“Hemangioendothelioma” is the name given to a group of vascular neoplasms that may be benign or malignant depending on their activity.

These tumors are typically categorized by an enlarged mass found in the head or neck or the soft tissues, such as the intestines, lungs, lymph nodes, pleura, retroperitoneum, or stomach.

These masses have been known to metastasize, or spread, to nearby tissue. Hemangioendotheliomas affect both men and women equally.

There are three common types of Hemangioendothelioma tumors:

• Epithelioid: A malignant mass found in the lining of the blood vessels in the liver and lungs. Extremely rare, affecting only 0.01% of patients with cancer.
• Kaposiform: A rare vascular tumor that usually affects infants and young children
• Retiform: A low-grade tumor that usually develops on the skin and is highly unlikely to become malignant

These masses tend to grow slowly and sometimes go into remission on their own; however, others have been known to recur and display very aggressive behavior and rapid growth.

Signs & Symptoms

Symptoms can vary from patient to patient, but the most common include:

• Weight loss
• Visceral pain
• Enlarged abdominal mass
• Enlarged liver or spleen
• Red skin nodules
• Blue nodules

Diagnosis & Treatment

Patients with these types of tumors should seek treatment from a medical professional who specializes in sarcoma.

These masses are usually detected with by a diagnostic scan, including an x-ray, ultrasound, CT or CAT scan, MRI, or blood test. An angiography—a medical imaging procedure that captures images of the lumen, or inside, of blood vessels and organs of the body, is also sometimes administered.

Treatment depends on the size, location, and stage of the mass. If the mass is benign, the patient’s physician may observe the tumor to see if it metastasizes or becomes malignant; some patients are given steroids as a preventative measure. Surgical excision has been found to be effective for certain malignant tumors; others have responded well to chemotherapy and/or radiation.

Prognosis

Patients should seek medical treatment immediately. Most patients whose disease is treated in the early stages have roughly a 50 percent chance of a 5-year survival rate from the time of diagnosis.