A report on kaposiform hemangioendothelioma in the. Kaposiform hemangioendothelioma khe is a rare vascular neoplasm that previously has been described only in children. 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. Kaposiform hemangioendothelioma khe is an uncommon vascular tumor of intermediate malignant potential, usually occurs in the extremities and retroperitoneum of infants and is characterized by its association with lymphangiomatosis and kasabachmerritt phenomenenon kmp in certain cases.
Impact of sirolimus treatment for refractory kaposiform hemangioendothelioma with exacerbation of the disease 10 years after initial diagnosis naoki sakata, soichi suenobu, munehiro okano, satoshi ueda, masatomo kimura, and tsukasa takemura. Kaposiform hemangioendothelioma khe is a rare neoplasm of vascular origin that. 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. It often manifests later than infantile hemangioma, and complication by kasabachmerritt syndrome is common. Kaposiform lymphangiomatosis genetic and rare diseases.
Kasabachmerritt phenomenon is a serious coagulopathy associated with kaposiform hemangio endothelioma khe, tufted angioma, and possibly other. Kaposiform hemangioendothelioma khe and tufted angioma ta are rare vascular tumors that typically occur in infancy and early childhood. The exact incidence and prevalence has not been accurately determined, but has been estimated to be 0. Kaposiform hemangioendothelioma khe is a benign tumor of the blood vessels. Examples of vascular tumors include hemangiomas, lymphangiomas, hemangioendotheliomas, kaposis sarcomas, angiosarcomas, and hemangioblastomas. Pathology of kaposiform hemangioendothelioma dr sampurna.
It generally originates on the skin, usually affecting deeper tissue by. Kaposiform hemangioendothelioma plastic surgery key. This rare condition mainly affects infants and young children, although cases have been reported in. Kaposiform hemangioendothelioma is a rare, lowgrade malignant vascular neoplasm. Kaposiform hemangioendothelioma is a rare disease of childhood. An angioma refers to any type of benign vascular tumor some vascular tumors. Kaposiform lymphangiomatosis kla is a rare type of tumor and vascular malformation that results from the abnormal development of the lymphatic system. Kaposiform hemangioendothelioma also known as infantile kaposiform hemangioendothelioma is an uncommon vascular tumor, first described by niedt, greco, et al. Kaposiform hemangioendothelioma khe is a rare vascular neoplasm with high morbidity and mortality. Retroperitoneum, subcutaneous or deep soft tissue of upper extremities, head and neck area. Mesh or free text words were executed during the search. Kaposiform hemangioendothelioma is a vascular tumor that commonly presents as a cutaneous mass, is observed in children, and is associated with kasabachmerritt phenomenon.
Kaposiform hemangioendothelioma khe is a relatively rare vascular tumor with an aggressive and infiltrating nature. Tufted angioma, kaposiform hemangioendothelioma, and the. In the case of kmp, it is speculated that the unique vessel architecture of kaposiform hemangioendothelioma khe and tufted angiomas ta promotes platelet trapping and the resultant consumptive coagulopathy. Many of us remember hearing about kasabachmerritt phenomenon kmp on rounds as an intern in the nursery and conjured visions of large hemangiomas.
Pdf kaposiform hemangioendothelioma khe is a rare vascular neoplasm that. Researchers are still searching for the cause of khe. It has rarely been observed in the head and neck region and at times, can present. Kaposiform hemangioendothelioma, tufted a ngioma, kasabachmerritt phenomenon, treatment and therapy. Kaposiform hemangioendothelioma has no gender predilection and most commonly presents in the first year of life. Kaposiform hemangioendothelioma with kasabachmerritt. Kaposiform hemangioendothelioma khe is a rare vascular tumor that arises during early childhood and has an equal prevalence in males and females. Kaposiform hemangioendothelioma khe is a rare vascular tumor. 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 spindle cells. Kaposiform hemangioendothelioma article about kaposiform.
Kaposiform hemangioendothelioma is a rare, highly vascular and aggressive soft tissue tumor frequently associated with kasabachmerritt. These tumors are histopathologically distinct from the common hemangiomas of infancy. Hemangioendothelioma article about hemangioendothelioma. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Kaposiform hemangioendothelioma khe boston childrens.
Kaposiform lymphangiomatosis kla and kaposiform hemangioendothelioma khe are rare and aggressive vascular disorders. Kaposiform hemangioendothelioma khe is a rare, locally aggressive vascular neoplasm that mainly occurs during childhood. Numerous khe in a broad range of locations have been reported e. Kaposiform hemangioendothelioma khe is a locally aggressive vascular tumor that usually occurs in infants. Tumor nodules composed with wellcanalized areas alternating with poorly canalized and solidappearing areas resembling a capillary. Typical features also include low fibrinogen and elevated ddimers. It can appear anywhere on the body, often on the head and neck but also on the stomach, back, arms and legs even inside the chest, abdomen or bones. Skin tumor nonmelanocytic hemangioendothelioma and. The purpose of this study was to evaluate the imaging findings of khe in children. Kaposiform hemangioendothelioma radiology reference. The initiating mechanism during the pathogenesis of khe has yet to be discovered. Herein we report a case of kaposiform hemangioendothelioma with chest wall deformity in an adult who did not show the kasabachmerritt phenomenon or cutaneous lesions.
Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. Khe can be confused with infantile hemangioma due to the age of presentation and presence of a vascular cutaneous lesion. 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. The search was restricted to the human population and intervention studies, including clinical trials. Kla involves multiple parts of the body, especially the lungs and chest. Kaposiform hemangioendothelioma khe is a rare, locally invasive, vascular tumor of infants and adolescents. This lesion is commonly associated with kasabachmerritt syndrome and lymphangiomatosis. It generally affects the soft tissues presenting as mass lesions with associated cutaneous abnormities and kasabachmerritt phenomenon kmp. 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. Response to paclitaxel in an adult patient with advanced. Pubmed is a searchable database of medical literature and lists journal articles that discuss kaposiform hemangioendothelioma. The lymphatic system is part of the immune system made up of vessels that help to protect the body from infection and foreign substances.
Though several case reports have discussed the imaging findings of khe, larger comprehensive studies are lacking. It typically grows just below the skin or, more rarely, into internal organs, between the lungs or in the abdominal cavity. Impact of sirolimus treatment for refractory kaposiform. This lesion typically presents as a bluishred mass in the superficial or deep soft tissue of the extremities, although it may also appear in the. Kaposiform lymphangiomatosis kla is a rare type of tumor and vascular. Kaposiform hemangioendothelioma khe may look like a birthmark, but is actually a rare benign tumor caused by the abnormal growth of blood vessels. Kaposiform hemangioendothelioma khe, an intermediate tumor of endothelial origin in childhood, is often associated with kasabachmerritt. Kaposiform hemangioendothelioma in tonsil of a child.
Tufted angioma and kaposiform hemangioendothelioma khe are rare vascular tumors that typically occur during infancy or early childhood. Tufted angioma, previously known as angioblastoma of nakagawa, is a benign tumor whose name derives from its histopathologic appearance characterized by tufts of capillaries within the dermis 1,2. Epithelioid and retiform variants are most commonly observed, but. Patients with kaposiform hemangioendothelioma in the absence of kasabachmerritt phenomenon patient sex age of onset location size in diameter initial and maximum if different appearance age at followup evaluation course 1 f 2 y left cheek and mandible 11. Kaposiform hemangioendothelioma khe is a rare and aggressive vascular tumor that is benign noncancerous and does not spread to other locations in the body. Treatment of kaposiform hemangioendothelioma and tufted angioma. Pseudomyogenic hemangioendothelioma was recognized as a distinctive entity under the name epithelioid sarcomalike hemangioendothelioma in 2003. The sites of predilection are the skin mainly, and deep anatomic regions such as retroperitoneum and so on. Hemangioendothelioma an overview sciencedirect topics.
Kaposiform hemangioendothelioma khe is rare, and classified as a locally aggressive vascular tumor by international society for the study of vascular anomalies. Pdf kaposiform hemangioendothelioma khe is a rare vascular neoplasm with high morbidity and. A study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Epithelioid hemangioendothelioma is a rare malignant neoplasm which can occur only in bone or as a multiorgan disease 230. Click on the link to view a sample search on this topic. Imaging findings of kaposiform hemangioendothelioma in. Tarja niini, sakari knuutila, in bone cancer second edition, 2015. Sirolimus, a promising treatment for refractory kaposiform. Hemangioendothelioma definition of hemangioendothelioma. The term has also been applied to those that show borderline behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Aptt activated partial thromboplastin time, khe kaposiform hemangioendothelioma, kms. Kaposiform hemangioendothelioma european journal of internal. Kaposiform hemangioendothelioma of the tongue in an adult.
The translocation places part of the camta1 gene under. Kaposiform hemangioendothelioma without kasabachmerritt. This is a vascular tumor associated with the kasabachmerritt phenomenon severe coagulopathy due to platelet trapping and spontaneous bleeding. It is not inherited, and while it typically occurs in infancy, khe can arise prenatally or in adults after trauma. Kaposiform hemangioendothelioma kh is a rare tumor of childhood often associated with kasabachmerritt phenomenon kmp and occasionally lymphangiomatosis. Treatment of kaposiform hemangioendothelioma and tufted. Lyons ll, north pe, macmoune lai f, stoler mh, folpe al, weiss sw. The importance of early diagnosis and treatment of. The patients, two men and one woman, were aged 64, 55, and 48 years, respectively. Expansile kaposiform hemangioendothelioma deformed.
Composite hemangioendothelioma american journal of. General rare tumor of childhood mean age 4 years, range 2 weeks to 20 years some cases may previously have been called acquired tufted angioma usually on extremities or head and neck. Kaposiform hemangioendothelioma khe is a rare vascular tumor, typically presenting in infancy as a distinctive cutaneous lesion with illdefined borders. Kaposiform hemangioendothelioma definition of kaposiform. Pathology outlines kaposiform hemangioendothelioma. Clinicopathological features of kaposiform hemangioendothelioma. The term hemangioendothelioma describes several types of vascular neosplasms and includes both noncancerous benign and cancerous malignant growths. Vincristine and sirolimus in the treatment of kaposiform.
One or a combination of the following terms was used. We examined 3 children with kaposiform hemangioendothelioma, all of whom were. The aim of this study was to examine the clinical features and prognosis of kla and khe involving the thorax. Niedt gw, greco ma, wieczorek r, blanc wa, knowles dm 2nd. Kaposiform hemangioendothelioma khe is a rare, aggressive vascular tumor that typically occurs during infancy or early childhood. Hemangioendotheliomas are caused by abnormal growth of blood vessel cells, although. Kaposiform hemangioendothelioma in a 3 months old infant. Kaposiform hemangioendothelioma is a locally aggressive, nonmetastasizing vascular tumour. Irregular tumor nodules growing in an infiltrative fashion and evoking a dense hyaline stromal response courtesy of h.
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