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1. Bisogno G, Carli M, Stevens M, Oberlin O, Treuner J, Scarzello G, Colombatti R, De Zen L, Pinkerton CR: Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue. Bone Marrow Transplant; 2002 Sep;30(5):297-302
Hazardous Substances Data Bank. MELPHALAN .

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  • [Title] Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue.
  • The MMT4 study was designed to explore an intensive chemotherapy regimen (MMT4-89) and the role of high-dose melphalan (MMT4-91) in children with metastatic soft tissue sarcoma, including extraosseous peripheral neuroectodermal tumor (PNET).
  • Thirty-one patients with PNET were treated between 1989 and 1995 (11 according to MMT4-89 and 20 according to MMT4-91).
  • Chemotherapy consisted of four CEVAIE cycles, each including three 3-week courses: CEV (carboplatin 500 mg/m(2), epirubicin 150 mg/m(2), vincristine 1.5 mg/m(2)), IVA ifosfamide 9 g/m(2), actinomycin 1.5 mg/m(2), vincristine 1.5 mg/m(2)), IVE (ifosfamide 9 g/m(2), etoposide 600 mg/m(2), vincristine 1.5 mg/m(2)).
  • Local control was achieved in 77% of irradiated patients vs 45% of non-irradiated.
  • In conclusion, despite the high CR rate, intensive chemotherapy with or without high-dose melphalan appeared to have little impact on the survival of patients with metastatic extraosseus PNET.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Melphalan / administration & dosage. Neuroectodermal Tumors / therapy. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Age Factors. Bone Marrow Transplantation. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Male. Peripheral Blood Stem Cell Transplantation. Remission Induction / methods. Survival Analysis. Treatment Outcome

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  • (PMID = 12209351.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] Q41OR9510P / Melphalan
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2. Furuno Y, Nishimura S, Kamiyama H, Numagami Y, Saito A, Kaimori M, Nishijima M: Intracranial peripheral-type primitive neuroectodermal tumor. Neurol Med Chir (Tokyo); 2008 Feb;48(2):72-6
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  • [Title] Intracranial peripheral-type primitive neuroectodermal tumor.
  • Magnetic resonance (MR) imaging revealed a large extraaxial tumor with cyst at the right frontotemporal region.
  • The solid part of the tumor was homogeneously enhanced on T(1)-weighted MR imaging after injection of gadolinium.
  • Digital subtraction angiography of the external carotid artery revealed sunburst appearance corresponding to the tumor, which was fed by the right middle meningeal artery.
  • His headache worsened and computed tomography revealed enlargement of the tumor and intracystic hemorrhage, so emergent operation was performed.
  • At surgery, the tumor strongly adhered to the dural membrane, and was obviously extraaxial.
  • The tumor and cyst were gross totally removed.
  • The histological diagnosis was peripheral-type primitive neuroectodermal tumor (pPNET).
  • Following surgery, radiation therapy and chemotherapy were given.
  • Ewing's sarcoma and pPNET form a family of small round cell tumors arising in the bone or soft tissue.
  • MIC-2 is a useful marker in the differential diagnosis.
  • [MeSH-major] Brain Neoplasms / pathology. Meningioma / pathology. Neuroectodermal Tumors, Primitive, Peripheral / pathology
  • [MeSH-minor] Adolescent. Antigens, CD / metabolism. Biomarkers, Tumor / metabolism. Cell Adhesion Molecules / metabolism. Diagnosis, Differential. Frontal Lobe / pathology. Headache / etiology. Headache / pathology. Humans. Ki-67 Antigen / metabolism. Magnetic Resonance Imaging. Male. Temporal Lobe / pathology. Tomography, X-Ray Computed

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  • (PMID = 18296876.001).
  • [ISSN] 0470-8105
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Ki-67 Antigen
  • [Number-of-references] 20
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3. Lee YY, Kim DH, Lee JH, Choi JS, In KH, Oh YW, Cho KH, Roh YK: Primary pulmonary Ewing's sarcoma/primitive neuroectodermal tumor in a 67-year-old man. J Korean Med Sci; 2007 Sep;22 Suppl:S159-63
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  • [Title] Primary pulmonary Ewing's sarcoma/primitive neuroectodermal tumor in a 67-year-old man.
  • Extraskeletal Ewing's sarcoma (EES) is a branch of neuroectodermal tumor (PNET), which is very rare soft tissue sarcoma.
  • We report a case of EES/PNET arising is the lung of a 67-yr-old man.
  • Computed tomography, bone scintigraphy, and positron emission tomography confirmed the mass to have a primary pulmonary origin.
  • The diagnosis was confirmed both pathologically and genetically.
  • The mass lesion was resected, and the patient is currently undergoing chemotherapy.
  • [MeSH-major] Lung Neoplasms / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Sarcoma, Ewing / diagnosis
  • [MeSH-minor] Aged. Calmodulin-Binding Proteins / genetics. Chromosome Breakage. Chromosomes, Human, Pair 22 / genetics. Diagnosis, Differential. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. RNA-Binding Proteins / genetics

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  • (PMID = 17923745.001).
  • [ISSN] 1011-8934
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Calmodulin-Binding Proteins; 0 / EWSR1 protein, human; 0 / RNA-Binding Proteins
  • [Other-IDs] NLM/ PMC2694395
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4. Bacci G, Ferrari S, Bertoni F, Donati D, Bacchini P, Longhi A, Brach Del Prever A, Forni C, Rimondini S: Neoadjuvant chemotherapy for peripheral malignant neuroectodermal tumor of bone: recent experience at the istituto rizzoli. J Clin Oncol; 2000 Feb;18(4):885-92
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  • [Title] Neoadjuvant chemotherapy for peripheral malignant neuroectodermal tumor of bone: recent experience at the istituto rizzoli.
  • PURPOSE: The results achieved in 44 patients with nonmetastatic peripheral neuroectodermal tumor (PNET) of bone treated with neoadjuvant chemotherapy are reported.
  • PATIENTS AND METHODS: A six-drug regimen of chemotherapy (vincristine, doxorubicin, dactinomycin, cyclophosphamide, ifosfamide, and etoposide) was administered to all patients.
  • Local treatment consisted of surgery in 20 patients, surgery followed by radiotherapy in 13, and radiotherapy only in 11.
  • RESULTS: At a mean follow-up of 4.5 years (range, 2 to 7 years), 23 patients (52%) remain event-free, 20 have relapsed (45%), and one has died of chemotherapy-related toxicity.
  • Of these, 103 (75%) remained continuously event-free, 34 (24%) relapsed, and one died of chemotherapy-related toxicity.
  • It follows that PNET patients treated with this chemotherapy regimen have a significantly worse prognosis than typical ES patients (5-year event-free survival, 54.2% v 70.6%, P <.012; 5-year overall survival, 62.7% v 78.3%, P <.002).
  • CONCLUSION: The authors conclude that studies into new adjuvant therapy for Ewing's sarcoma modulated according to risk of relapse should also consider neural differentiation as a risk factor.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / surgery. Neoadjuvant Therapy. Neuroectodermal Tumors, Primitive, Peripheral / surgery
  • [MeSH-minor] Adolescent. Adult. Antibiotics, Antineoplastic / administration & dosage. Antibiotics, Antineoplastic / adverse effects. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Alkylating / adverse effects. Antineoplastic Agents, Phytogenic / administration & dosage. Antineoplastic Agents, Phytogenic / adverse effects. Chi-Square Distribution. Cyclophosphamide / administration & dosage. Cyclophosphamide / adverse effects. Dactinomycin / administration & dosage. Dactinomycin / adverse effects. Disease-Free Survival. Doxorubicin / administration & dosage. Doxorubicin / adverse effects. Female. Follow-Up Studies. Humans. Male. Neoplasm Recurrence, Local / pathology. Prognosis. Radiotherapy, Adjuvant. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / radiotherapy. Sarcoma, Ewing / surgery. Survival Rate. Treatment Outcome. Vincristine / administration & dosage. Vincristine / adverse effects

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  • [CommentIn] J Clin Oncol. 2000 May;18(10):2187-8 [10811686.001]
  • (PMID = 10673532.001).
  • [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] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Phytogenic; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide
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5. Konoplia NE, Strongin IuS, Talabaev MV, Aleĭnikova OV: [Effectiveness of intensive chemotherapy in the treatment of medulloblastoma/primitive neuroectodermal tumor in children]. Vopr Onkol; 2008;54(2):157-63
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  • [Title] [Effectiveness of intensive chemotherapy in the treatment of medulloblastoma/primitive neuroectodermal tumor in children].
  • Standard and high-risk groups of 77 children with neuroectodermal medulloblastoma were given sandwich chemotherapy.
  • The former group was treated with high-dose chemotherapy complemented with autotransplantation of bone marrow and peripheral stem cells.
  • The treatment proved effective: 7-year recurrence-free survival (0.66 +/- 0.05) (overall survival--0.67 +/- 0.05; recurrence-free--0.62 +/- 0.06).
  • Sandwich chemotherapy administered in standard risk group was followed by 7-year recurrence-free survival (0.84 +/- 0.08).
  • High-dose chemotherapy complemented with autotransplantation of bone marrow and peripheral stem cells in conjunction with high-dose chemotherapy resulted in 6-year recurrence-free survival: 0.77 +/- 0.08 in patients after high-dose chemotherapy and 0.46 +/- 0.10--without it.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy
  • [MeSH-minor] Adolescent. Bone Marrow Transplantation. Cerebellar Neoplasms / drug therapy. Chemotherapy, Adjuvant. Child. Child, Preschool. Disease-Free Survival. Drug Administration Schedule. Female. Humans. Male. Peripheral Blood Stem Cell Transplantation. Radiotherapy, Adjuvant. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 18522163.001).
  • [ISSN] 0507-3758
  • [Journal-full-title] Voprosy onkologii
  • [ISO-abbreviation] Vopr Onkol
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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6. Laffosse JM, Accadbled F, Abid A, Kany J, Darodes P, Sales De Gauzy J: [Reconstruction of long bone defects with a vascularized fibular graft after tumor resection in children and adolescents: thirteen cases with 50-month follow-up]. Rev Chir Orthop Reparatrice Appar Mot; 2007 Oct;93(6):555-63
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  • [Title] [Reconstruction of long bone defects with a vascularized fibular graft after tumor resection in children and adolescents: thirteen cases with 50-month follow-up].
  • [Transliterated title] Reconstruction osseuse des os longs après exérèse carcinologique par l'utilisation de greffons fibulaires vascularisés chez l'enfant et l'adolescent.
  • PURPOSE OF THE STUDY: The vascularized fibular graft is a widely used technique for the reconstruction of long bone defects after tumor resection.
  • We report our experience with long bone reconstructions in children and adolescents after resection of primary malignant bone tumors.
  • Preoperatively, the pathological diagnosis was Ewing tumor (n=7), osteogenic sarcoma (n=5), neuroepithelioma (n=1).
  • All patients except one were given chemotherapy preoperatively and postoperatively and four received adjuvant radiotherapy.
  • Tumor resection created a gap (n=8) or involved resection-arthrodesis (n=5, three knees, one ankle, one elbow).
  • Eleven of the twelve patients who underwent tumor resection involving the lower limb were able to walk with full weight bearing at 13.9 months (range 841 months), half of them without any supportive device.
  • Among the fibular grafts which healed, primary healing of the distal end was noted in all cases, but not for the proximal end.
  • Healing was always achieved for the distal focus but not for the proximal focus which receives its blood supply from a branch of the anterior tibial artery which is not harvested.
  • The defective blood supply can thus hinder bone healing.
  • CONCLUSION: Long bone reconstruction using an autologous vascularized fibular graft is a reliable technique providing satisfactory functional results.
  • [MeSH-major] Bone Neoplasms / surgery. Bone Transplantation / methods. Reconstructive Surgical Procedures / methods. Surgical Flaps
  • [MeSH-minor] Adolescent. Arthrodesis / methods. Cause of Death. Chemotherapy, Adjuvant. Child. Child, Preschool. Follow-Up Studies. Fractures, Bone / etiology. Fractures, Spontaneous / etiology. Humans. Knee Joint / surgery. Lung Neoplasms / secondary. Neuroectodermal Tumors, Primitive, Peripheral / surgery. Osteosarcoma / surgery. Postoperative Complications. Radiotherapy, Adjuvant. Retrospective Studies. Sarcoma, Ewing / surgery. Treatment Outcome. Walking / physiology

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  • (PMID = 18065864.001).
  • [ISSN] 0035-1040
  • [Journal-full-title] Revue de chirurgie orthopédique et réparatrice de l'appareil moteur
  • [ISO-abbreviation] Rev Chir Orthop Reparatrice Appar Mot
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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7. Suarez CR, Bertolone SJ, Raj AB, Coventry S: Second malignant neoplasms in childhood acute lymphoblastic leukemia: primitive neuroectodermal tumor of the chest wall with germline p53 mutation as a second malignant neoplasm. Am J Hematol; 2004 May;76(1):52-6
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  • [Title] Second malignant neoplasms in childhood acute lymphoblastic leukemia: primitive neuroectodermal tumor of the chest wall with germline p53 mutation as a second malignant neoplasm.
  • Second malignant neoplasm (SMNs) are a devastating sequelae observed on these children, with an estimated cumulative risk of 2-3.3% fifteen years after diagnosis.
  • Primitive neuroectodermal tumor of bone (PNET) is rarely observed as a SMN following treatment of childhood ALL.
  • The authors described the occurrence of a chest wall PNET of the bone at the site of a central line placement associated with both germ-line and tumor cell p53 mutation in a 8-year-old boy 1 year after completing therapy for standard risk ALL.
  • A review of the literature of 25,051 children treated for ALL discovered 230 SMNs (0.99%), and only one case of PNET of the bone was noted among this group.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neoplasms, Second Primary / genetics. Neuroectodermal Tumors, Primitive, Peripheral / genetics. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Thoracic Wall / pathology. Tumor Suppressor Protein p53 / genetics
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Humans. Male. Mutation. Treatment Outcome

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15114597.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Tumor Suppressor Protein p53
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8. Yoshida Y, Osaka S, Tokuhashi Y: Analysis of limb function after various reconstruction methods according to tumor location following resection of pediatric malignant bone tumors. World J Surg Oncol; 2010;8:39
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  • [Title] Analysis of limb function after various reconstruction methods according to tumor location following resection of pediatric malignant bone tumors.
  • BACKGROUND: In the reconstruction of the affected limb in pediatric malignant bone tumors, since the loss of joint function affects limb-length discrepancy expected in the future, reconstruction methods that not only maximally preserve the joint function but also maintain good limb function are necessary.
  • We analysis limb function of reconstruction methods by tumor location following resection of pediatric malignant bone tumors.
  • PATIENTS AND METHODS: We classified the tumors according to their location into 3 types by preoperative MRI, and evaluated reconstruction methods after wide resection, paying attention to whether the joint function could be preserved.
  • The mean age of the patients was 10.6 years, Osteosarcoma was observed in 26 patients, Ewing's sarcoma in 3, and PNET(primitive neuroectodermal tumor) and chondrosarcoma (grade 1) in 1 each.
  • RESULTS: Type I were those located in the diaphysis, and reconstruction was performed using a vascularized fibular graft(vascularized fibular graft).
  • Type 2 were those located in contact with the epiphyseal line or within 1 cm from this line, and VFG was performed in 1, and distraction osteogenesis in 1.
  • Type III were those extending from the diaphysis to the epiphysis beyond the epiphyseal line, and a Growing Kotz was mainly used in 10 patients.
  • The mean functional assessment score was the highest for Type I (96%: n = 4) according to the type and for VFG (99%) according to the reconstruction method.
  • CONCLUSION: The final functional results were the most satisfactory for Types I and II according to tumor location.
  • Therefore, considering the function of the affected limb, a limb reconstruction method allowing the maximal preservation of joint function should be selected after careful evaluation of the effects of chemotherapy and the location of the tumor.
  • [MeSH-major] Bone Neoplasms / surgery. Extremities / physiology. Reconstructive Surgical Procedures / methods
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Chondrosarcoma / pathology. Chondrosarcoma / surgery. Female. Follow-Up Studies. Humans. Male. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Neuroectodermal Tumors, Primitive, Peripheral / surgery. Osteosarcoma / pathology. Osteosarcoma / surgery. Sarcoma, Ewing / pathology. Sarcoma, Ewing / surgery. Surgical Flaps. Survival Rate. Treatment Outcome

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  • (PMID = 20482815.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2881919
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9. Wolff JE, Finlay JL: High-dose chemotherapy in childhood brain tumors. Onkologie; 2004 Jun;27(3):239-45
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  • [Title] High-dose chemotherapy in childhood brain tumors.
  • Early attempts to use high-dose chemotherapy technology in order to improve the effect of nitrosourea on high-grade gliomas resulted in minimal benefit as well as in severe toxicity.
  • Since then, other drugs have been applied in conjunction with either autologous bone marrow or peripheral blood stem cells, including thiotepa, etoposide, melphalan, cyclophosphamide, and busulfan.
  • The data suggest benefit in recurrent primitive neuroectodermal tumors (PNET), in newly diagnosed young children with PNET and possibly in young children with newly diagnosed ependymoma, as a strategy not only to improve tumor-free survival but also to avoid exposure of the young brain to irradiation.
  • In other tumors such as recurrent ependymoma and newly diagnosed or recurrent brain stem glioma, high-dose chemotherapy remains ineffective.
  • New protocols under evaluation include new agents, multiple cycles of high-dose chemotherapy and allogeneic transplantation as immunotherapeutic approach.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Brain Neoplasms / drug therapy. Drug Therapy / methods
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy / methods. Ependymoma / drug therapy. Ependymoma / surgery. Glioma / drug therapy. Glioma / surgery. Humans. Infant. Infant, Newborn. Medulloblastoma / drug therapy. Medulloblastoma / surgery. Neuroectodermal Tumors / drug therapy. Neuroectodermal Tumors / surgery. Practice Patterns, Physicians'

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  • [Copyright] Copyright 2004 S. Karger GmbH, Freiburg
  • (PMID = 15249712.001).
  • [ISSN] 0378-584X
  • [Journal-full-title] Onkologie
  • [ISO-abbreviation] Onkologie
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 65
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10. Fangusaro JR, Jubran RF, Allen J, Gardner S, Dunkel IJ, Rosenblum M, Atlas MP, Gonzalez-Gomez I, Miller D, Finlay JL: Brainstem primitive neuroectodermal tumors (bstPNET): results of treatment with intensive induction chemotherapy followed by consolidative chemotherapy with autologous hematopoietic cell rescue. Pediatr Blood Cancer; 2008 Mar;50(3):715-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Brainstem primitive neuroectodermal tumors (bstPNET): results of treatment with intensive induction chemotherapy followed by consolidative chemotherapy with autologous hematopoietic cell rescue.
  • We have evaluated the response rate and survival utilizing intensified chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) and adjuvant radiation therapy in six young children with newly diagnosed brainstem primitive neuroectodermal tumors (bstPNET).
  • Following maximum surgical resection of the tumor, patients received high dose induction chemotherapy including vincristine, cisplatin, cyclophosphamide, and etoposide.
  • Eligible patients received a single cycle of myeloablative chemotherapy followed by AuHCR.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow Transplantation. Brain Stem. Infratentorial Neoplasms / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Peripheral Blood Stem Cell Transplantation
  • [MeSH-minor] Brain Damage, Chronic / etiology. Carboplatin / administration & dosage. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cranial Irradiation. Cyclophosphamide / administration & dosage. Etoposide / administration & dosage. Humans. Infant. Leucovorin / administration & dosage. Mesna / administration & dosage. Methotrexate / administration & dosage. Remission Induction. Thiotepa / administration & dosage. Transplantation, Autologous. Vincristine / administration & dosage

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17009232.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; 905Z5W3GKH / Thiotepa; BG3F62OND5 / Carboplatin; NR7O1405Q9 / Mesna; Q20Q21Q62J / Cisplatin; Q573I9DVLP / Leucovorin; YL5FZ2Y5U1 / Methotrexate
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11. Moschovi M, Trimis G, Stefanaki K, Anastasopoulos J, Syriopoulou V, Koultouki E, Tzortzatou-Stathopoulou F: Favorable outcome of Ewing sarcoma family tumors to multiagent intensive preoperative chemotherapy: a single institution experience. J Surg Oncol; 2005 Mar 15;89(4):239-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Favorable outcome of Ewing sarcoma family tumors to multiagent intensive preoperative chemotherapy: a single institution experience.
  • BACKGROUND: Aim of our study was to evaluate the efficacy of multiagent intensive preoperative chemotherapy in patients with Ewing sarcoma family tumors (ESFT), in order to succeed a better percentage of necrosis before surgical resection.
  • PROCEDURE: Eighteen patients with ESFT were treated with the same multiagent intensive preoperative protocol.
  • 5/18 patients had bone Ewings sarcoma (EWS) and 13/18 had peripheral primitive neuroectodermal tumor (PNET).
  • None had metastases at diagnosis.
  • Chemotherapy consisted of 5 or 6 cycles with vincristine, cisplatin, cyclophosphamide, and Adriamycin, followed by 12 cycles of vincristine, cyclophosphamide, and actinomycin-D.
  • Five patients with EWS underwent total resection after 5-6 cycles of preoperative chemotherapy and prosthetic replacement was performed in two of them.
  • In 3/13 patients with PNET the tumor was resected at diagnosis and in 1/13 after 5 cycles of chemotherapy, while 9/13 patients received chemotherapy only and/or radiotherapy.
  • RESULTS: In patients with EWS, the histologic specimens of the resected tumors showed that tissue necrosis was 100% in four patients and 95% in one patient.
  • No patient had topical recurrence or developed metastatic disease during follow-up period (2-13 years, mean time 7.4 years).
  • There were no major side effects of chemotherapy.
  • CONCLUSIONS: The intensive chemotherapy schedule, comprising of 5-6 cycles preoperatively, seems to maximize the percentage of tumor necrosis, thus improving outcome.
  • Our study implies that this combined therapy may improve the prognosis of ESFT.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Bone Neoplasms / genetics. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / genetics
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Doxorubicin / administration & dosage. Drug Administration Schedule. Female. Humans. Infant. Male. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / genetics. Neuroectodermal Tumors, Primitive, Peripheral / mortality. Neuroectodermal Tumors, Primitive, Peripheral / surgery. Preoperative Care. Radiotherapy Dosage. Survival Rate. Treatment Outcome. Vincristine / administration & dosage

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  • [Copyright] (c) 2005 Wiley-Liss, Inc.
  • (PMID = 15726621.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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12. Strother D, Ashley D, Kellie SJ, Patel A, Jones-Wallace D, Thompson S, Heideman R, Benaim E, Krance R, Bowman L, Gajjar A: Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study. J Clin Oncol; 2001 May 15;19(10):2696-704
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study.
  • PURPOSE: This study was designed to determine the feasibility and safety of delivering four consecutive cycles of high-dose cyclophosphamide, cisplatin, and vincristine, each followed by stem-cell rescue, every 4 weeks, after completion of risk-adapted craniospinal irradiation to children with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor (PNET).
  • After surgical resection, high-risk patients were treated with topotecan in a 6-week phase II window followed by craniospinal radiation therapy and four cycles of high-dose cyclophosphamide (4,000 mg/m2 per cycle), with cisplatin (75 mg/m2 per cycle), and vincristine (two 1.5-mg/m2 doses per cycle).
  • Support with peripheral blood stem cells or bone marrow and with granulocyte colony-stimulating factor was administered after each cycle of high-dose chemotherapy.
  • Treatment of average-risk patients consisted of surgical resection and craniospinal irradiation, followed by the same chemotherapy given to patients with high-risk disease.
  • The expected duration of the chemotherapy was 16 weeks, with a cumulative cyclophosphamide dose of 16,000 mg/m2 and a planned dose-intensity of 1,000 mg/m2/wk.
  • RESULTS: Fifty of the 53 patients commenced high-dose chemotherapy, and 49 patients completed all four cycles.
  • The median length of chemotherapy cycles one through four was 28, 27, 29, and 28 days, respectively.
  • Engraftment occurred at a median of 14 to 15 days after infusion of stem cells or autologous bone marrow.
  • No deaths were attributable to the toxic effects of high-dose chemotherapy.
  • For the high-risk patients, the 2-year progression-free survival is 73.7% +/- 10.5% from the start of therapy and 84.2% +/- 8.6% from the start of radiation therapy.
  • CONCLUSION: Administering four consecutive cycles of high-dose chemotherapy with stem-cell support after surgical resection and craniospinal irradiation is feasible in newly diagnosed patients with medulloblastoma/supratentorial PNET with aggressive supportive care.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy
  • [MeSH-minor] Adolescent. Adult. Blood Transfusion. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Feasibility Studies. Female. Humans. Male. Stem Cells / drug effects. Topotecan / administration & dosage. Vincristine / administration & dosage

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  • (PMID = 11352962.001).
  • [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 / R25 CA023944; United States / NCI NIH HHS / CA / CA 21765; United States / NCI NIH HHS / CA / P01 CA 23009
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7M7YKX2N15 / Topotecan; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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13. Cebrián JL, Ibarzabal A, Garcia-Crespo R, Marco F, Ortega L, López-Durán L: Peripheral primitive neuroectodermal tumor after radiotherapy. Clin Orthop Relat Res; 2003 Aug;(413):255-60

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Peripheral primitive neuroectodermal tumor after radiotherapy.
  • A 41-year-old man had a peripheral neuroectodermal tumor develop at the distal third of the fibula 4 years after radiotherapy for relapsed villonodular synovitis.
  • This type of sarcoma usually is classified into the heterogeneic group of small round-cell bone tumors as a subdivision of Ewing's sarcomas.
  • The immuno-staining positivity of the neoplastic cells for the neuron-specific enolase allowed the authors to make the diagnosis of a tumor with neuroectodermal origin.
  • When the histologic study confirmed the diagnosis, the patient was treated with chemotherapy, surgical excision of the tumor, and adjuvant radiotherapy.
  • Radiotherapy is thought to be involved in the genesis of osteogenic sarcomas as it has been shown in several reports, but there is no evidence in the literature of a peripheral neuroectodermal tumor developing after radiotherapy.
  • [MeSH-major] Neoplasms, Second Primary / etiology. Neuroectodermal Tumors, Primitive, Peripheral / etiology. Synovitis, Pigmented Villonodular / radiotherapy

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  • (PMID = 12897617.001).
  • [ISSN] 0009-921X
  • [Journal-full-title] Clinical orthopaedics and related research
  • [ISO-abbreviation] Clin. Orthop. Relat. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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14. Asano K, Kikuchi J, Munakata A, Ohkuma H, Kubo O: An infant case of intracranial peripheral-type primitive neuroectodermal tumor with long-term survival. Brain Tumor Pathol; 2007;24(2):69-74
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  • [Title] An infant case of intracranial peripheral-type primitive neuroectodermal tumor with long-term survival.
  • Supratentorial primitive neuroectodermal tumors (S-PNET) that develop in children have recently been classified into two types: central-type PNET (C-PNET), which has been reported over the years, and peripheral-type PNET (P-PNET), which develops intracranially and was referred to as Ewing's sarcoma in the past.
  • P-PNET is fundamentally a malignant tumor, but the patient reported here represents a case of long-term survival from onset without recurrence.
  • At the age of 21 months, a male infant developed a cranial bone deformity and symptoms of high intracranial pressure.
  • A CT scan revealed a cystic tumor attaching to the falx, and cyst drainage operation was immediately performed.
  • The intracranial tumor was then resected.
  • The tumor was an intradural extramedullary tumor, and it was totally excised with the falx attachment.
  • The tumor was initially diagnosed as a neuroblastoma, and postoperative treatment consisted of administration of radiotherapy and chemotherapy using cyclophosphamide and vincristine.
  • Recent repeated performance of histopathological analysis resulted in a diagnosis of P-PNET.
  • In recent years, studies in molecular biology have demonstrated that P-PNET involves the EWS-FLI1 chimeric gene, and immunohistochemical staining has shown P-PNET to be MIC2 positive.
  • P-PNET also differs from C-PNET with regard to prognosis, and for this reason it is believed that P-PNET and C-PNET should be considered separate entities.
  • That is, in spite of the fact that P-PNET is a malignant tumor, patient survival can be comparatively long.
  • Because P-PNET originates intracranially, it is fundamentally an intradural extramedullary tumor.
  • For this reason, treatment should consist of surgical excision that is as complete as possible, followed by appropriate radiotherapy and chemotherapy.
  • [MeSH-major] Diagnostic Errors. Neuroblastoma / pathology. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Male. Neurosurgical Procedures. Radiotherapy. Tomography, X-Ray Computed

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  • (PMID = 18095134.001).
  • [ISSN] 1433-7398
  • [Journal-full-title] Brain tumor pathology
  • [ISO-abbreviation] Brain Tumor Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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15. Attabib NA, West M, Rhodes RH: Peripheral primitive neuroectodermal tumor of the cavernous sinus: case report. Neurosurgery; 2006 May;58(5):E992; discussion E992
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Peripheral primitive neuroectodermal tumor of the cavernous sinus: case report.
  • OBJECTIVE: Ewing sarcoma/peripheral primitive neuroectodermal tumors (pPNET family) are small, round, blue cell tumors that have a decided predilection for young patients and commonly arise in bone and soft tissue.
  • INTERVENTION: The patient underwent debulking of the tumor, and the diagnosis of a pPNET was made based on histological, immunohistochemical, and molecular genetics (EWS-FLI1 fusion gene) findings.
  • Bone scans, bone marrow aspiration, and biopsy and chest computed tomographic scans showed no evidence of systemic involvement.
  • The patient had adjuvant treatment with radiotherapy and chemotherapy.
  • After 14 months, the patient had no neurological deficits, and neuroimaging showed stable disease, although some chemotherapy complications occurred.
  • CONCLUSION: This is a case of cavernous sinus pPNET in a 48-year-old woman, in whom the diagnosis is supported by the presence of EWS-FLI1 fusion gene.
  • [MeSH-major] Cavernous Sinus / pathology. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / genetics

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  • (PMID = 16639307.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS
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16. Kim KJ, Jang BW, Lee SK, Kim BK, Nam SL: A case of peripheral primitive neuroectodermal tumor of the ovary. Int J Gynecol Cancer; 2004 Mar-Apr;14(2):370-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of peripheral primitive neuroectodermal tumor of the ovary.
  • Peripheral primitive neuroectodermal tumor (PNET) belongs to the PNET/Ewing's sarcoma family.
  • PNET is a small round cell tumor of putative neuroectoderm origin and is the second most common sarcoma among children and young adults.
  • It may occur anywhere in the body and within any age group; however, it is most likely to occur in the bone and soft tissues.
  • There have been a small number of case reports of PNET arising in the ovary.
  • We presented a case of PNET arising in the right ovary of an 18-year-old woman.
  • The tumor was metastased to the lymph nodes of the pelvis and para-aorta at surgical staging.
  • We had persecuted Taxol/carboplatin chemotherapy, pelvic cavity radiotherapy, and Vincristine/Actinomycin, Cyclophosphamide/Doxorubicin (VACA).
  • [MeSH-major] Neuroectodermal Tumors / diagnosis. Ovarian Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Combined Modality Therapy. Diagnosis, Differential. Fatal Outcome. Female. Humans. Tomography, X-Ray Computed

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  • (PMID = 15086740.001).
  • [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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17. Saada E, Thariat J, Follana P, Birtwisle-Peyrottes I, Haudebourg J, Trojani C, Bacque P, Thyss A: Primitive neuroectodermal tumor of the pelvis in an elderly patient. Onkologie; 2009 Sep;32(8-9):499-502
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  • [Title] Primitive neuroectodermal tumor of the pelvis in an elderly patient.
  • BACKGROUND: Peripheral primitive neuroectodermal tumors (PNET) belong to the rare family of primary bone neoplasms.
  • Recent clinicopathological studies have revealed that Ewing's sarcoma and PNET have overlapping features and they are now included in the same classification, the Ewing's sarcoma family of tumors (EFTs).
  • PNET have a marked predilection for the extremities and are very rare in the pelvis.
  • CASE REPORT: We report the case of a 69-year-old man with PNET sarcoma.
  • Outcome was favorable after combined modality treatment including chemotherapy based on the Memphis protocol - adapted from that used for Ewing's sarcoma in children - and surgery.
  • CONCLUSION: Our case is uncommon because of the age at diagnosis, the fortuitous way of revelation, and the choice of dose-intense chemotherapy adapted from the Memphis protocol (cyclophosphamide- and doxorubicin-based) for children, which was efficient and safe.
  • It supports the fact that an adult, and even an old patient, with good physical status, may be treated safely and radically even with dose-adapted aggressive chemotherapy.
  • [MeSH-major] Bone Neoplasms / pathology. Ilium / pathology. Neuroectodermal Tumors, Primitive / pathology. Pelvic Neoplasms / pathology

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  • (PMID = 19745594.001).
  • [ISSN] 1423-0240
  • [Journal-full-title] Onkologie
  • [ISO-abbreviation] Onkologie
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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18. Heikaus S, Schaefer KL, Eucker J, Hogrebe E, Danebrock R, Wai DH, Krenn V, Gabbert HE, Poremba C: Primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis in a 46-year-old man-differential diagnosis and review of the literature. Hum Pathol; 2009 Jun;40(6):893-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis in a 46-year-old man-differential diagnosis and review of the literature.
  • Peripheral primitive neuroectodermal tumor/Ewing's tumors are rare bone and soft tissue malignancies with a highly aggressive clinical course and early metastases occurring at multiple peripheral sites.
  • Here, we present for the first time a case of a 46-year-old man with a primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis.
  • The diagnosis of peripheral primitive neuroectodermal tumor/Ewing's tumor was established by histology, immunohistochemistry, and molecular pathology.
  • The tumor revealed a rapid progress in 2 months' time.
  • Therefore, the patient was included in the EURO-E.W.I.N.G.99 study and was placed on chemotherapy.
  • However, the tumor progressed during ongoing therapy, and the patient died in March 2008.
  • In conclusion, though being reported here for the first time, peripheral primitive neuroectodermal tumor/Ewing's tumors should be considered in the differential diagnosis of blue round cell tumors of the testis.
  • A rapid and correct diagnosis of this entity is crucial for fast and accurate therapy, which is stressed by the fatal case presented here.
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / pathology. Sarcoma, Ewing / pathology. Testicular Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Diagnosis, Differential. Fatal Outcome. Humans. Male. Middle Aged

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  • (PMID = 19269015.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 23
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19. Harder Y, Buechler U, Vögelin E: Primitive neuroectodermal tumor of the thumb metacarpal bone: a case report and literature review. J Hand Surg Am; 2003 Mar;28(2):346-52
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  • [Title] Primitive neuroectodermal tumor of the thumb metacarpal bone: a case report and literature review.
  • A 27-year-old otherwise healthy patient was diagnosed with a primitive neuroectodermal tumor of the thumb metacarpal bone of the left hand.
  • Based on a common chromosomal translocation this tumor shows a close relationship to Ewing's sarcoma.
  • The treatment consisted of neo- and adjuvant chemotherapy and marginal resection of the affected thumb metacarpal bone including periosseous soft tissue and reconstruction of the thumb by an intercalated segmental index pollicization.
  • [MeSH-major] Bone Neoplasms / surgery. Metacarpus / pathology. Neuroectodermal Tumors, Primitive, Peripheral / surgery. Thumb / pathology. Thumb / surgery

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  • (PMID = 12671870.001).
  • [ISSN] 0363-5023
  • [Journal-full-title] The Journal of hand surgery
  • [ISO-abbreviation] J Hand Surg Am
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 31
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20. Llombart-Bosch A, Pellín A, Carda C, Noguera R, Navarro S, Peydró-Olaya A: Soft tissue Ewing sarcoma--peripheral primitive neuroectodermal tumor with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript. Diagn Mol Pathol; 2000 Sep;9(3):137-44
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  • [Title] Soft tissue Ewing sarcoma--peripheral primitive neuroectodermal tumor with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript.
  • This study describes a new case of Ewing sarcoma (ES)-peripheral primitive neuroectodermal tumor (pPNET) with unusual phenotype and fusion gene structure.
  • The tumor located in the inguinal area of a 15-year-old boy showed a highly aggressive behavior with hematogenous metastases after intensive chemotherapy and bone marrow transplant, causing death 28 months after diagnosis.
  • The tumor displayed a clear cell pattern, and several neuroectodermal markers proved positive both in the original tumor and in xenografts.
  • This neuroectodermal character was confirmed by electron microscopy.
  • Moreover, cytogenetically the tumor has an unusual chromosomal rearrangement, t(2;22)(q13;q22,t(3;18)(p21;q23); representing a new EWS-FEV fusion type in which exon 7 of EWS gene is fused with exon 2 of FEV gene.
  • This is the third published study of an ES-pPNET showing EWS-FEV fusion described, but it is the first study of a tumor with the aforementioned fusion points.
  • [MeSH-major] Chromosomes, Human, Pair 2 / genetics. Chromosomes, Human, Pair 22 / genetics. Neuroectodermal Tumors, Primitive / genetics. Oncogene Proteins, Fusion / genetics. Sarcoma, Ewing / genetics. Soft Tissue Neoplasms / genetics. Translocation, Genetic / genetics
  • [MeSH-minor] Adolescent. Animals. Combined Modality Therapy. Disease Progression. Exons / genetics. Fatal Outcome. Groin. Humans. Karyotyping. Male. Mice. Mice, Nude. Neoplasm Metastasis. Neoplasm Proteins / analysis. Prognosis. Reverse Transcriptase Polymerase Chain Reaction. Transplantation, Heterologous. Tumor Cells, Cultured

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  • (PMID = 10976720.001).
  • [ISSN] 1052-9551
  • [Journal-full-title] Diagnostic molecular pathology : the American journal of surgical pathology, part B
  • [ISO-abbreviation] Diagn. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / EWS-FEV fusion protein, human; 0 / Neoplasm Proteins; 0 / Oncogene Proteins, Fusion
  • [Number-of-references] 45
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21. Gunluoglu MZ, Kara HV, Demir A, Dincer SI: Results of multimodal treatment of two patients with thoracic primitive neuroectodermal tumor. Is surgery really helpful for survival? Thorac Cardiovasc Surg; 2007 Oct;55(7):460-1
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  • [Title] Results of multimodal treatment of two patients with thoracic primitive neuroectodermal tumor. Is surgery really helpful for survival?
  • Primitive neuroectodermal tumors (PNET) belong to the group of small round cell tumors and are rarely seen.
  • They are rapidly progressive tumors, despite usually being treated by a multimodal therapy which includes surgery and chemoradiotherapy.
  • We present two patients with PNET of the thorax treated in our clinic.
  • The first patient had a huge tumor in the right hemithorax, which shifted the mediastinum to the contralateral hemithorax.
  • Diagnosis was established by transthoracic fine-needle aspiration biopsy and the tumor was treated by surgical resection.
  • The second patient had a small tumor on the right costovertebral angle which protruded towards the skin and was diagnosed by incisional biopsy.
  • The tumor responded very well to preoperative chemotherapy and complete resection was achieved surgically.
  • This patient had bone metastasis, local recurrence and pleural pulmonary metastasis after 6, 18 and 28 months, respectively, and died 30 months after the operation.We discuss the limited effect of surgery on the treatment of thoracic PNET on the basis of the results of these patients in whom we performed surgery.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Brain Neoplasms / therapy. Neoplasm Recurrence, Local / therapy. Neuroectodermal Tumors, Primitive, Peripheral / therapy. Pleural Neoplasms / therapy. Thoracic Neoplasms / therapy. Thoracotomy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Combined Modality Therapy. Fatal Outcome. Female. Humans. Male. Neoadjuvant Therapy. Radiotherapy, Adjuvant. Tomography, X-Ray Computed

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  • (PMID = 17902071.001).
  • [ISSN] 0171-6425
  • [Journal-full-title] The Thoracic and cardiovascular surgeon
  • [ISO-abbreviation] Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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22. Utsunomiya A, Uenohara H, Suzuki S, Shimosaka S, Numagami Y, Nishimura S, Nishino A, Suzuki H, Sakurai Y: [A case of peripheral-type primitive neuroectodermal tumor arising in the dura mater at the frontal base]. No To Shinkei; 2004 Mar;56(3):237-41
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  • [Title] [A case of peripheral-type primitive neuroectodermal tumor arising in the dura mater at the frontal base].
  • Magnetic resonance image (MRI) revealed a large tumor in the frontal base with tumoral hemorrhage.
  • Angiography showed the tumor was fed by anterior meningeal arteries.
  • At surgery, the tumor was arising in the dura mater at the frontal base, and was removed totally.
  • Histological examination showed the tumor to be composed of small cells with uniform round nuclei and minimal cytoplasm.
  • The tumor was histologically confirmed to be peripheral-type primitive neuroectodermal tumor(pPNET).
  • Following surgery, he underwent whole brain, whole spine and local radiation therapy(30 Gy in total respectively) and received two 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day, etoposide 60 mg/m2/day and IFOS 900 mg/m2/day.
  • After these therapies, follow-up radiological examination showed there was no recurrence of the tumor for 24 months.
  • Ewing sarcoma and pPNET(ES/pPNET) is the designation given to a family of small round cell tumor arising in bone or soft tissues.
  • Intracranial PNETs are devided into central nervous system PNET(cPNET) and pPNET.
  • It is necessary that intracranial PNETs are divided into two types of PNETs because of different prognosis between these tumors.
  • MIC-2 is a specific marker for pPNET/ES family and is useful in the differential diagnosis of these two types of tumors.
  • [MeSH-major] Dura Mater. Meningeal Neoplasms / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis
  • [MeSH-minor] Antigens, CD / analysis. Biomarkers, Tumor / analysis. Cell Adhesion Molecules / analysis. Chemotherapy, Adjuvant. Child. Diagnosis, Differential. Humans. Magnetic Resonance Angiography. Male. Radiotherapy, Adjuvant. Treatment Outcome

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  • (PMID = 15112448.001).
  • [ISSN] 0006-8969
  • [Journal-full-title] Nō to shinkei = Brain and nerve
  • [ISO-abbreviation] No To Shinkei
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
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23. Somers GR, Shago M, Zielenska M, Chan HS, Ngan BY: Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and an unusual karyotype: case report. Pediatr Dev Pathol; 2004 Sep-Oct;7(5):538-45
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  • [Title] Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and an unusual karyotype: case report.
  • Primitive neuroectodermal tumor/Ewing sarcoma (PNET/ES) rarely occurs in the skin and subcutaneous tissues.
  • We present a case of a 16-year-old girl with primary cutaneous and subcutaneous PNET/ES of the abdominal wall.
  • Despite wide local excision and chemotherapy, she rapidly developed cranial bone and brain metastases, followed by lung and skeletal metastases, and died shortly thereafter.
  • The recurrent tumor exhibited light microscopic features of a small, round, blue cell tumor with intracytoplasmic glycogen.
  • Cytogenetic analysis of the relapsed tumor showed a complex karyotype: 47,XX,i(1)(q10), der(4)t(4;19) (q33 approximately q35;q13.1), + 8,t(15;17)(q24;p11.2 approximately p12),der(19)t (19;20)(q13.1;p11.2),der(22)t(20;22)(q13;q13).
  • The clinical behavior and atypical and complex cytogenetic abnormalities exhibited by the tumor in this patient are unusual and represent the most aggressive end of the clinical spectrum of cutaneous and subcutaneous PNET/ES.
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / secondary. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / pathology. Subcutaneous Tissue / pathology
  • [MeSH-minor] Adolescent. Biomarkers, Tumor / analysis. Bone Neoplasms / secondary. Brain Neoplasms / metabolism. Brain Neoplasms / secondary. Chromosome Aberrations. Fatal Outcome. Female. Humans. Immunohistochemistry. Lung Neoplasms / secondary. Microscopy, Electron, Transmission. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15547779.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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24. 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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  • [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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25. Ludwig K: [Musculoskeletal lymphomas]. Radiologe; 2002 Dec;42(12):988-92
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  • Primary lymphomas of bone or skeletal muscle are rare entities.
  • The most frequent among these diseases are primary non-Hodgkin's lymphomas of bone.
  • They account for 3-5% of all bone tumors and 5% of all primary extranodal non-Hodgkin's lymphomas.
  • Primary manifestations of Hodgkin's disease in bone or skeletal muscle are rarities.
  • Primary non-Hodgkin's lymphomas of skeletal muscle are rarities as well.
  • Primary non-Hodgkin's lymphomas of bone can be found in any patient age.
  • The radiographic appearance of these entities resembles other aggressive bone tumors.
  • Their differential diagnosis includes -- depending on the patient's age -- Ewing's sarcoma,malignant fibrous histiocytoma,metastases of small cell tumors and osteomyelitis.Further differential diagnoses are the peripheral primitive neuroectodermal tumor (PNET), osteosarcoma, eosinophilic granuloma and fibrosarcoma.
  • Treatment of primary non-Hodgkin's lymphomas uses combinations of chemotherapy and radiation therapy.
  • Operative treatment is reserved for the treatment of complications.
  • The prognosis of primary non-Hodgkin's lymphomas is reflected by 10-year-survival-rates without recurrence of more than 80% in unifocal manifestations.
  • [MeSH-major] Bone Neoplasms / diagnosis. Hodgkin Disease / diagnosis. Lymphoma, Non-Hodgkin / diagnosis. Magnetic Resonance Imaging. Muscle Neoplasms / diagnosis. Tomography, X-Ray Computed
  • [MeSH-minor] Bone and Bones / pathology. Humans. Muscle, Skeletal / pathology. Neoplasm Staging. Prognosis. Sensitivity and Specificity

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  • (PMID = 12486552.001).
  • [ISSN] 0033-832X
  • [Journal-full-title] Der Radiologe
  • [ISO-abbreviation] Radiologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 0
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26. Kano T, Sasaki A, Tomizawa S, Shibasaki T, Tamura M, Ohye C: Primary Ewing's sarcoma of the orbit: case report. Brain Tumor Pathol; 2009;26(2):95-100
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  • Computed tomography revealed a left intraorbital mass measuring 3 cm x 3 cm involving the left lateral wall of the orbit and the greater wing of the left sphenoid bone.
  • During surgery, the tumor was seen to arise from the lateral wall of the orbit and infiltrate into the left temporal muscle.
  • Following the surgery, the patient was administered radiation therapy for the whole cranium and chemotherapy for the residual tumors.
  • However, the tumor recurred, and the patient died about 2 years following the first surgery because the tumor had metastasized to the lung.
  • On light microscopy, the tumor cells were closely packed with uniform, small, and round cells.
  • Immunohistochemical studies showed that the tumor cell membrane stained positive for MIC2.
  • Based on these results, the tumor was diagnosed to be primary Ewing's sarcoma.
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Orbital Neoplasms / diagnosis. Sarcoma, Ewing / diagnosis

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  • (PMID = 19856222.001).
  • [ISSN] 1861-387X
  • [Journal-full-title] Brain tumor pathology
  • [ISO-abbreviation] Brain Tumor Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] TDQ283MPCW / Eosine Yellowish-(YS); YKM8PY2Z55 / Hematoxylin
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27. Ziegler DS, Cohn RJ, McCowage G, Alvaro F, Oswald C, Mrongovius R, White L, Australian and New Zealand Children's Study Group: Efficacy of vincristine and etoposide with escalating cyclophosphamide in poor-prognosis pediatric brain tumors. Neuro Oncol; 2006 Jan;8(1):53-9
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  • Three consecutive studies by the Australia and New Zealand Children's Cancer Study Group--VETOPEC I, Baby Brain 91, and VETOPEC II--have used a specific chemotherapy regimen of vincristine (VCR), etoposide (VP-16) and escalating CPA in patients with relapsed, refractory, or high-risk solid tumors.
  • Patients in the VETOPEC II cohort were treated with very high dose CPA with peripheral blood stem cell (PBSC) rescue.
  • We also assessed whether the use of very high dose chemotherapy with stem cell rescue improved the response rate or affected toxicity.
  • Seventy-one brain tumor patients were treated with VETOPEC-based protocols.
  • Of the 54 patients evaluable for tumor response, 17 had a complete response (CR) and 20 a partial response (PR) to treatment, which yielded an overall response rate of 69%.
  • The CR + PR was 83% (19/23) for medulloblastomas, 56% (5/9) for primitive neuroectodermal tumors, 55% (6/11) for grade 3 and 4 astrocytomas, and 80% (6/8) for ependymomas.
  • There were no toxic deaths within the PBSC-supported VETOPEC II cohort, despite higher CPA doses, compared with 7% among the non-PBSC patients.
  • The response rates observed support further development of this chemotherapy regimen.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Brain Neoplasms / drug therapy. Brain Neoplasms / mortality

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  • (PMID = 16443948.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Meta-Analysis; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; VETOPEC protocol
  • [Other-IDs] NLM/ PMC1871918
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28. Yeoh EJ, Cunningham JM, Yee GC, Hunt D, Houston JA, Richardson SL, Stewart CF, Houghton PJ, Bowman LC, Gajjar AJ: Topotecan-filgrastim combination is an effective regimen for mobilizing peripheral blood stem cells. Bone Marrow Transplant; 2001 Sep;28(6):563-71
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  • [Title] Topotecan-filgrastim combination is an effective regimen for mobilizing peripheral blood stem cells.
  • We compared the efficacy, toxicity, and cost of topotecan-filgrastim and filgrastim alone for mobilizing peripheral blood stem cells (PBSCs) in 24 consecutive pediatric patients with newly diagnosed medulloblastoma.
  • PBSCs were mobilized with an upfront window of topotecan-filgrastim for 11 high-risk patients (residual tumor > or =1.5 cm2 after resection; metastases limited to neuraxis) and with filgrastim alone for 13 average-risk patients.
  • All patients subsequently underwent craniospinal irradiation and four courses of high-dose chemotherapy with stem cell rescue.
  • Target yields of CD34+ cells (> or =8 x 10(6)/kg) were obtained with only one apheresis procedure for each of the 11 patients treated with topotecan-filgrastim, but with a mean of 2.3 apheresis procedures for only six (46%) of the 13 patients treated with filgrastim alone (P = 0.0059).
  • Mean times to neutrophil and platelet engraftment were similar.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Granulocyte Colony-Stimulating Factor / administration & dosage. Hematopoietic Stem Cell Mobilization / methods. Topotecan / administration & dosage
  • [MeSH-minor] Child. Costs and Cost Analysis. Female. Filgrastim. Hematopoietic Stem Cell Transplantation / adverse effects. Hematopoietic Stem Cell Transplantation / economics. Hematopoietic Stem Cell Transplantation / methods. Humans. Male. Medulloblastoma / complications. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / complications. Neuroectodermal Tumors, Primitive / therapy. Radiotherapy, Adjuvant. Recombinant Proteins. Retrospective Studies. Therapeutic Equivalency

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  • (PMID = 11607769.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P01 CA 23099; United States / NCI NIH HHS / CA / P30 CA 21765
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Recombinant Proteins; 143011-72-7 / Granulocyte Colony-Stimulating Factor; 7M7YKX2N15 / Topotecan; PVI5M0M1GW / Filgrastim
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29. De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A: Temozolomide in resistant or relapsed pediatric solid tumors. Pediatr Blood Cancer; 2006 Jul;47(1):30-6
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  • The drug was administered at the dose of 215 mg/m2/day x 5 days or 180 mg/m2/day x 5 days in patients with prior craniospinal irradiation (CSI) or autologous bone marrow transplantation (ABMT).
  • Tumor types were: neuroblastoma (NB; n = 17), medulloblastoma (MB; 8), brain stem glioma (BSG; 8), extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor (EOES; 4), Ewing's sarcoma (ES; 4), anaplastic astrocytoma (AA; 3), rhabdomyosarcoma (RMS; 2), ependymoma (EP; 2), cerebral primitive neuroectodermal tumor (cPNET; 2), hepatocarcinoma (HC; 1), and osteosarcoma (OS; 1).
  • RESULTS: Objective response-rate (CR + PR + MR) in our series was 13.4% (1.9% CR, 3.8% PR, and 7.7% MR), SD occurred in 38.4% of patients and 48% had PD.
  • The median survival was 7.8 months (range 1-37) and median time to progression was 3.4 months (range 1-20); these data were significantly correlated with histology and previous nitrosureas administration in multivariate analysis.
  • CONCLUSION: Oral TMZ was well tolerated in children with resistant or relapsed solid tumors and showed activity in NB and CNS tumours refractory to standard chemotherapy.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Dacarbazine / analogs & derivatives. Drug Resistance, Neoplasm. Neoplasm Recurrence, Local / drug therapy. Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease-Free Survival. Dose-Response Relationship, Drug. Female. Humans. Male. Survival Analysis

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  • [Copyright] Copyright 2006 Wiley-Liss, Inc.
  • [ErratumIn] Pediatr Blood Cancer. 2006 Oct 15;47(5):647-8
  • (PMID = 16047361.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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30. Sato S, Mitsuyama T, Ishii A, Kawakami M, Kawamata T: Multiple primary cranial Ewing's sarcoma in adulthood: case report. Neurosurgery; 2009 Feb;64(2):E384-6; discussion E386
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: Ewing's sarcoma is a malignant bone tumor occurring most frequently in the long bones and flat bones as a solitary lesion during the first 2 decades of life.
  • Ewing's sarcoma and peripheral primitive neuroectodermal tumor have recently been considered to be the same entity because of histological and molecular similarities.
  • A computed tomographic scan revealed osteolytic changes of the inner calvarial bone.
  • Electron microscopy showed little differentiation to neuronal tissue, indicating Ewing's sarcoma.
  • After surgical treatment, conventional whole cranial irradiation of 40 Gy and chemotherapy were conducted.
  • The tumor in the left frontal region disappeared.
  • CONCLUSION: Although quite rare, Ewing's sarcoma should be taken into consideration as a differential diagnosis of multiple cranial mass lesions in adulthood.
  • [MeSH-major] Neoplasms, Multiple Primary / diagnosis. Neoplasms, Multiple Primary / therapy. Sarcoma, Ewing / diagnosis. Sarcoma, Ewing / therapy. Skull Neoplasms / diagnosis. Skull Neoplasms / therapy
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male. Treatment Outcome

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  • (PMID = 19190443.001).
  • [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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31. Deutsch M, Wollman MR: Radiotherapy for metastases to the mandible in children. J Oral Maxillofac Surg; 2002 Mar;60(3):269-71
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Six children had a neuroblastoma, 1 had angiosarcoma of the liver, 1 had adenocarcinoma of the rectum, and 1 had peripheral primitive neuroectodermal tumor (Ewing's sarcoma) of the spine.
  • In 3 children, the mandible was the first bone involved by metastases.
  • All children had received chemotherapy.
  • RESULTS: All children died of disseminated disease at 5 to 59 months from their initial diagnosis, 5 to 29 months from the detection of metastases to bone, and only 6 days to 17 months (median, 2 months) from the first treatment of metastases to the mandible.
  • CONCLUSIONS: The outlook for children with metastases that involve the mandible is very poor, and we recommend short intensive courses of radiotherapy consisting of 1 to 3 treatments to total doses of 400 to 1,200 cGy for palliation of pain.

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  • [Copyright] Copyright 2002 American Association of Oral and Maxillofacial Surgeons
  • (PMID = 11887137.001).
  • [ISSN] 0278-2391
  • [Journal-full-title] Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons
  • [ISO-abbreviation] J. Oral Maxillofac. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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32. Lowichik A, Zhou H, Pysher TJ, Smith L, Lemons R, Coffin CM: Therapy associated changes in childhood tumors. Adv Anat Pathol; 2000 Nov;7(6):341-59
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Therapy associated changes in childhood tumors.
  • Contemporary treatment regimens for the common solid tumors of childhood have led to increased numbers of post-treatment pathologic specimens from survivors.
  • Current therapeutic strategies for childhood cancers in North America require an accurate pathologic diagnosis and stratify patients based on combinations of clinical, biological, and pathologic features.
  • In several tumor systems, the pathologic response to therapy also modifies the treatment regimen.
  • Accurate pathologic interpretation of such specimens is critical in providing useful prognostic information for therapeutic decisions.
  • Standardized handling of post-therapy pathologic specimens, appropriate use of molecular and genetic studies, consideration of the differential diagnoses, and assessment of the potential biologic significance of therapy-induced pathologic changes are, therefore, critical for patient management and determination of treatment protocols.
  • [MeSH-minor] Adolescent. Bone Neoplasms / drug therapy. Bone Neoplasms / pathology. Bone Neoplasms / surgery. Child. Child, Preschool. Fibrosarcoma / pathology. Fibrosarcoma / therapy. Hepatoblastoma / pathology. Hepatoblastoma / therapy. Histocytochemistry. Humans. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Liver Neoplasms / pathology. Liver Neoplasms / therapy. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Osteosarcoma / pathology. Osteosarcoma / surgery. Prognosis. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / surgery. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / drug therapy. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery. Wilms Tumor / drug therapy. Wilms Tumor / pathology. Wilms Tumor / surgery

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  • (PMID = 11078058.001).
  • [ISSN] 1072-4109
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 149
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33. Krasin MJ, Rodriguez-Galindo C, Billups CA, Davidoff AM, Neel MD, Merchant TE, Kun LE: Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors. Int J Radiat Oncol Biol Phys; 2004 Nov 1;60(3):830-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors.
  • PURPOSE: To assess the effect of radiation dose on local tumor control of the Ewing sarcoma family of tumors in 79 patients with localized disease treated at a single institution.
  • METHODS AND MATERIALS: Thirty-seven patients received vincristine, actinomycin D, cyclophosphamide, and doxorubicin, and 42 received vincristine, actinomycin D, and cyclophosphamide, with alternating cycles of ifosfamide and etoposide; all underwent definitive radiotherapy (median dose, 37.5 Gy) with either low-dose (<40 Gy) or standard dose (> or =40 Gy) radiation delivered according to the protocol.
  • We calculated the cumulative incidence of local treatment failure, disease recurrence, and overall survival and analyzed the effect of known prognostic factors and radiation dose.
  • RESULTS: The cumulative incidence of local treatment failure at 10 years was 30.4% and that of disease recurrence was 40%.
  • Patient age > or =14 years and tumor size > or =8 cm were adverse prognostic factors for local treatment failure; patient age > or =14 years was also associated with worse survival.
  • Although the radiation dose alone did not predict for local treatment failure, the cumulative incidence of local failure at 10 years was 19% when tumors <8 cm were treated with <40 Gy, and no patient treated with standard doses (> or =40 Gy) developed local recurrence (p = 0.084).
  • CONCLUSION: Tumor size and patient age predict for local tumor control in patients with Ewing sarcoma family of tumors treated with systemic therapy and definitive radiotherapy.
  • [MeSH-major] Bone Neoplasms / radiotherapy. Sarcoma, Ewing / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Age Factors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Infant. Male. Neoplasm Recurrence, Local. Neoplasms, Second Primary. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / radiotherapy. Prognosis. Radiotherapy Dosage. Retrospective Studies. Treatment Failure. Vincristine / administration & dosage

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  • (PMID = 15465200.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide
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34. Scurr M, Judson I: How to treat the Ewing's family of sarcomas in adult patients. Oncologist; 2006 Jan;11(1):65-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Ewing's sarcoma, peripheral primitive neuroectodermal tumor, and Askin's tumor comprise a single family of tumors, the Ewing's family of tumors, which is characterized by chromosomal translocation.
  • Ewing's sarcoma is known as a malignancy of childhood, but with a median age of 15 years at diagnosis, it should equally be regarded as a malignancy of adolescence and young adulthood.
  • There is much controversy regarding the role of age at diagnosis, with some studies showing older age to be associated with poorer outcome and others showing no association between age and survival.
  • This article examines whether age does affect outcome and treatment in this group of tumors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Sarcoma, Ewing / drug therapy

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  • (PMID = 16401715.001).
  • [ISSN] 1083-7159
  • [Journal-full-title] The oncologist
  • [ISO-abbreviation] Oncologist
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 51
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35. Sturla LM, Westwood G, Selby PJ, Lewis IJ, Burchill SA: Induction of cell death by basic fibroblast growth factor in Ewing's sarcoma. Cancer Res; 2000 Nov 1;60(21):6160-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Ewing's sarcoma is thought to arise after developmental arrest of primitive neural cells during embryogenesis.
  • Treatment of NuNu mice with bFGF decreased growth of the highly tumorigenic Ewing's sarcoma cell lines.
  • This novel observation may provide a new therapeutic strategy for Ewing's sarcomas.
  • [MeSH-major] Bone Neoplasms / pathology. Fibroblast Growth Factor 2 / pharmacology. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Animals. Apoptosis / drug effects. Cell Count. Cell Death / drug effects. Cell Differentiation / drug effects. Cell Division / drug effects. Cell Survival / drug effects. Female. Humans. Mice. Mice, Nude. Necrosis. Nerve Growth Factor / pharmacology. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / metabolism. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Receptors, Fibroblast Growth Factor / biosynthesis. Tumor Cells, Cultured. Xenograft Model Antitumor Assays

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  • (PMID = 11085540.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Receptors, Fibroblast Growth Factor; 103107-01-3 / Fibroblast Growth Factor 2; 9061-61-4 / Nerve Growth Factor
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