title: Outcome of radiation therapy for patients with Kasabach-Merritt syndrome.
abstract: OBJECTIVE
  The efficacy of radiation therapy for Kasabach-Merritt syndrome, which is characterized by a huge hemangioma with consumption coagulopathy, remains controversial. In this study, we retrospectively investigated the treatment outcome of radiation therapy for seven neonates with Kasabach-Merritt syndrome.
  
  
  METHODS
  During the past 25 years we have seen seven children with Kasabach-Merritt syndrome who were treated with radiation therapy. Their ages ranged from 1 day to 5 months, with a median age of 1 month. The hemangioma was located in the extremities in four of seven children. Tumor sizes ranged from 70 cm to more than 150 cm in greatest diameter. Initial platelet counts were all less than 40,000/mm3 except for one patient. In principle, the total dose applied to the hemangioma was 8-10 Gy, with a daily dose of 1 Gy five times a week.
  
  
  RESULTS
  Four of seven hemangiomas responded dramatically, with a concomitant rise of the platelet count to radiation therapy. Although the remaining three hemangiomas, all of which were ill circumscribed by widespread overlying shiny, dusky purple skin, became less tense during radiation therapy. Disseminated intravascular coagulopathy was not improved, but they have responded favorably to two or three courses of radiation therapy with an extended radiation field by 1.5 years of age. As a result, all seven patients are now surviving with no evidence of hemangioma or hematological abnormalities. Shortening of the extremity was observed in three patients who received multiple courses of radiation therapy.
  
  
  CONCLUSIONS
  Radiation therapy appears to be one of the effective treatment options for Kasabach-Merritt syndrome despite the risk of growth delay and malignancy.
authors.affiliation: Department of Radiology and Radiation Oncology, Gunma University School of Medicine, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Mitsuhashi
authors.forename: M
authors.initials: M
authors.lastname: Furuta
authors.forename: H
authors.initials: H
authors.lastname: Sakurai
authors.forename: T
authors.initials: T
authors.lastname: Takahashi
authors.forename: S
authors.initials: S
authors.lastname: Kato
authors.forename: M
authors.initials: M
authors.lastname: Nozaki
authors.forename: Y
authors.initials: Y
authors.lastname: Saito
authors.forename: K
authors.initials: K
authors.lastname: Hayakawa
authors.forename: H
authors.initials: H
authors.lastname: Niibe
mesh_terms: D004211:Disseminated Intravascular Coagulation / Q000097:blood / Q000532:radiotherapy*; D005260:Female; D006391:Hemangioma / Q000097:blood / Q000532:radiotherapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010976:Platelet Count / Q000528:radiation effects; D011878:Radiotherapy / Q000009:adverse effects; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome