Pediatric Rhabdomyosarcoma Follow-up

• Chemotherapy: Chemotherapy cycles are usually administered every 3 weeks (although vincristine is periodically given weekly) in patients with rhabdomyosarcoma (RMS), depending on recovery of the bone marrow. Patients receiving cycles that include cyclophosphamide, ifosfamide, and etoposide generally require inpatient admission for drug administration and monitoring.
• Fever and neutropenia: Admission is required to administer intravenous (IV) antibiotics and to monitor patients.
• Other reasons for inpatient care: Patients may require admission for a multitude of other medical problems during the chemotherapy phase of treatment, including varicella infection (to administer IV acyclovir and to monitor), mucositis (resulting from narcotics use), dehydration, meningitis, constipation, fungal pneumonia, and cystitis, among others.

• CBC count: Perform a CBC count twice each week in patients receiving therapy by using granulocyte-colony stimulating factor (G-CSF) so that G-CSF can be discontinued when the absolute neutrophil count has reached a predetermined level (usually 1 or 5 X 10⁹/L [1000 or 5000/µL]).
• Blood chemistry: Monitor blood chemistry results and liver function in patients receiving parenteral nutrition or in those who have a history of toxicity, especially if the patient continues to receive nephrotoxic or hepatotoxic antibiotics or other drugs.
• Chemotherapy: Depending on the protocol, some chemotherapy regimens (eg, vincristine, dactinomycin in particular) can be administered on an outpatient basis.
• Monitoring for recurrence: Continue to perform blood tests and radiographic scans on an outpatient basis, with the frequency decreasing over time. In general, patients should be examined every 3 months for the first year, every 6 months for the second and third years, and yearly thereafter.
• Long-term follow-up care: At 5 or longer after the end of therapy, patients are considered to be long-term survivors. Patients should be examined annually at a late-effects clinic and monitored with appropriate studies depending on the type of therapy they received. Visits may include hormonal, psychosocial, and neurologic evaluations, as well as follow-up examinations by the radiotherapist.

• Trimethoprim-sulfamethoxazole: Prophylaxis against pneumocystic pneumonia should continue until 6 months after the end of chemotherapy.
• Fluconazole: Systemic fungal prophylaxis is not necessary.
• Clotrimazole: Prophylactic therapy for thrush may be discontinued after chemotherapy is completed.
• Chlorhexidine mouth rinse: Prophylaxis against gingivitis and other mouth infections may be discontinued after chemotherapy is completed.

• Although major cancer therapy should take place at a center with pediatric oncologists, the child's referring pediatrician or general practitioner should continue to play an important role in the child's care throughout treatment.
• The referring physician can be critical in performing the first evaluation of an illness, particularly if the child lives far from an oncology center.

• No preventive measures are known for childhood cancers.

The treatment of rhabdomyosarcoma results in a multitude of potential long-term adverse effects.^[24] The most common findings include the following:

• Cardiomyopathy

  • In patients who receive an anthracycline, cardiac function must be monitored to assess for the development of cardiomyopathy.
  • Cardiomyopathy may also from cyclophosphamide use.
• Pulmonary failure
• Metabolic derangements: Ifosfamide use, in particular, can lead to renal electrolyte wasting (Fanconi syndrome).
• Secondary malignant neoplasms

  • Secondary malignant neoplasms may arise as a result of radiotherapy and chemotherapy, particularly with alkylating agents.
  • Etoposide markedly increases the risk for acute myelogenous leukemia, particularly when regimens with frequent dosing schedules are used.
  • Radiation therapy increases the risk of second malignancies, including skin and bone tumors.

• Chemotherapy: Parents and patients (if appropriate) must undergo formal training to learn about the adverse effects of chemotherapy. They must know what is expected to happen as a result of the therapy and are encouraged to ask questions.
• Central venous catheters

  • When patients have central venous catheters that exit the skin (eg, Hickman or Broviac catheters), the parents or the patient must learn to properly care for the line. This care usually involves daily heparin flushes.
  • Patients and parents must understand the limitations on activities because of central venous catheters. For example, swimming is not permitted.
  • Patients with subcutaneous catheters (eg, Mediport catheters) do not need to perform daily care, but they should learn to apply a topical anesthetic (eg, EMLA cream, or lidocaine-prilocaine cream) at least 1 hour before an anticipated needle stick.