A Case of Superficial Kaposiform Hemangioendothelioma Treated with Oral Propranolol Combined with Topical Sirolimus

Abstract

Kaposiform hemangioendothelioma(KHE) without Kasabach-Merritt phenomenon is a rare tumor primarily observed in pediatric patients; however, its documentation in the literature remains limited. We reported about a 1-year-old boy diagnosed with superficial KHE who received oral propranolol in combination with topical sirolimus and reviewed relevant reports and treatment of superficial KHE.

Keywords: case treatment; pediatric patients; rare tumor; superficial kaposiform hemangioendothelioma.

Figure 1

(a) Prior to treatment. (b) The plaques were lighter in color after one year of treatment.

Figure 2

(a) B-ultrasound examination showed hyperechoic area with vascular echo. (b) Follow-up B-ultrasound showed scattered hyperechoic areas with a little blood flow signals.

Figure 3

(a) Histopathological showing focal, well-circumscribed nodules comprised of atypical spindle cells (HE, ×50). (b) The spindle-shaped endothelial cells aligned to form a slit-like vascular channels (arrow, HE, ×100). (c) The proliferated vessels were positive for CD31(CD31, ×50). (d) The proliferated vessels were positive for CD34(CD34, ×50). (e) The proliferated vessels were negative for GLUT-1 (GLUT-1, ×50). (f) D2-40 staining was negative in the center of the lesion and positive in the surrounding dilated lymphatic vessels (D2-40, ×50).