Rhabdomyosarcoma in Childhood (Soft Tissue Sarcoma in Kids)

Rhabdomyosarcoma in Childhood (Soft Tissue Sarcoma in Kids)

Overview of Sarcoma

Soft tissue sarcoma (STS) is a rare type of cancer that originates in tissues that support and connect the body, such as fat, muscles, nerves, blood vessels, tendons, and lymphatic vessels.

Sarcomas can be benign (non-cancerous) or malignant (cancerous). Although they account for only 1% of all cancers, they require specific treatments and follow-up care.

What is Rhabdomyosarcoma?

Rhabdomyosarcoma is a type of soft tissue sarcoma that develops in skeletal muscles—voluntary muscles that control body movements. Though it can occur anywhere in the body, it is most commonly seen in children and adolescents.

Common Locations of Rhabdomyosarcoma

• Head and neck (40% cases):

• Parameningeal sites (near brain): 25%

• Eye socket (orbit): 8%

• Other head/neck regions: 7%

• Genitourinary system (30% cases): Bladder, reproductive organs

• Limbs (arms/legs): 15% cases

• Other areas: Trunk, chest, abdomen, pelvis, and biliary tract

Types of Rhabdomyosarcoma

• Favourable Tumors:

• Embryonal Rhabdomyosarcoma: Most common; seen in head, neck, bladder, and reproductive organs.

• Subtypes: Botryoid and Spindle Cell Rhabdomyosarcoma

• Unfavourable Tumors:

• Alveolar Rhabdomyosarcoma: Aggressive; occurs in limbs or trunk.

• Pleomorphic and Undifferentiated Rhabdomyosarcoma: Rare in children but common in young adults.

Statistics

• 400 to 500 cases are reported annually in the United States.

• 5-year survival rate: 71% for children under 15 years old.

Risk Factors

Though the exact cause is unknown, some genetic conditions increase the risk of rhabdomyosarcoma:

• Li-Fraumeni Syndrome

• Beckwith-Wiedemann Syndrome

• Neurofibromatosis

• Costello Syndrome

• Cardio-facio-cutaneous Syndrome

Symptoms of Rhabdomyosarcoma

The symptoms vary based on the tumor’s location:

• Nose: Bleeding, sinus-like symptoms

• Ear: Pain, discharge, or bleeding

• Eye socket: Swelling or crossed eyes

• Urinary/Reproductive organs:

• Blood in urine

• Difficulty urinating

• Vaginal bleeding or lump in testicles

• Abdomen/Pelvis: Pain, vomiting, constipation

• Muscles (Arms/Legs): Painful or painless lumps

Diagnostic Methods

• Physical examination

• Biopsy: Removes tissue sample to confirm cancer.

• Immunocytochemistry Test: Checks for proteins like actin, desmin, MyoD-1, and myogenin.

• Genetic Testing (FISH Test): Detects chromosome translocations.

• Bone Marrow Biopsy: Checks if cancer has spread to the bone marrow.

• Imaging Tests:

• X-ray

• CT Scan

• MRI

• Bone Scan

• PET-CT

Treatment Options

1. Surgery

• Removes the tumor and surrounding healthy tissue (margin).

• If the tumor is large or not completely removable, chemotherapy or radiation may be used before surgery.

• Even if the tumor is entirely removed, chemotherapy is usually administered to prevent recurrence.

2. Chemotherapy

• Uses drugs to destroy cancer cells.

• Neoadjuvant chemotherapy: Given before surgery to shrink the tumor.

• Adjuvant chemotherapy: Administered after surgery to eliminate remaining cancer cells.

Common Chemotherapy Drugs for Rhabdomyosarcoma:

• Vincristine (Oncovin, Vincasar)

• Dactinomycin (Cosmegen)

• Cyclophosphamide (Cytoxan)

• Combination: VAC regimen

Side Effects: Fatigue, nausea, vomiting, infections, hair loss, loss of appetite, diarrhea. Most side effects subside after treatment.

3. Radiation Therapy

• External Beam Radiation Therapy (EBRT) is used when the tumor cannot be fully removed by surgery.

• Helps shrink tumors and reduce the chance of recurrence.

Side Effects: Fatigue, skin irritation, nausea, and diarrhea. These effects usually diminish after treatment.

Follow-Up Care

• Regular follow-ups with physical exams, blood tests, and imaging tests are crucial to monitor for recurrence.

• Management of side effects is also part of the follow-up plan.

Rhabdomyosarcoma treatment involves early detection, surgery, chemotherapy, and radiation, with long-term follow-up care essential to ensure the best outcomes.

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