Is epithelioid hemangioendothelioma curable?
Although localized epithelioid hemangioendothelioma can be surgically resected, there is no effective therapy for systemic disease, and mortality from the cancer ranges between 13% and 18% when confined to soft tissue, but life expectancy in metastatic cases is unpredictable and ranges from a couple of months to 15 to …
What is epithelioid hemangioendothelioma?
Epithelioid hemangioendothelioma, or EHE, is a rare cancer that grows from the cells that make up the blood vessels. This cancer can occur anywhere in the body with the most common sites being the liver, lungs, and bone.
What is Kaposiform Hemangioendothelioma?
Kaposiform hemangioendothelioma, or KHE, is a type of tumor that grows in the blood vessels. Blood vessels help move blood around the body. This tumor usually grows just underneath the skin, but it can also grow deeper inside the chest or abdomen.
What is Kasabach-Merritt syndrome?
Kasabach-Merritt phenomenon (KMP) is a rare, potentially life-threatening condition. It occurs when certain fast-growing vascular tumors [kaposiform hemangioendothelioma (KHE) or tufted angioma (TA)] trap and destroy platelets, interfering with blood clotting and raising the risk of serious bleeding.
What is Kaposiform Lymphangiomatosis?
Kaposiform lymphangiomatosis (KLA) is an extremely rare condition in which the vessels that carry lymphatic fluid throughout the body expand and interconnect. These abnormal lymphatic vessels invade and cause damage to surrounding tissue, bones and organs.
What does Hemangioendothelioma mean?
The term hemangioendothelioma describes several types of vascular neosplasms and includes both non-cancerous (benign) and cancerous (malignant) growths. The term has also been applied to those that show “borderline” behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas.
What is platelet trapping?
Kasabach-Merritt syndrome (KMS) consists of large and rapidly growing vascular tumors associated with thrombocytopenia, generalized petechiae, and bleeding. The cause of the thrombocytopenia is thought to be related to the trapping of platelets by the abnormal endothelium of the tumor.
What is pseudomyogenic hemangioendothelioma?
First described in 2003 as epithelioid-sarcoma-like hemangioendotheliomaand later in 2011 as pseudomyogenic hemangioendothelioma, this rare vascular tumor is of intermediate malignant potential. It was officially included for the first time in the most recent World Health Organization’s Classification of Tumours of Soft Tissue and Bone.
Is denosumab effective for primary pseudomyogenic hemangioendothelioma?
Pseudomyogenic hemangioendothelioma (PMHE) is a rare endothelial neoplasm that involves the bones in only 14% of all cases. The optimal treatment strategy has not been established. We herein report a case of primary PMHE in which denosumab treatment showed activity in both imaging studies and the clinical outcome.
What is the best treatment for hemangioendothelioma?
Treatment Treatment. Treatment for hemangioendothelioma may depend on the type of hemangioendothelioma present in the affected individual and the risk of recurrence or metastases. In most reported cases, surgical excision (removal) of the mass has been the only treatment.
What are the different types of hemangioendothelioma?
Examples of types of hemangioendotheliomas include spindle cell hemangioma; papillary intralymphatic (Dabska tumor); retiform; kaposiform; epithelioid; pseudomyogenic (epithelioid sarcoma-like hemangioendothelioma); and composite.