Soft tissue, lung formerly known as intravascular bronchioalveolar tumour, pleura, liver, peritoneum, bone, lymph nodes and skin. Epithelioid hemangioma angiolymphoid hyperplasia with. We present the case of a young man, 24yearold, with ehe of the forearm with secondary involvement of the distal radius. An alternate yap1tfe3 fusion has been detected in a small and distinct subset of cases. Other than the name, classical type epithelioid sarcoma shares only the distinctive immunophenotype of proximal type epithelioid sarcoma. Case report epithelioid hemangioendothelioma of the anterior. Clinically, this lesion presents as single or multiple pink to redbrown papules and nodules on the face, scalp and ear. Epithelioid cell histiocytoma is a well recognised variant of cutaneous fibrous histiocytoma that may be confused with other benign and malignant mesenchymal lesions. Differential diagnosis epithelioid sarcoma surgical. Histopathology images of epithelioid hemangioendothelioma. Epithelioid hemangioendothelioma epithelioid hemangioendothelioma, liver. Epithelioid hemangioendothelioma ehe is a rare vascular tumor with.
Additional and relevant useful information for epithelioid 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. However, the peak age range is the midadult period, between 2050 years. 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. Our case involved a 49yearold female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing heh. Hepatic epithelioid hemangioendothelioma hehe is a rare vascular tumor which has an aggressiveness graded between hemangioma and hepatic hemangiosarcoma hhs. Epithelioid hemangiomas may occur spontaneously, due to an injury or a trauma. Epithelioid hemangioma, gingival growth, vasculoproliferative neoplasm. Epithelioid sarcoma sclerosing epithelioid fibrosarcoma. 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. Epithelioid hemangioendothelioma tumors most commonly affect the soft tissues, liver, lungs and bones.
Pdf epithelioid hemangioendothelioma of the tongue. Epithelioid hemangioma of the temporal artery clinically. It is a superficial vascular entity which can either be due to tumor or reactive lesion, but the exact etiology is still unknown. 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. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized. Case report open access epithelioid hemangioma of the penis. We hereby present a rare case which has been reported with the history of small nodularlike growth on mandibular buccal and lingual area. Pdf epithelioid hemangioma of penis mimicking malignancy. 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.
Skin nonmelanocytic tumor vascular tumors epithelioid hemangioendothelioma. Bone ehe comprises less than 1% of the bone neoplasms. Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. Epithelioid hemangioendothelioma is a malignant vascular tumour. Epithelioid hemangioendothelioma is a rare type of vascular tumor that affects the epithelial cells, which line the inside of blood vessels.
Substantial advances have been made in recent years in identifying the underlying recurrent chromosomal and genomic alterations in a significant subset of soft tissue tumours, and this continues to enrich our understanding of the biological mechanisms of tumour. Although kimuras disease has often been considered to be identical to angiolymphoid hyperplasia with eosinophilia epithelioid haemangioma, recent studies suggest that they are different clinicop. Epithelioid hemangioma, though rare, can occur in pediatric patients and appears to involve the epiphyses in these patients. Most lesions have a nonspecific nodular appearance with frequent secondary changes such as. 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. Pdf epithelioid hemangioendothelioma is an uncommon vascular neoplasm. Epithelioid hemangioma may occur in individuals of all age group. Epitheliod hemangioendothelioma of liver is a rare lowgrade malignancy that can also occurs in other organs as a primary malignancy.
Pulmonary epithelioid hemangioendothelioma misdiagnosed as. Epithelioid hemangioendothelioma ehe is a rare vascular tumor of intermediate malignancy. Results of a tagged red blood cell scan were negative for hemangioma, and a fineneedle aspiration biopsy showed benign liver tissue. Richard l kempson md robert v rouse md department of pathology stanford university school of medicine stanford ca 943055342. Hepatic epithelioid hemangioendothelioma and adult liver. Ches are rare vascular tumors in which various types of hemangioendothelioma are combined in 1 lesion. Epithelioid hemangioma may be considered for an epiphyseal lesion in a skeletally immature patient. Epithelium based vascular tumors are controversial due. Epithelioid hemangioma arises in adult patients and is usually solitary, although up to 25% may be multifocal. 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. Thirtynine tumors, from 24 females and 15 males with an age range 985 years. It is essential to avoid misdiagnosis with peyronies disease and penile cancer, as management differs significantly.
Hepatic epithelioid hemangioendothelioma heh is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Case report open access epithelioid hemangioma of the. Spinal ehe is a quite rare disease with few case reports and series reported in the literature. Pathology outlines epithelioid hemangioendothelioma. This series represents a novel group of vascular tumors in domestic animals of which there are only 3 previous cases reported in the veterinary literature. Epithelioid hemangioma is a rare benign vascular lesion that presents as a nodular lesion in the skin of head and neck region. Within the spectrum of epithelioidtype vascular tumors, epithelioid hemangioendothelioma and epithelioid angiosarcoma have overlapping imaging characteristics with eh. Epithelioid hemangioendothelioma of the forearm with. Epithelioid variant of hemangioma and hemangiosarcoma in. Epithelioid hemangioendothelioma is a malignant, often indolent vascular tumor which occurs at various anatomic sites. But they are slowgrowing and do not usually spread. Pdf epithelioid hemangioendothelioma of cavernous sinus.
Epithelioid hemangioendothelioma ehe is a subtype of. Angiolymphoid hyperplasia with eosinophilia archives of. Fletcher cdm, beham a, bekir s, clarke amt, marler nje. An epithelioid hemangioendothelioma ehe is a tumor involving the blood vessels and surrounding epithelioid cells. However, a distinct, diseasedefining genetic alteration recently described for ehe indicates that it is an entirely separate entity from both angiosarcoma and hemangioma.
Hemangioendotheliomas are caused by abnormal growth of blood. Soft tissue ehe equally affects both sexes, but liver and. Epithelioid and epithelial neoplasms of bone archives of. The patient had been treated with corticosteroids predonine 5 mgday p. Epithelioid hemangioma is a special type of hemangioma characterized by a benign although. Specifically, the distinction from epithelioid hemangioendothelioma. In epithelioid histiocytoma, sections show a centrally located, circumscribed tumour underlying an. Epithelioid hemangioendothelioma ehe is a rare malignant vascular tumor that typically involves the soft tissues, skin, viscera, bone, and, in rare cases, the oral cavity. Recent advances in the diagnosis of soft tissue tumours. Two extremely rare cases of ehe arising in the tongue of two young children. Epithelioid haemangioma angiolymphoid hyperplasia with. Pdf epithelioid hemangioma eh is an uncommon benign vascular tumor. Treatment of hepatic epithelioid hemangioendothelioma. Histopathological features diagnosed the case definitive of eh.
Article pdf available in the korean journal of pathology 485. 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. It is otherwise known as epithelioid fibrous histiocytoma and epithelioid dermatofibroma. Epithelioid hemangioma of bone and soft tissue full text. A rare case of epithelioid hemangioma angiolymphoid hyperplasia with eosinophilia arising in the right temporal artery of a 68yearold japanese woman was investigated. This is in contrast to the usual age and location reported. The term hemangioendothelioma describes several types of vascular neosplasms and includes both noncancerous benign and cancerous malignant growths. Cytologic features of epithelioid hemangioendothelioma. 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. Intraoral epithelioid hemangioendothelioma discussion the analysis of cases of intraoral epithelioid hemangioma reported in the literature is a relatively difficult task, once the lesion from years ago has received various names, such as hemangioendothelioma, malig. Skin nonmelanocytic tumor epithelioid hemangioma angiolymphoid hyperplasia with eosinophils. Pathology of angiolymphoid hyperplasia with eosinophilia. The various histopathologic variants of hemangiomas may present as an aggressive hemangioma, with destruction of the adjacent bone cortex and softtissue extension.
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