2017
DOI: 10.1055/s-0037-1604358
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Pancreatic Kaposiform Hemangioendothelioma Not Responding to Sirolimus

Abstract: Background  Kaposiform hemangioendothelioma (KHE) is a vascular tumor frequently associated with Kasabach–Merritt phenomenon (KMP), characterized by severe thrombocytopenia and consumptive coagulopathy. Visceral involvement in KHE is rare. In our recent experience, sirolimus has shown to be an effective treatment in cutaneous KHE, becoming indeed the treatment of choice in KMP. We report a case of pancreatic KHE associated with KMP and refractory to sirolimus. Case Report  A 4-month-old infant is referred for… Show more

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Cited by 16 publications
(15 citation statements)
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“…One prospective nonrandomized study and 17 retrospective studies described the treatment of lymphatic malformations with oral sirolimus. 14,19,26,32,41,48,56,61,[64][65][66][67][68][69][70][71][72][73] The median age was 12.8 years (0-34 years); 20% of patients were younger than 1 year, and 90% were younger than 18 years. Nonspecified decrease in lesion size was reported in 77.8% patients, partial response in 22.2%, complete response in 3.7%, improvement of pleural effusion in 9.9%, decrease of skin exudate and vesicles in 16.5%, and improvement of pain in 4.9%.…”
Section: Vascular Malformationsmentioning
confidence: 99%
“…One prospective nonrandomized study and 17 retrospective studies described the treatment of lymphatic malformations with oral sirolimus. 14,19,26,32,41,48,56,61,[64][65][66][67][68][69][70][71][72][73] The median age was 12.8 years (0-34 years); 20% of patients were younger than 1 year, and 90% were younger than 18 years. Nonspecified decrease in lesion size was reported in 77.8% patients, partial response in 22.2%, complete response in 3.7%, improvement of pleural effusion in 9.9%, decrease of skin exudate and vesicles in 16.5%, and improvement of pain in 4.9%.…”
Section: Vascular Malformationsmentioning
confidence: 99%
“…While prednisolone, vincristine and sirolimus showed some effectiveness in decreasing the tumor size 16,17 , can cause side effects such as abdominal pain, elevations of aspartate aminotransferase (AST) and alanine aminotransferase (ALT), opportunistic infection, loss of appetite, etc 18 . Moreover, there are case reports of visceral KHE not responding to sirolimus 19,20 . In addition, for those KHE patients unresponsive to glucocorticoids, propranolol, vincristine or sirolimus treatment, surgical therapy reduced skin petechia and coagulopathy to normal levels.…”
Section: Discussionmentioning
confidence: 99%
“…6 Medical therapies for KHE and KMP include systemic steroids, vincristine, inteferon-alpha and aspirin. 7 In cases with associated biliary obstruction, complete surgical resection with a Whipple operation has been reported and there have also been successful attempts at biliary stenting and external drainage (palliative surgery), pending spontaneous tumour regression. 1,6 Historically, angiography and embolisation have been used for the diagnosis and treatment of KHE.…”
Section: Discussionmentioning
confidence: 99%
“…Mammalian target of rapamycin (mTOR) inhibitors such as sirolimus have proven to be effective in the treatment of cutaneous KHE. 7 Triana et al described a case of pancreatic KHE not responding to sirolimus and suggested the possibility of a different molecular profile compared to the cutaneous form as a result of the resistance to mTOR inhibitors treatment. 7…”
Section: Discussionmentioning
confidence: 99%