blood vessels are lined by epithelioid cells, which grow abnormally to form the tumor. Epithelioid hemangioendothelioma is a malignant, often indolent vascular tumor which occurs at various anatomic sites. Composite hemangioendothelioma american journal of. Histopathology images of epithelioid hemangioendothelioma by.