Successful Management of Pancreatic Kaposiform Hemangioendothelioma With Sirolimus

Wang, Chuan; Li, Yanan; Xiang, Bo; Li, Fuyu; Chen, Siyuan; Li, Li; Ji, Yi

doi:10.1097/mpa.0000000000000801

Cited by 19 publications

(14 citation statements)

References 9 publications

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“…In the present study, although the frequency of KMP in the retroperitoneal lesions was high, we identified two patients with retroperitoneal lesions that were not associated with KMP. Similar findings have been reported by other investigators . Theoretically, small retroperitoneal lesions without KMP might not be detectable unless they become clinically evident …”

Section: Discussionsupporting

confidence: 91%

Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon

et al. 2018

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Section: Discussionsupporting

confidence: 91%

Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon

et al. 2018

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“…The aim of this systematic review was to assess the efficacy and safety of vincristine and sirolimus for the treatment of KHE. Aside from the 15 studies that were included in this meta‐analysis, there have many case reports describing vincristine and/or sirolimus in the treatment of KHE . Because of the inherent selection bias, these case reports have not been included in the current review.…”

Section: Discussionmentioning

confidence: 99%

Vincristine and sirolimus in the treatment of kaposiform haemangioendothelioma

Peng

Yang

et al. 2019

J Paediatrics Child Health

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Aim Kaposiform haemangioendothelioma (KHE) is a rare, potentially life‐threatening vascular tumour that is often associated with thrombocytopenia and coagulopathy, known as the Kasabach‐Merritt phenomenon (KMP). Because of the rarity and complexity of KHE, the optimal paradigm for treating KHE has yet to be elucidated. We aim to assess the efficacy and safety of vincristine and sirolimus for the treatment of KHE. Methods A comprehensive review of the literature was conducted from January 1993 to June 2018. A total of 15 studies were selected for the meta‐analysis. Five studies included 75 individuals and reported the response and side effects to vincristine in the treatment of KHE with or without KMP. A total of 10 studies that included 127 individuals reported the response and safety of sirolimus for treating KHE with or without KMP. Results The pooled odds ratio (OR) for the effectiveness of vincristine was 0.72. The pooled OR for the effectiveness of sirolimus was 0.91. The side effects associated with vincristine during the treatment included neuropathy, abdominal pain, loss of appetite and mild elevations of aspartate aminotransferase (AST) and alanine aminotransferase (ALT). The side effects associated with sirolimus therapy included bronchitis; lymphopenia; elevated AST, ALT and platelets; hyperlipidaemia; opportunistic infection; mild reversible leukopenia; mucositis; fever; pain and skin rash/vomiting and diarrhoea. Conclusions This systematic review showed a high efficacy of vincristine and sirolimus in the treatment of KHE. Based on the available data in the literature, it appears that sirolimus is potentially an efficacious and safe treatment option for KHE. Further randomised, controlled trials are recommended.

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“…Evidence for the efficacy of sirolimus in treatment of KHE is plentiful, albeit no controlled studies exist . However, it is noteworthy that patients should not receive a live vaccine while being treated with sirolimus.…”

Section: Discussionmentioning

confidence: 99%

Development of Kasabach–Merritt phenomenon following vaccination: More than a coincidence?

Chen

Yang

et al. 2018

The Journal of Dermatology

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Kasabach-Merritt phenomenon (KMP) occurred uniquely in patients with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). We report the clinical characteristics of two patients with KHE involving the right upper arm. The patients demonstrated rapid enlargement of the lesion with severe KMP shortly after vaccination. Sirolimus was used to treat the KHE with KMP. The patients showed a quick normalization of the platelet level. The follow-up examination revealed that the size of the mass was significantly decreased. This report raises the intriguing possibility that extrinsic factors may contribute to the development of KMP in the context of an already existing KHE.

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Successful Management of Pancreatic Kaposiform Hemangioendothelioma With Sirolimus

Cited by 19 publications

References 9 publications

Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon

Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon

Vincristine and sirolimus in the treatment of kaposiform haemangioendothelioma

Development of Kasabach–Merritt phenomenon following vaccination: More than a coincidence?

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