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1. Ashford RU, McCloskey EV, Purohit OP, Ingram CE, Grimer RJ, Coleman RE: Ten-Year Follow-Up of a Patient with Metastatic Ewing's Sarcoma of the Pelvis. Sarcoma; 2002;6(4):131-3

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ten-Year Follow-Up of a Patient with Metastatic Ewing's Sarcoma of the Pelvis.
  • PATIENT: We report a 32-year-old women with a pelvic Ewing's sarcoma, who developed skeletal metastases within 20 months of diagnosis but following treatment remains disease-free at 10 years.
  • DISCUSSION: Ewing's sarcoma is a highly malignant tumour of bone.
  • In our patient there was little or no response to salvage chemotherapy, but, against the odds, remission was induced with pamidronate and palliative radiotherapy to some but not all sites of disease.This remission has been maintained without additional therapy for a further 5 years.

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  • [Cites] Cancer. 1999 Feb 15;85(4):869-77 [10091764.001]
  • [Cites] Ann Surg. 1999 Jul;230(1):79-86 [10400040.001]
  • [Cites] Cancer. 1995 Oct 15;76(8):1388-97 [8620413.001]
  • [Cites] J Bone Joint Surg Am. 1993 Oct;75(10):1457-65 [8408134.001]
  • [Cites] Br J Cancer. 2001 Apr 20;84(8):1126-34 [11308265.001]
  • (PMID = 18521349.001).
  • [ISSN] 1357-714X
  • [Journal-full-title] Sarcoma
  • [ISO-abbreviation] Sarcoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2395498
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2. Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS: Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med; 2003 Feb 20;348(8):694-701
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  • [Title] Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
  • BACKGROUND: Ewing's sarcoma and primitive neuroectodermal tumor of bone are closely related, highly malignant tumors of children, adolescents, and young adults.
  • A new drug combination, ifosfamide and etoposide, was highly effective in patients with Ewing's sarcoma or primitive neuroectodermal tumor of bone who had a relapse after standard therapy.
  • We designed a study to test whether the addition of these drugs to a standard regimen would improve the survival of patients with newly diagnosed disease.
  • METHODS: Patients 30 years old or younger with Ewing's sarcoma, primitive neuroectodermal tumor of bone, or primitive sarcoma of bone were eligible.
  • The patients were randomly assigned to receive 49 weeks of standard chemotherapy with doxorubicin, vincristine, cyclophosphamide, and dactinomycin or experimental therapy with these four drugs alternating with courses of ifosfamide and etoposide.
  • Of 120 patients with metastatic disease, 62 were randomly assigned to the standard-therapy group and 58 to the experimental-therapy group.
  • There was no significant difference in five-year event-free survival between the treatment groups (P=0.81).
  • Among the 398 patients with nonmetastatic disease, the mean (+/-SE) five-year event-free survival among the 198 patients in the experimental-therapy group was 69+/-3 percent, as compared with 54+/-4 percent among the 200 patients in the standard-therapy group (P=0.005).
  • Overall survival was also significantly better among patients in the experimental-therapy group (72+/-3.4 percent vs. 61+/-3.6 percent in the standard-therapy group, P=0.01).
  • CONCLUSIONS: The addition of ifosfamide and etoposide to a standard regimen does not affect the outcome for patients with metastatic disease, but it significantly improves the outcome for patients with nonmetastatic Ewing's sarcoma, primitive neuroectodermal tumor of bone, or primitive sarcoma of bone.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Bone Neoplasms / drug therapy. Etoposide / administration & dosage. Ifosfamide / administration & dosage. Neuroectodermal Tumors, Primitive / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Disease Progression. Disease-Free Survival. Doxorubicin / administration & dosage. Drug Administration Schedule. Female. Humans. Male. Prognosis. Sarcoma / drug therapy. Sarcoma / mortality. Sarcoma / secondary. Survival Rate. Treatment Failure. Vincristine / administration & dosage

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  • [Copyright] Copyright 2003 Massachusetts Medical Society
  • [CommentIn] N Engl J Med. 2003 Feb 20;348(8):747-9 [12594321.001]
  • [CommentIn] Natl Med J India. 2004 Mar-Apr;17(2):84-5 [15141601.001]
  • (PMID = 12594313.001).
  • [ISSN] 1533-4406
  • [Journal-full-title] The New England journal of medicine
  • [ISO-abbreviation] N. Engl. J. Med.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Randomized Controlled Trial
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide
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3. Souid AK, Fahey RC, Aktas MK, Sayin OA, Karjoo S, Newton GL, Sadowitz PD, Dubowy RL, Bernstein ML: Blood thiols following amifostine and mesna infusions, a pediatric oncology group study. Drug Metab Dispos; 2001 Nov;29(11):1460-6
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  • The Pediatric Oncology Group study for metastatic Ewing's sarcoma used amifostine and mesna with the alkylating agents.
  • To determine the fate of combined drug thiols, we measured thiol levels in plasma, red blood cells (RBC), and peripheral blood mononuclear cells (PBMC) of four patients.
  • We also conducted analogous measurements on two patients who received mesna alone and a volunteer's blood following in vitro treatment.
  • Incubation of a volunteer's blood with mesna, WR-1065, or both revealed that cellular uptake of total reducible drug was approximately 10% of plasma level for mesna but approximately 60% for WR-1065.
  • Cellular drugs were mainly the thiol form, whereas half of the plasma drugs were disulfides.
  • WR-1065 and mesna appeared in the cells by the end of the drug infusions, although WR-1065 uptake was more efficient than mesna.
  • The concentration-time profiles of mesna in RBC paralleled those in plasma.
  • Adequate dosing of either drug is necessary for protecting the cells from toxic effects of alkylating agents.
  • [MeSH-major] Amifostine / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / blood. Mesna / administration & dosage. Protective Agents / administration & dosage. Radiation-Protective Agents / administration & dosage. Sulfhydryl Compounds / blood
  • [MeSH-minor] Adolescent. Adult. Child. Chromatography, High Pressure Liquid. Disulfides / metabolism. Female. Humans. Infusions, Intravenous. Leukocytes, Mononuclear / drug effects. Leukocytes, Mononuclear / metabolism. Male. Mercaptoethylamines / administration & dosage. Mercaptoethylamines / blood. Mercaptoethylamines / therapeutic use. Sarcoma, Ewing / blood. Sarcoma, Ewing / drug therapy

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  • (PMID = 11602522.001).
  • [ISSN] 0090-9556
  • [Journal-full-title] Drug metabolism and disposition: the biological fate of chemicals
  • [ISO-abbreviation] Drug Metab. Dispos.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Disulfides; 0 / Mercaptoethylamines; 0 / Protective Agents; 0 / Radiation-Protective Agents; 0 / Sulfhydryl Compounds; 31098-42-7 / WR 1065; M487QF2F4V / Amifostine; NR7O1405Q9 / Mesna
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4. Pamukçu B, Bilge AK, Meriç M, Atilgan D: Metastatic Ewing's sarcoma involving the right ventricle. Turk Kardiyol Dern Ars; 2008 Dec;36(8):546-8
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  • [Title] Metastatic Ewing's sarcoma involving the right ventricle.
  • Cardiac metastasis of Ewing's sarcoma is rare.
  • She had a seven-year history of radical right tibial resection for Ewing's sarcoma and was also receiving chemotherapy for lung metastasis of Ewing's sarcoma.
  • The mass showed partial contrast enhancement, suggesting a malignant metastatic mass.
  • [MeSH-major] Bone Neoplasms / pathology. Echocardiography / methods. Heart Neoplasms / diagnosis. Heart Neoplasms / secondary. Sarcoma, Ewing / pathology

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  • (PMID = 19223721.001).
  • [ISSN] 1016-5169
  • [Journal-full-title] Türk Kardiyoloji Derneği arşivi : Türk Kardiyoloji Derneğinin yayın organıdır
  • [ISO-abbreviation] Turk Kardiyol Dern Ars
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Turkey
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5. Miser JS, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Grier HE: 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; 2004 Jul 15;22(14):2873-6
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  • [Title] 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.
  • PURPOSE: One hundred twenty patients with metastatic Ewing's sarcoma or primitive neuroectodermal tumor (PNET) of bone were entered onto a randomized trial evaluating whether the addition of ifosfamide and etoposide to vincristine, doxorubicin, cyclophosphamide, and dactinomycin improved outcomes.
  • Treatment comprised 9 weeks of chemotherapy before local control and 42 weeks of chemotherapy; thereafter, regimen A consisted of vincristine 2 mg/m(2), cyclophosphamide 1,200 mg/m(2), and either doxorubicin 75 mg/m(2) or dactinomycin 1.25 mg/m(2).
  • The event-free survival (EFS) and survival (S) at 8 years were 20% (SE, 5%) and 32% (SE, 6%), respectively, for those treated on regimen A and 20% (SE, 6%) and 29% (SE, 6%), respectively, for those treated on regimen B.
  • Patients who had only lung metastases had EFS and S of 32% (SE, 8%) and 41% (SE, 9%), respectively, at 8 years.
  • CONCLUSION: Adding ifosfamide and etoposide to standard therapy does not improve outcomes of patients with Ewing's sarcoma or PNET of bone with metastases at diagnosis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Humans. Ifosfamide / administration & dosage. Neoplasm Metastasis. Survival Analysis. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15254055.001).
  • [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
  • [Publication-type] Clinical Trial; Journal Article; Randomized Controlled Trial
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide
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6. Dimou AT, Syrigos KN, Saif MW: Neuroendocrine tumors of the pancreas: what's new. Highlights from the "2010 ASCO Gastrointestinal Cancers Symposium". Orlando, FL, USA. January 22-24, 2010. JOP; 2010;11(2):135-8
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  • [Title] Neuroendocrine tumors of the pancreas: what's new. Highlights from the "2010 ASCO Gastrointestinal Cancers Symposium". Orlando, FL, USA. January 22-24, 2010.
  • Surgical excision has been the mainstay of treatment for neuroendocrine tumors of the pancreas (PNET).
  • Compounds like streptozocin and dacarbazin have been traditionally used in inoperable cases and somatostatin to treat syndromes deriving from functional tumors.
  • However, a lot of progress has taken place in the area of molecular characterization of these tumors, revealing activation of mammalian target of rapamycin (mTOR) and VEGF pathways.
  • The role of modern biologic compounds in the treatment of PNET is not clear yet.
  • In addition, combination of resection and transarterial chemoembolization (TACE) has been proven effective over either modality alone in the treatment of PNET metastatic to the liver in a retrospective analysis.
  • This comes to address the problem of selecting local intervention in a metastatic disease, which has been a reasonable choice for this group of tumors in the past.
  • Last but not least the role of Ki-67 in decision-making in PNET is being discussed.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Medical Oncology / trends. Neuroendocrine Tumors / drug therapy. Pancreatic Neoplasms / drug therapy
  • [MeSH-minor] Chemoembolization, Therapeutic / methods. Clinical Trials as Topic / methods. Clinical Trials as Topic / trends. Combined Modality Therapy. Congresses as Topic. Everolimus. Gastrointestinal Neoplasms / pathology. Gastrointestinal Neoplasms / therapy. Humans. Indoles / administration & dosage. Pyrroles / administration & dosage. Retrospective Studies. Sirolimus / administration & dosage. Sirolimus / analogs & derivatives

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  • (PMID = 20208321.001).
  • [ISSN] 1590-8577
  • [Journal-full-title] JOP : Journal of the pancreas
  • [ISO-abbreviation] JOP
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Indoles; 0 / Pyrroles; 0 / sunitinib; 9HW64Q8G6G / Everolimus; W36ZG6FT64 / Sirolimus
  • [Number-of-references] 23
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7. Bernstein ML, Devidas M, Lafreniere D, Souid AK, Meyers PA, Gebhardt M, Stine K, Nicholas R, Perlman EJ, Dubowy R, Wainer IW, Dickman PS, Link MP, Goorin A, Grier HE, Pediatric Oncology Group, Children's Cancer Group Phase II Study 9457, Children's Oncology Group: Intensive therapy with growth factor support for patients with Ewing tumor metastatic at diagnosis: Pediatric Oncology Group/Children's Cancer Group Phase II Study 9457--a report from the Children's Oncology Group. J Clin Oncol; 2006 Jan 1;24(1):152-9
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  • [Title] Intensive therapy with growth factor support for patients with Ewing tumor metastatic at diagnosis: Pediatric Oncology Group/Children's Cancer Group Phase II Study 9457--a report from the Children's Oncology Group.
  • PURPOSE: Prognosis is poor for Ewing sarcoma patients with metastasis at diagnosis.
  • We intensified a five-drug therapy (ifosfamide, etoposide alternated with vincristine, doxorubicin, and cyclophosphamide) using filgrastim but not stem-cell support.
  • We studied topotecan alone and combined with cyclophosphamide in therapeutic windows before the five-drug therapy.
  • PATIENTS AND METHODS: Eligible patients were < or = 30 years old and had histologically proven Ewing sarcoma or primitive neuroectodermal tumor (PNET) and metastasis at diagnosis.
  • The best responses to the overall therapy included 45 complete responses, 41 PRs, stable disease in 14 patients, and PD in five patients.
  • CONCLUSION: Topotecan had limited activity in patients with Ewing sarcoma or PNET metastatic at diagnosis.
  • [MeSH-major] Amifostine / therapeutic use. Bone Neoplasms / drug therapy. Cyclophosphamide / administration & dosage. Sarcoma, Ewing / drug therapy. Topotecan / therapeutic use

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  • (PMID = 16382125.001).
  • [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] Clinical Trial, Phase II; Journal Article; Randomized Controlled Trial
  • [Publication-country] United States
  • [Chemical-registry-number] 7M7YKX2N15 / Topotecan; 8N3DW7272P / Cyclophosphamide; M487QF2F4V / Amifostine
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8. 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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  • [Title] Topotecan combined with myeloablative doses of thiotepa and carboplatin for neuroblastoma, brain tumors, and other poor-risk solid tumors in children and young adults.
  • Twenty-one patients with poor-prognosis tumors and intact function of key organs received topotecan 2 mg/m2 by 30-min intravenous (i.v.) infusion on days -8, -7, -6, -5, -4; thiotepa 300 mg/m2 by 3 h i.v. infusion on days -8, -7, -6; and carboplatin by 4 h i.v. infusion on days -5, -4, -3 with a daily dose derived from the pediatric Calvert formula, using a targeted area under the curve of seven mg/ml* min ( approximately 500 mg/m2/day).
  • 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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9. Hendershot E: Treatment approaches for metastatic Ewing's sarcoma: a review of the literature. J Pediatr Oncol Nurs; 2005 Nov-Dec;22(6):339-52
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  • [Title] Treatment approaches for metastatic Ewing's sarcoma: a review of the literature.
  • The Ewing's sarcoma family of tumors (ESFT) is an aggressive group of neoplasms that represent approximately 3% of all pediatric malignancies.
  • The outcome for the 25% of patients who present with metastatic disease, however, remains poor, with long-term survival rates of less than 30%.
  • Outcomes of metastatic treatment protocols to date will be examined as well as the rationale for current and future therapies.
  • Nursing considerations in caring for patients with metastatic ESFT will be discussed.
  • A case scenario will be reviewed to highlight treatment and supportive care issues in the management of the disease.
  • Cancer therapy in general is becoming more complex; treatment approaches involve different ways of targeting tumor cells.
  • It is crucial that nurses caring for these patients understand the rationale behind treatment strategies so that appropriate patient education and support may be given.
  • [MeSH-major] Neoplasm Metastasis / drug therapy. Sarcoma, Ewing / therapy

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  • (PMID = 16216896.001).
  • [ISSN] 1043-4542
  • [Journal-full-title] Journal of pediatric oncology nursing : official journal of the Association of Pediatric Oncology Nurses
  • [ISO-abbreviation] J Pediatr Oncol Nurs
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 43
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10. Faulkner CF, Godbolt AM, DeAmbrosis B, Triscott J: Hand, foot and mouth disease in an immunocompromised adult treated with aciclovir. Australas J Dermatol; 2003 Aug;44(3):203-6
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  • A 27-year-old man, immunosuppressed from recent chemotherapy for metastatic Ewing's sarcoma, presented with a 1-week history of a painful, pruritic, papulovesicular eruption on the hands and feet.
  • A diagnosis of hand, foot and mouth disease was made based on histology, detection of Enterovirus ribonucleic acid by polymerase chain reaction on a swab from a vesicle, and a four-fold increase in Enterovirus antibody levels.
  • After 3 1/2 weeks he was commenced on oral aciclovir 200 mg five times daily, with subsequent resolution of all lesions within 5 days.
  • [MeSH-minor] Acyclovir / therapeutic use. Adult. Antiviral Agents / therapeutic use. Diagnosis, Differential. Enterovirus / isolation & purification. Humans. Male. Sarcoma, Ewing / complications. Sarcoma, Ewing / therapy. Treatment Outcome

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  • (PMID = 12869047.001).
  • [ISSN] 0004-8380
  • [Journal-full-title] The Australasian journal of dermatology
  • [ISO-abbreviation] Australas. J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Antiviral Agents; X4HES1O11F / Acyclovir
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11. 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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  • [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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12. Kushner BH, Meyers PA: How effective is dose-intensive/myeloablative therapy against Ewing's sarcoma/primitive neuroectodermal tumor metastatic to bone or bone marrow? The Memorial Sloan-Kettering experience and a literature review. J Clin Oncol; 2001 Feb 01;19(3):870-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] How effective is dose-intensive/myeloablative therapy against Ewing's sarcoma/primitive neuroectodermal tumor metastatic to bone or bone marrow? The Memorial Sloan-Kettering experience and a literature review.
  • PURPOSE: Attempts to improve outcomes of patients with Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) metastatic to bone/bone marrow (BM) have focused on chemotherapy dose intensification strategies.
  • PATIENTS AND METHODS: Twenty-one unselected MSKCC patients with newly diagnosed ES/PNET metastatic to bone/BM received the "P6" protocol which includes cycles of cyclophosphamide (4.2 g/m(2))/doxorubicin (75 mg/m(2))/vincristine and cycles of ifosfamide (9 g/m(2))/etoposide (500 mg/m(2)).
  • Patients in complete/very good partial remission (CR/VGPR) after P6 received myeloablative therapy with either total-body irradiation (TBI) (hyperfractionated 15 Gy)/melphalan (180 mg/m(2)) or thiotepa (900 mg/m(2))/carboplatin (1,500 mg/m(2)).
  • Initial responses to P6 were CR/VGPR in 19 patients, but eight of them plus two others developed PD while receiving or shortly after completing P6.
  • Eight patients were treated with TBI/melphalan: four relapsed 2 to 7 months after transplantation; two died early of toxicity; one died of pulmonary failure 17 months after transplantation (no evidence of ES/PNET); and one remains in CR at more than 7 years.
  • All reports on large series of unselected patients with ES/PNET metastatic to bone/BM showed similarly unsatisfactory results.
  • Poor outcome was seen with use of active agents for ES/PNET-cyclophosphamide, ifosfamide, doxorubicin, dactinomycin, vincristine, etoposide - at standard dosages for prolonged periods of time and at higher dosages in intensive regimens for short or prolonged periods of time.
  • CONCLUSION: The MSKCC experience and findings reported in the literature suggest that dose-intensive use of the chemotherapy agents with established activity against ES/PNET is reaching its efficacy and toxicity limits.
  • A major impact on prognosis awaits the development of entirely novel therapies.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow Neoplasms / drug therapy. Bone Neoplasms / drug therapy. Brain Neoplasms / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Male. Melphalan / administration & dosage. Thiotepa / administration & dosage. Vincristine / administration & dosage. Whole-Body Irradiation

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  • (PMID = 11157041.001).
  • [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
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 905Z5W3GKH / Thiotepa; Q41OR9510P / Melphalan; UM20QQM95Y / Ifosfamide; CAV protocol
  • [Number-of-references] 63
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13. Brames M, Ehrlich Y, Einhorn L: Malignant transformation of teratoma to primitive neuroectodermal tumor (PNET): Outcome analysis with PNET based chemotherapy. J Clin Oncol; 2009 May 20;27(15_suppl):e16121

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant transformation of teratoma to primitive neuroectodermal tumor (PNET): Outcome analysis with PNET based chemotherapy.
  • : e16121 Background: Metastatic testis cancer is uniquely chemosensitive and highly curable.
  • We report results of malignant transformation of teratoma to metastatic PNET treated with PNET based chemotherapy.
  • METHODS: Retrospective review of 42 patients with PNET in testis or at metastatic sites seen at Indiana University from 1999-2007.
  • Of these 42 patients, 10 had inoperable or unresectable PNET treated with cyclophosphamide 1200 mg/M<sup>2</sup>, doxorubicin 75 mg/M<sup>2</sup>, and vincristine 2 mg IV alternating with ifosfamide 1.8 grams/M<sup>2</sup> × 5 days plus etoposide 100 mg/M<sup>2</sup> × 5 days.
  • RESULTS: 9 of 10 patients had at least 1 prior platinum based combination chemotherapy regimen for their germ cell tumor.
  • Tumor markers (human chorionic gonadotropin and alphafetoprotein) were normal at start of chemotherapy for biopsy proven PNET.
  • Two of 10 are currently disease-free for 16 and 33 months from initiation of PNET specific chemotherapy and 3 other patients are alive with disease at 73, 34, and 30 months.
  • CONCLUSIONS: PNET specific chemotherapy has a high objective response rate for malignant transformation of teratoma to PNET, with some patients capable of long-term survival with chemotherapy followed by surgical resection.

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  • (PMID = 27963389.001).
  • [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. Ateser G, Yildiz O, Leblebici C, Mandel NM, Unal F, Turna H, Arikan I, Colcaki D: Metastatic primitive neuroectodermal tumor of the ovary in pregnancy. Int J Gynecol Cancer; 2007 Jan-Feb;17(1):266-9
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  • [Title] Metastatic primitive neuroectodermal tumor of the ovary in pregnancy.
  • Primitive neuroectodermal tumor (PNET) is a small round tumor belonging to the PNET/Ewing's sarcoma family.
  • We hereby report a case of PNET of the ovary, which was detected at the second trimester of pregnancy.
  • Chemotherapy was administered and a healthy baby was delivered by cesarean section.
  • After the pregnancy, the mother was found to have metastatic disease.
  • Chemotherapy was continued, but she died due to progressive disease 13 months after the initial diagnosis.
  • In this case report, we discuss chemotherapy options during pregnancy and the importance of multidisciplinary approach to unusual presentations of rare tumors.
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Ovarian Neoplasms / drug therapy. Ovarian Neoplasms / pathology. Pregnancy Complications, Neoplastic / drug therapy. Pregnancy Complications, Neoplastic / pathology

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  • (PMID = 17291265.001).
  • [ISSN] 1048-891X
  • [Journal-full-title] International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
  • [ISO-abbreviation] Int. J. Gynecol. Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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15. Shamberger RC, Grier HE: Ewing's sarcoma/primitive neuroectodermal tumor of the chest wall. Semin Pediatr Surg; 2001 Aug;10(3):153-60
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  • [Title] Ewing's sarcoma/primitive neuroectodermal tumor of the chest wall.
  • Ewing's sarcoma/primitive neuroectodermal tumor is the most common tumor of the chest wall in children and adolescents.
  • It is extremely malignant with a high frequency of both metastatic spread and of local recurrence.
  • Cure requires intensive therapy to control both distant and local disease.
  • The optimal therapeutic sequence is initial biopsy followed by induction chemotherapy with subsequent resection of the primary tumor.
  • This approach will achieve the lowest incidence of tumor present at the margins of resection and, hence, need for postoperative radiotherapy.
  • The chest wall is a rare site for tumors in children and adolescents.
  • In a series reported from St Jude's Children's Research Hospital, chest wall tumors constituted only 1.8% of the solid childhood tumors.
  • They are primarily mesenchymal in origin and the Ewing's sarcoma/primitive neuroectodermal tumors (PNET) predominate.
  • This report concentrates on the later tumors.
  • They are recognized to be extremely malignant, and cure in those who present with metastatic disease is very difficult to achieve.
  • Recent advances in our understanding of their cytogenetic basis and optimal treatment are presented.
  • [MeSH-major] Neuroectodermal Tumors, Primitive / pathology. Neuroectodermal Tumors, Primitive / therapy. Sarcoma, Ewing / pathology. Sarcoma, Ewing / therapy
  • [MeSH-minor] Chemotherapy, Adjuvant. Child. Child, Preschool. Disease-Free Survival. Female. Humans. Male. Retrospective Studies. Treatment Outcome

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  • [Copyright] Copyright 2001 by W.B. Saunders Company
  • (PMID = 11481653.001).
  • [ISSN] 1055-8586
  • [Journal-full-title] Seminars in pediatric surgery
  • [ISO-abbreviation] Semin. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. 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
  •  go-up   go-down


17. Meyers PA, Krailo MD, Ladanyi M, Chan KW, Sailer SL, Dickman PS, Baker DL, Davis JH, Gerbing RB, Grovas A, Herzog CE, Lindsley KL, Liu-Mares W, Nachman JB, Sieger L, Wadman J, Gorlick RG: 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; 2001 Jun 01;19(11):2812-20
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  • [Title] 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.
  • PURPOSE: To determine whether consolidation therapy with high-dose melphalan, etoposide, and total-body irradiation (TBI) with autologous stem-cell support would improve the prognosis for patients with newly diagnosed metastatic Ewing's sarcoma (ES).
  • PATIENTS AND METHODS: Thirty-two eligible patients with newly diagnosed ES metastatic to bone and/or bone marrow were enrolled onto this study.
  • Treatment was initially comprised of five cycles of induction chemotherapy (cyclophosphamide, doxorubicin, and vincristine alternating with ifosfamide and etoposide) and local control.
  • Peripheral-blood stem-cell collection was performed after the second cycle of chemotherapy, with delay if the bone marrow was persistently involved.
  • If patients had a good response to initial therapy, they proceeded to consolidation therapy with melphalan, etoposide, TBI, and stem-cell support.
  • RESULTS: Of the 32 eligible patients, 23 proceeded to high-dose therapy consolidation.
  • Three patients died from toxicity during the high-dose phase of the therapy.
  • The majority of the patients who underwent high-dose consolidation therapy experienced relapse and died with progressive disease.
  • The 2-year post-stem-cell reconstitution EFS for the subset of 23 patients who received consolidation therapy is 24%.
  • Analysis of peripheral-blood stem-cell collections by molecular techniques for minimal residual disease showed contamination of at least some samples by tumor cells in all three patients with available data.
  • CONCLUSION: Consolidation with high-dose melphalan, etoposide, TBI, and autologous stem-cell support failed to improve the probability of EFS in this cohort of patients with newly diagnosed metastatic ES.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Hematopoietic Stem Cell Transplantation. Sarcoma, Ewing / therapy. Whole-Body Irradiation
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease Progression. Dose-Response Relationship, Drug. Etoposide / administration & dosage. Female. Humans. Infant. Male. Melphalan / administration & dosage. Neoplasm Metastasis. Prognosis. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 11387352.001).
  • [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 03750; United States / NCI NIH HHS / CA / CA 07306; United States / NCI NIH HHS / CA / R25 CA092049; United States / NCI NIH HHS / CA / CA 14560; United States / NCI NIH HHS / CA / CA 28882; United States / NCI NIH HHS / CA / CA 02971; United States / NCI NIH HHS / CA / CA 26044; United States / NCI NIH HHS / CA / CA 17829; United States / NCI NIH HHS / CA / CA 27678; United States / NCI NIH HHS / CA / CA 13539; United States / NCI NIH HHS / CA / CA 05426; United States / NCI NIH HHS / CA / CA 26126; United States / NCI NIH HHS / CA / CA03888
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q41OR9510P / Melphalan
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18. Peng RJ, Sun XF, Xiang XJ, Zhen ZJ, Ling JY, Tong GL, Xia Y, Xu GC, Jiang WQ: [Efficacy and survival of 92 cases of Ewing's sarcoma family of tumor initially treated with multidisciplinary therapy]. Ai Zheng; 2009 Dec;28(12):1304-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Efficacy and survival of 92 cases of Ewing's sarcoma family of tumor initially treated with multidisciplinary therapy].
  • BACKGROUND AND OBJECTIVE: Ewing's sarcoma family of tumor (ESFT) is aggressive.
  • The optimal therapy modality for ESFT is still to be found.
  • This study was to explore the clinical characteristics and therapy for ESFT.
  • RESULT: Of 92 cases, 23 were Ewing's sarcoma of bone, 21 extraosseous Ewing's sarcoma, 43 peripheral primitive neuroectodermal tumor, and 5 Askin tumor.
  • Median follow-up time was 31.5 months (range, 10-137 months).
  • Thirty-eight patients received multidisciplinary therapy and 19 single model therapy in non-metastasis group.
  • Three-year overall survival (OS) and event-free survival (EFS) were significantly different between non-metastatic multidisciplinary therapy group and non-metastatic single model group (63% vs. 20%, 46% vs. 18%, respectively, P<0.001).
  • The patients who received surgery plus chemotherapy and plus radiation or not had longer survival than those treated with chemotherapy plus radiation in non-metastatic multidisciplinary therapy group (Chi2=7.591, 9.212; P=0.006, 0.002).
  • Cox regression analysis suggested therapy model and response to treatment were independent prognostic factors for ESFT.
  • CONCLUSIONS: Our studying showed multidisciplinary therapy could significantly improve non-metastatic ESFT patients' survival.
  • Chemotherapy plus surgery and plus radiation or not were superior to chemotherapy plus radiation in local control for the non-metastatic ESFT.
  • Therapy model and response were independent prognostic factors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Cyclophosphamide / therapeutic use. Disease-Free Survival. Doxorubicin / therapeutic use. Female. Follow-Up Studies. Humans. Infant. Lymphatic Metastasis. Male. Middle Aged. Pelvic Neoplasms / drug therapy. Pelvic Neoplasms / pathology. Pelvic Neoplasms / radiotherapy. Pelvic Neoplasms / surgery. Survival Rate. Vincristine / therapeutic use. Young Adult

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  • (PMID = 19958626.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; CAV protocol
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19. Jimenez RE, Folpe AL, Lapham RL, Ro JY, O'Shea PA, Weiss SW, Amin MB: Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases. Am J Surg Pathol; 2002 Mar;26(3):320-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases.
  • Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors, including blastema-predominant Wilms' tumor (WT).
  • Approximately 90% of ES/PNET have a specific t(11;22), which results in a chimeric EWS-FLI-1 protein.
  • Immunohistochemistry for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET.
  • WT-1, the WT-associated tumor suppressor gene, is overexpressed in WT but not in ES/PNET.
  • No study has examined FLI-1 or WT-1 expression in renal ES/PNET.
  • The clinicopathologic features of 11 renal ES/PNET were studied along with immunohistochemistry for cytokeratin, desmin, CD99, FLI-1, and WT-1.
  • The mean tumor size was 11.8 +/- 3.8 cm (mean +/- standard deviation).
  • Grossly, all tumors showed necrosis and hemorrhage, and 4 had cystic change.
  • Microscopically, all tumors showed vaguely lobular growth, primitive round cells, and variable rosette formation.
  • Immunohistochemical results on the renal ES/PNET were cytokeratin (2/8 focal), desmin (0/9), CD99 (8/8), FLI-1 (5/8), and WT-1 (0/8).
  • Follow-up on 8 cases (mean, 28 mo; range, 6-64 mo) showed 4 lung and pleural metastases, 1 bone metastasis, liver metastasis, 2 local recurrences, and 5 deaths from disease (median time to death, 16.8 mo).
  • No case had distant metastatic disease at presentation.
  • Adjuvant therapy included chemotherapy (8 cases), radiation (3 cases), and bone marrow transplantation (1 case).
  • Our study affirms a unique proclivity of renal ES/PNET for young adults and that it is a highly aggressive neoplasm, with rapid death in many cases, usually after the development of treatment-resistant lung metastases.
  • These tumors must be distinguished from blastema-predominant WT and other primitive renal tumors that require different therapy.
  • FLI-1 and WT-1 immunohistochemistry may be valuable in this differential diagnosis, given the known immunophenotypic overlap between ES/PNET and blastema-predominant WT with regard to CD99, cytokeratin, and desmin.
  • The accurate distinction between these two entities has clear prognostic and therapeutic implications.
  • [MeSH-major] Kidney Neoplasms / pathology. Neuroectodermal Tumors, Primitive / pathology. Proto-Oncogene Proteins. Sarcoma, Ewing / pathology
  • [MeSH-minor] Adult. Aged. Antigens, CD / analysis. Cell Adhesion Molecules / analysis. Child. Combined Modality Therapy. DNA-Binding Proteins / analysis. Desmin / analysis. Diagnosis, Differential. Female. Genes, Tumor Suppressor. Humans. Immunohistochemistry. Keratins / analysis. Male. Middle Aged. Neoplasm Metastasis. Proto-Oncogene Protein c-fli-1. Trans-Activators / analysis. WT1 Proteins / analysis. Wilms Tumor / chemistry. Wilms Tumor / genetics. Wilms Tumor / pathology

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  • [CommentIn] Am J Surg Pathol. 2003 Aug;27(8):1177 [12883255.001]
  • (PMID = 11859203.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / DNA-Binding Proteins; 0 / Desmin; 0 / Proto-Oncogene Protein c-fli-1; 0 / Proto-Oncogene Proteins; 0 / Trans-Activators; 0 / WT1 Proteins; 68238-35-7 / Keratins
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20. Casella R, Moch H, Rochlitz C, Meier V, Seifert B, Mihatsch MJ, Gasser TC: Metastatic primitive neuroectodermal tumor of the kidney in adults. Eur Urol; 2001 May;39(5):613-7
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  • [Title] Metastatic primitive neuroectodermal tumor of the kidney in adults.
  • OBJECTIVE: Primitive neuroectodermal tumors (PNET) of the kidney are rare and highly aggressive malignancies.
  • The purpose of our study was to present information about the management of patients with metastatic disease.
  • METHODS: The records of 2 patients (30-year-old female and 32-year-old male) with metastatic PNET of the kidney were reviewed and our data compared with the literature.
  • RESULTS: Neither clinical evaluation nor radiological methods allowed to distinguish PNET from renal cell carcinoma.
  • Immunohistochemistry revealed strong positivity for CD99 in tumor 1 and weak positivity for NSE and vimentin in both tumors.
  • In tumor 2, EWS/FLI1 translocation was detected by RT-PCR.
  • Patient 1 underwent nephrectomy, seven cycles of polychemotherapy, two cycles of high-dose chemotherapy, autologous bone marrow rescue, radiotherapy of suspicious skeletal foci and is without evidence of recurrent disease 28 months after therapy.
  • Patient 2 underwent six cycles of polychemotherapy, nephrectomy, high-dose chemotherapy with cyclophosphamide and abdominal radiotherapy.
  • Because of relapse high-dose chemotherapy with stem cell rescue was not performed.
  • CONCLUSIONS: The diagnosis of renal PNET must be considered in young patients with renal neoplasm, particularly those with advanced disease at presentation.
  • Achieving exact diagnosis has important clinical consequences because polychemotherapy and high-dose chemotherapy may lead to dramatic tumor reduction or even complete remission.
  • [MeSH-major] Kidney Neoplasms / pathology. Neuroectodermal Tumors, Primitive / pathology
  • [MeSH-minor] Adult. Antigens, CD / analysis. Cell Adhesion Molecules / analysis. Combined Modality Therapy. Fatal Outcome. Female. Humans. Liver Neoplasms / secondary. Lung Neoplasms / secondary. Male

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  • (PMID = 11464048.001).
  • [ISSN] 0302-2838
  • [Journal-full-title] European urology
  • [ISO-abbreviation] Eur. Urol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
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21. Schwinger W, Klass V, Benesch M, Lackner H, Dornbusch HJ, Sovinz P, Moser A, Schwantzer G, Urban C: Feasibility of high-dose interleukin-2 in heavily pretreated pediatric cancer patients. Ann Oncol; 2005 Jul;16(7):1199-206
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: The administration of high-dose interleukin-2 (IL-2) seems to be a therapeutic option for children with refractory and metastatic solid malignancies.
  • METHODS: We prospectively studied treatment-related toxicities, quality of life and laboratory parameters in 10 children with progressive or metastatic solid tumors (metastatic osteosarcoma, n=4; neuroblastoma stage IV, n=3; metastatic Ewing's sarcoma, n=2; metastatic Wilms' tumor, n=1) during IL-2 therapy.
  • RESULTS: All patients developed fever >39 degrees C and influenza-like symptoms, with a significant decrease in Karnofsky score.
  • In two patients treatment had to be stopped after three cycles because of severe side-effects.
  • During IL-2 therapy a statistical significant increase in white blood cells (WBC), creatinine, gamma-glutamyltransferase, C-reactive protein, glucose and body weight was observed.
  • No constant quantitative changes in total lymphocytes and subsets were observed during IL-2 therapy.
  • CONCLUSIONS: IL-2 treatment in children with refractory and relapsed solid malignancies is associated with severe, but reversible, side-effects.
  • However, five of the 10 patients with diseases of worst prognosis could be rescued by this treatment.

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  • (PMID = 15849223.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Interleukin-2
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22. Saran F, Baumert BG, Creak AL, Warrington AP, Ashley S, Traish D, Brada M: Hypofractionated stereotactic radiotherapy in the management of recurrent or residual medulloblastoma/PNET. Pediatr Blood Cancer; 2008 Mar;50(3):554-60
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  • [Title] Hypofractionated stereotactic radiotherapy in the management of recurrent or residual medulloblastoma/PNET.
  • PURPOSE: To evaluate the efficacy and toxicity of hypofractionated stereotactic radiotherapy in the management of locally recurrent or residual central nervous system (CNS) primitive neuroectodermal tumors (PNETs).
  • PATIENTS AND METHODS: Between 1991 and 2005, 12 patients with locally recurrent medulloblastoma and two patients with residual supratentorial PNET were treated with hypofractionated stereotactic conformal radiotherapy (SCRT).
  • Nine of 12 patients underwent resection at recurrence and 13 patients received at least one cycle of chemotherapy prior to SCRT.
  • All received focal SCRT (30-40 Gy/6-8 #) using non-coplanar arcs (n = 6) or fixed conformal non-coplanar fields (n = 8).
  • Causes of death were recurrent CNS disease (n = 7), herpes encephalitis (n = 1), and metastatic PNET outside the CNS (n = 1).
  • CONCLUSION: Hypofractionated SCRT provides effective local control with acceptable toxicity for patients with recurrent localized PNET.
  • However, overall long-term disease control is rare and limited by the occurrence of CSF mediated relapses, which thus could benefit from intensive systemic chemotherapy as part of the primary relapse strategy even in local recurrences.
  • Larger multi-national studies will be necessary to assess the value of such combined treatment approaches.
  • [MeSH-major] Cerebellar Neoplasms / radiotherapy. Dose Fractionation. Medulloblastoma / radiotherapy. Neuroectodermal Tumors, Primitive / radiotherapy. Radiotherapy, Conformal / methods. Supratentorial Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Male. Neoplasm Recurrence, Local / radiotherapy. Neoplasm, Residual. Palliative Care. Retrospective Studies. Stereotaxic Techniques

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17941071.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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23. Kara IO, Gonlusen G, Sahin B, Ergin M, Erdogan S: A general aspect on soft-tissue sarcoma and c-kit expression in primitive neuroectodermal tumor and Ewing's sarcoma. Is there any role in disease process? Saudi Med J; 2005 Aug;26(8):1190-6
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  • [Title] A general aspect on soft-tissue sarcoma and c-kit expression in primitive neuroectodermal tumor and Ewing's sarcoma. Is there any role in disease process?
  • OBJECTIVE: Within soft-tissue sarcoma, primitive neuroectodermal tumors have been shown to cover a wide spectrum of small round cell sarcomas, including Ewing's sarcomas (ES) and primitive neuroectodermal tumors (PNET).
  • The role of the stem cell factor/kit pathway has been investigated in different human tumors especially in chronic myelocytic leukemia and gastrointestinal stromal tumor and an autocrine loop has been assumed in small cell lung carcinoma, and recently in ES and PNET.
  • Our aim is to investigate the c-kit expression in ES and PNET and also to assessed if c-kit has any role in disease process.
  • METHODS: We thoroughly searched the archives of the Department of Pathology, Faculty of Medicine, Cukurova University Turkey, between 2000 and 2004; and found 14 ES and 14 PNET paraffin embedded tissues.
  • We carried out the detection of the c-kit expression by immunohistochemical staining.
  • Five were diagnosed as metastatic disease whereas 23 were diagnosed as non-metastatic disease at admission.
  • According to treatment modalities, 10 were treated with surgery alone, 11 with surgery and chemotherapy, and 7 with surgery, radiation therapy and also with chemotherapy.
  • The primary tumor was lower than 5 cm in its dimension in 21 patients.
  • While in 5 patients, tumor was more than 5 cm but did not exceed 10 cm, it was >10 cm in 2 patients.
  • In 5 PNET patients, c-kit expression were stained immunohistochemically in over 50% and in 3 of ES patients.
  • There was no significant correlation between c-kit expression and gender, localization, metastatic status, treatment modalities and tumor.
  • CONCLUSION: We suggest that therapy with tyrosine kinase inhibitor for PNET and ES patients may be an alternative in addition to standard therapy modalities, especially in patients non-responsive to standard therapy.
  • [MeSH-major] Neuroectodermal Tumors, Primitive / metabolism. Neuroectodermal Tumors, Primitive / pathology. Proto-Oncogene Proteins c-kit / metabolism. Sarcoma, Ewing / metabolism. Sarcoma, Ewing / pathology

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  • (PMID = 16127511.001).
  • [ISSN] 0379-5284
  • [Journal-full-title] Saudi medical journal
  • [ISO-abbreviation] Saudi Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Saudi Arabia
  • [Chemical-registry-number] EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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