Fosb may be helpful in distinguishing epithelioid hemangioma from ehe. Epithelioid hemangioma of bone is a rare type of angiomatous tumor. Always think of epithelioid sarcoma when an extremity lesions looks granulomatous keratin positivity is typically seen in both. The blood vessels are lined by epithelioid cells, which grow abnormally to form the tumor. 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. Pathology of angiolymphoid hyperplasia with eosinophilia. 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. Is it named after its classification between angiosarcoma and hemangioma, which are also vascular tumors comprising endothelial cells. Ahle morphology and to evaluate its value in differential diagnosis of epithelioid vascular tumors. It is essential to avoid misdiagnosis with peyronies disease and penile cancer, as management differs significantly.
Epithelioid endothelial cell tumors currently are classified as epithelioid hemangioma, epithelioid hemangioendothelioma, and epithelioid angiosarcoma. After developing a rash from sorafenib, thalidomide was started with the progression of disease. Based on the two index cases, a particular emphasis for selection for the extended study cohort was given to lesions with increased cellularity and evidence of atypical histologic features. Cutaneous epithelioid hemangioma of atypical location. Epithelioid cell histiocytoma is regarded as a variant of fibrous histiocytoma dermatofibroma. After surgical resection, histopathological examination of the resected specimen confirmed the diagnosis of epithelioid hemangioma of the colon, which was backed up by the immunohistochemical.
Involvement of the we report an unusual case of eh alhe that arose on the lower back in a zosteriform array. As such, epithelioid hemangioma might be comparable to another benign vascular. Congenital hemangioma retiform hemangioendothelioma epitheloid hemangioendothelioma lymphatic malformation lm lvm, clvm tufted hemangioma pila, dabska tumor venous malformation vm cavm spindlecell hemangioma composite hemanigoendothioma arteriovenous malformation avm clavm epithelioid hemangioma kaposi sarcoma arteriovenous fistula. Epithelioid hemangioendothelioma ehe represents the most aggressive member of the hemangioendothelioma family, which is intermediate between hemangioma and angiosarcoma in terms of recurrence and metastatic potential. Epithelioid hemangioma eh is a benign neoplasm with distinctive vasoformative features, which occasionally shows increased cellularity, cytologic atypia, andor locoregional aggressive growth, resulting in challenging differential diagnosis from malignant vascular neoplasms. May 04, 2020 vascular tumors of the liver in adult patients include cavernous hemangioma, a common benign tumor. A case of a 48yearold man presented with radiating pain to the lower thoracic region for two years. Although epithelioid hemangioma is a rare disease, knowledge. 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. Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. Skin nonmelanocytic tumor epithelioid hemangioma angiolymphoid hyperplasia with eosinophils. Angiolymphoid hyperplasia with eosinophilia wikipedia. Cytologic features of epithelioid hemangioendothelioma.
Kato h, hatori m, kokubun s, watanabe m, smith ra, hotta t, ogose a, morita t, murakami t, aiba s. Although, a progressive clinical course with tumorrelated fatality has been documented in some instances, this lesion does not behave as aggressively as a conventional angiosarcoma 14. Pdf epithelioid hemangioma of bone and soft tissue. Epithelioid hemangioma eh is an uncommon benign vascular tumor, of controversial etiology that usually presents as a slowly growing. Although they were once considered different stages of the same disease, they are now known to represent separate entities. Epithelioid hemangioendothelioma is a rare type of vascular tumor that affects the epithelial cells, which line the inside of blood vessels.
Because ehe, a lowgrade tumor with metastatic potential, is intermediate between epithelioid hemangioma, a benign tumor, and epithelioid angiosarcoma, a highgrade malignant tumor, we included 15 cases of epithelioid hemangioma and 5 cases of epithelioid angiosarcoma to determine if there was any relationship between them. Epithelioid hemangioma of the bone is a local ly aggressive uncommon vascular neoplasm composed of cells with an endothelial pheno. Case outline a 52yearold male patient presented with a oneyear history of a nodular lesion in the left buccal mucosa measuring 3 cm. Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin, first defined as a specific entity by weiss and enzinger in 1982. Eh is defined as an intermediategrade malignant vascular neoplasm whose clinical course is between those of epithelioid hemangioma and angiosarcoma.
However, a distinct, diseasedefining genetic alteration recently described for ehe indicates that it is an entirely separate entity from both angiosarcoma and hemangioma. Epithelioid hemangioma involving three contiguous bones. Epithelioid hemangioendothelioma is a rare form of cancer that occurs in the thin layer of cells, or endothelium, that line the blood vessels of the human body specifically in the liver and lungs. We sought to investigate the fosb staining pattern in eh with angiolymphoid hyperplasia with eosinophilia eh. Epithelioid hemangioma in the oral mucosa a case report. We present a case of epithelioid hemangioma of the penis in a 50yearold caucasian man. We describe ultrasound, ct and ctpet features of an uncommon disease presentation in the upper arm area. Epithelioid hemangioma eh is a benign vascular lesion that is characterized by wellformed, capillarysized vessels lined by histiocytoid or epithelioid endothelial cells. 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. Epithelioid sarcoma sclerosing epithelioid fibrosarcoma. We report a documented case of epithelioid hemangioma occurring in the distal femur of a 35yearold man. The pathology files of mskcc and the personal consultations of the corresponding authors cra, cdf were searched for cases of epithelioid hemangioma eh. Introduction epithelioid hemangioma is an uncommon benign vasoproliferative neoplasm that usually manifests as multiple red nodules in middleaged adults. It is rare in bone, where it grows in a more solid fashion than its soft tissue counterpart.
Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Treatment of hepatic epithelioid hemangioendothelioma. Differential diagnosis epithelioid sarcoma surgical. 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. Epithelioid hemangioma is a special type of hemangioma characterized by a benign although locally aggressive behavior, composed by proliferated smallcaliber blood vessels lined by plump endothelial cells with an epithelioid morphology.
This study uses an internet registry to identify clinical patterns with prognostic significance in ehe. A study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and highgrade angiosarcoma. Clinical manifestation is variable, usually with nonspecific symptoms such as right upper quadrant pain and weight loss. An epithelioid hemangioendothelioma ehe is a tumor involving the blood vessels and surrounding epithelioid cells. Jun 25, 2007 angiolymphoid hyperplasia with eosinophilia alhe and kimuras disease kd share many clinical and histopathological features. 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.
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. 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. Thirtynine tumors, from 24 females and 15 males with an age range 985 years. It is most frequently localized in the skin of the head and neck region. Hemangioendothelioma is used to describe a group of vascular tumors included. Case report epithelioid angiosarcoma of the middle ear. Epithelioid hemangioma eh is a rare benign vascular lesion that presents as a nodular mass in the skin, usually in the head and neck region. Zfp36fosb fusion defines a subset of epithelioid hemangioma. Epithelioid cells growing in single files, strands, cords. Histopathological examination shows vascular spaces of various sizes lined by prominent endothelium, and inflammatory infiltrates composed of eosinophils, histiocytes, mast cells and lymphocytes. These tumors are well recognized in soft tissues, but less so in bone.
Sep 10, 2018 an epithelioid hemangioma eh is a rare, benign tumor involving the blood vessels and surrounding epithelioid cells. The cutaneous epithelioid hemangioma is a benign vascular tumor, of mesenchymal origin, with no systemic involvement, which may be differentiated from lesions of similar aspects, such as lipoma, cylindroma, sebaceous and epidermoid cyst, lymphocytoma, insect bite reaction, vaccines, sarcoidosis, dermatofibroma, pyogenic granuloma, and kimuras. Other vascular proliferations which can be morphological mimics were also tested. 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.
Hemorrhagic epithelioid and spindle cell hemangioma keel. The clinical, radiographic, mr imaging and histologic findings of the tumor are described. Angiolymphoid hyperplasia with eosinophilia alhe and kimuras disease kd share many clinical and histopathological features. Fosb immunoreactivity in endothelia of epithelioid. Hemangioma bone benign vascular tumor epithelioid aggressive. Utility of immunohistochemistry for endothelial markers in. Article information, pdf download for epithelioid hemangioma of bone.
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. It is usually asymptomatic, and its exact etiology is still unknown. From the authors files, 15 representative cases of eh. Epithelioid hemangioma is a rare vascular tumor found in the penis. An alternate yap1tfe3 fusion has been detected in a small and distinct subset of cases. 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. Angiolymphoid hyperplasia with eosinophilia also known as. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized.
Mar 12, 2018 from the authors files, 15 representative cases of eh. The epithelioid hemangioma, also called angiolymphoid hyperplasia with. Case report a case of epithelioid hemangioma of the right third rib. Epithelioid hemangioma eh is a benign vascular lesion that is characterized by wellformed, capillarysized vessels lined by histiocytoid or epithelioid endothelial cells and often accompanied. Our case involved a 49yearold female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing heh. Likewise, for individuals with retiform hemangioendothelioma, local recurrence is common but metastatic disease is rare. A novel specific genetic translocation in epithelioid. Radiographs showed a welldefined expanding, osteolytic lesion in the diaphysis of the femur.
Specifically, the distinction from epithelioid hemangioendothelioma. Epithelioid hemangioma eh was described originally in 1969 as angiolymphoid hyperplasia with eosinophilia alhe. Ehe, malignant endovascular papillary angio endothelioma. Epithelium based vascular tumors are controversial due to challenging differentiation between various types including. However, a distinct, diseasedefining genetic alteration recently described for ehe indicates that it is an entirely separate entity from. Epithelioid hemangioendothelioma tumors most commonly affect the soft tissues, liver, lungs and bones. Recently, alhe is being called epithelioid hemangioma eh, a term that better describes the possible neoplastic nature of the entity. The epithelioid hemangioma, also called angiolymphoid hyperplasia with eosinophilia, is a rare benign vascular tumor that manifests as nodules. Pdf the controversy surrounding diagnosis of an epithelioid hemangioma eh, particularly when.
Benign and malignant vascular tumors of the liver in adults. Epithelioid hemangioendothelioma is a malignant, often indolent vascular tumor which occurs at various anatomic sites. Case report epithelioid hemangioendothelioma of the anterior. Multiple epithelioid hemangiomas with orbital involvement. Epithelioid cells are polyhedral with abundant cytoplasm and a large, roundtooval nucleus with peripheral margination of chromatin and prominent eosinophilic or purple nucleolus. 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. Hepatic epithelioid hemangioendothelioma heh is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Sep 10, 2018 an epithelioid hemangioendothelioma ehe is a tumor involving the blood vessels and surrounding epithelioid cells. Case outline a 52yearold male patient presented with a oneyear history of a nodular lesion in the. Epithelioid hemangioma of bone and soft tissue full text. 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. An epithelioid hemangioma eh is a rare, benign tumor involving the blood vessels and surrounding epithelioid cells. Clinically, this lesion presents as single or multiple pink to redbrown papules and nodules on the face, scalp and ear.
Hemangioendothelioma genetic and rare diseases information. Benign endothelial cell neoplasms hemangiomas are very common while malignant endothelial cell cancers hemangioendotheliomas and. Epithelioid hemangioma, also known as angiolymphoid hyperplasia with eosinophilia, is a cutaneous angioproliferative lesion that follows a benign clinical course. 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 hemangioendothelioma of bone radiographics. Epithelioid hemangioma angiolymphoid hyperplasia with.
Fosb immunoreactivity in endothelia of epithelioid hemangioma. Alhe were selected and evaluated for their fosb immunostaining pattern. Recently, alhe is being called epithelioid hemangioma eh, a term that better describes the possible neoplastic. Lesions usually present as single or multiple redbrown papules or subcutaneous nodules with a predilection for the head and neck region. Epithelioid hemangioendothelioma ehe, first described by weiss and enzinger in 1982, is a malignant vascular neoplasm with indolent behavior in the majority of cases. 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 hemangioendothelioma ehe is a rare vascular neoplasm of endothelial origin with clinical behavior intermediate between hemangioma and angiosarcoma. But they are slowgrowing and do not usually spread. Lm lvm, clvm tufted hemangioma pila, dabska tumor venous malformation vm cavm spindlecell hemangioma composite hemanigoendothioma arteriovenous malformation avm clavm epithelioid hemangioma kaposi sarcoma arteriovenous fistula pyogenic granuloma. For language access assistance, contact the ncats public information officer.
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