Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options.
BACKGROUND
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor affecting infants and young children. Although benign, it can be associated with an aggressive locally growing tumor and/or a life-threatening Kasabach-Merritt phenomenon (KMP). To date, only reviews of limited cases have been performed. We, therefore, conducted a comprehensive literature search to collect relevant data and make recommendations for future treatment trials.


METHODS
Review of the available literature between 1993 and 2017 revealed a total of 105 publications involving 215 patients of less than 21 years of age. To this, we added 12 from our department and 4 from the Cooperative Weichteilsarkomstudie database.


RESULTS
We found that KMP was present in 79% of the infants, in 47% of the 1-5-year olds, in 43% of the 6-12-year olds, and in 10% of the 13-21-year-old patients. KMP was present in nearly all (94%) patients with retroperitoneal tumors and in all patients with extra-regional tumors. The median size of a KHE without KMP was 12 cm2 as compared to 49 cm2 when associated with a KMP. With complete (not further classifiable if R0 or R1) resection, all patients were cured. If inoperable, response regarding KMP/regression of tumor size was seen in 29/28% with steroid-, 47/39% with vincristine-, 44/43% with interferon alpha-, 65/61% with anti-platelet agents-, and in 97/100% with sirolimus-containing therapies.


CONCLUSIONS
Patients with progressive KHE should undergo resection whenever it is considered a safe option. If inoperable, sirolimus should be the first choice for treating KMP and reducing tumor size.
['Schmid|Irene|I|', 'Klenk|Anne K|AK|', 'Sparber-Sauer|Monika|M|', 'Koscielniak|Ewa|E|', 'Maxwell|Rebecca|R|', 'Häberle|Beate|B|']
D000293:Adolescent D017668:Age of Onset D002423:Cause of Death D002648:Child D002675:Child, Preschool D015331:Cohort Studies D003131:Combined Modality Therapy D016208:Databases, Factual D004621:Embolization, Therapeutic D005260:Female D005858:Germany D006390:Hemangioendothelioma D006801:Humans D007223:Infant D059885:Kasabach-Merritt Syndrome D008297:Male D011878:Radiotherapy D012189:Retrospective Studies D018570:Risk Assessment D012514:Sarcoma, Kaposi D020123:Sirolimus D018709:Statistics, Nonparametric D016019:Survival Analysis D016896:Treatment Outcome D019043:Vascular Neoplasms D055815:Young Adult
Kaposiform hemangioendothelioma Kasabach–Merritt phenomenon Pediatric