title: Steroid-resistant kaposiform hemangioendothelioma: a retrospective study of 37 patients treated with vincristine and long-term follow-up.
abstract: BACKGROUND
  Kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP) still remains a particular and life-threatening disease. The purpose of this study was to evaluate the efficacy of vincristine (VCR) and the possibility of replacement with steroids in the treatment of steroid-resistant KHE with KMP.
  
  
  METHODS
  We retrospectively reviewed the medical records of 37 patients with steroid-resistant KHE who were treated at the Children's Hospital of Fudan University between March 2003 and March 2013.
  
  
  RESULTS
  The age of initial diagnosis with KHE was between 1 day and 10 months. Eight and 29 cases were located in the superficial and deep soft tissues, respectively. Thirty-seven KHE lesions did not respond well to steroids before starting VCR treatment. Twenty-six KHE lesions achieved complete remission, with platelet counts reaching normal levels within7.6 ± 5.2 weeks after VCR treatment. The vascular tumor began to decrease in size or soften at an average of 4.9 ± 2.7 weeks. Two KHE lesions had partial responses and one remains in treatment. Eight KHE lesions had no apparent response to VCR and thus received other therapies. Twenty-eight patients have ended treatment with VCR; the average length of treatment was 31.2 ± 5.9 weeks. Side effects occurred in 48.6% of patients who received steroids, and in 11.4% of patients who received VCR treatment. The mean follow-up time was 3.5 years. No recurrences have been reported.
  
  
  CONCLUSIONS
  VCR appears to be a safe and effective treatment option in the management of steroid-resistant KHE with KMP, and recommended as first-choice treatment.
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
authors.forename: Zuopeng
authors.initials: Z
authors.lastname: Wang
authors.forename: Kai
authors.initials: K
authors.lastname: Li
authors.forename: Wei
authors.initials: W
authors.lastname: Yao
authors.forename: Kuiran
authors.initials: K
authors.lastname: Dong
authors.forename: Xianmin
authors.initials: X
authors.lastname: Xiao
authors.forename: Shan
authors.initials: S
authors.lastname: Zheng
mesh_terms: D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage*; D019008:Drug Resistance, Neoplasm / Q000187:drug effects*; D005260:Female; D005500:Follow-Up Studies; D006390:Hemangioendothelioma / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D059885:Kasabach-Merritt Syndrome / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D010976:Platelet Count; D012189:Retrospective Studies; D012514:Sarcoma, Kaposi / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D013256:Steroids / Q000008:administration & dosage*; D013997:Time Factors; D014750:Vincristine / Q000008:administration & dosage*
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D013256:Steroids; D014750:Vincristine
keywords: K-M phenomenon; VCR; kaposiform hemangioendothelioma; treatment