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The use of mTOR inhibitors for the treatment of kaposiform hemangioendothelioma. A systematic review.
Maza-Morales, Mariana; Valdés-Loperena, Sofía; Durán-McKinster, Lourdes Carola; García-Romero, María Teresa.
Afiliação
  • Maza-Morales M; Dermatology Department, National Institute of Pediatrics, Mexico City, Mexico.
  • Valdés-Loperena S; Dermatology Department, National Institute of Pediatrics, Mexico City, Mexico.
  • Durán-McKinster LC; Dermatology Department, National Institute of Pediatrics, Mexico City, Mexico.
  • García-Romero MT; Dermatology Department, National Institute of Pediatrics, Mexico City, Mexico.
Pediatr Dermatol ; 40(3): 440-445, 2023.
Article em En | MEDLINE | ID: mdl-36716766
BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a locally aggressive and potentially lethal vascular tumor of infancy. Current consensus recommendations include the use of vincristine and/or systemic steroids as first-line treatment. Mammalian target of rapamycin (mTOR) inhibitors represent a promising therapy for patients with KHE. The goal of our study is to critically assess the existing literature on outcomes of patients with KHE treated with mTOR inhibitors. METHODS: We conducted a literature search from 1 January 2000, to 30 April 2022. Articles reporting outcomes of patients treated with mTOR inhibitors for KHE were included. Descriptive statistics were used to describe and summarize the outcomes of the treatment. RESULTS: We included 327 patients with a mean age at diagnosis of 9.1 months (SD ± 9). Patients were treated with an mTOR inhibitor for a mean of 15.2 months (SD ± 4.1). A total of 315 (96.3%) patients had positive outcomes including improvement of the tumor size, symptoms and/or laboratory parameters in 227 (85%) and complete remission in 38 (12%). Seven (2%) patients did not respond to treatment and seven (2%) died of sepsis (4), Kasabach-Merritt phenomenon complications (1), cardiac and liver failure due to ductus arteriosus (1), or metastatic disease (1). CONCLUSION: This systematic review supports the efficacy and safety of mTOR inhibitors for KHE. Their use resulted in positive outcomes in terms of decreased symptoms, reduction in tumor size and improvement in biochemical parameters with a mortality rate of 2%. According to these findings, we suggest revised consensus treatment guidelines for KHE with mTOR inhibitors potentially considered first-line therapy.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Sarcoma de Kaposi / Síndrome de Kasabach-Merritt / Hemangioendotelioma Tipo de estudo: Diagnostic_studies / Guideline / Systematic_reviews Limite: Humans / Infant Idioma: En Revista: Pediatr Dermatol Ano de publicação: 2023 Tipo de documento: Article País de afiliação: México País de publicação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Sarcoma de Kaposi / Síndrome de Kasabach-Merritt / Hemangioendotelioma Tipo de estudo: Diagnostic_studies / Guideline / Systematic_reviews Limite: Humans / Infant Idioma: En Revista: Pediatr Dermatol Ano de publicação: 2023 Tipo de documento: Article País de afiliação: México País de publicação: Estados Unidos