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1. Bar-Sela G, Peer A, Rothschild S, Haim N: Treatment of patients aged over 50 years with non-osseous Ewing's sarcoma family tumors: five cases and review of literature. Tumori; 2008 Nov-Dec;94(6):809-12
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  • [Title] Treatment of patients aged over 50 years with non-osseous Ewing's sarcoma family tumors: five cases and review of literature.
  • BACKGROUND: Most clinical trials on Ewing's sarcoma family of tumors include pediatric and adolescent populations, whereas clinical data on older patients are limited.
  • PATIENTS AND METHODS: We report on 5 patients older than 50 years with a tumor of the Ewing's sarcoma family treated recently in our department.
  • Major dose reductions and/or treatment delays were required in all 5 patients.
  • Complete remission was achieved in the remaining 4 patients with the addition of different treatment modalities.
  • One patient had lung metastasis 3 years after starting chemotherapy, and 3 patients have remained without evidence of recurrent disease for 1-6 years from the onset of chemotherapy CONCLUSIONS: There is no definite answer as to whether older age is a poor prognostic factor in patients with a tumor of the Ewing's sarcoma family.
  • In our experience, patients over 50 poorly tolerated the standard chemotherapy protocol used in the pediatric population.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Sarcoma, Ewing / therapy
  • [MeSH-minor] Aged. Chemotherapy, Adjuvant. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Prognosis. Radiotherapy, Adjuvant

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  • (PMID = 19267097.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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2. Bagatell R, Gore L, Egorin MJ, Ho R, Heller G, Boucher N, Zuhowski EG, Whitlock JA, Hunger SP, Narendran A, Katzenstein HM, Arceci RJ, Boklan J, Herzog CE, Whitesell L, Ivy SP, Trippett TM: Phase I pharmacokinetic and pharmacodynamic study of 17-N-allylamino-17-demethoxygeldanamycin in pediatric patients with recurrent or refractory solid tumors: a pediatric oncology experimental therapeutics investigators consortium study. Clin Cancer Res; 2007 Mar 15;13(6):1783-8
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  • [Title] Phase I pharmacokinetic and pharmacodynamic study of 17-N-allylamino-17-demethoxygeldanamycin in pediatric patients with recurrent or refractory solid tumors: a pediatric oncology experimental therapeutics investigators consortium study.
  • We conducted a phase I trial of 17-AAG in pediatric patients with recurrent or refractory neuroblastoma, Ewing's sarcoma, osteosarcoma, and desmoplastic small round cell tumor to determine the maximum tolerated dose, define toxicity and pharmacokinetic profiles, and generate data about molecular target modulation.
  • Caution should be used in treatment of patients with bulky pulmonary disease.
  • [MeSH-major] Benzoquinones / administration & dosage. Benzoquinones / pharmacokinetics. Lactams, Macrocyclic / administration & dosage. Lactams, Macrocyclic / pharmacokinetics. Neoplasms / drug therapy. Pediatrics
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / administration & dosage. Antineoplastic Agents / adverse effects. Antineoplastic Agents / pharmacokinetics. Biomarkers, Tumor / analysis. Child. Child, Preschool. Female. Humans. Male. Maximum Tolerated Dose. Recurrence. Treatment Failure

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  • [CommentIn] Clin Cancer Res. 2007 Mar 15;13(6):1625-9 [17363512.001]
  • (PMID = 17363533.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Grant] United States / NCRR NIH HHS / RR / M01 RR-00069; United States / NCRR NIH HHS / RR / M01 RR-00082; United States / NCRR NIH HHS / RR / M01 RR-00095; United States / NCI NIH HHS / CA / U54 CA090821
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzoquinones; 0 / Biomarkers, Tumor; 0 / Lactams, Macrocyclic; 4GY0AVT3L4 / tanespimycin
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3. DeLaney TF, Liebsch NJ, Pedlow FX, Adams J, Dean S, Yeap BY, McManus P, Rosenberg AE, Nielsen GP, Harmon DC, Spiro IJ, Raskin KA, Suit HD, Yoon SS, Hornicek FJ: Phase II study of high-dose photon/proton radiotherapy in the management of spine sarcomas. Int J Radiat Oncol Biol Phys; 2009 Jul 1;74(3):732-9
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  • Negative surgical margins are uncommon; hence, doses of >or=66 Gy are recommended.
  • Treatment included pre- and/or postoperative photon/proton XRT with or without radical resection; patients with osteosarcoma and Ewing's sarcoma received chemotherapy.
  • Shrinking fields delivered 50.4 cobalt Gray equivalent (Gy RBE) to subclinical disease, 70.2 Gy RBE to microscopic disease in the tumor bed, and 77.4 Gy RBE to gross disease at 1.8 Gy RBE qd.
  • Spinal cord dose was limited to 63/54 Gy RBE to surface/center.
  • Intraoperative boost doses of 7.5 to 10 Gy could be given by dural plaque.
  • Two of 36 (5.6%) patients treated for primary versus 7/14 (50%) for recurrent tumor developed local recurrence (p < 0.001).
  • Five patients developed late radiation-associated complications; no myelopathy developed but three sacral neuropathies appeared after 77.12 to 77.4 Gy RBE.
  • CONCLUSIONS: Local control with this treatment is high in patients radiated at the time of primary presentation.
  • Sacral nerves receiving 77.12-77.4 Gy RBE are at risk for late toxicity.

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  • (PMID = 19095372.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA021239; United States / NCI NIH HHS / CA / CA021239-27; United States / NCI NIH HHS / CA / CA021239-28; United States / NCI NIH HHS / CA / P01CA021239; United States / NCI NIH HHS / CA / P01 CA021239-27; United States / NCI NIH HHS / CA / P01 CA021239-28
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protons
  • [Other-IDs] NLM/ NIHMS121640; NLM/ PMC2734911
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4. Kushner BH, Cheung NK, Kramer K, Dunkel IJ, Calleja E, Boulad F: Topotecan combined with myeloablative doses of thiotepa and carboplatin for neuroblastoma, brain tumors, and other poor-risk solid tumors in children and young adults. Bone Marrow Transplant; 2001 Sep;28(6):551-6
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  • Post-transplant treatment included radiotherapy alone (four patients) or plus biological agents (11 patients with neuroblastoma).
  • With a follow-up of 6+ to 32+ (median 11+) months, event-free survivors include 10/11 neuroblastoma patients (first CR), 4/5 brain tumor patients (second PR or CR), 1/3 patients with metastatic Ewing's sarcoma (first or second CR), and a patient transplanted for multiply recurrent immature ovarian teratoma; a patient with desmoplastic small round-cell tumor (second PR) had progressive disease at 8 months.
  • Favorable results for disease control, manageable toxicity, and the antitumor profiles of topotecan, thiotepa, and carboplatin, support use of this three-drug regimen in the treatment of neuroblastoma and brain tumors; applicability to other tumors is still uncertain.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Carboplatin / administration & dosage. Neuroblastoma / drug therapy. Thiotepa / administration & dosage. Topotecan / administration & dosage
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Hematopoietic Stem Cell Transplantation. Humans. Infant. Male. Radiotherapy, Adjuvant. Remission Induction. Treatment Outcome

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  • (PMID = 11607767.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA61017; United States / NCI NIH HHS / CA / CA72868
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Clinical Trial, Phase III; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 7M7YKX2N15 / Topotecan; 905Z5W3GKH / Thiotepa; BG3F62OND5 / Carboplatin
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5. Karosas AO: Ewing's sarcoma. Am J Health Syst Pharm; 2010 Oct 1;67(19):1599-605
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  • [Title] Ewing's sarcoma.
  • PURPOSE: The current treatments of and new therapeutic options for the management of Ewing's sarcoma (ES) are reviewed.
  • Much progress has been made in the treatment of ES since the disease was first described in the 1920s.
  • With current multimodality treatment including chemotherapy, radiation, and surgery, patients with localized disease have a long-term survival rate of approximately 50%.
  • To date, the role of high-dose chemotherapy supported by stem cell rescue as a consolidation therapy for high-risk ES tumors has yet to be conclusively determined.
  • Much effort is being invested in treating cancer with targeted therapies, and the EWS-ETS fusion gene would likely provide an important tumor-specific target.
  • Tyrosine kinases (TKs) are overexpressed in human sarcoma tumors, and cell lines may serve as potential targets for new therapies.
  • One TK receptor that is a promising therapeutic target is insulinlike growth factor-1 receptor.
  • CONCLUSION: Treatments for ES include surgery, radiation, and cytotoxic regimens, many of which include vincristine.
  • Treatment for recurrent ES has included topotecan, cyclophosphamide, temozolomide, and irinotecan.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adult. Child. Combined Modality Therapy. Drug Delivery Systems. Humans. Neoplasm Recurrence, Local. Survival Rate. Young Adult

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  • (PMID = 20852160.001).
  • [ISSN] 1535-2900
  • [Journal-full-title] American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists
  • [ISO-abbreviation] Am J Health Syst Pharm
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
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6. Desai KI, Nadkarni TD, Goel A, Muzumdar DP, Naresh KN, Nair CN: Primary Ewing's sarcoma of the cranium. Neurosurgery; 2000 Jan;46(1):62-8; discussion 68-9
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  • [Title] Primary Ewing's sarcoma of the cranium.
  • OBJECTIVE: We analyzed the data for a series of 14 patients with primary Ewing's sarcomas of the cranium who were treated since 1985.
  • Our aim was to assess the long-term outcomes and the selection of appropriate treatment methods.
  • METHODS: The patients were reviewed with respect to their clinical presentations, treatment, and outcomes.
  • Skeletal surveys with routine radiographs and technetium-99 bone scans to detect extracranial Ewing's sarcomas were performed for all patients.
  • For all 14 patients, radical tumor excision was achieved surgically.
  • All patients were then subjected to adjuvant multidrug chemotherapy and radiotherapy.
  • This recurrent tumor was completely excised, and additional chemotherapy was administered.
  • CONCLUSION: Although primary Ewing's sarcoma of the cranium is a malignant bone tumor, it is associated with a good prognosis when treated with radical surgery, aggressive multidrug chemotherapy, and radiotherapy.
  • [MeSH-major] Sarcoma, Ewing / therapy. Skull Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Infant. Male. Time Factors. Treatment Outcome

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  • (PMID = 10626936.001).
  • [ISSN] 0148-396X
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 37
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7. Bar-Sever Z, Cohen IJ, Connolly LP, Horev G, Perri T, Treves T, Hardoff R: Tc-99m MIBI to evaluate children with Ewing's sarcoma. Clin Nucl Med; 2000 Jun;25(6):410-3
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  • [Title] Tc-99m MIBI to evaluate children with Ewing's sarcoma.
  • PURPOSE: Tc-99m MIBI has been used increasingly to evaluate benign and malignant tumors because of its tumor-seeking properties and ability to provide an imaging assessment of multiple-drug resistance.
  • This study investigated the clinical utility of Tc-99m MIBI in the management of Ewing's sarcoma in children.
  • METHODS: Thirteen Tc-99m MIBI studies in nine (six male, three female) patients ages 6.5 to 20 years (mean, 13.4 years) with Ewing's sarcoma were reviewed.
  • All patients had imaging studies at diagnosis, and four had follow-up studies during or after therapy.
  • Scintigraphy was evaluated for Tc-99m MIBI uptake within the tumor and in metastases, which other imaging modalities had shown to be present in four patients.
  • Scintigraphic results were correlated with the clinical course in all patients and with tumor P-glycoprotein status in six patients.
  • RESULTS: Tc-99m MIBI accumulated in 6 of 9 primary tumors and did not accumulate in one recurrent tumor.
  • The presence or absence of Tc-99m MIBI uptake at diagnosis or after therapy carried no prognostic significance.
  • CONCLUSION: Tc-99m MIBI imaging does not appear to be useful in Ewing's sarcoma.
  • [MeSH-major] Bone Neoplasms / radionuclide imaging. Radiopharmaceuticals. Sarcoma, Ewing / radionuclide imaging. Technetium Tc 99m Sestamibi
  • [MeSH-minor] Adolescent. Adult. Child. Drug Resistance, Multiple. Drug Resistance, Neoplasm. Female. Femoral Neoplasms / radionuclide imaging. Femoral Neoplasms / therapy. Follow-Up Studies. Humans. Lung Neoplasms / radionuclide imaging. Lung Neoplasms / secondary. Male. Neoplasm Recurrence, Local / radionuclide imaging. P-Glycoprotein / analysis. Pelvic Bones / radionuclide imaging. Prognosis. Retrospective Studies

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  • (PMID = 10836685.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / P-Glycoprotein; 0 / Radiopharmaceuticals; 971Z4W1S09 / Technetium Tc 99m Sestamibi
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8. Subbiah V, Anderson P, Lazar AJ, Burdett E, Raymond K, Ludwig JA: Ewing's sarcoma: standard and experimental treatment options. Curr Treat Options Oncol; 2009 Apr;10(1-2):126-40
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  • [Title] Ewing's sarcoma: standard and experimental treatment options.
  • OPINION STATEMENT: Ewing sarcoma family tumors (EWS), which include classic Ewing's sarcoma in addition to primitive neuroectodermal tumor and Askin tumor, are the second most common variety of primary bone cancer to afflict adolescents and young adults.
  • Multi-disciplinary care incorporating advances in diagnosis, surgery, chemotherapy, and radiation has substantially improved the survival rate of patients with localized Ewing sarcoma to nearly 70%.
  • Unfortunately, those advances have not significantly changed the long-term outcome for those with metastatic or recurrent disease; 5-year survival remains less than 25%.
  • This apparent therapeutic plateau exists despite extensive effort during the last four decades to optimize the efficacy of cytotoxic chemotherapy through combination of chemotherapies of mechanistically diverse action, dose-dense scheduling (provided as frequently as every 2 weeks), increased adjuvant treatment duration, and higher dosage per cycle (facilitated with parallel strides in supportive care incorporating growth factors).
  • As has already occurred for malignancies such as breast or colon cancer, the "-omics-based" revolution has enhanced our understanding of the molecular changes responsible for Ewing's tumor formation and identified a number of potential targets (such as IGF-1R or mTOR) amenable to biological therapy.
  • It has also created both a challenge and an opportunity to develop predictive biomarkers capable of selecting patients most likely to benefit from targeted therapy.
  • In this review, we discuss current standard-of-care for patients with Ewing's sarcoma and highlight the most promising experimental therapies in early-phase clinical trials.
  • [MeSH-major] Bone Neoplasms / surgery. Sarcoma, Ewing / surgery. Therapies, Investigational
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / pharmacology. Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Clinical Trials as Topic. Combined Modality Therapy. Drug Delivery Systems. Drug Screening Assays, Antitumor. Humans. Lung Neoplasms / drug therapy. Lung Neoplasms / mortality. Lung Neoplasms / secondary. Lung Neoplasms / surgery. Multicenter Studies as Topic. Oncogene Proteins, Fusion / antagonists & inhibitors. Oncogene Proteins, Fusion / genetics. Proto-Oncogene Protein c-fli-1. RNA-Binding Protein EWS. Receptor, IGF Type 1 / antagonists & inhibitors. Survival Rate. Transcription Factors / antagonists & inhibitors. Translocation, Genetic. Young Adult

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  • (PMID = 19533369.001).
  • [ISSN] 1534-6277
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Transcription Factors; EC 2.7.10.1 / Receptor, IGF Type 1
  • [Number-of-references] 94
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9. Drabko K, Zawitkowska-Klaczynska J, Wojcik B, Choma M, Zaucha-Prazmo A, Kowalczyk J, Gorczynska E, Toporski J, Kałwak K, Turkiewicz D, Chybicka A: Megachemotherapy followed by autologous stem cell transplantation in children with Ewing's sarcoma. Pediatr Transplant; 2005 Oct;9(5):618-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Megachemotherapy followed by autologous stem cell transplantation in children with Ewing's sarcoma.
  • Twenty-one children with high-risk Ewing's tumor received high-dose chemotherapy with a PBSCT.
  • Aim of the study was evaluation of efficiency and safety of this procedure.
  • Megachemotherapy consisted of melphalan 140 mg/m2/busulfan 16 mg/kg in 12 patients, melphalan 140 mg2/treosulfan 10.0 g/m2 in two patients and melphalan with other drugs in seven patients.
  • Children transplanted without remission died: Two of them due to transplant related causes and eight had progression of disease in a median time 7 month after PBSCT.
  • Megachemotherapy with PBSCT is a safe procedure in children with Ewing's sarcoma in remission.
  • Autologos transplantation in children with metastatic Ewing's sarcoma seems to improve their outcome.
  • Patients with Ewing's sarcoma, resistant to conventional therapy and with recurrent disease did not benefit from megachemotherapy.
  • New approaches such as anti-tumor vaccination or using of imatinib are reasonable to introduce in patients with relapsed or resistant to therapy Ewing's tumor.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Hematopoietic Stem Cell Transplantation. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Alkylating / adverse effects. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Male. Melphalan / administration & dosage. Melphalan / adverse effects. Survival Rate. Transplantation, Autologous

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  • (PMID = 16176419.001).
  • [ISSN] 1397-3142
  • [Journal-full-title] Pediatric transplantation
  • [ISO-abbreviation] Pediatr Transplant
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; Q41OR9510P / Melphalan
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10. Anderson P, Kopp L, Anderson N, Cornelius K, Herzog C, Hughes D, Huh W: Novel bone cancer drugs: investigational agents and control paradigms for primary bone sarcomas (Ewing's sarcoma and osteosarcoma). Expert Opin Investig Drugs; 2008 Nov;17(11):1703-15
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  • [Title] Novel bone cancer drugs: investigational agents and control paradigms for primary bone sarcomas (Ewing's sarcoma and osteosarcoma).
  • BACKGROUND: New investigational agents and chemotherapy regimens including cyclophosphamide + topotecan, temozolomide + irinotecan, and anti-IGF-1R antibodies in Ewing's sarcoma (ES) and liposomal muramyltripeptide phosphatidylethanolamine (L-MTP-PE), aerosol therapy, and bone-specific agents in osteosarcoma (OS) may improve survival and/or quality of life on 'continuation' therapy.
  • OBJECTIVE: Review of investigational approaches and control paradigms for recurrent or metastatic primary bone tumors.
  • Review some current state-of-the-art approaches for OS including L-MTP-PE, anti-IGF-1R inhibition, aerosol therapies and bone specific agents.
  • RESULTS/CONCLUSION: L-MTP-PE with chemotherapy in OS has been shown to improve survival; compassionate access is available for recurrence and/or metastases.
  • Aerosol therapy (granulocyte-macrophage colony stimulating factor, cisplatin, gemcitabine) for lung metastases is a promising approach to reduce systemic toxicity.
  • The bone-specific agents including denosumab (anti-receptor activator of NF-kappaB ligand antibody) and bisphosphonates may have benefit against giant cell tumor, ES and OS.
  • Anti-IGF-1R antibody SCH717454 has preclinical activity in OS but best effectiveness will most likely be in combination with chemotherapy earlier in therapy.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Osteosarcoma / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Animals. Drug Evaluation, Preclinical. Humans. Immunotherapy. Neoplasm Metastasis / pathology


11. Mutafoğlu Uysal K, Olgun N, Sarialioğlu F, Kargi A, Cevik N: A case with extraosseous Ewing's sarcoma: a late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome? Pediatr Hematol Oncol; 2000 Jul-Aug;17(5):415-9
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  • [Title] A case with extraosseous Ewing's sarcoma: a late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome?
  • A case is presented here of extraosseous Ewing's sarcoma that developed 8 years after allogeneic bone marrow transplantation performed for beta-thalassemia major.
  • The patient was treated with chemotherapy and radiotherapy and died with recurrent disease.
  • To the authors' knowledge, this is the first case of extraosseous Ewing's sarcoma after bone marrow transplantation for thalassemia.
  • The possible contribution of transplantation procedure and the genetic factors as well as the primary genetic hemoglobinopathy to the development of this malignant tumor are discussed.
  • [MeSH-major] Sarcoma, Ewing / etiology. Soft Tissue Neoplasms / etiology
  • [MeSH-minor] Adult. Bone Marrow Transplantation / adverse effects. Child. Family Health. Female. Genetic Predisposition to Disease. Humans. Male. Neoplastic Cells, Circulating. Pedigree. Transplantation, Homologous / adverse effects. beta-Thalassemia / complications. beta-Thalassemia / therapy

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  • (PMID = 10914053.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] ENGLAND
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12. Saylors RL 3rd, Stine KC, Sullivan J, Kepner JL, Wall DA, Bernstein ML, Harris MB, Hayashi R, Vietti TJ, Pediatric Oncology Group: Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study. J Clin Oncol; 2001 Aug 01;19(15):3463-9
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  • [Title] Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study.
  • PURPOSE: To determine the response rate of the combination of cyclophosphamide and topotecan in pediatric patients with recurrent or refractory malignant solid tumors.
  • All patients received filgrastim (5 mcg/kg) daily until the absolute neutrophil count (ANC) was > or = 1,500 microL after the time of the expected ANC nadir.
  • RESULTS: A total of 307 treatment courses were given to the 83 fully assessable patients.
  • Responses (complete response plus partial response) were seen in rhabdomyosarcoma (10 of 15 patients), Ewing's sarcoma (six of 17 patients), and neuroblastoma (six of 13 patients).
  • Partial responses were seen in two of 18 patients with osteosarcoma and in one patient with a Sertoli-Leydig cell tumor.
  • CONCLUSION: The combination of cyclophosphamide and topotecan is active in rhabdomyosarcoma, neuroblastoma, and Ewing's sarcoma.
  • The therapy can be given with acceptable hematopoietic toxicity with the use of filgrastim support.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Bone Neoplasms / drug therapy. Child. Child, Preschool. Cyclophosphamide / administration & dosage. Female. Humans. Infant. Infusions, Intravenous. Male. Neuroblastoma / drug therapy. Osteosarcoma / drug therapy. Rhabdomyosarcoma / drug therapy. Sarcoma, Ewing / drug therapy. Topotecan / administration & dosage

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  • (PMID = 11481351.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 05587; United States / NCI NIH HHS / CA / CA 07431; United States / NCI NIH HHS / CA / CA 11233; United States / NCI NIH HHS / CA / CA 15089; United States / NCI NIH HHS / CA / CA 20549; United States / NCI NIH HHS / CA / CA 25408; United States / NCI NIH HHS / CA / CA 28476; United States / NCI NIH HHS / CA / CA 29139; United States / NCI NIH HHS / CA / CA 29293; United States / NCI NIH HHS / CA / CA 29691; United States / NCI NIH HHS / CA / CA 30969; United States / NCI NIH HHS / CA / CA 32053; United States / NCI NIH HHS / CA / CA 33603; United States / NCI NIH HHS / CA / CA 35587; United States / NCI NIH HHS / CA / CA 53128; United States / NCI NIH HHS / CA / CA 69428
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 7M7YKX2N15 / Topotecan; 8N3DW7272P / Cyclophosphamide
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13. Chow WA, Chu P, Chung V, Lawrence J, Garcia D, Doroshow JH: Imatinib mesylate therapy for recurrent Ewing's family of tumors (EFT). J Clin Oncol; 2004 Jul 15;22(14_suppl):9054

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imatinib mesylate therapy for recurrent Ewing's family of tumors (EFT).
  • : 9054 Background: The prognosis for patients with recurrent Ewing's sarcoma (ES) and primitive neuroectodermal tumors (PNET) remains poor.
  • Novel therapies are urgently needed.
  • Based upon these findings, we treated a patient with recurrent EFT that expressed both c-Kit and PDGFR-α with imatinib mesylate.
  • Chemotherapy with vincristine, doxorubicin and cyclophosphamide, altenating with ifosfamide and etoposide was initiated.
  • Progression was documented after 11 cycles of chemotherapy.
  • IHC of the original tumor revealed 3+/3 IHC positivity for both c-Kit and PDGFR-α.
  • CONCLUSIONS: This case report demonstrates that imatinib mesylate was an effective alternative therapy for this patient with recurrent EFT whose tumor expressed the c-Kit and PDGFR-α TKRs.

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  • (PMID = 28014099.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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14. Karseladze AI, Filipova NA, Navarro S, Llombart-Bosch A: Primitive neuroectodermal tumor of the uterus. A case report. J Reprod Med; 2001 Sep;46(9):845-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primitive neuroectodermal tumor of the uterus. A case report.
  • Histologically the tumor was composed of uniform, rounded, oval and sometimes spindle shaped cells with a narrow rim of eosinophilic cytoplasm.
  • The cells were positive for neurogenic marker protein gene product, neuron-specific enolase and Ewing's sarcoma-related HBA-71.
  • The patient received combined therapy, external radiation to the pelvis and chemotherapy.
  • Four years later she was alive, without signs of recurrent tumor.
  • [MeSH-minor] Adolescent. Antibodies, Neoplasm / isolation & purification. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Diagnosis, Differential. Female. Humans. Hysterectomy. Immunohistochemistry

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  • (PMID = 11584489.001).
  • [ISSN] 0024-7758
  • [Journal-full-title] The Journal of reproductive medicine
  • [ISO-abbreviation] J Reprod Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Neoplasm
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15. Cohen DD, Zorn K, Bernard C, Rajan R, Kuzmarov IW: A para-testicular primitive neuroectodermal tumor in an adult: a case report and literature review. Can J Urol; 2000 Aug;7(4):1081-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A para-testicular primitive neuroectodermal tumor in an adult: a case report and literature review.
  • OBJECTIVE: The authors describe the salient clinical, radiologic and histopathologic features of an extremely rare para-testicular primitive neuroectodermal tumor in a 25 year-old man.
  • INTERVENTION: Excisional biopsy of the tumor en bloc was performed.
  • Adjuvant VAdriaC-based chemotherapy (Vincristine, Doxorubicin, and Cyclophosphamide) was given post-operatively.
  • MAIN OUTCOME MEASURES: Histopathologic examination and immunohistochemical studies were performed on formaldehyde-fixed, paraffin-embedded tumor tissue.
  • RESULTS: Histologic examination showed an undifferentiated small cell tumor.
  • The tumor cells stained positively with MIC-2, a marker specific for primitive neuroectodermal tumors.
  • The patient is 12 months post surgery and has completed adjuvant chemotherapy with no evidence of recurrent disease.
  • CONCLUSIONS: This highly unusual, peripheral primitive neuroectodermal tumor should be considered in the differential diagnosis of undifferentiated small cell neoplasms of the genitourinary system in adults, from the kidney to the testicle.
  • We present a patient with a PNET treated based on a Ewing's family of tumors protocol.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Testicular Neoplasms / pathology

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  • (PMID = 11109079.001).
  • [ISSN] 1195-9479
  • [Journal-full-title] The Canadian journal of urology
  • [ISO-abbreviation] Can J Urol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 28
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