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1. Mittal R, Al Awadi S, Sahar O, Behbehani AM: Ewing's sarcoma as second malignant neoplasm after retinoblastoma: a case report. Med Princ Pract; 2008;17(1):84-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ewing's sarcoma as second malignant neoplasm after retinoblastoma: a case report.
  • OBJECTIVES: To report a case of a child with the hereditary form of unilateral retinoblastoma (RB), who developed Ewing's sarcoma of the right fibula 3 years after the enucleation of the right eye.
  • He was fully investigated and found to have locally advanced RB with bone marrow involvement (Reese-Ellsworth stage IVA).
  • The patient received chemotherapy and diode laser thermotherapy in Kuwait and the UK.
  • After extensive investigations, it was reported as Ewing's sarcoma.
  • He was treated with chemotherapy, surgery (complete excision of the fibula) and high-dose chemotherapy followed by autologous stem cell transplantation.
  • The child is now nearly 2 years after completing the treatment and is disease free.
  • These children need a very close follow-up for the early diagnosis of SMNs or even subsequent malignancies.
  • [MeSH-major] Bone Neoplasms / diagnosis. Fibula. Neoplasms, Second Primary / diagnosis. Retinal Neoplasms / diagnosis. Retinoblastoma / diagnosis. Sarcoma, Ewing / diagnosis
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Diagnosis, Differential. Eye Enucleation. Humans. Infant. Male. Stem Cell Transplantation. Treatment Outcome

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  • [Copyright] (c) 2007 S. Karger AG, Basel.
  • (PMID = 18059108.001).
  • [ISSN] 1423-0151
  • [Journal-full-title] Medical principles and practice : international journal of the Kuwait University, Health Science Centre
  • [ISO-abbreviation] Med Princ Pract
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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2. Hotte SJ, Smith AM, Bramwell VH, Howson-Jan K: High-Dose Chemotherapy Followed by Peripheral and/or Bone Marrow Stem Cell Transplant in Patients With Advanced Sarcoma: Experience of a Canadian Centre. Sarcoma; 2004;8(2-3):63-9

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  • [Title] High-Dose Chemotherapy Followed by Peripheral and/or Bone Marrow Stem Cell Transplant in Patients With Advanced Sarcoma: Experience of a Canadian Centre.
  • We present results of 24 patients with sarcoma undergoing autotransplantation at a Canadian institution.
  • PATIENTS AND METHODS: Twenty-four patients were treated between 1988 and 1998: 23 were >/=18 years (median 27; range 12-56); genders were equal; 12 patients had Ewing's sarcoma.
  • At diagnosis, 12 (50%) had metastatic disease.
  • Prior to autotransplant, all had >/=1 chemotherapy regimen.
  • Of the four alive, three had Ewing's sarcoma, one alveolar rhabdomyosarcoma, and all were in CR at transplant.
  • Median time to relapse was 6 months (2-59).
  • CONCLUSIONS: Stem cell autotransplantation does not benefit most patients with sarcoma.

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  • (PMID = 18521397.001).
  • [ISSN] 1357-714X
  • [Journal-full-title] Sarcoma
  • [ISO-abbreviation] Sarcoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2395608
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3. Horn B, Reiss U, Matthay K, McMillan A, Cowan M: Veno-occlusive disease of the liver in children with solid tumors undergoing autologous hematopoietic progenitor cell transplantation: a high incidence in patients with neuroblastoma. Bone Marrow Transplant; 2002 Mar;29(5):409-15
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  • We retrospectively analyzed the incidence and risk factors for veno-occlusive disease (VOD) in 83 consecutive children with solid tumors, who underwent autologous blood or bone marrow (BM) transplantation at UCSF between 1992 and 2000.
  • Forty-one patients were diagnosed with neuroblastoma and 42 had another solid tumor (Ewing's sarcoma, soft tissue sarcomas, germ cell tumors, etc).
  • Twenty patients (24%) developed VOD.
  • Univariate analysis identified the following risk factors for VOD: diagnosis of neuroblastoma (odds ratio 6.1, P < 0.01), abdominal radiation (odds ratio 4.1, P < 0.01), abdominal surgery (odds ratio 4.1, P < 0.01), and age < or =7 years of age (odds ratio 3.3, P = 0.02).
  • Disease status at transplant, intensity of previous chemotherapy, conditioning regimen, progenitor cell source, ALT, AST, albumin level, renal function prior to transplant, or use of amphotericin, growth-factor or heparin during transplant, did not affect the incidence of VOD.
  • On multivariate analysis, only the diagnosis of neuroblastoma remained significant (odds ratio 7.8, P = 0.03).
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Humans. Incidence. Infant. Multivariate Analysis. Neoplasms / complications. Neoplasms / epidemiology. Neoplasms / therapy. Retrospective Studies. Risk Factors. Transplantation, Autologous. Treatment Outcome


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4. Kerst JM, Van Coevorden F, Peterse J, Haas RL, Linn SC: [Young adults with Ewing's sarcoma]. Ned Tijdschr Geneeskd; 2004 Jul 3;148(27):1355-8
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  • [Title] [Young adults with Ewing's sarcoma].
  • Ewing's sarcoma was diagnosed in three men, one aged 22 and two aged 30.
  • In the youngest patient with localised disease, supplementary radiotherapy was withheld in view of the good results of induction chemotherapy, surgery and consolidation chemotherapy.
  • However, four months later, there was a localised recurrence, again followed by induction chemotherapy, chemotherapy at high dosage, stem cell transplantation, radiotherapy and finally surgical intervention, after which a complete remission was achieved.
  • The 30-year-old man with localised disease was given induction chemotherapy, surgery, consolidation chemotherapy and radiotherapy; 14 months after the diagnosis he was in good condition.
  • Despite intensive therapy he died 8 months after diagnosis.
  • Ewing's sarcoma is a musculoskeletal malignancy that occurs in children and adolescents but also in young adults.
  • It generally manifests itself as a painful swelling originating in bone or soft tissue.
  • In 20-25% of cases there are already metastases (to the lungs, bone and bone marrow) by the time of diagnosis.
  • The diagnosis and treatment of this rare, therapy-sensitive disease should take place in a study setting and in co-operation with a multidisciplinary sarcoma working group.
  • [MeSH-major] Bone Neoplasms / diagnosis. Sarcoma, Ewing / diagnosis
  • [MeSH-minor] Adult. Combined Modality Therapy. Fatal Outcome. Humans. Lung Neoplasms / secondary. Male. Neoplasm Metastasis. Neoplasm Recurrence, Local. Treatment Outcome

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  • (PMID = 15283029.001).
  • [ISSN] 0028-2162
  • [Journal-full-title] Nederlands tijdschrift voor geneeskunde
  • [ISO-abbreviation] Ned Tijdschr Geneeskd
  • [Language] dut
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Netherlands
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5. Kushen M, Gulbahce HE, Lam CH: Ewing's sarcoma of the cavernous sinus: case report. Neurosurgery; 2005 Jun;56(6):E1375; discussion E1375
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  • [Title] Ewing's sarcoma of the cavernous sinus: case report.
  • OBJECTIVE AND IMPORTANCE: The Ewing's sarcoma (ES) family of tumors is a relatively rare entity, presenting most commonly in children.
  • Histopathological findings yielded a diagnosis of ES.
  • The tumor was treated with radiation therapy to both sites and, subsequently, after confirmed metastases to other sites, 11 cycles of doxorubicin-based chemotherapy, as well as bone marrow transplantation.
  • However, the patient succumbed to the illness 18 months after her initial diagnosis.
  • CONCLUSION: The cavernous sinus is an unusual site for ES, but given the vascularity and the frequency of this tumor in childhood, the diagnosis should be entertained.
  • [MeSH-major] Bone Neoplasms / pathology. Cavernous Sinus / pathology. Sarcoma, Ewing / pathology. Vascular Neoplasms / pathology

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  • (PMID = 15918955.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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6. Madhar M, Latifi M, Chafik R, Saidi H, Masmejean E, Sabti A, Essadki B, Fikry T: [Ewing's sarcoma of the hand: a case report]. Chir Main; 2005 Jun-Aug;24(3-4):196-8
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  • [Title] [Ewing's sarcoma of the hand: a case report].
  • Ewing's sarcoma of the hand is rare.
  • We report a case of Ewing's sarcoma of the left hand, presenting as a swelling of the hand gradually enlarging over six months.
  • Despite amputation and post-operative chemotherapy, death occurred two months later due marrow aplasia.
  • [MeSH-major] Bone Neoplasms / diagnosis. Hand / surgery. Sarcoma, Ewing / diagnosis

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  • (PMID = 16121630.001).
  • [ISSN] 1297-3203
  • [Journal-full-title] Chirurgie de la main
  • [ISO-abbreviation] Chir Main
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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7. Schovanec J, Mrácek J, Havlas V, Trc T: [Ewing's sarcoma in children--current surgical treatment options, evaluation of our patients]. Acta Chir Orthop Traumatol Cech; 2004;71(4):220-7
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  • [Title] [Ewing's sarcoma in children--current surgical treatment options, evaluation of our patients].
  • [Transliterated title] Ewingův sarkom u detí--moznosti soucasné chirurgické terapie, zhodnocení naseho souboru pacientů.
  • This also applies to bone tumors.
  • Until recently, these neoplasms, most frequently occurring in children and adolescents, were considered to have the worst possible prognosis with a minimal opportunity for a successful outcome of treatment.
  • This is a likely reason for the still deeply rooted belief that amputation of the affected limb is necessary and disease prognosis is uncertain, which also applies to Ewing's sarcoma.
  • Only lately could these patients be offered a hope of successful treatment including limb salvage.
  • MATERIAL: In the period from 1984 to mid-2003, 78 patients with Ewing's sarcoma were treated in our department.
  • In 13 patients, the procedure was restricted to diagnostic biopsy only, because the tumor was inoperable.
  • METHODS: The diagnosis was made on the basis of diagnostic biopsy and, subsequently, neoadjuvant chemotherapy was administered.
  • When this resulted in tumor regression, definitive surgery was performed, involving tumor resection and bone replacement either with autograft or allograft, or with an individual prosthesis.
  • Inoperable tumors were managed by megadose chemotherapy and by radiotherapy.
  • The patients after orthopedic surgery receive adjuvant therapy, including bone marrow transplantation in indicated cases.
  • RESULTS: The evaluation of our 78 patients showed that patient survival is not related to the surgical procedure used.
  • In patients with an early diagnosis and a positive response of the tumor to chemotherapy, the reconstruction procedure appeared to be sufficiently radical in terms of cancer control while preserving limb function; these patients showed neither a significant increase in disease recurrence nor metastatic dissemination.
  • However, amputation and exarticulation cannot completely be avoided and they are necessary in the patients whose tumor failed to respond to chemotherapy or in whom radical removal of the tumor is not possible.
  • The time between the onset of pain and initiation of therapy is also an important factor affecting the treatment outcome.
  • Complications of reconstructive surgery for Ewing's sarcoma recorded in our patient group included osteomyelitis and graft fracture in addition to relapse and metastatic dissemination followed by death.
  • DISCUSSION: Reconstructive surgery for Ewing's sarcoma is carried out in our department in patients with a confirmed diagnosis, in whom neoadjuvant therapy has resulted in tumor regression and in whom the extent and site of a tumor permit this sort of procedure.
  • If amputation is not possible due to tumor localization, megadose chemotherapy is administered.
  • The results of long-term survival evaluation of our patients undergoing resection and replacement show that the procedure has been sufficiently radical, because no local recurrence or metastatic dissemination followed by death were recorded.
  • CONCLUSIONS: This study evaluated the current techniques of treating Ewing's sarcoma, with emphasis on reconstructive surgery leading to limb salvation and maintenance of its full function.
  • [MeSH-major] Bone Neoplasms / surgery. Sarcoma, Ewing / surgery

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  • (PMID = 15456100.001).
  • [ISSN] 0001-5415
  • [Journal-full-title] Acta chirurgiae orthopaedicae et traumatologiae Cechoslovaca
  • [ISO-abbreviation] Acta Chir Orthop Traumatol Cech
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Czech Republic
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8. 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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  • [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).
  • Immunohistochemistry for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET.
  • 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).
  • 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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9. 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.
  • METHODS: Thirty-two patients had metastases to lungs only, 12 patients had metastases to bone marrow or bones only, 64 patients had metastases in multiple sites, and five patients had metastases in other sites; seven patients could not be assessed precisely.
  • 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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10. Grosso F, Dileo P, Sanfilippo R, Stacchiotti S, Bertulli R, Piovesan C, Jimeno J, D'Incalci M, Gescher A, Casali PG: Steroid premedication markedly reduces liver and bone marrow toxicity of trabectedin in advanced sarcoma. Eur J Cancer; 2006 Jul;42(10):1484-90
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  • [Title] Steroid premedication markedly reduces liver and bone marrow toxicity of trabectedin in advanced sarcoma.
  • Trabectedin is a marine-derived cytoxic alkaloid which has shown promising antitumour activity in a variety of human malignancies including sarcoma.
  • Fifty-four patients with advanced sarcoma (age 43 yrs, range 18-70), all pretreated with prior chemotherapy, were enrolled on a named individual basis for treatment with trabectedin.
  • Diagnosis was adult soft tissue sarcoma (STS) in 46 patients, Ewing's family tumour (EFT) in 4, and osteosarcoma (OS) in 4.
  • The initial 23 patients (total number of courses administered: 68) did not receive premedication prior to trabectedin, while the other 31 patients (total number of courses administered: 134) received premedication with dexamethasone 4 mg po bid 24 hours before therapy.
  • In this unselected patient series, premedication with dexamethasone strongly reduced drug-induced hepatotoxicity and myelosuppression.
  • [MeSH-major] Antineoplastic Agents, Alkylating / adverse effects. Bone Marrow Diseases / chemically induced. Dioxoles / adverse effects. Drug-Induced Liver Injury. Premedication. Sarcoma / drug therapy. Steroids / therapeutic use. Tetrahydroisoquinolines / adverse effects

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  • (PMID = 16737808.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Dioxoles; 0 / Steroids; 0 / Tetrahydroisoquinolines; 114899-77-3 / trabectedin
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11. Fraser CJ, Weigel BJ, Perentesis JP, Dusenbery KE, DeFor TE, Baker KS, Verneris MR: Autologous stem cell transplantation for high-risk Ewing's sarcoma and other pediatric solid tumors. Bone Marrow Transplant; 2006 Jan;37(2):175-81
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  • [Title] Autologous stem cell transplantation for high-risk Ewing's sarcoma and other pediatric solid tumors.
  • The prognosis for many pediatric and young adult patients with solid tumors that have metastasized at the time of diagnosis or have relapsed after therapy remains very poor.
  • The steep dose-response curve of many of these tumors to alkylating agents makes myeloablative chemotherapy followed by autologous stem cell transplantation (ASCT) an attractive potential therapy.
  • Patients with a diagnosis of Ewing's sarcoma (ES) or desmoplastic small round cell tumor (DSRCT) had significantly better survival than those with other diagnoses with estimated 3-year OS of 54% (95% CI: 29-79%) for this group of patients (P = 0.03).
  • These data justify continued investigation of ASCT as a consolidation therapy in patients with metastatic or relapsed ES and DSRCT.
  • [MeSH-major] Bone Neoplasms / mortality. Bone Neoplasms / therapy. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / therapy. Sarcoma, Ewing / mortality. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease-Free Survival. Female. Fibroma, Desmoplastic / complications. Fibroma, Desmoplastic / mortality. Fibroma, Desmoplastic / pathology. Fibroma, Desmoplastic / therapy. Follow-Up Studies. Hepatic Veno-Occlusive Disease / etiology. Hepatic Veno-Occlusive Disease / mortality. Humans. Male. Risk Factors. Stem Cell Transplantation / methods. Stem Cell Transplantation / mortality. Survival Rate. Transplantation, Autologous

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  • (PMID = 16273111.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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12. Koscielniak E, Morgan M, Treuner J: Soft tissue sarcoma in children: prognosis and management. Paediatr Drugs; 2002;4(1):21-8
ClinicalTrials.gov. clinical trials - ClinicalTrials.gov .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Soft tissue sarcoma in children: prognosis and management.
  • Soft tissue sarcomas (STS) account for approximately 7% of malignant neoplasms in children.
  • The heterogeneity of STS makes the diagnosis and therapy particularly difficult and should be reserved for specialized centers with expertise in treating cancer in children.
  • Major progress in the accuracy of diagnosis and classification has been made by the identification of specific, recurring genetic alterations t(2;13)(q35;q14) and t(1;13)(p36;q14) in alveolar rhabdomyosarcomas (RMS), t(X;18)(p11;q11) for synovial sarcoma (SS) and t(11;22)(q24;q12) or t(21;22)(q22;q12) for Ewing's tumor family.
  • As a result of large multicenter STS studies, such as the North-American Intergroup Rhabdomyosarcoma Study, the German Pediatric Soft Tissue Sarcoma Study Group (CWS), Italian Gruppo Cooperativo Italiano study and Sociètè Internationale d'Oncologie Pèdiatrique (SIOP) Malignant Mesenchymal Tumors study, the identification of more effective treatment strategies and improvement in prognosis have been made in the last 30 years.
  • Prognostic variables were identified and, by exploring novel therapeutic strategies, criteria were established for the use of preoperative chemotherapy and radiotherapy, and primary and delayed second-look surgery.
  • As a result of evaluation of different drugs active in STS, refinements in the utilization of chemotherapy have been made possible.
  • Clinical trials have also been instrumental in defining the late effects of treatment.
  • In STS the following drugs have proven to be useful: dactinomycin, vincristine, alkylating agents such as cyclophosphamide and ifosfamide, as well as anthracyclines such as doxorubicin (adriamycin) and epi-doxorubicin.
  • In general, with conventional fractionation (1 x 1.8 to 2 Gy/day) radiotherapy doses between 40 and 50 Gy should be administered.
  • The German CWS group explored the effectiveness of 32 Gy when accelerated and hyperfractionated, and given simultaneously to chemotherapy, and found it adequate for local tumor control in patients with selected favorable prognostic factors.
  • When treated with a combination of chemotherapy and local therapy, STS showed an event-free survival between 50 and 80% [RMS 70%, extraskeletal Ewing sarcoma (EES) and peripheral neuroectodermal tumor (PNET) circa 50%, and SS 70 to 80%].
  • Age (>10 years), bone, and/or bone marrow metastases are associated with a very poor prognosis (survival rate of about 5%).
  • The value of high dose chemotherapy with hematopoietic stem cell rescue in patients with poor prognostic STS remains unclear and should be performed in controlled studies only.
  • [MeSH-major] Sarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols. Child. Combined Modality Therapy. Humans. Neoplasm Staging. Prognosis. Radiotherapy. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / surgery. Rhabdomyosarcoma / therapy

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  • (PMID = 11817983.001).
  • [ISSN] 1174-5878
  • [Journal-full-title] Paediatric drugs
  • [ISO-abbreviation] Paediatr Drugs
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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13. Burdach S, van Kaick B, Laws HJ, Ahrens S, Haase R, Körholz D, Pape H, Dunst J, Kahn T, Willers R, Engel B, Dirksen U, Kramm C, Nürnberger W, Heyll A, Ladenstein R, Gadner H, Jürgens H, Go el U: Allogeneic and autologous stem-cell transplantation in advanced Ewing tumors. An update after long-term follow-up from two centers of the European Intergroup study EICESS. Stem-Cell Transplant Programs at Düsseldorf University Medical Center, Germany and St. Anna Kinderspital, Vienna, Austria. Ann Oncol; 2000 Nov;11(11):1451-62
ClinicalTrials.gov. clinical trials - ClinicalTrials.gov .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • In order to evaluate a possible therapeutic benefit after allogeneic SCT in patients with advanced Ewing tumors (AET), we compared outcome after autologous and allogeneic stem-cell transplantation (SCT).
  • All patients underwent remission induction chemotherapy and local treatment before myeloablative therapy.
  • Seventeen of thirty-six patients had multifocal primary Ewing's tumor, eighteen of thirty-six had early, multiple or multifocal relapse, one of thirty-six patients had unifocal late relapse.
  • We analyzed the following risk factors, that could possibly influence the event-free survival (EFS): number of involved bones, degree of remission at time of SCT, type of graft, indication for SCT, bone marrow infiltration, bone with concomitant lung disease, age at time of diagnosis, pelvic involvement, involved compartment radiation, histopathological diagnosis.
  • Eighteen of thirty-six patients suffered relapse or died of disease, nine of thirty-six died of treatment related toxicity (DOC).
  • Age > or = 17 years at initial diagnosis (P < 0.005) significantly deteriorated outcome.
  • According to the type of graft, EFS was 0.25 +/- 0.08 after autologous and 0.20 +/- 0.13 after allogeneic SCT.

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  • (PMID = 11142486.001).
  • [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] Comparative Study; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunologic Factors; 0 / Interleukin-2
  • [Number-of-references] 56
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