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Outcome of radiation therapy for patients with Kasabach-Merritt syndrome
N Mitsuhashi1, M Furuta, H Sakurai
1Department of Radiology and Radiation Oncology, Gunma University School of Medicine, Japan.
Insights
Radiation therapy effectively treats Kasabach-Merritt syndrome, improving hemangiomas and platelet counts in neonates. While risks exist, all patients survived with no residual abnormalities.
Area of Science:
- Pediatric Oncology
- Radiation Oncology
- Hematology
Background:
- Kasabach-Merritt syndrome involves large hemangiomas causing consumption coagulopathy.
- The efficacy of radiation therapy for this condition is debated.
- This study evaluates radiation therapy outcomes in neonates with Kasabach-Merritt syndrome.
Purpose of the Study:
- To retrospectively investigate the treatment outcomes of radiation therapy for Kasabach-Merritt syndrome in neonates.
- To assess the efficacy of radiation in managing hemangioma size and associated coagulopathy.
Main Methods:
- Retrospective analysis of seven neonates diagnosed with Kasabach-Merritt syndrome.
- Treatment involved radiation therapy with a total dose of 8-10 Gy, delivered at 1 Gy/day, five times weekly.
- Patients ranged from 1 day to 5 months old, with hemangiomas often in extremities and initial platelet counts below 40,000/mm³.
Main Results:
- Four out of seven hemangiomas showed dramatic response with improved platelet counts.
- Remaining hemangiomas showed reduced tension, and disseminated intravascular coagulopathy improved with extended radiation courses.
- All seven patients survived with no evidence of hemangioma or hematological issues; extremity shortening occurred in three patients.
Conclusions:
- Radiation therapy is a viable and effective treatment for Kasabach-Merritt syndrome.
- Potential risks include growth delay and secondary malignancy.
- Successful outcomes were achieved in all studied neonates.
Purpose:
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 And Materials:
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.
