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1. 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.
  • [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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2. 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.
  • Computed tomography, bone scintigraphy, and positron emission tomography confirmed the mass to have a primary pulmonary origin.
  • Fluorescence in situ hybridization (FISH) was performed, which revealed an EWSR1 (Ewing sarcoma breakpoint region 1) 22q12 rearrangement.
  • 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

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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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3. 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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  • [Title] Induction of cell death by basic fibroblast growth factor in Ewing's sarcoma.
  • Ewing's sarcoma is thought to arise after developmental arrest of primitive neural cells during embryogenesis.
  • Because basic fibroblast growth factor (bFGF) has a critical role in the regulation of cell survival, proliferation, and differentiation during embryogenesis, we have tested the hypothesis that bFGF and FGF receptors may contribute to the development of Ewing's sarcoma and may provide a mechanism for the modulation of their behavior.
  • All four of the Ewing's sarcoma cell lines examined expressed bFGF and FGF receptors, which were detected by immunofluorescence and Western blotting. bFGF-induced a significant dose-dependent decrease in Ewing's sarcoma cell proliferation on plastic and reduced anchorage-independent growth in soft agar.
  • Induction of cell death was dependent on dosage of, and period of exposure to, bFGF. bFGF did not induce differentiation of Ewing's sarcoma cells in either the presence or the absence of serum or nerve growth factor.
  • Treatment of NuNu mice with bFGF decreased growth of the highly tumorigenic Ewing's sarcoma cell lines.
  • In summary, bFGF decreases Ewing's sarcoma growth in vitro and in vivo by the induction of cell death.
  • 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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4. 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

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  • [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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5. 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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  • [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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6. 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.
  • To assess the prognostic significance of neural differentiation in the family of Ewing's sarcoma, these results have been compared with the outcomes of 138 concomitant patients with typical Ewing's sarcoma (TES) who were treated according to the same protocol.
  • 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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7. 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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  • [Title] How to treat the Ewing's family of sarcomas in adult patients.
  • 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.
  • This has led to uncertainty in how best to manage nonpediatric patients with Ewing's sarcoma.
  • 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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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. 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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  • [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.
  • 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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10. 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.
  • 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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11. 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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12. Peng RJ, Sun XF, Xiang XJ, Zhen ZJ, Ling JY, Tong GL, Xia Y, Xu GC, Jiang WQ: [Efficacy and survival of 92 cases of Ewing's sarcoma family of tumor initially treated with multidisciplinary therapy]. Ai Zheng; 2009 Dec;28(12):1304-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Efficacy and survival of 92 cases of Ewing's sarcoma family of tumor initially treated with multidisciplinary therapy].
  • BACKGROUND AND OBJECTIVE: Ewing's sarcoma family of tumor (ESFT) is aggressive.
  • The optimal therapy modality for ESFT is still to be found.
  • This study was to explore the clinical characteristics and therapy for ESFT.
  • RESULT: Of 92 cases, 23 were Ewing's sarcoma of bone, 21 extraosseous Ewing's sarcoma, 43 peripheral primitive neuroectodermal tumor, and 5 Askin tumor.
  • Median follow-up time was 31.5 months (range, 10-137 months).
  • Thirty-eight patients received multidisciplinary therapy and 19 single model therapy in non-metastasis group.
  • Three-year overall survival (OS) and event-free survival (EFS) were significantly different between non-metastatic multidisciplinary therapy group and non-metastatic single model group (63% vs. 20%, 46% vs. 18%, respectively, P<0.001).
  • The patients who received surgery plus chemotherapy and plus radiation or not had longer survival than those treated with chemotherapy plus radiation in non-metastatic multidisciplinary therapy group (Chi2=7.591, 9.212; P=0.006, 0.002).
  • Cox regression analysis suggested therapy model and response to treatment were independent prognostic factors for ESFT.
  • CONCLUSIONS: Our studying showed multidisciplinary therapy could significantly improve non-metastatic ESFT patients' survival.
  • Chemotherapy plus surgery and plus radiation or not were superior to chemotherapy plus radiation in local control for the non-metastatic ESFT.
  • Therapy model and response were independent prognostic factors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Cyclophosphamide / therapeutic use. Disease-Free Survival. Doxorubicin / therapeutic use. Female. Follow-Up Studies. Humans. Infant. Lymphatic Metastasis. Male. Middle Aged. Pelvic Neoplasms / drug therapy. Pelvic Neoplasms / pathology. Pelvic Neoplasms / radiotherapy. Pelvic Neoplasms / surgery. Survival Rate. Vincristine / therapeutic use. Young Adult

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  • (PMID = 19958626.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; CAV protocol
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13. 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).
  • 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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14. 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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  • [Title] Primary Ewing's sarcoma of the orbit: case report.
  • 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.
  • Primary orbital Ewing's sarcoma of the skull has been considered to be extremely rare, and a review of the literature was performed.
  • [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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15. 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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  • [Title] Multiple primary cranial Ewing's sarcoma in adulthood: case report.
  • 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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16. 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 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.
  • [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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17. 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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  • 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.
  • [MeSH-minor] Adenocarcinoma / radiotherapy. Adenocarcinoma / secondary. Adolescent. Child. Child, Preschool. Female. Hemangiosarcoma / radiotherapy. Hemangiosarcoma / secondary. Humans. Infant. Liver Neoplasms / pathology. Male. Neuroblastoma / radiotherapy. Neuroblastoma / secondary. Pain, Intractable / radiotherapy. Radiotherapy Dosage. Rectal Neoplasms / pathology. Retrospective Studies. Sarcoma, Ewing / radiotherapy. Sarcoma, Ewing / secondary. Spinal Neoplasms / pathology

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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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18. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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).
  • 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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