Ewing Sarcoma and Primitive Neuroectodermal Tumors Follow-up
- Author: Jeffrey A Toretsky, MD; Chief Editor: Robert J Arceci, MD, PhD more...
Further Inpatient Care
- Chemotherapy can be administered on an inpatient or outpatient basis, depending on patient tolerance and proximity to the hospital.
- Patients often develop episodes of fever while neutropenic, resulting in 3- to 7-day hospitalizations between cycles of chemotherapy.
Further Outpatient Care
- Chemotherapy care and follow-up
- Most patients require RBC and platelet support starting approximately 2 months after the start of therapy and continuing to the completion of therapy. Although G-CSF is given for neutrophil support, biweekly CBC counts are necessary.
- A full physical examination is required before each cycle of chemotherapy and any time suspicious signs or symptoms arise between cycles. Suspicious signs include signs similar to those observed at presentation, as well as unexplained fever or pain.
- Primary and metastatic sites are evaluated approximately every 10-12 weeks during therapy and every 3-4 months during the first year after therapy.
- Reevaluations are spaced out gradually for 5-6 years after the completion of therapy. At that time, no further scanning is indicated; however, the patient should have annual follow-up visits to monitor function of the primary site and late effects of therapy.
- Long-term follow-up
- Late effects from chemotherapy require regular follow-up with a provider trained to evaluate the such sequelae.
- Recurrence of primary disease is the major risk in the first 10 years after diagnosis.
- Second malignancy occurs in approximately 1-2% of patients beginning after 5 years after diagnosis. The most common second malignancy is acute myeloid leukemia.
- Therapeutic toxicities to the heart and kidneys and to the nervous, endocrine, and mental systems should be monitored in patients who had acute toxicity and in those who developed symptoms after therapy.
Transfer
- Patient care during chemotherapy is generally under the direct supervision of the pediatric oncologist. The primary care physician should be kept informed about the patient's progress and complications.
- After therapy is completed, the primary physician should increase his or her involvement in patient care.
Deterrence/Prevention
- No prevention methods are known.
Complications
- Chemotherapy complications
- Vincristine primarily causes neuropathy, including constipation, myalgias, arthralgias, and cholestasis.
- Doxorubicin causes myocardial dysfunction and pancytopenia.
- Cyclophosphamide causes pancytopenia and a dosage-dependent hemorrhagic cystitis.
- Ifosfamide is similar to cyclophosphamide, although it is associated with an increased incidence of hemorrhagic cystitis, which requires the use of mesna. Patients near the end of therapy occasionally develop the Fanconi syndrome of electrolyte wasting.
- Etoposide can result in pancytopenia as well as anaphylactic reactions, and it is implicated in the development of second malignancies, particularly acute myelogenous leukemia.
- In general, combination chemotherapy results in alopecia, nausea, vomiting, and, occasionally, diarrhea. The nutritional and psychologic statuses of patients undergoing this therapy must be closely monitored.
- Surgical complications
- Surgical complications generally include infection and bleeding.
- Specific complications are related to the site of surgery and to the patient's overall condition at the time of surgery.
- Radiation complications
- Complications of radiation therapy are a direct result of the sites of radiation.
- Patients who receive large pelvic doses of radiation often have increased problems with pancytopenia, malnutrition, and diarrhea.
- Radiation increases the likelihood of second malignancies, particularly in the radiation field.
Prognosis
- At this time, the only significant factor that determines the prognosis is the presence or absence of metastatic disease.
Patient Education
- Any patient with a malignancy needs extensive education, as does their family.
- For the patient, education includes age and developmentally appropriate information about their disease and its therapy. Patients should be informed about their specific disease and prognosis. Education also includes information about expected complications, particularly fever and its management.
Kim DH, Kim SY, Lee HJ, Song BS, Kim DH, Cho JB, et al. Assessment of Chemotherapy Response Using FDG-PET in Pediatric Bone Tumors: A Single Institution Experience. Cancer Res Treat. Sep 2011;43(3):170-5. [Medline]. [Full Text].
Miser JS, Krailo MD, Tarbell NJ, et al. Treatment of metastatic Ewing's sarcoma or primitive neuroectodermal tumor of bone: evaluation of combination ifosfamide and etoposide--a Children's Cancer Group and Pediatric Oncology Group study. J Clin Oncol. Jul 15 2004;22(14):2873-6. [Medline].
Womer, West, Krailo, Pawel, Dickman. hemotherapy intensification by interval compression in localized Ewing Sarcoma Family Tumors. Seattle, WA: Connective Tissue Oncology Society Annual Meeting; 2007.
Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. Feb 20 2003;348(8):694-701. [Medline].
Dunst J, Jurgens H, Sauer R, Pape H, Paulussen M, Winkelmann W, et al. Radiation therapy in Ewing's sarcoma: an update of the CESS 86 trial. Int J Radiat Oncol Biol Phys. Jul 15 1995;32(4):919-30. [Medline].
Israelsen RB, Ilium BE, Crabtree S, Randall RL, Jones KB. Extremity sarcoma surgery in younger children: ten years of patients ten years and under. Iowa Orthop J. 2011;31:145-53. [Medline]. [Full Text].
Gurney JG, Swensen AR, Bulterys M. Malignant bone tumors. In: Ries LA, Smith MAS, Gurney JG, et al, eds. Cancer Incidence and Survival Among Children and Adolescents: United States SEER Program 1975-1995. Publication 99-4649. Bethesda, MD: National Cancer Institute; 1999:99-110.
Meyers PA, Krailo MD, Ladanyi M, Chan KW, Sailer SL, Dickman PS, et al. High-dose melphalan, etoposide, total-body irradiation, and autologous stem-cell reconstitution as consolidation therapy for high-risk Ewing's sarcoma does not improve prognosis. J Clin Oncol. Jun 1 2001;19(11):2812-20. [Medline].
Paulussen M, Ahrens S, Dunst J, Winkelmann W, Exner GU, Kotz R, et al. Localized Ewing tumor of bone: final results of the cooperative Ewing's Sarcoma Study CESS 86. J Clin Oncol. Mar 15 2001;19(6):1818-29. [Medline].
Saylors RL 3rd, Stine KC, Sullivan J, et al. Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study. J Clin Oncol. Aug 1 2001;19(15):3463-9. [Medline].
Uren A, Toretsky JA. Ewing's sarcoma oncoprotein EWS-FLI1: the perfect target without a therapeutic agent. Future Oncol. Aug 2005;1(4):521-8. [Medline].
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