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Rhabdomyosarcoma
Disease information
Overview
Rhabdomyosarcoma is a serious kind of cancer that starts in the cells that are supposed to grow into the body's skeletal muscles—the ones we can control to move around. This type of cancer is most often found in the body's soft tissues. It's actually the most common kind of soft tissue sarcoma in kids and teenagers, making up about 60-70% of these cancers in young people. It mostly shows up in two age groups: little kids between 2 and 6 years old, and then again in teenagers. For the younger kids, it usually pops up in the head and neck area or in girls' urinary and reproductive systems. Teenagers, on the other hand, tend to get it in their arms and legs, their body's main trunk, or, for boys, in the urinary and reproductive systems.
Causes and Symptoms
We're not quite sure what causes rhabdomyosarcoma, but kids with certain genetic conditions seem to have a higher chance of getting it than other kids. These conditions include:
- Li-Fraumeni syndrome
- Costello syndrome
- Gorlin syndrome (also known as basal cell nevus syndrome)
- Type I neurofibromatosis (von Recklinghausen disease)
- Beckwith-Wiedemann syndrome
- Hereditary retinoblastoma
- Rubinstein-Taybi syndrome
Since rhabdomyosarcoma can pop up almost anywhere in the body, the symptoms really depend on where it starts growing and how big it gets. Here's how it usually breaks down: About 35% of the time, it's in the head and neck area. Around 25% show up in the urogenital tract. Roughly 20% occur in the limbs. And about 10% in the trunk.
If the tumor is in the head or neck, it might block the nose, push on the eye (that's called proptosis), cause droopy eyelids (ptosis), change vision, cause headaches, lead to facial nerve paralysis, or even cause hearing loss. When it's in the arms or legs, you might feel a lump. And if it's in the urogenital area, symptoms can include blood in the urine (hematuria), pain when peeing (dysuria), and getting infections like urethritis and cystitis more often.
Treatment
Treating rhabdomyosarcoma involves a team effort, combining surgery, chemotherapy, and radiation therapy, all with the goal of getting rid of the cancer while keeping as much normal function as possible. Here's what doctors look at to figure out the best treatment plan: Can the whole tumor be taken out with surgery? Has the cancer spread to other parts of the body when it's first found? Where exactly is the tumor? Is there a particular genetic change in the cancer cells, like the PAX/FKHR gene translocation? What does the staging tell us about how advanced the cancer is? How does everything look after surgery?
The ideal scenario is to completely remove the tumor with surgery without losing the body part's normal function. If the tumor can't be totally removed, then radiation therapy steps in to target what's left. And chemotherapy is there to shrink the tumor before surgery and also to go after any cancer cells that have spread elsewhere in the body. All three of these treatments are part of a well-thought-out plan that's tailored to each child's specific situation.