Multimodal treatment of Kasabach-Merritt syndrome arising from tufted angioma: A case report.

Kasabach-Merritt syndrome (KMS) is a rare type of vascular tumor associated with a severely decreased platelet count. No standard guidelines for the treatment of the disease have been established so far. In the present study, a 1-year-old pediatric patient with KMS arising from tufted angioma was successfully and variously treated with steroids, vincristine, surgery and propranolol for 18 months. Systemic steroids stabilized the platelet count stable, while vincristine reduced the size of the tumor. Due to unpredictable response, the patient was operated. Combination of vincristine and propranolol was introduced post-surgery to improve the severely low platelet count of the patient. Following multimodal therapy for 18 months, there has been no evidence of recurrence or metastasis during 2 years of follow-up. Currently, the patient is alive and well. The management of KMS presents a challenge, and well-designed studies are required to clearly determine the benefits and risks of multidisciplinary treatment.