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Adult Ocular and Orbital (Ocular Adnexa) Tumors
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
P.N. Plowman, Rachel Lewis, J.L. Hungerford
Malignant hemangioendothelioma is much less common. It is not encapsulated, and local recurrence is likely after attempted surgical excision unless a wide margin of normal tissue is excised. Recurrence should be managed by orbital exenteration. There is a high mortality rate from distant spread. Proton beam therapy may in the future widen the indications for radiotherapy in this population of patients.
Oncology
Published in Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss, Understanding Medical Terms, 2020
Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss
One of the most common sites for both primary and metastatic tumors is the liver. Primary tumors of the benign type include cavernous hemangioma (hem-, meaning "blood"; angi-, meaning "vessel"; plus -oma, "tumor"), focal nodular hyperplasia, hepatic cell adenoma (aden-, "gland"), and infantile hemangioendothelioma (endothel-, arising from linings of the blood vessel). The most prevalent malignant tumor of the liver is hepatocellular (hepato-, "liver") carcinoma (HCC). In children it is a hepatoblastoma. Others occurring less frequently are intrahepatic cholangiocarcinoma, angiosarcoma, epithelioid hemangioendothelioma, and undifferentiated sarcoma.
Liver transplantation
Published in Prem Puri, Newborn Surgery, 2017
Alastair J. W. Millar, CWN Spearman
Hemangioendothelioma in infancy is an occasional indication, if medical treatment with steroids, propranolol, and surgical treatment with resection, hepatic artery ligation, or embolization fails, but outcome is guarded.10
Efficacy of transcatheter arterial chemoembolization combined with sirolimus for treating Kasabach–Merritt phenomenon in infants, a retrospective study
Published in Annals of Medicine, 2023
Chuan-gao Yin, Wei-Wei Qi, Song Wang, Deng Pan, Xiao-Li Chen, Shi-Yu Li
Kasabach–Merritt syndrome (KMS), which refers to giant haemangioma combined with thrombocytopenia syndrome, was first reported by Kasabach and Merritt in 1940 [1]. In the 1990s, it was found that this disease was caused by Kaposiform hemangioendothelioma (KHE) and tufted haemangioma (TA) rather than infantile haemangioma. In the same year, Professor Sarker suggested that severe thrombocytopenia, microangiopathic haemolytic anaemia, secondary fibrinogenopenia, and consumptive coagulation disorder caused by KHE and TA should be known as the Kasabach–Merritt phenomenon (KMP). Infants and young children are prone to KMP. Although the condition is rare, the fatality rate is as high as 10%–37% [2], and there is no agreed treatment regimen. Traditional treatment methods include compression, support, drug therapy, radiotherapy, endovascular embolization, and surgery. Drug therapy includes treatment with prednisone, interferon, propranolol, and sirolimus, added in recent years. However, compression and drug therapies are slow to work. For large lesions, it is usually difficult to surgically resect them completely, and the risk is high. The action time of endovascular embolization alone on platelet elevation is usually limited.
The landscape of tyrosine kinase inhibitors in sarcomas: looking beyond pazopanib
Published in Expert Review of Anticancer Therapy, 2019
Christopher P Wilding, Mark L Elms, Ian Judson, Aik-Choon Tan, Robin L Jones, Paul H Huang
A further cohort of 15 patients with metastatic or locally advanced epithelioid hemangioendothelioma (EHE) not amenable to curative resection were enrolled onto this trial [92]. PFS at 9 months was chosen as the primary end-point given the indolent nature of EHE [93]. Seven of the 15 patients (46%) had undergone previous surgery and 5 patients (33%) had received prior systemic anticancer therapy. mPFS was 6 months, with a non-progression rate at 9 months of 30.7% (4 of 13 assessable patients). Best response rate on cross-sectional imaging per RECIST following sorafenib was a partial response in 2 of 13 assessable patients (13.3%) and stable disease in 9 of 13 (69.2%). In the French Sarcoma Group study, a sorafenib dose reduction was required in 3 of 15 patients (20%), whilst 5 patients (33.3%) required a transient drug discontinuation due to toxicity.
What’s the latest with investigational drugs for soft tissue sarcoma?
Published in Expert Opinion on Investigational Drugs, 2022
Elena Cojocaru, Andrea Napolitano, Cyril Fisher, Paul Huang, Robin L Jones, Khin Thway
Angiosarcoma and epithelioid hemangioendothelioma (EHE) are both highly vascular tumors but have different underlying mechanisms of development and biology. EHE has been histologically compared to angiosarcoma but is characterized by WWTR1(TAZ)-CAMTA, or YAP1-TFE3 fusions [72]. EHE are tumors with a spectrum of clinical behaviors, ranging from slow-growing and indolent disease to very aggressive and rapidly progressing disease which sometimes necessitates treatment with chemotherapy agents used in other types of STS [73,74]. In EHE, the use of mechanistic target of rapamycin (mTOR) inhibitors has shown the most meaningful clinical benefit and should be offered to patients with advanced disease [73,75].