Jul 10, 20 the term hemangioendothelioma describes several types of vascular neosplasms and includes both noncancerous benign and cancerous malignant growths. Typical features also include low fibrinogen and elevated ddimers. Kaposiform hemangioendothelioma khe is a rare vascular neoplasm that previously has been described only in children. 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.
It generally affects the soft tissues presenting as mass lesions with associated cutaneous abnormities and kasabachmerritt phenomenon kmp. This disambiguation page lists articles associated with the same title. This is a vascular tumor associated with the kasabachmerritt phenomenon severe coagulopathy due to platelet trapping and spontaneous bleeding. She underwent simultaneous tumor biopsy, spinal deformity correction and fusion.
The patients, two men and one woman, were aged 64, 55, and 48 years, respectively. The importance of early diagnosis and treatment of kaposiform. Though several case reports have discussed the imaging findings of khe, larger comprehensive studies are lacking. Pathology outlines kaposiform hemangioendothelioma.
Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor associated with kasabach merritt syndrome. The lymphatic system is part of the immune system made up of vessels that help to protect the body from infection and foreign substances. May 23, 2011 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. These tumors are histopathologically distinct from the common hemangiomas of infancy. Jan 30, 2015 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.
Article pdf available in orphanet journal of rare diseases 151 december 2020 with 83 reads. Kaposiform hemangioendothelioma definition of kaposiform. For language access assistance, contact the ncats public information officer. It generally originates on the skin, usually affecting deeper tissue by. We examined 3 children with kaposiform hemangioendothelioma, all of whom were. Kaposiform hemangioendothelioma khe is a rare vascular tumor that arises during early childhood and has an equal prevalence in males and females. Abstractkaposiform hemangioendothelioma khe is an uncommon vascular tumor of intermediate. Kaposiform hemangioendothelioma in tonsil of a child. Hepatic epithelioid hemangioendothelioma hehe is a rare, low to intermediate grade malignant hepatic vascular tumor. Kaposiform hemangioendothelioma kh is a rare vascular tumor of intermediate malignancy that occurs mainly in the childhood and is often associated with kasabachmerritt phenomenon or lymphangiomatosis or both. Kaposiform hemangioendothelioma in a 3 months old infant.
Kaposiform lymphangiomatosis genetic and rare diseases. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. 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. Kaposiform hemangioendothelioma is a rare, highly vascular and aggressive soft tissue tumor frequently. Musculoskeletal complication in kaposiform hemangioendothelioma. Abdominal kaposiform hemangioendothelioma associated with ly. Early diagnosis by means of mri and tissue biopsy portends a better outcome. Clinicopathological features of kaposiform hemangioendothelioma. Kaposiform hemangioendothelioma article about kaposiform. Ches are rare vascular tumors that are clinically similar to more common vascular tumors but histologically exhibit a composite of hemangioendothelioma variants. Primary cardiac kaposiform hemangioendothelioma ecronicon. Congenital fulminant kaposiform hemangioendothelioma of the. Kaposiform hemangioendothelioma khe is a benign tumor of the blood vessels.
Kaposiform hemangioendothelioma involving the neck sciencedirect. Kaposiform hemangioendothelioma genetic and rare diseases. We report the case of a 1monthold infant who presented with stridor and was found to. Nov 01, 2012 soft tissue kaposiform hemangioendothelioma. Kaposiform hemangioendothelioma is a rare pediatric neoplasm that presents most commonly in the soft tissues.
Kaposiform hemangioendothelioma khe is composed of several solid. Kaposiform hemangioendothelioma of the tongue in an adult. Hemangioendothelioma of the testis pdf free download. Kaposiform lymphangiomatosis kla and kaposiform hemangioendothelioma khe are rare and aggressive vascular disorders. 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. Pdf kaposiform hemangioendothelioma khe is a rare vascular neoplasm with high. Imaging findings of kaposiform hemangioendothelioma in. We report a unique spinal khe with painless thoracic scoliosis in a 14yearold girl. Researchers are still searching for the cause of khe. Kaposiform hemangioendothelioma khe is a unique locally aggressive vascular tumor with poor prognosis.
Kaposiform hemangioendothelioma without kasabachmerritt. This article is from world journal of surgical oncology, volume 9. Epidemiology there may be a greater female incidence with reported maletofemale ratio, 3. The term has also been applied to those that show borderline behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. We present a case of congenital kaposiform hemangioendothelioma of the leg in a female infant who was born to a mother treated with various medications including etanercept, a tnf antagonist, due to rheumatoid arthritis. Kaposiform hemangioendothelioma is a rare tumor of childhood often accompanied by kasabachmerritt syndrome and occasionally lymphangiomatosis. 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. Kaposiform hemangioendothelioma also known as infantile kaposiform hemangioendothelioma is an uncommon vascular tumor, first described by niedt, greco, et al. Kaposiform lymphangiomatosis kla is a rare type of tumor and vascular malformation that results from the abnormal development of the lymphatic system. Current knowledge and future perspectives article pdf available in orphanet journal of rare diseases 151. 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, a rare vascular neoplasm of infancy and.
Kaposiform hemangioendothelioma khe is a rare, locally aggressive, and nonmetastatic vascular tumor of intermediate malignancy. Kaposiform hemangioendothelioma is a rare locally aggressive vascular neoplasm of infancy and childhood. Kaposiform hemangioendothelioma khe may look like a birthmark, but is actually a rare benign tumor caused by the abnormal growth of blood vessels. Download fulltext pdf download fulltext pdf kaposiform hemangioendothelioma.
Wed like to understand how you use our websites in order to improve them. Cutaneous kaposiform hemangioendothelioma khe and tufted angioma ta are vascular. It is not inherited, and while it typically occurs in infancy, khe can arise prenatally or in adults after trauma. Examples of vascular tumors include hemangiomas, lymphangiomas, hemangioendotheliomas, kaposis sarcomas, angiosarcomas, and hemangioblastomas. Niedt gw, greco ma, wieczorek r, blanc wa, knowles dm 2nd. Click on the link to view a sample search on this topic. Tufted angioma, kaposiform hemangioendothelioma, and the. A report on kaposiform hemangioendothelioma in the cervical.
The purpose of this study was to evaluate the imaging findings of khe in children. Kaposiform hemangioendothelioma khe boston childrens. While both on the spectrum of vascular tumors, tufted angiomas ta have a more benign. Kaposiform hemangioendothelioma involving the neck core. Congenital fulminant kaposiform hemangioendothelioma of. 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. Kaposiform hemangioendothelioma of the choledochus malignant hemangioendothelioma of the uterus. Kaposiform hemangioendothelioma and tufted angioma epi. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree.
It has rarely been observed in the head and neck region and at times, can present without kmp. Journal of surgical case reports, volume 2019, issue 4, april 2019, rjz1. A report on kaposiform hemangioendothelioma in the. This rare condition mainly affects infants and young children, although cases have been reported in older children and adults. Composite hemangioendothelioma american journal of clinical. Request pdf kaposiform hemangioendothelioma kaposiform hemangioendothelioma khe, first described by zuckerberg et al. The aim of this study is to assess the clinical and imaging features of khe, and to compare the differences between solitary and diffusive infiltrative subtype further. Kaposiform hemangioendothelioma khe is a rare, locally invasive, vascular tumor of infants and adolescents. Composite hemangioendothelioma che has been recently recognized as a low to intermediategrade vascular tumor.
Kla involves multiple parts of the body, especially the lungs and chest. Article pdf available in american journal of clinical pathology. Hepatic epithelioid hemangioendothelioma radiology. Kaposiform hemangioendothelioma khe is a rare, aggressive vascular tumor that typically occurs during infancy or early childhood. Hemangioendothelioma genetic and rare diseases information. Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. Kaposiform hemangioendothelioma khe is a rare and aggressive vascular tumor that is benign noncancerous and does not spread to other locations in the body. Clinical and imaging features of kaposiform hemangioendothelioma. Tufted angioma and kaposiform hemangioendothelioma khe are rare vascular tumors that typically occur during infancy or early childhood. Infantile hemangioendothelioma of the liver hemangioendothelioma of the salivary gland kaposiform hemangioendothelioma of the choledochus malignant hemangioendothelioma of the uterus epithelioid hemangioendothelioma of. Kaposiform hemangioendothelioma khe, an intermediate tumor of endothelial origin in childhood, is often associated with kasabachmerritt. Kaposiform hemangioendothelioma with adolescent thoracic. 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.
Pdf kaposiform hemangioendothelioma khe is a rare vascular neoplasm that previously has. It often manifests later than infantile hemangioma, and complication by kasabachmerritt syndrome is common. Kaposiform hemangioendothelioma and tufted angioma. Pubmed is a searchable database of medical literature and lists journal articles that discuss kaposiform hemangioendothelioma. Morphologically, the tumor consisted of dense spindle cells with a nodular growth. If you have problems viewing pdf files, download the latest version of adobe reader. Microscopically, from the differential diagnostic point of view, pathologies such as welldifferentiated angiosarcoma early ks lesion, fibrosarcoma advanced ks lesion, arteriovenous malformations, kaposiform hemangioendothelioma, spindle cell hemangioendothelioma and bacillary angiomatosis, should be included.
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