A case report of Kaposiform haemangioendothelioma; response with propranolol and steroids

Saurav Verma,Adarsh Barwad,Venkatesan S Kumar,Sameer Rastogi,Ekta Dhamija

doi:10.1186/s13569-020-00134-8

Abstract

BackgroundKaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone. It is a locally aggressive tumor usually seen in infants. Here we report a case of kaposiform hemagioendothelioma in a child who responded to propranolol and steroids.Case presentationA 3-year-old male child presented with a swelling below his right knee with characteristic violet skin lesion. There was no evidence of Kasabach–Merritt phenomenon. After no improvement with several attempts at debridement and anti-tubercular treatment; a diagnosis of Kaposiform Haemangioendothelioma was reached on the basis of overall clinical picture and histology. The child was treated with propranolol and steroids and had an excellent clinical response and a near complete resolution on imaging at 5 months.ConclusionsThese cases are often misdiagnosed and despite a delay in diagnosis have good outcomes with appropriate multimodality management. This case highlights the unique and typical characteristics of kaposiform haemangioendothelioma.

Highlights

Kaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone
When tumor is unresectable or surgery is associated with unacceptable morbidity other treatment modalities like angiography followed by embolization, radiotherapy, vincristine chemotherapy, pharmacotherapy with propranolol, steroids or sirolimus have been used with varying rates of success [5,6,7,8,9]
There was no evidence of Kasabach–Merritt phenomenon (KMP), profound thrombocytopenia resulting from intralesional platelet trapping, in our patient

Summary

Introduction

Kaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone. Background Kaposiform haemangioendothelioma (KH), first described by Zuckerberg et al in 1993, is a rare vascular tumor and may involve skin, deep soft tissue or bone [1]. When tumor is unresectable or surgery is associated with unacceptable morbidity other treatment modalities like angiography followed by embolization, radiotherapy, vincristine chemotherapy, pharmacotherapy with propranolol, steroids or sirolimus have been used with varying rates of success [5,6,7,8,9]. A radiograph of right leg showed lytic sclerotic destructive lesion with midzone of transition seen involving right proximal tibia and fibula with soft tissue component (Fig. 1c).

Results

Conclusion