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1. Watt AJ: Chest wall lesions. Paediatr Respir Rev; 2002 Dec;3(4):328-38
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Malignant lesions include neuroblastoma, rhabdomyosarcoma, Ewings sarcoma, Askin tumour and primitive neuroectodermal tumours.
  • Manifestations of systemic diseases such as leukaemia, lymphoma, Langerhans cell histocytosis and infections such as tuberculosis and actinomycosis may also cause chest wall lesions.
  • The imaging characteristics of the above are reviewed but only a minority of lesions show diagnostic imaging characteristics.
  • The role of different imaging modalities is discussed, with an emphasis on magnetic resonance imaging for demonstrating lesion morphology and local spread, with computed tomography and nuclear medicine being used mainly to assess remote disease.

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  • (PMID = 12457604.001).
  • [ISSN] 1526-0542
  • [Journal-full-title] Paediatric respiratory reviews
  • [ISO-abbreviation] Paediatr Respir Rev
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 59
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2. Van Valen F, Fulda S, Truckenbrod B, Eckervogt V, Sonnemann J, Hillmann A, Rödl R, Hoffmann C, Winkelmann W, Schäfer L, Dockhorn-Dworniczak B, Wessel T, Boos J, Debatin KM, Jürgens H: Apoptotic responsiveness of the Ewing's sarcoma family of tumours to tumour necrosis factor-related apoptosis-inducing ligand (TRAIL). Int J Cancer; 2000 Oct 15;88(2):252-9
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  • [Title] Apoptotic responsiveness of the Ewing's sarcoma family of tumours to tumour necrosis factor-related apoptosis-inducing ligand (TRAIL).
  • We investigated the cytotoxic responsiveness of 40 cell lines derived from representatives of the Ewing's sarcoma family of tumours (ESFT), i.e., Ewing's sarcoma (ES), peripheral primitive neuroectodermal tumour (pPNET) and Askin tumour (AT), to tumour necrosis factor-related apoptosis-inducing ligand (TRAIL).
  • Incubation with TRAIL at 100 ng/ml induced cell death at 24 hr in 19 of 26 ES, 11 of 12 pPNET and 2 of 2 AT cell lines.
  • Half-maximal cell death concentrations (IC(50) values) varied from 0.1 to 20 ng/ml.
  • TRAIL displayed potent cytotoxic activity against freshly derived ESFT cell isolates.
  • The apoptotic programme in the sensitive ESFT VH-64 cell line revealed TRAIL-induced activation of FLICE/MACH1 (caspase-8) and CPP32/Yama/apopain (caspase-3) and processing of the prototype caspase substrate poly(ADP-ribose) polymerase.
  • While macromolecule synthesis inhibitors (actinomycin D, cycloheximide) augmented susceptibility to TRAIL in TRAIL-responsive cell lines, these agents did not render TRAIL-resistant cell lines susceptible to TRAIL.
  • However, the proteasome inhibitor MG132 sensitised to TRAIL in resistant cell lines.
  • Collectively, these results show that TRAIL initiates effective death in the vast majority (80%) of cell lines derived from ESFT.
  • Since TRAIL provoked cell death in ESFT ex vivo, this cytokine may be a promising drug for the treatment of ESFT in vivo.
  • [MeSH-major] Apoptosis / drug effects. Membrane Glycoproteins / toxicity. Tumor Necrosis Factor-alpha / toxicity
  • [MeSH-minor] Adenosine Triphosphate / metabolism. Apoptosis Regulatory Proteins. Bone Neoplasms. Cell Survival / drug effects. Cytochrome c Group / analysis. Humans. Intracellular Membranes / drug effects. Intracellular Membranes / pathology. Intracellular Membranes / physiology. Kinetics. Membrane Potentials / drug effects. Mitochondria / pathology. Mitochondria / physiology. Neuroectodermal Tumors, Primitive. Sarcoma, Ewing. TNF-Related Apoptosis-Inducing Ligand. Tumor Cells, Cultured

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  • [Copyright] Copyright 2000 Wiley-Liss, Inc.
  • (PMID = 11004677.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Apoptosis Regulatory Proteins; 0 / Cytochrome c Group; 0 / Membrane Glycoproteins; 0 / TNF-Related Apoptosis-Inducing Ligand; 0 / TNFSF10 protein, human; 0 / Tumor Necrosis Factor-alpha; 8L70Q75FXE / Adenosine Triphosphate
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3. 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.
  • 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.
  • [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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4. Ma C, Bower KA, Chen G, Shi X, Ke ZJ, Luo J: Interaction between ERK and GSK3beta mediates basic fibroblast growth factor-induced apoptosis in SK-N-MC neuroblastoma cells. J Biol Chem; 2008 Apr 4;283(14):9248-56
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  • The Ewing's sarcoma family of tumors (ESFT) includes Ewing's sarcoma (ES), Askin's tumor of the chest wall, and peripheral primitive neuroectodermal tumor.
  • Using a human peripheral primitive neuroectodermal tumor cell line, SK-N-MC, we demonstrated FGF2 stimulated phosphorylation of ERK1 and ERK2 (pERK1/2) and GSK3beta (pGSK3beta(Tyr-216)), all of which were primarily retained in the cytoplasm.
  • Similarly, expression of a kinase-deficient (K85R) GSK3beta or GSK3beta-small interfering RNA inhibited FGF2-regulated ERK/pGSK3beta(Tyr-216) association and translocated pERK to the nucleus.
  • Both K85R GSK3beta and small interfering RNA offered protection against FGF2-induced cell death.
  • In contrast, overexpression of wild-type GSK3beta sensitized cells to FGF2 cytotoxicity.
  • As a result, they potentiated FGF2-induced cell death.

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  • (PMID = 18263590.001).
  • [ISSN] 0021-9258
  • [Journal-full-title] The Journal of biological chemistry
  • [ISO-abbreviation] J. Biol. Chem.
  • [Language] ENG
  • [Grant] United States / NIAAA NIH HHS / AA / AA015407-03; United States / NIAAA NIH HHS / AA / R01 AA015407; United States / NIAAA NIH HHS / AA / AA 015407; United States / NIAAA NIH HHS / AA / R01 AA015407-03
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; 0 / Multienzyme Complexes; 0 / Neoplasm Proteins; 0 / Oxidants; 0 / RNA, Small Interfering; 0 / Solvents; 103107-01-3 / Fibroblast Growth Factor 2; 3K9958V90M / Ethanol; BBX060AN9V / Hydrogen Peroxide; EC 2.7.11.1 / glycogen synthase kinase 3 beta; EC 2.7.11.24 / Mitogen-Activated Protein Kinase 1; EC 2.7.11.24 / Mitogen-Activated Protein Kinase 3; EC 2.7.11.26 / Glycogen Synthase Kinase 3
  • [Other-IDs] NLM/ PMC2431019
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5. Fresco R, Saldombide L, Suárez L: Synchronous presentation of an Askin tumor and a plasmacytoma in an adult patient. Tumori; 2003 May-Jun;89(3):324-7
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  • [Title] Synchronous presentation of an Askin tumor and a plasmacytoma in an adult patient.
  • Askin tumor, or malignant small round cell tumor of the thoracopulmonary region, is an extremely infrequent entity occurring primarily in children and adolescents.
  • Its histopathologic and cytogenetic features suggest that it belongs to the family of Ewing's sarcoma and primitive neuroectodermal tumors.
  • We report the case of a 43-year-old woman affected by an Askin tumor with bone metastases at diagnosis, presenting synchronously with a plasmacytoma.
  • This is the first reported case of the simultaneous occurrence of an Askin tumor and a malignant hemopathy.
  • The progression of the former and the remission of the plasmacytoma during chemotherapy were remarkable, since Askin tumor treatment shares drugs used for the treatment of plasma cell tumors.
  • Given the infrequent presentation of these diseases in a young adult and the coexistence of two neoplasias characterized by typical chromosomal abnormalities, we consider the possibility of a genetic cancer susceptibility in our patient.
  • [MeSH-major] Bone Neoplasms / secondary. Carcinoma, Small Cell / secondary. Neoplasms, Second Primary / pathology. Plasmacytoma / pathology. Thoracic Neoplasms / pathology

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  • (PMID = 12908792.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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6. Subbiah V, Anderson P, Lazar AJ, Burdett E, Raymond K, Ludwig JA: Ewing's sarcoma: standard and experimental treatment options. Curr Treat Options Oncol; 2009 Apr;10(1-2):126-40
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  • OPINION STATEMENT: Ewing sarcoma family tumors (EWS), which include classic Ewing's sarcoma in addition to primitive neuroectodermal tumor and Askin tumor, are the second most common variety of primary bone cancer to afflict adolescents and young adults.
  • As has already occurred for malignancies such as breast or colon cancer, the "-omics-based" revolution has enhanced our understanding of the molecular changes responsible for Ewing's tumor formation and identified a number of potential targets (such as IGF-1R or mTOR) amenable to biological therapy.
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / pharmacology. Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Clinical Trials as Topic. Combined Modality Therapy. Drug Delivery Systems. Drug Screening Assays, Antitumor. Humans. Lung Neoplasms / drug therapy. Lung Neoplasms / mortality. Lung Neoplasms / secondary. Lung Neoplasms / surgery. Multicenter Studies as Topic. Oncogene Proteins, Fusion / antagonists & inhibitors. Oncogene Proteins, Fusion / genetics. Proto-Oncogene Protein c-fli-1. RNA-Binding Protein EWS. Receptor, IGF Type 1 / antagonists & inhibitors. Survival Rate. Transcription Factors / antagonists & inhibitors. Translocation, Genetic. Young Adult

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  • (PMID = 19533369.001).
  • [ISSN] 1534-6277
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Transcription Factors; EC 2.7.10.1 / Receptor, IGF Type 1
  • [Number-of-references] 94
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7. Huang DS, Tang SQ, Wang JW, Liu L, Lu S: [Two cases of Askin tumor misdiagnosed as pulmonary tuberculosis]. Zhonghua Er Ke Za Zhi; 2004 Apr;42(4):286
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  • [Title] [Two cases of Askin tumor misdiagnosed as pulmonary tuberculosis].
  • [MeSH-major] Diagnostic Errors. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Thoracic Neoplasms / diagnosis. Tuberculosis, Pulmonary / diagnosis
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carcinoma, Small Cell / diagnosis. Carcinoma, Small Cell / drug therapy. Child. Diagnosis, Differential. Humans. Male. Prognosis. Treatment Outcome

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  • (PMID = 15157391.001).
  • [ISSN] 0578-1310
  • [Journal-full-title] Zhonghua er ke za zhi = Chinese journal of pediatrics
  • [ISO-abbreviation] Zhonghua Er Ke Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
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8. Papi A, Ferreri AM, Rocchi P, Guerra F, Orlandi M: Epigenetic modifiers as anticancer drugs: effectiveness of valproic acid in neural crest-derived tumor cells. Anticancer Res; 2010 Feb;30(2):535-40
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  • [Title] Epigenetic modifiers as anticancer drugs: effectiveness of valproic acid in neural crest-derived tumor cells.
  • Valproic acid (VPA) is an established drug in the long-term therapy of epilepsy.
  • In this study, the anticancer properties of VPA on neural crest-derived human tumor cell lines G361 melanoma, U87MG glioblastoma and SKNMC Askin tumor cells were investigated.
  • The effect of VPA on cell growth, apoptotic activity and invasive ability were evaluated.
  • Firstly, VPA induced cell growth inhibition and apoptotic activity, as demonstrated by sulforhodamine B protein assay, annexin V assay and by Western blot analysis for Bcl2 and Bax expression levels, in all three cell lines.
  • Treatment with VPA caused a decrease in the invasive ability of all three cell lines.
  • Taken together, our results, besides providing further evidence that VPA may represent a promising therapeutic strategy in cancer treatment, may help in the design of new protocols geared at the treatment of neural crest-derived tumors.
  • [MeSH-major] Anticonvulsants / pharmacology. Brain Neoplasms / drug therapy. Glioblastoma / drug therapy. Melanoma / drug therapy. Thoracic Neoplasms / drug therapy. Valproic Acid / pharmacology
  • [MeSH-minor] Apoptosis / drug effects. Blotting, Western. Cell Line, Tumor. Cell Proliferation / drug effects. Humans. Matrix Metalloproteinases / metabolism. Neoplasm Invasiveness. Tissue Inhibitor of Metalloproteinase-1 / metabolism

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  • (PMID = 20332466.001).
  • [ISSN] 1791-7530
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Anticonvulsants; 0 / Tissue Inhibitor of Metalloproteinase-1; 614OI1Z5WI / Valproic Acid; EC 3.4.24.- / Matrix Metalloproteinases
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9. Seddon BM, Whelan JS: Emerging chemotherapeutic strategies and the role of treatment stratification in Ewing sarcoma. Paediatr Drugs; 2008;10(2):93-105
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  • The Ewing sarcoma family of tumors (ESFT) is one of the most common groups of malignancies arising in children, adolescents, and young adults up to approximately 25 years of age.
  • It comprises Ewing sarcoma arising from bone and extraosseous Ewing sarcoma arising from soft tissues (which includes peripheral neuroectodermal tumors and Askin tumor arising from the chest wall).
  • These can be categorized as tumor-related factors (presence of metastases, tumor site, volume, lactic dehydrogenase level, chromosomal translocation type, presence of fusion transcripts in blood and bone marrow), treatment-related factors (local therapy, histologic response to chemotherapy, radiologic response to chemotherapy, chemotherapy regimen), and patient-related factors (gender, age).
  • Newer chemotherapeutic agents are currently being investigated, and there is now increasing interest in the identification of molecular targets in ESFT that could be exploited therapeutically, which include the mammalian target of rapamycin (mTOR) and insulin growth factor-1 (IGF-1) receptor pathways.
  • [MeSH-minor] Age Factors. Antineoplastic Protocols. Combined Modality Therapy. Humans. Pediatrics. Prognosis. Protein Kinases / physiology. Receptor, IGF Type 1 / physiology. Signal Transduction. TOR Serine-Threonine Kinases

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  • (PMID = 18345719.001).
  • [ISSN] 1174-5878
  • [Journal-full-title] Paediatric drugs
  • [ISO-abbreviation] Paediatr Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.10.1 / Receptor, IGF Type 1
  • [Number-of-references] 126
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10. Iwamoto Y: Diagnosis and treatment of Ewing's sarcoma. Jpn J Clin Oncol; 2007 Feb;37(2):79-89
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  • Ewing's sarcoma is a small round-cell tumor typically arising in the bones, rarely in soft tissues, of children and adolescents.
  • Ewing's sarcoma has retained the most unfavorable prognosis of all primary musculoskeletal tumors.
  • Prior to the use of multi-drug chemotherapy, long-term survival was less than 10%.
  • In addition, the preferred method of tumor resection has changed; limb salvage has nearly replaced amputation of the affected limb.
  • Recent studies have revealed that the pathognomonic translocations involving the EWS gene on chromosome 22 and an ETS-type gene, which is most commonly the Fli1 gene on chromosome 11, are implicated in more than 95% of Ewing's sarcomas, primitive neuroectodermal tumors and Askin's tumors.
  • Therefore, these lesions have become regarded as a single entity, dubbed the Ewing's family of tumors.
  • RT-PCR to detect EWS-ETS gene arrangements is widely used to confirm the diagnosis of Ewing's family of tumors.
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Humans

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  • (PMID = 17272319.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 61
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11. Schiavetti A, Varrasso G, Maurizi P, Cappelli C, Clerico A, Properzi E, Castello MA: Ten-day schedule oral etoposide therapy in advanced childhood malignancies. J Pediatr Hematol Oncol; 2000 Mar-Apr;22(2):119-24
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  • Between April 1995 and February 1999, 15 pretreated patients with high-risk tumors received oral VP-16.
  • RESULTS: Twelve patients were evaluable for tumor response.
  • After 2 to 4 months of treatment, one patient had complete remission (CR), two had partial response (PR), two had minor response (MR), two had mixed response (MxR), three had stable disease (SD), and two had progressive disease (PD).
  • Stable disease was observed in three patients, one with an Askin tumor, one with medulloblastoma, and one with hepatoblastoma.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / therapeutic use. Etoposide / therapeutic use. Neoplasms / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Child. Child, Preschool. Disease Progression. Drug Administration Schedule. Feasibility Studies. Female. Humans. Male. Patient Compliance. Treatment Outcome

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  • (PMID = 10779024.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 6PLQ3CP4P3 / Etoposide
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