kaposiform haemangioendothelioma

Last reviewed 01/2018

It is a rare, locally aggressive vascular tumour that is often associated with the Kasabach-Merritt syndrome.

Children and neonates are predominantly affected by this condition (1).

The tumour is usually solitary and occurs in the proximal arms and legs and the trunk (including the retroperitoneum). Craniofacial lesions are rare. Invasion of nearby organs is possible (1).

The lesion is characterised by rapid growth and variable shrinkage.

It doesn't regress spontaneously.

It manifests as a slightly raised subcutaneous mass and may appear purple and shiny with an advancing ecchymotic margin. Lesions are large in size (>5 cm in diameter). Tumours greater than 10 cm in diameter may be associated with thrombocytopaenia (1).

Kaposiform haemangioendotheliomas may be associated with lymphatic anomalies (2).

Kaposiform haemangioendothelioma should be differentiated from infantile haemangioma. Diagnosis is assisted by MRI (1).

Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon is associated with high mortality. Death may be caused by haemorrhage, sepsis, or invasion of vital structures (1).

Treatment options include corticosteroids, interferon alfa-2a, vincristine and cyclophosphamide. Excision, arterial embolization, and radiotherapy have all been used to treat the condition. Heparin is contraindicated (1).

Reference:

• 1. Mulliken JB et al. Case 13-2004. A newborn girl with a large cutaneous lesion, thrombocytopenia, and anemia. N Engl J Med. 2004;350(17):1764-75.
• 2. Garzon MC et al. Vascular malformations: Part I. J Am Acad Dermatol. 2007;56(3):353-70