KHE and Tufted Angioma

  • What is a KHE?

    KHE stands for ‘Kaposiform Haemangioendothelioma’.  It is a rare tumour that occurs in infants and young children.  It has a distinctive appearance that doctors who work in this area can usually recognise, but they can be mistaken for haemangiomas of infancy, particularly by clinicians who don’t see them very often.  They can sometimes be quite large, and can continue to grow much more than would be expected for a haemangioma.    KHEs are generally more worrying than haemangiomas.   When treated in a specialist centre, most children survive and recover from a KHE but this was not generally the case in the past.  . The biggest concern is that the platelet count in children with KHE can drop dangerously low (platelets are parts of the blood which are essential for blood clotting).  If this is not recognised children can be at risk of internal bleeding, so if a KHE is suspected, a blood test needs to be performed.

    KHE’s must be treated in a specialised centre with expertise in the management of vascular anomalies.  They are often treated in consultation with an oncology (cancer) clinic.  Even though KHE is not a cancer because it does not spread to other parts of the body, the experience of the oncologist in drug treatments can be very helpful.

    What is a Tufted Angioma?

    Tufted angioma is a condition which is very similar to KHE, although they tend to be smaller and nearer the surface.  They  are less likely to have problems with low platelet count.

    How are KHE and Tufted angioma diagnosed?

    Although the diagnosis may be suspected based on the appearance, it is very important to be certain that the diagnosis is correct.  The first investigation is usually an ultrasound, which is followed by an MRI scan.  Most often a definitive diagnosis will require a tissue biopsy which requires a small operation under general anaesthetic.

    What treatments are available for KHE and Tufted Angioma?

    The usual treatment for both these conditions is drug therapy.  Treatment is not always required for small lesions but even if treatment is not commenced, regular reviews are required to check on progress.

    The most common drugs used to treat KHE and Tufted angioma are steroids and vincristine, which needs to be given by a special indwelling intravenous catheter. There are new drug treatments available for this condition which appear to be very promising. A trial of the drug Rapamycin (sirolimus) in the treatment of KHE is underway in several centres including Melbourne. Propranolol is of no use in treating KHE or Tufted angioma.