Fig. 8
From: Kaposiform hemangioendothelioma: current knowledge and future perspectives
Congenital KHE associated with KMP on the right face. Congenital KHEs with KMP likely represent a period of temporary and partial remission shortly after birth. The signs and symptoms may alleviate spontaneously. However, rebound growth of the lesions accompanying severe KMP would reoccur within the next several days or weeks. a The parents’ photograph revealed a bluish, swollen and firm vascular mass on the right face after birth (2 days of age). Her platelet count was 7 × 109/L. Without any special treatment, the tumor became soft and was stagnant in size. Her platelet count reached a highest value of 161 × 109/L (b: 1 week of age, c: 6.5 weeks of age). Subsequently, however, the tumor became progressively enlarged and displayed obvious ecchymosis (d: 8 weeks of age). The patient’s platelet count dropped to 3 × 109/L. She was treated with a combination therapy of sirolimus (0.8 mg/m2 administered twice daily) and prednisolone (2 mg/kg/d). One (e) and 4 weeks (f) after treatment. Within 10 days of combination therapy, the girl’s platelet level normalized. The prednisolone was tapered and discontinued within the following 4 weeks, and sirolimus was continued. G, Twelve months after treatment. H, Photograph at 26 months of age (24 months of treatment) demonstrates a nearly complete involution of the lesion. Sirolimus was then tapered and discontinued