Chavez ‘s mystery cancer: metastatic rhabdomyosarcoma

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    Venezuela’s President Hugo Chavez is reportedly suffering from metastatic rhabdomyosarcoma (RMS), a rare soft tissue cancer diagnosed usually in children and teens, with very few cases in adults over the age of 30.

    Retired anchor Dan Rather has reported the news, although these have not been officially confirmed by Venezuela’s government officials.

    How serious is Chavez’s condition?

    • Rhabdomyosarcoma: a rare but aggressive cancer

    According to the Sarcoma Foundation of America, rhabdomyosarcoma is most often found in children and teenagers. It is a malignant soft tissue tumor that originates from skeletal muscle cells and in average affects more males than females.

    More than half of the reported cases occur in children under the age of 10, and this form of cancer represents approximately 4 to 8 percent of all malignant diseases in children under the age of 15.

    In this photo released by Miraflores Press Office, Venezuela’s President Hugo Chavez speaks as he leads a televised Cabinet meeting at Miraflores presidential palace in Caracas, Venezuela, Tuesday, May 22, 2012. Chavez has reappeared on television a week and a half after returning from cancer treatment in Cuba. (AP Photo/Miraflores Press Office, Marcelo Garcia)

    Adult RMS, however, is very uncommon with a total of 400 documented cases in the United States and Europe during the last 30 years.

    The tumor often develops in specific areas like the head, neck, genital area, prostate, and bladder, though it can appear in any part of the body. It is often painless, but some patients may experience bleeding or pain at the tumor’s location.

    Chavez’s cancer seems to be located mostly in the pelvic area, according to his own initial reports, although based on Rather’s report, it would now be in the terminal state.

    The National Center for Biotechnology Information reports that symptoms of RMS vary significantly based on their location in the body, but usually include:

    • Painful muscle lumps often mistaken for an injury.
    • Tumors in the nose or throat can cause trouble swallowing, congestion, bleeding, or neurological issues.
    • Eye tumors can impair vision, cause eye bulging, and cause pain or swelling.
    • Bladder and vaginal tumors may cause trouble urinating, incontinence, and trouble moving the bowels.
    • Ear tumors may cause hearing loss, pain, or swelling.

    RMS tumors can spread locally, regionally, or distantly, according to the  Liddy Shriver Sarcoma Initiative.

    • Local tumors spread to tissue within the  immediate vicinity of where the cancer started.
    • Regional tumors have traveled to the lymph nodes in the area where the mass originated.
    • Distant tumors have traveled through the bloodstream to another area within the body, often the bones, bone marrow, or the lungs.
    • What does this mean for Chavez?

    If indeed Chavez’s cancer has metastasised, it probably went through all these stages without being detected during the first two:

    Stage I: Localized tumor can be identified and can be removed with surgery. If it is not, it progresses to stage II.

    Stage II: Tumor is still localized and has not spread to lymph nodes or other organs. Size has increased but remains smaller than 2 inches. It can be removed with surgery but microscopic residue could stay in the body and regenerate the tumor.

    Stage III: Tumor is now larger than 2 inches and might have spread to lymph nodes. It is more difficult to completely remove with surgery and tumors tend to reappear in the same area.

    Stage IV: Cancer has affected other areas in the body (spread) and the only option to control it is chemo and radiation therapy.

    Treatment for this form of cancer involves chemotherapy and surgical removal of the tumor in combination with radiotherapy.

    Chavez has reportedly received surgical intervention in Cuba, as early as last June. However, he announced in February this year that a new tumor had developed in the same pelvic area and another operation was required. At the beginning of May, he traveled to Cuba once more for surgery and chemotherapy.

    • What could be the prognosis for Chavez?

    Due to the rarity of adult RMS, little clinical data exists on the condition, but the information available indicates adults have poorer outcomes compared to children.

    The American Cancer Association states that within the RMS high-risk group, or among people with metastasized tumors, a 5-year survival rate could be expected for 20 to  40 percent of the patients, and depending on where the cancer is located. Patients with minimally spread RMS (between 1 to 2 new locations) are more likely to be placed in the 40 percentile group.

    Aggressive treatment for RMS is necessary, and successful treatment will depend on the location and size of the tumor. Metastasized RMS carries a significantly poorer prognosis. Accordingly, if indeed Chavez’s cancer has spread to other organs, it is now inoperable and less responsive to chemo and radiation therapy.

    At this time, Rather’s report claims Chavez might only have a few months to live.

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