Ewing Sarcoma and Primitive Neuroectodermal Tumors Treatment & Management
- Author: Jeffrey A Toretsky, MD; Chief Editor: Robert J Arceci, MD, PhD more...
Medical Care
The most recent Children's Oncology Group (COG) clinical trial randomized patients between chemotherapy cycles either 2 or 3 weeks apart.[2, 3] The patients who received chemotherapy had an improved outcome to those who received chemotherapy every 3 weeks. The doses of chemotherapy were similar between the groups and both groups of patients received granulocyte-colony stimulating factor (G-CSF) to support adequate neutrophil counts.
Medical therapy varies slightly among European and North American pediatric oncologists.
- Patients should be treated under the supervision of a pediatric oncologist; staff at a comprehensive pediatric oncology center should direct care.
- A multidisciplinary team should evaluate and treat the patient. The team may include pediatric oncologists, radiation oncologists, surgeons, radiologists, pathologists, nurses, social workers, occupational and/or physical therapists, blood bank specialists, psychologists, school tutors, and pharmacists.
- In-house expertise from infectious disease specialists is often required.
- Treatment lasts 6-9 months and consists of alternating courses of 2 chemotherapeutic regimens: (1) vincristine, doxorubicin, and cyclophosphamide and (2) ifosfamide and etoposide.[4]
- Management of the primary tumor site is critical to long-term cure. Definitive surgical margins are desirable (eg, removal of fibula, limb salvage with extensive margins).
- In the absence of a minimally morbid surgical procedure, local control may be achieved with radiation thera py. Doses to the tumor and fractionation are site dependent.[5]
Surgical Care
Any surgery should be performed under the supervision of experienced oncologic surgeons specializing in the area of the body where the tumor is found. The specific surgery is highly patient dependent.[6]
Consultations
- Orthopedic surgeon
- If the patient has a lesion close to bone that is potentially resectable, consultation with an orthopedic oncologist is required biopsy.
- Biopsy planning is critical because an inappropriately conducted procedure can contaminate tissue planes.
- Neurologist: Lesions close to nerve roots, ganglia, or plexuses might result in neurologic symptoms.
- Pathologist: When a mass is resected from a pediatric patient, a pathologist should be aware of the procedure and the differential diagnosis. This information and involvement is critical for appropriate diagnostic studies to be performed.
Diet
- P atients require close monitoring of their caloric intake during treatment.
- Services of a dietitian are often needed. However, no special diets are required for treatment.
Activity
- Activity limitations depend on the location of primary and metastatic lesions.
- No general restrictions are indicated.
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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