Retiform Hemangioendothelioma - All About It!

Retiform Hemangioendothelioma is a low-grade tumour of the subcutaneous tissues and the skin. The condition is most commonly observed in young adults on the extremities. RH manifests as a subcutaneous nodule or a cutaneous plaque that is either asymptotic or causes local discomfort. 

RH typically occurs in the lower limbs, but recently, few cases have been reported with tumours in the penis, head, and trunk. 

Diagnosing Retiform Hemangioendothelioma - 

The only way to diagnose RH is to undergo a biopsy. It is histologically characterized by long arborizing vascular channels that are lined with hobnail endothelial cells. The vascular channels are arranged in a retiform pattern, which resembles rete testis. 

What is the treatment for this condition?

Treatment for Retiform Hemangioendothelioma is controversial.

Surgery is performed to remove the tissues after a thorough microscopic examination is done to study the manifestation of the disease. However, there is a high tendency for recurrence. Patients are advised to follow-up in the long-term. 

According to studies, radiation therapy has proved successful in two cases where the disease had spread to the lymph nodes. In one of the cases, the patient had lesions over mons pubis with lymph node metastases. Lymph node dissection was used to resect the lesion, and radiotherapy was provided as a post-operative procedure. 

In another case of unresectable RH, the patient was successfully treated with a low-dose of chemotherapy drug and moderate radiotherapy. As a result, the patient was seen remaining free from the disease at 36 months. 

Yet another treatment approach practised by doctors is immunotherapy - the mode of treatment uses the patient’s immune system to fight the disease. 

Therefore, as reports suggest, all three lines of treatment - radiation therapy, surgery, and a combination of both have produced mixed results. 

Retiform Hemangioendothelioma is a rare, low-grade cancerous tumour. It is essential that you distinguish it from its aggressive counterparts, such as angiosarcoma, as they differ markedly in their prognosis and behaviour. It is therefore important that you consult a specialist in this field of medical science, and get a proper diagnosis and appropriate treatment on time.