Phase III Trial Adding Vincristine-Topotecan-Cyclophosphamide to the Initial Treatment of Patients With Nonmetastatic Ewing Sarcoma: A Children's Oncology Group Report.

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Abstract

[Indexed for MEDLINE] Conflict of interest statement: Patrick J. LeaveyResearch Funding: Eleison Pharmaceuticals Nadia N. LaackResearch Funding: Bristol Myers Squib Mark D. KrailoConsulting or Advisory Role: Merck Sharp & DohmeTravel, Accommodations, Expenses: Merck Sharp & Dohme R. Lor RandallHonoraria: Daiichi Sankyo, Onkos Surgical, OncliveTravel, Accommodations, Expenses: Biomet, Daiichi Sankyo/Lilly Steven G. DuBoisResearch Funding: Merck (Inst), Roche/Genentech (Inst), Lilly (Inst), Curis (Inst), Loxo (Inst), BMS (Inst), Eisai (Inst), Pfizer (Inst), Turning Point Therapeutics (Inst), Bayer (Inst), Salarius Pharmaceuticals (Inst)Travel, Accommodations, Expenses: Roche/Genentech, Salarius PharmaceuticalsUncompensated Relationships: Y-mAbs Therapeutics Inc Damon R. ReedConsulting or Advisory Role: Eisai, PfizerTravel, Accommodations, Expenses: Salarius Pharmaceuticals Douglas S. HawkinsResearch Funding: Loxo (Inst), Bristol Myers Squibb (Inst), Merck Sharp & Dohme (Inst), Bayer (Inst), Lilly (Inst), Eisai (Inst), Amgen (Inst), Seattle Genetics (Inst), Incyte (Inst), Jazz Pharmaceuticals (Inst), Pfizer (Inst)Travel, Accommodations, Expenses: Bayer, AstraZeneca Helen NadelConsulting or Advisory Role: ICON Clinical Research G. Douglas LetsonEmployment: StrykerConsulting or Advisory Role: StrykerTravel, Accommodations, Expenses: Stryker Mark BernsteinResearch Funding: Merck Paul ChubaEmployment: Radiation Oncology Specialists Neyssa MarinaEmployment: Five Prime Therapeutics, Genentech/Roche (I), Synthorx, Sanofi PasteurStock and Other Ownership Interests: Five Prime Therapeutics, Genentech/Roche (I), Synthorx, Sanofi PasteurTravel, Accommodations, Expenses: Five Prime Therapeutics, SynthorxUncompensated Relationships: Stanford University School of MedicineOpen Payments Link: https://openpaymentsdata.cms.gov/physician/1210881 Richard GorlickResearch Funding: Eisai Katherine A. JanewayHonoraria: Foundation MedicineConsulting or Advisory Role: Bayer, IpsenTravel, Accommodations, Expenses: Bayer Leo MascarenhasConsulting or Advisory Role: BayerResearch Funding: AstraZeneca/MedImmune, Lilly, Bayer, Salarius Pharmaceuticals, Turning Point Therapeutics, Pfizer, Incyte, Amgen, E.R. Squibb Sons, LLC, Jazz Pharmaceuticals, MerckTravel, Accommodations, Expenses: Bayer, Lilly, Thermo Fisher Scientific, Salarius PharmaceuticalsUncompensated Relationships: Children's Oncology Group Foundation, The Pablove Foundation, American Society of Pediatric Hematology/OncologyNo other potential conflicts of interest were reported. 9. Gan To Kagaku Ryoho. 2004 Mar;31(3):346-50. [Ewing's sarcoma]. [Article in Japanese] Goto T(1), Hozumi T, Kondo T. Author information: (1)Dept. of Orthopaedic Surgery and Musculoskeletal Oncology, Tokyo Metropolitan Komagome Hospital, 3-18-22 Hon-Komagome, Bunkyo-ku, Tokyo 113-8677, Japan. Ewing's sarcomas account for 6.8% of all primary malignant bone tumors and are probably a neurogenic, undifferentiated, high-grade malignancy, which usually affects the bones of children 5-15 years of age. Pain and swelling are the most common symptoms. Increase of CRP and erythrocyte sedimentation rate, leucocytosis, and anemia are frequently seen. Radiologically, they show permeative bone destruction on plain radiographs. When arising in the diaphysis of long bones, laminated, "onion-skin" periosteal reaction is seen. The tumor shows muscle density on CT, iso-signal intensity on T1-weighted MR images, and high signal intensity on T2-weighted MR images. Intramedullary invasion and skip lesions can be detected on MR images. Histologically, the tumor is uniformly composed of sheets of small round cells closely packed and without any matrix product. Glycogen granules are demonstrated in the cytoplasm by periodic acid-Schiff (PAS) and diastase reactions. Immunohistochemically, Ewing's sarcomas are positive for vimentin and MIC-2 gene product (CD99). Reciprocal translocation, i.e., t(11;22) (q24;q12), is seen in the tumor cells. EWS/FLI-1 fusion gene can be demonstrated, which can be a complementary method in diagnosing this tumor. Because Ewing's sarcomas are chemosensitive and radiosensitive, they are treated by a combination of chemotherapy, surgery, and radiotherapy. Neoadjuvant chemotherapy consists of preoperative chemotherapy and postoperative chemotherapy. Preoperative chemotherapy aims at eradicating distant micrometastasis, reducing the primary tumor volume, and evaluating the efficacy of the chemotherapeutic agents. Surgery is performed as a local treatment by excising the tumor using the wide procedure. If surgery is impractical, curative radiotherapy is performed instead of excision. When surgery is performed without complete wide procedure, adjuvant radiotherapy is carried out to eradicate the residual tumor cells. Postoperative chemotherapy aims to eradicate the distant micrometastasis. Recently, myeloablative, high-dose chemotherapy followed by autologous bone marrow transplantation is being attempted for poor-prognosis patients and good results have been reported.