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

Saurav Verma1, Ekta Dhamija2, Adarsh Barwad3

  • 1Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.

Clinical Sarcoma Research
|August 9, 2020
PubMed

Insights

Kaposiform haemangioendothelioma, a rare vascular tumor, can be effectively treated with propranolol and steroids. This case study shows a child with kaposiform haemangioendothelioma achieving excellent response and near-complete resolution with this treatment.

Area of Science:

  • Vascular Oncology
  • Pediatric Oncology
  • Dermatology

Background:

  • Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor typically affecting infants.
  • KHE can involve skin, deep soft tissue, or bone, presenting diagnostic challenges.

Observation:

  • A 3-year-old male presented with a swelling below the right knee and a characteristic violet skin lesion.
  • Initial treatments including debridement and anti-tubercular therapy were ineffective.
  • Diagnosis was confirmed via clinical presentation and histology, ruling out Kasabach-Merritt phenomenon.

Findings:

  • Treatment with propranolol and steroids resulted in an excellent clinical response.
  • Imaging at 5 months post-treatment showed near-complete resolution of the kaposiform haemangioendothelioma.

Implications:

  • This case underscores the importance of recognizing KHE's unique characteristics for timely diagnosis.
  • Multimodality management, including propranolol and steroids, offers favorable outcomes for KHE, even after diagnostic delays.
  • Highlights potential therapeutic options for challenging pediatric vascular tumors.
Abstract

Keywords:
Kaposiform hemangioendotheliomaPropranololSteroids

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