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.

PROCEDURE:

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: Zuopeng Wang, Kai Li, Wei Yao, Kuiran Dong, Xianmin Xiao, Shan Zheng
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 62 Issue 4 Pg. 577-80 (Apr 2015) ISSN: 1545-5017 [Electronic] United States
• PMID: 25346262 (Publication Type: Evaluation Study, Journal Article)
• Copyright: © 2014 Wiley Periodicals, Inc.

Chemical References

• Antineoplastic Agents, Phytogenic
• Steroids
• Vincristine

Topics

• Antineoplastic Agents, Phytogenic (administration & dosage)
• Drug Resistance, Neoplasm (drug effects)
• Female
• Follow-Up Studies
• Hemangioendothelioma (blood, drug therapy, pathology)
• Humans
• Infant, Newborn
• Kasabach-Merritt Syndrome (blood, drug therapy, pathology)
• Male
• Platelet Count
• Retrospective Studies
• Sarcoma, Kaposi (blood, drug therapy, pathology)
• Steroids (administration & dosage)
• Time Factors
• Vincristine (administration & dosage)