This condition is a rare cancerous tumor composed of cells that make up the blood vessels, called endothelial cells. It is primarily seen in the lungs. 

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What is a Hemangioendothelioma?

A hemangioendothelioma is a cancerous tumor of bone, made of cells named endothelial cells. There is a low rate of metastasis, or spreading, to other parts of the body. 

Who is usually affected?
Who is usually affected?
  • • This condition is very rare, with only one in one million people diagnosed worldwide.
  • • There is a wide distribution of age, ranging from 5-70 years old. 
  • • The majority of cases occur between 15 to 40 years of age.
  • • There is a slight male dominance, with a male to female ratio of 1.5:1. 
  • • Most hemangioendotheliomas have a genetic mutation, called a chromosomal translocation. This is when chromosomes break apart and combine together again in the wrong way. 
  • • The WWTR gene and the CAMTA1 gene are associated genetic markers. The YAP gene and the TFE3 gene are associated genetic markers but less common. 
Common Bones Involved
Common Bones Involved
  • • Most commonly seen in the skull, spine, ribs and lower extremity.
Signs and Symptoms
Signs and Symptoms
  • • Pain and swelling.
Biological Behavior
Biological Behavior
  • • There is no heredity component to this condition, so it is not passed down in families. 
  • • There is a low rate of metastasis, or spreading, to other parts of the body. 
  • • This cancer is caused by uncontrolled cell growth in the cells that make up the blood vessels, called endothelial cells. 
  • • A very rare type of cancer. 
  • • The work-up often consists of a physical examination, X-rays, CT scans, MRI, and sometimes bone scans are required. 
  • • CT of the chest is necessary to check for spread, or metastasis, of the cancer.
  • • The diagnosis is often confirmed with a biopsy, which means taking a sample of tumor and having it analyzed under a microscope by a pathologist.

Risk to your limbs

Hemangioendotheliomas are cancerous aggressive tumors that, if left unchecked, will grow and destroy your normal bone. Clinically, local pain and swelling may be the first signs of a growing hemangioendothelioma. As the tumor slowly grows, the bone is weakened and you are at an increased risk of breaking the bone due to the tumor (called a pathological fracture). They may also spread to your lungs or other bones.

Radiographic imaging is used to help form a diagnosis. These include X-Ray, MRI, CT and Bone Scans

An example of a X-Ray is shown.


Treatment of Hemangioendothelioma

Limb sparing surgery is the preferred treatment. In low-grade lesions, the standard of treatment is commonly curettage, or scooping out the tumor with a instrument called a curette. This is often sufficient for curing the disease, if the cancer has not spread to other parts of the body (metastasis). Other treatments include surgical ablation and radiation. 

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Hey! I am Dr. Wittig.

Myself and my amazing team are dedicated to saving your life and your limb. Losing a limb because of a tumor can be a terrifying experience. But, it does not have to be the only option. I’ve spent 20+ years as a Board-Certified Orthopedic Surgeon and Orthopedic Oncologist.

I’ve devoted my career to helping children and adults afflicted with bone and soft tissue masses by performing complex limb saving surgeries. Most patients can have their limb saved, which may require innovative techniques.

Patients afflicted with musculoskeletal tumors have complex conditions that are best taken care of at large hospitals. I am the Chairman of Orthopedics and Chief of Orthopedic Oncology at Morristown Medical Center. My philosophy is a multidisciplinary team approach, working together to tailor treatment to individual patients. Education and research are essential to my practice, providing the best setting for extraordinary patient care. Because of this, we have some of the top results in the country.