No treatment is uniformly effective or relapse and complicationfree. 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. Pseudomyogenic hemangioendothelioma was recognized as a distinctive entity under the name epithelioid sarcomalike hemangioendothelioma in 2003. If you previously purchased this article, log in to readcube. This lesion is commonly associated with kasabachmerritt syndrome and lymphangiomatosis. Pathology outlines kaposiform hemangioendothelioma. Hepatic epithelioid hemangioendothelioma heh is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Herein, we report a rare case of bifocal tibial kaposiform hemangioendothelioma. Treatment of kaposiform hemangioendothelioma and tufted. Rare case of epithelioid hemangioendothelioma in a child.
Epithelioid hemangioendothelioma evidence search nice. On examination, a spherical swelling in the right ear was found. Kaposiform hemangioendothelioma in children oup academic. Retiform hemangioendothelioma is a rare intermediate or borderline neoplasm of the blood vessels that mostly occurs in extremities. Pubmed is a searchable database of medical literature and lists journal articles that discuss kaposiform hemangioendothelioma. Hemangioendothelioma article about hemangioendothelioma by. Khe is commonly associated with kasabachmerritt syndrome kms and lymphangiomatosis. Composite hemangioendothelioma american journal of. Here we report a unique case of retiform hemangioendothelioma presented in the external auditory canal. A hemangioendothelioma is typically a slow growing benign tumor that does not cause major symptoms, but it can. We report a case of rh of the foot of a 19yearold white female. Pathology of kaposiform hemangioendothelioma dr sampurna roy md. Kla involves multiple parts of the body, especially the lungs and chest.
Sep 10, 2018 in spite of all treatment measures, some epithelioid hemangioendothelioma can metastasize causing multiorgan dysfunction syndrome mods additional and relevant useful information for epithelioid hemangioendothelioma. Eventually, multifocal lesions of kaposiform hemangioendothelioma disappeared on the images of magnetic resonance imaging and have remained in remission for 27 months after sirolimus cessation. Kaposiform hemangioendothelioma khe is a rare locally aggressive vascular tumor that usually presents as a superficial or deep soft tissue mass with associated cutaneous lesions. A 5 year 20092014 retrospective study on the challenges and outcome of two hundred forty five children with vascular anomalies referred for surgical management was undertaken at the tashkent medical academy, uzbekistan. Intratesticular kaposiform haemangioendothelioma in adults. Epidemiology there may be a greater female incidence with reported maletofemale ratio, 3. The thoracoabdominal computed tomography showed three well limited opacities of the right lung, among them one shows some calcifications. Successful management of kaposiform hemangioendothelioma. View the article pdf and any associated supplements and figures for a period of 48 hours. Definition and pathogenesis intermediategrade vascular neoplasm less aggressive than angiosarcoma frequently involves the spine, skull, and les multifocal in 50% of cases importance rare clinical features epithelioid variant has a m. We demonstrated that the aktmammalian target of rapamycin signaling pathway played a pivotal role in the kaposiform hemangioendothelioma growth. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized.
Kaposiform hemangioendothelioma without kasabachmerritt. Radiation oncologysarcomahemangioendothelioma wikibooks. The term hemangioendothelioma has been applied to name several vascular proliferations, including both benign and malignant neoplasms. Ehe, malignant endovascular papillary angio endothelioma.
Epithelioid hemangioendothelioma is a rare type of vascular tumor that affects the epithelial cells, which line the inside of blood vessels. Epithelioid hemangioendothelioma is a vascular tumor of low to intermediate malignancy that was initially described in the soft tissue. The initiating mechanism during the pathogenesis of khe has yet to be discovered. However, hepatoblastoma may have similar imaging features and clinical. Epithelioid hemangioendothelioma ehe is a rare cancer that belongs to the group of cancers called sarcomas. Click on the link to view a sample search on this topic. Jul 10, 20 if you have problems viewing pdf files, download the latest version of adobe reader. Retroperitoneum, subcutaneous or deep soft tissue of upper extremities, head and neck area. Bifocal tibial kaposiform hemangioendothelioma responsive. Subash a, senthil gk, ramamoorthy r, appasamy a, selvarajan n. A report on kaposiform hemangioendothelioma in the. Kaposiform hemangioendothelioma khe is a rare, aggressive tumour caused by the abnormal growth of blood vessels that can look like a birthmark. Metastatic disease was reported in more than 100 cases in the literature. Abstractkaposiform hemangioendothelioma khe is an uncommon vascular tumor of intermediate.
A mass can potentially form anywhere in the body, but the condition most commonly affects blood vessels near the skin or in the liver or spleen. Cutaneous kaposiform hemangioendothelioma khe is a vascular tumor with a propensity to cause kasabachmerritt phenomenon kmp. Epithelioid hemangioendothelioma is a vascular tumor of endothelial origin with an intermediate histological and clinical behavior somewhere between that of. It is difficult to manage epithelioid hemangioendothelioma, due to the following factors.
Kaposiform hemangioendothelioma khe is a locally aggressive vascular tumor that may be complicated by kasabachmerritt phenomenon kmp, a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either khe or tufted angioma ta. Clinical manifestations of hepatic epithelioid hemangioendothelioma hehe are nonspecific, such as right upper quadrant pain, hepatomegaly and weight loss while many patients remain asymptomatic at diagnosis. Hemangioendothelioma definition of hemangioendothelioma at. This study evaluated ultrasonography us and magnetic resonance imaging mri. Kaposiform hemangioendothelioma khe is a rare vascular neoplasm with high morbidity and mortality. Kaposiform hemangioendothelioma is a locally aggressive, nonmetastasizing vascular tumour. Kaposiform hemangioendothelioma with kasabachmerritt. While both on the spectrum of vascular tumors, tufted angiomas ta have a more benign. Sep 23, 2014 epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. Kaposiform hemangioendothelioma with adolescent thoracic. It is a lowgrade malignant neoplasm that is locally aggressive but without proven metastatic. This means it originates in the cells that line the inside of blood vessels.
The annual incidence of khe has been estimated at 0. Kaposiform hemangioendothelioma khe is a rare vascular neoplasm that previously has been described only in children. Pathology subtypes subtypes dependent on location include hemangioendothelioma msk hemangioendothelioma of liver see also epithelioid hemangioendothelioma. Kaposiform hemangioendothelioma and tufted angioma epigenetic analysis including genomewide methylation profiling. Epithelioid hemangioendothelioma is an exceedingly rare primary breast tumor. The absence of urinary catecholamines supports the diagnosis of hemangioendothelioma. Trial hemangioendothelioma 3 unread trial of sunitinib plus nivolumab after standard treatment in advanced soft tissue and bone sarcomas. The official journal of isfahan university of medical sciences, volume 14. Haemangioendothelioma article about haemangioendothelioma.
Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infiltrating nodules and sheets of. Development of kasabachmerritt syndrome in a child with multifocal kaposiform hemangioendothelioma in the cervical region reported. Full text musculoskeletal complication in kaposiform. Primary hepatic epithelioid hemangioendothelioma hehe is a very rare type of malignant tumor first reported by ishak et al in 1984. Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma. We examined 3 children with kaposiform hemangioendothelioma, all of whom were. Oct 01, 2016 if you have problems viewing pdf files, download the latest version of adobe reader. Khes are clinically heterogeneous and may develop into a lifethreatening thrombocytopenia and consumptive. The lungs and liver are the two common primary organs affected. The patient had a high plasma level of the ddimer and fibrinogen.
Our recent results showed that ectopic overexpression of human prox1 gene, a lymphatic endothelial nuclear transcription factor, promoted an aggressive behavior in 2. It is locally aggressive and potentially life threatening when associated with consumptive coagulopathy, known as kasabachmerritt syndrome kms. Histologically, the tumor grew as numerous elongated vessels resembling the shape of rete testis with involvement of the skin adnexal structures and. The importance of early diagnosis and treatment of. Kaposiform lymphangiomatosis genetic and rare diseases.
Kaposiform hemangioendothelioma khe is a locally aggressive vascular tumor that usually occurs in infants. Epithelioid hemangioendothelioma tumors most commonly affect the soft tissues, liver, lungs and bones. Since then, epithelioid hemangioendothelioma has been described in various anatomic sites including parenchymal tissues and the bone. Unlimited viewing of the articlechapter pdf and any associated supplements. Case report epithelioid hemangioendothelioma of the anterior. It is commonly associated with kasabachmerritt phenomenon which is directly responsible for the significant morbidity and mortality, including hemodynamic instability, local invasion, and compression of vital structures. Treatment of hepatic epithelioid hemangioendothelioma. The patients, two men and one woman, were aged 64, 55, and 48 years, respectively. A hemangioendothelioma is a tumor derived from blood vessels.
At present, the patient is 6 years old and remains disease free. Kaposiform lymphangiomatosis kla is a rare type of tumor and vascular malformation that results from the abnormal development of the lymphatic system. Epithelioid hemangioendothelioma ehe is a rare vascular neoplasm that occurs more often in women than men and usually arises within the liver, but can also present within other tissues including lung, bone, or peritoneum 1. For language access assistance, contact the ncats public information officer. The aim of this study was to examine the clinical features and prognosis of kla and khe involving the thorax. Infantile hepatic hemangioendothelioma is strongly suggested by the presence of a vascular lesion on imaging studies. However, no firm conclusions can be determined for recommended treatment options. A 9yearold female presented with a 2year history of pain and swelling in the left leg. Kaposiform hemangioendothelioma is composed of several solid poorly circumscribed nodules, and each nodule is composed of a mixture of small capillaries and solid lobules of endothelial cells arranged in a glomeruloid pattern. Kaposiform hemangioendothelioma in adolescentonset scoliosis. Hemangioendotheliomas are locally aggressive vascular tumors with. One case of hepatoblastoma had prolonged activated partial thromboplastin time aptt, while one case of infantile haemangioendothelioma was detected with clinical jaundice and had bilirubin levels of 14. Spectrum of appearances on ct and mri of hepatic epithelioid. Kaposiform hemangioendothelioma and tufted angioma epi.
Hepatic epithelioid hemangioendothelioma hehe is a rare, low to intermediate grade malignant hepatic vascular tumor. In patients with hemangioendotheliomas, elevations in. Hemangioendothelioma an overview sciencedirect topics. Mediastinal epithelioid hemangioendothelioma mimicking a teratoma. Clinical characteristics of epithelioid hemangioendothelioma.
Contrastenhanced ct typically demonstrates inhomogeneous intratumoral uptake that is generally less intense than in the normal liver parenchyma, and hemangioendothelioma remains relatively hypoattenuating compared with infantile hemangioendothelioma, 1, 5, 7. Hemangioendothelioma of bone portnotes orthopaedicsone. Apr 06, 2020 a hemangioendothelioma is a very rare type of tumor that appears inside a blood vessel. Hemangioendotheliomas, along with hemangiopericytomas and angiosarcomas, are tumors that arise from vascular structures. Kaposiform hemangioendothelioma khe is a rare, locally invasive, vascular tumor of infants and adolescents. A free application to browse and search the journals content is now available for iphoneipad. Results for epithelioid hemangioendothelioma 1 10 of 16 sorted by relevance date click export csv or ris to download the entire page or use the checkboxes to select a subset of records to download. Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. Kaposiform haemangioendothelioma khe is a rare vascular neoplasm, locally aggressive, that can be congenital, with 50% presenting at birth. The current case presents a patient with metastatic pulmonary epithelioid. Epithelioid hemangioendothelioma is an important diagnosis, since longterm disease free survival is possible, especially in the setting of orthotopic liver transplantation. This article is from journal of research in medical sciences.
She underwent simultaneous tumor biopsy, spinal deformity correction and fusion. Hemangioendothelioma definition at, a free online dictionary with pronunciation, synonyms and translation. Composite hemangioendothelioma of the forehead and right eye. 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.
Typical features also include low fibrinogen and elevated ddimers. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. The observation that extremity kaposiform hemangioendothelioma could possibly be associated with lymphedema has implications for the care of patients with kaposiform hemangioendothelioma. Intermediategrade malignant tumors, clinically behaving between benign hemangioma and malignant angiosarcoma. Sirolimus, a promising treatment for refractory kaposiform. Although khe mainly presents during early childhood, some cases have been described in adolescents and adults. A 45yearold man, he shows an isolated chronic cough with a preserved general state of health. Kaposiform hemangioendothelioma khe is a rare vascular tumour of the infancy and the first decade of life. Trial hemangioendothelioma 3 unread trial of sunitinib plus nivolumab after standard treatment in advanced soft tissue and bone sarcomas posted. Kaposiform haemangioendothelioma complicated by kasabach. It often manifests later than infantile hemangioma, and complication by kasabachmerritt syndrome is common. Some have rather benign course while others show much more malignant progression.
The main pathological features of khe are abnormal angiogenesis and lymphangiogenesis. The pulmonary epithelioid hemangioendothelioma is a rare vascular intermediate malignancy tumour. Resources for hemangioendothelioma of bone and related topics on orthopaedicsone. Transformation between kaposiform hemangioendothelioma and. Kaposiform hemangioendothelioma khe is a rare vascular neoplasm that can be potentially low malignant.
Epithelioid and retiform variants are most commonly observed, but. Kaposiform lymphangiomatosis kla and kaposiform hemangioendothelioma khe are rare and aggressive vascular disorders. We report the identification of a kaposiform hemangioendothelioma kh in the oropharynx of a 3. Epithelioid hemangioendothelioma is an uncommon vascular tumor of intermediate malignant potential. We report a unique spinal khe with painless thoracic scoliosis in a 14yearold girl. Kaposiform hemangioendothelioma khe is a rare, locally aggressive, and nonmetastatic vascular tumor of intermediate malignancy. If you have problems viewing pdf files, download the latest version of adobe reader. Epithelioid refers to the elongated shape of the cells when seen under the microscope. Kaposiform hemangioendothelioma involving whole of a leg in a neonate with kasabachmerritt. This study reports the role of surgery and laser approach in management children kaposiform hemangioendothelioma. Kaposiform hemangioendothelioma khe is a rare vascular endothelial neoplasm of intermediate malignancy which usually occurs in infancy and early childhood, although adult cases have recently been reported 14. Hemangioendothelioma genetic and rare diseases information.
Our case involved a 49yearold female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing heh. Kaposiform hemangioendothelioma khe is a rare, locally aggressive vascular neoplasm that mainly occurs during childhood. Musculoskeletal hemangioendothelioma radiology reference. Haemangioendothelioma definition of haemangioendothelioma. Kaposiform hemangioendothelioma with associated kasabach.
The sites of predilection are the skin mainly, and deep anatomic regions such as retroperitoneum and so on. A rare presentation of retiform hemangioendothelioma in the. A neoplasm derived from blood vessels, characterized by numerous prominent endothelial cells that occur singly, in aggregates, and as the lining of congeries of vascular tubes or channels. Kaposiform hemangioendothelioma is a rare vascular tumor that almost exclusively occurs in children. Clinically it appears as illdefined red to purple indurated plaque. Impact of sirolimus treatment for refractory kaposiform. Mar 16, 2020 webpathology is a free educational resource with 10276 high quality pathology images of benign and malignant.
It generally affects the soft tissues presenting as mass lesions with associated cutaneous abnormities and kasabachmerritt phenomenon kmp. Composite hemangioendothelioma che has been recently recognized as a low to intermediategrade vascular tumor. Hemangioendothelioma is treated with wide surgical excision. A frequent finding consists of the presence of areas of lymphangiomatosis adjacent to the solid nodules. May, 2015 epithelioid hemangioendothelioma is a rare vascular tumor of borderline or lowgrade malignancy. Kaposiform hemangioendothelioma is a rare locally aggressive vascular endothelialderived spindle cells neoplasm. But they are slowgrowing and do not usually spread. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free. Kaposiform hemangioendothelioma genetic and rare diseases.
Hepatic epithelioid hemangioendothelioma radiology. Hemangioendothelioma aka angioendothelioma kaposiform hemangioendothelioma may be locally aggressive epithelioid hemangioendothelioma, not otherwise specified massons vegetant intravascular hemangioendothelioma intravascular endothelial hyperplasia spindle cell hemangioendothelioma aka spindle cell angioendothelioma, spindle cell. Hemangioendothelioma definition of hemangioendothelioma by. Kaposiform hemangioendothelioma khe and tufted angioma ta primarily occurring in infants are difficult to distinguish. Hemangioendothelioma is used to describe a group of vascular tumors included.
Kaposiform haemangioendothelioma is a very rare vascular tumour of intermediate borderline malignancy, typically occurring in the skin and soft tissues of the extremities in infants and children. In the case of hemangioendotheliomas, the cell of origin is the endothelial cell, and they range from benign to frankly malig. Kaposiform hemangioendothelioma khe is a locally aggressive vascular tumor which usually occurs in infants. This article is from world journal of surgical oncology, volume 9.
The lymphatic system is part of the immune system made up of vessels that help to protect the body from infection and foreign substances. Ches are rare vascular tumors that are clinically similar to more common vascular tumors but histologically exhibit a composite of hemangioendothelioma variants. It generally originates on the skin, usually affecting deeper tissue by. Hemangioendothelioma and angiosarcoma springerlink.
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