What is Hemangiopericytoma & How is it Treated?

Hemangiopericytoma is a rare type of tumor, which develops in the soft tissue of the body including fat, tendons, muscles, blood vessels, nerves and other fibrous tissue. Patients in adolescence suffering from Hemangiopericytoma may have a tumor which can be malignant or benign. This tumor is commonly found in bones; however, it can metastasize to the lungs also. Hemangiopericytoma developing in a baby is known as infantile myofibromatosis; where the tumor may usually go away on its own.

The exact cause of the growth of Hemangiopericytoma is not clear. According to research, it is thought that genetic mutations can lead to development of Hemangiopericytoma. Other than this, it is also thought that there can be a possible connection between soft-tissue sarcomas and development of other cancers.

Hemangiopericytoma affects the soft tissue which has flexibility and can be easily removed. A patient can have a tumor for a long time before it gets diagnosed from to its growing size and compression against the surrounding tissue. Symptoms differ from patient to patient and depend on the tumor’s size, location and spread. Some of the common symptoms of Hemangiopericytoma are:

• Development of a painless lump or swelling.
• Patient will experience soreness or pain.
• Patient will have difficulty using legs or feet or will limp when walking.
• Patient will suffer from low blood sugar (hypoglycemia).

Symptoms of Hemangiopericytoma affecting the lungs consist of:

• Pain when breathing.
• Difficulty in breathing.
• Chest pain.
• Coughing with or without blood.
• Night sweats.

Medical history and physical examination of the patient is done.

Biopsy is the conclusive diagnostic tool for diagnosis of not only Hemangiopericytoma but also other cancers. A sample of the affected tissue is taken from the tumor and sent to the lab where it is analyzed microscopically.

Blood tests, such as complete blood count (CBC) and blood chemistry tests are also done for diagnosis.

X-rays are helpful in diagnosis of bone tumors and they are also helpful in distinguishing between benign and malignant bone tumors.

Computerized Tomography Scan (CT scan) is an imaging test which generates detailed images of the body, including the bones, fat, muscles and organs. CT scan gives more detailed images when compared to x-rays and are mainly done to check the chest and lungs for metastatic tumors.

Bone Scan is a nuclear imaging test which helps in detecting bone and metastatic tumors. Bone scans also help in detecting abnormalities in other bones.

Magnetic Resonance Imaging (MRI) Scan is also an imaging test that helps in determining the extent of the tumor or the spread of the cancer within the bone and joint. MRI scan also helps in assessing the relationship of the tumor to the nerves, muscles and blood vessels.

If the Hemangiopericytoma is malignant, then staging of the tumor is done to determine the severity of the cancer and how far the tumor has spread from its original location. The tumor stage and degree of the aggressiveness of the tumor helps in determining the best form of treatment possible for the patient and also gives an idea regarding the patient’s prognosis. A localized Hemangiopericytoma means that the tumor or the cancer hasn’t spread further than its original location or the site at which it has developed.

Treatment of Hemangiopericytoma consists of:

• Surgery: Surgery is done to remove the tumor (Hemangiopericytoma) in its entirety along with the surrounding healthy tissue. Depending on the tumor’s size and location, it may be necessary to remove a part of the limb or the complete limb. Limb-sparing surgery can be done to avoid amputation where all the affected bone and cartilage with some of the surrounding muscle is removed and the nearby nerves, vessels and tendons are saved. A metal prosthesis or a bone graft is used to replace the bone which is removed. Additional surgeries may be needed to replace or repair the rods, which break or become loose. Patients need intensive rehabilitation after undergoing limb-sparing surgery. It can take as long as one year for the patient to regain the complete use of the limb after a limb-sparing surgery. In rare cases, patients who have undergone a limb-sparing surgery may eventually need to have amputation of the limb due to recurrence of the tumor or due to a severe complication.
• Amputation: Amputation is the only surgical option left if the tumor (Hemangiopericytoma ) involves important blood vessels, nerves and cannot be removed. During the surgery for amputation, the doctors ensure that the skin and muscles form a cuff around the bone which is amputated. After a week to 10 days of the surgery, the patient will be fitted for a temporary socket and prosthesis that can be used for two to four months till there is complete healing of the stump. After which a permanent artificial limb is placed.
• Radiation Therapy: Radiation is done in malignant hemangiopericytoma in combination with surgery either before or after the tumor is removed. In rare cases, radiation alone is done for treating primary hemangiopericytoma.
• Chemotherapy: Chemotherapy is also done if Hemangiopericytoma is malignant. Chemotherapy consists of drugs which kill the cancer cells. Most of the times, a combination of chemotherapy drugs are used to fight the cancer cells and to shrink Hemangiopericytoma. Depending on the type of cancer, certain chemotherapy drugs can be given in a specific order. The drugs in chemotherapy don’t differentiate between the cancer cells and normal healthy cells. Due to this, the patient experiences many adverse side effects during the treatment with chemotherapy. Chemotherapy can be given in various forms which include: pill (oral route), injection (intramuscular or intravenous) and intrathecal where the chemotherapy drugs are injected into the spinal column.

The prognosis for Hemangiopericytoma depends on the following factors:

• The location and size of the tumor.
• The absence or presence of metastasis.
• The spread or extent of the Hemangiopericytoma.
• The response of the tumor to treatment.
• Patient’s age and overall health.
• Patient’s ability to tolerate specific therapies, medications or procedures.
• It is important that immediate and aggressive treatment is started for a good prognosis.

The patient needs to visit a cancer survivorship clinic every year where physical exam, laboratory tests and imaging scans are done for:

• Screening to detect early recurrence of cancer.
• Managing any complications.
• Managing late effects of the treatment.

Also Read:

• What is Fibrous Histiocytoma