Epithelioid hemangioendothelioma is a malignant, often indolent vascular tumor which occurs at various anatomic sites. Jul 10, 20 likewise, for individuals with retiform hemangioendothelioma, local recurrence is common but metastatic disease is rare. Most of the cases of oral epithelioid hemangioendothelioma are asymptomatic, and diagnosis is hampered by the fact that the. We use cookies to offer you a better experience, personalize content, tailor advertising, provide social media features, and better understand the use of our services.
Epithelioid hemangioma of bone is a rare, locally aggressive but benign vascular tumor that is now recognized as a distinct entity from other vascular neoplasms. Epithelioid hemangioendothelioma of the suprasellar area pdf file primary pleural epithelioid haemangioendothelioma with metastases to the skin. We present a case of epithelioid hemangioma of the penis in a 50yearold caucasian man. Mar 14, 2015 pulmonary epithelioid hemangioendothelioma peh is a rare vascular tumor of borderline malignancy that originates from endothelial cells. 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. Epithelioid hemangioma eh, also known as angiolymphoid hyperplasia with eosinophilia, is a benign vascular proliferation of unknown etiology that was firstly. Although no specific radiographic appearance has emerged to date for the epithelioid subtype of hemangioma, these lesions most typically exhibit welldefined osteolysis. Eh is defined as an intermediategrade malignant vascular neoplasm whose clinical course is between those of epithelioid hemangioma and angiosarcoma. The term hemangioendothelioma describes several types of vascular neosplasms and includes both noncancerous benign and cancerous malignant growths. Involvement of the we report an unusual case of eh alhe that arose on the lower back in a zosteriform array. Hepatic epithelioid hemangioendothelioma heh is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Histopathologic analysis showed lobular vascular proliferations containing plump.
Although benign hemangiomas are among the most common diagnoses amid connective tissue tumors, sarcomas showing endothelial differentiation ie, angiosarcoma and epithelioid hemangioendothelioma represent under 1% of all sarcoma diagnoses, and thus it is likely that fewer than 500 people in the united states are affected each year. In this case, the uncommon location in the skin of the upper limb stands out, once the frequent involved sites are generally head and neck, what made it even more difficult to suspect epithelioid hemangioma. Other relatively common features include surrounding sclerosis, cortical expansion and cortical destruction. We report a documented case of epithelioid hemangioma occurring in the distal femur of a 35yearold man. Multicentric epithelioid hemangioendothelioma involving the same. Several cutaneous vascular proliferations are composed of epithelioid endothelial cells, including epithelioid hemangioma eh, also named angiolymphoid. The epithelioid hemangioma, also called angiolymphoid hyperplasia with eosinophilia, due to its histopathological characteristics, is a rare benign vascular tumor, first described by wells and whimster, in 1969. However, a distinct, diseasedefining genetic alteration recently described for ehe indicates that it is an entirely separate entity from both angiosarcoma and hemangioma. 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. Epithelioid hemangioendothelioma of the skin and femur.
Malignant vascular tumorsan update modern pathology nature. Epithelioid hemangioma is a special type of hemangioma characterized by a benign although locally aggressive behavior, composed. 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. Solid form of epithelioid hemangioma 395 were clear.
We present the case of a young man, 24yearold, with ehe of the forearm with secondary involvement of the distal radius. The initial presumptive diagnosis was epithelioid vascular tumor of borderline malignancy. Epithelioid hemangioma of bone and soft tissue full text. 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. Epithelioid hemangioma is a rare benign vascular lesion that presents as a nodular lesion in the skin of head and neck region. It was first described as a distinct entity by weiss and enzinger in 1982 4. Jun 19, 2015 epithelioid hemangioendothelioma ehe is a rare, lowgrade malignant vascular tumor. Epithelioid hemangioma involving three contiguous bones. Most lesions have a nonspecific nodular appearance with frequent secondary changes such as. Radiographs showed a welldefined expanding, osteolytic lesion in the diaphysis of the femur. Although epithelioid hemangioma is a rare disease, knowledge. Oral epithelioid hemangioendothelioma unusual location of a.
No tumour involvement was microscopically shown in the surgical margin. Eight years tumor control with pazopanib for a metastatic. Epithelioid hemangioma of bone is a rare type of angiomatous tumor. Hepatic epithelioid hemangioendothelioma radiology. Soft tissue, lung formerly known as intravascular bronchioalveolar tumour, pleura, liver, peritoneum, bone, lymph nodes and skin. Histology showed sheets of epithelioid cells lining vascular structures in a hobnailed tombstonelike arrangement, with additional areas containing trabeculae of. Additional and relevant useful information for epithelioid hemangioma. Most of the cases of oral epithelioid hemangioendothelioma are asymptomatic, and diagnosis is hampered by the fact that the histological features are somewhat between. Histologically, this was a superficial illdefined le. Bone ehe comprises less than 1% of the bone neoplasms. Angiolymphoid hyperplasia with eosinophilia also known as epithelioid hemangioma is a distinctive vascular lesion which usually occurs during early and mid adult life 20 40 years. Pathology of angiolymphoid hyperplasia with eosinophilia.
Epithelioid hemangioendothelioma is a malignant vascular tumour. Epithelioid hemangioma eh was described originally in 1969 as angiolymphoid hyperplasia with eosinophilia alhe. Select the file that you have just downloaded and select import option. Pathology of epithelioid hemangioendothelioma dr sampurna. It is more common in adult females over 30 years old. It is a superficial vascular entity which can either be due to tumor or reactive lesion, but the exact etiology is still unknown. Jun 30, 2011 epithelioid hemangioma is a rare vascular tumor found in the penis. Although they were once considered different stages of the same disease, they are now known to represent separate entities. Most lesions have a nonspecific nodular appearance with frequent secondary changes such as excoriation and bleeding. Imaging of hepatic epithelioid hemangioendothelioma. Epithelioid hemangioma in the oral mucosa a case report. The purpose of this study is to evaluate the pathological, clinical, and radiological characteristics of epithelioid hemangioma arising within bone and soft tissue, in the first cohort of cases where the definitive diagnosis is supplemented by molecular.
Epithelioid hemangiomas have strong clinical and histopathological. Angiolymphoid hyperplasia with eosinophilia wikipedia. Epithelioid hemangioendothelioma is a rare vascular neoplasm, considered to have clinical behavior intermediate between a benign hemangioma and malignant angiosarcoma. Epithelioid hemangiomas may occur spontaneously, due to an injury or a trauma.
Treatment of hepatic epithelioid hemangioendothelioma. Epithelioid hemangioendothelioma of bone radiographics. It can occur at almost any age, 1, most frequently during the 2nd and 3rd decades of life, with a male predilection of approximately 2. Journal of the american academy of dermatology can be distinguished from. Epithelioid hemangioendothelioma eh is a rare vascular soft tissue tumor of. Explore more on epithelioid hemangioma of skin below. Back to issva classification for vascular anomalies overview causal genes in blue type alt for previous view when associated with overgrowth, some of these lesions belong to the pik3carelated overgrowth spectrum see details some of these lesions may be associated with thrombocytopenia andor consumptive coagulopathy see details.
However, a distinct, diseasedefining genetic alteration recently described for ehe indicates that it is an entirely separate entity from. Epithelioid hemangioendothelioma ehe is a rare, lowgrade malignant vascular tumor. Epitheliod hemangioendothelioma of liver is a rare lowgrade malignancy that can also occurs in other organs as a primary malignancy. Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin, first defined as a specific entity by weiss and enzinger in 1982. The term has also been applied to those that show borderline behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Epithelioid hemangioma eh, also known as angiolymphoid hyperplasia with eosinophilia, may affect the eyelids and orbit, mimic idiopathic orbital inflammation, and respond poorly to corticosteroids. Case outline a 52yearold male patient presented with a oneyear history of a nodular lesion in the left buccal mucosa measuring 3 cm. Pdf multifocal epithelioid hemangioma of the metacarpal. Pulmonary epithelioid hemangioendothelioma misdiagnosed as a.
Pulmonary epithelioid hemangioendothelioma misdiagnosed as. The clinical, radiographic, mr imaging and histologic findings of the tumor are described. Epithelioid hemangioendothelioma epithelioid hemangioendothelioma, liver. It was first described as a distinct entity by weiss and enzinger in 1982. Hemangioendotheliomas are caused by abnormal growth of blood. Epithelioid hemangioma of bone is typically found in adult patients, presenting most commonly as a solitary lesion, but approximately 25% may be. Epithelioid hemangioendothelioma epithelioid sarcoma. Author links open overlay panel stephen tyring md, phd a pamela. Article information, pdf download for epithelioid hemangioma and epithelioid. When epithelioid hemangioma eh of the bone is suspected, the differential diagnosis can include epithelioid hemangioendothelioma ehe, epithelioid angiosarcoma ea, and metastatic carcinoma. Spectrum of appearances on ct and mri of hepatic epithelioid.
The blood vessels are lined by epithelioid cells, which grow abnormally to form the tumor. Until recently it was confused with kimuras disease. Volume 20, issue 2, part 2, february 1989, pages 362366. Hepatic epithelioid hemangioendothelioma hehe is a rare, low to intermediate grade malignant hepatic vascular tumor. We hereby present a rare case which has been reported with the history of small nodularlike growth on mandibular buccal and lingual area. 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. If you have problems viewing pdf files, download the latest version of adobe reader. Differential diagnosis epithelioid sarcoma surgical.
Lesions usually present as single or multiple redbrown papules or subcutaneous nodules with a predilection for the head and neck region. The hepatic epithelioid hemangioendothelioma heh is developing from the vascular elements of mesenchymal tissue. Epithelioid hemangioma eh is an uncommon benign vascular tumor, of controversial etiology that usually presents as a slowly growing nodule on the face or digit of a middleaged woman. Most tumors are diagnosed in an advanced unresectable phase and when clinically aggressive, require systemic. Clinical manifestation is variable, usually with nonspecific symptoms such as right upper quadrant pain and weight loss. Epithelioid hemangioma and epithelioid hemangioendothelioma. Pdf share on tweeters share on facebook share on linkedin share on. Epithelioid hemangioma of skin bioinformatics tool laverne is a handy bioinformatics tool to help facilitate scientific exploration of related genes, diseases and pathways based on cocitations. Epithelioid hemangioendothelioma encasing the left.
Epithelioid hemangioendothelioma of the forearm with. Case outline a 52yearold male patient presented with a oneyear history of a nodular lesion in the. The lesion was excised with the safety margin of 10 mm. Oral epithelioid hemangioendothelioma unusual location. The epithelioid hemangioma, also called angiolymphoid hyperplasia with eosinophilia, is a rare benign vascular tumor that manifests as nodules. 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. Angiolymphoid hyperplasia with eosinophilia alhe and kimuras disease kd share many clinical and histopathological features.
These findings made diagnosis of epithelioid hemangioendothelioma eh. Pulmonary epithelioid hemangioendothelioma peh is a rare vascular tumor of borderline malignancy that originates from endothelial cells. Recently, alhe is being called epithelioid hemangioma eh, a term that better describes the possible neoplastic nature of the entity. Sep 10, 2018 an epithelioid hemangioma eh is a rare, benign tumor involving the blood vessels and surrounding epithelioid cells. 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. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized.
Conventional epithelioid hemangioendothelioma, pathological findings. Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. A case of a 48yearold man presented with radiating pain to the lower thoracic region for two years. Although the first part of this description is correct, the second part is not entirely accurate. Pdf epithelioid hemangioma of bone and soft tissue. Epithelioid hemangioendothelioma with extensive cystic change and camta1 rearrangement. Epithelioid hemangioma is a rare vascular tumor found in the penis. An epithelioid hemangioma eh is a rare, benign tumor involving the blood vessels and surrounding epithelioid cells. Thirtynine tumors, from 24 females and 15 males with an age range 985 years. Our case involved a 49yearold female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing heh. Thirtynine tumors, from 24 females and 15 males with an age. We present a case of epithelioid hemangioma of the spine with an unusual radiological appearance which to our. A reappraisal of a controversial entity costantino errani, lei zhang, david m.
Histopathological examination shows vascular spaces of various sizes lined by prominent endothelium, and inflammatory infiltrates composed of eosinophils, histiocytes, mast cells and lymphocytes. Angiolymphoid hyperplasia with eosinophilia also known as. 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. Histopathology images of epithelioid hemangioendothelioma by.
Epithelioid hemangioma eh is an uncommon benign vascular tumor, of controversial etiology that usually presents as a slowly growing nodule on the face or digit of a. For more information on how to use laverne, please read the how to guide. Cutaneous epithelioid hemangioma of atypical location. Wide surgical excision of epithelioid hemangioma with removal of the entire lesion is the preferred treatment method. The more pleomorphic varieties of epithelioid hemangioendothelioma are often initially confused with a metastatic carcinoma. Jun 25, 2007 angiolymphoid hyperplasia with eosinophilia alhe and kimuras disease kd share many clinical and histopathological features. A case report article pdf available in srpski arhiv za celokupno lekarstvo 144910. Hemangioendothelioma genetic and rare diseases information. Liver involvement occurs most often as a primary tumour and has a behaviour between benign hemangioma and aggressive hemangiosarcoma with extra hepatic spread. Introduction epithelioid hemangioma is an uncommon benign vasoproliferative neoplasm that usually manifests as multiple red nodules in middleaged adults.
Histopathology images of epithelioid hemangioendothelioma. Multifocal epithelioid hemangioma with reactive bone formation. Clinically, this lesion presents as single or multiple pink to redbrown papules and nodules on the face, scalp and ear. Epithelioid hemangioma, histiocytoid hemangioma, inflammatory angiomatous nodule, intravenous atypical vascular proliferation, papular angioplasia, inflammatory arteriovenous hemangioma, and pseudopyogenic granuloma usually presents with pink to redbrown, domeshaped, dermal papules or nodules of the head or neck. Epithelioid hemangioendothelioma is a rare connective tissue tumor of vascular origin. It is essential to avoid misdiagnosis with peyronies disease and penile cancer, as management differs significantly.
An alternate yap1tfe3 fusion has been detected in a small and distinct subset of cases. Epidemiology there may be a greater female incidence with reported maletofemale ratio, 3. Epithelioid hemangioma angiolymphoid hyperplasia with. The medical literature almost every paper about ehe begins with a statement that reads something like, ehe is a vascular neoplasm intermediate between hemangioma benign and angiosarcoma. Introduction epithelioid hemangioma eh, also known as angiolymphoid hyperplasia with eosinophilia, is a lesion of vascular origin that was described for the first time in 1969. Ehe occurs mostly in soft tissues of the extremities and various visceral organs lung, bone, brain and intestine, etc. For spindle cell hemangioma, simple excision is reportedly curative.
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