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1. Windham TC, Pisters PW: Retroperitoneal sarcomas. Cancer Control; 2005 Jan-Feb;12(1):36-43
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  • [Title] Retroperitoneal sarcomas.
  • BACKGROUND: The evaluation and treatment of retroperitoneal sarcomas are challenging because the tumors are relatively rare and frequently present with advanced disease in an anatomically complex location.
  • METHODS: We reviewed the literature on experience in the management of retroperitoneal sarcomas, and we present our own experience in the treatment of these tumors.
  • RESULTS: The identification of prognostic factors other than the adequacy of resection has been inconsistent.
  • Due to a lack of associated symptoms, retroperitoneal sarcomas smaller than 5 cm are rare.
  • Computed tomography is the most useful tool in the evaluation of retroperitoneal tumors.
  • Surgery, radiation therapy, and chemotherapy are treatment options, but the most important factor in the treatment of primary tumors is complete surgical resection.
  • The role of neoadjuvant and adjuvant therapies is not defined and should be considered within the context of clinical trials.
  • CONCLUSIONS: Early referral of patients with retroperitoneal soft tissue tumors will help to ensure that they will receive the benefits of multidisciplinary evaluation and treatment of their disease and ready access to clinical trials.
  • [MeSH-major] Retroperitoneal Neoplasms / diagnosis. Retroperitoneal Neoplasms / therapy. Sarcoma / diagnosis. Sarcoma / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Humans. Liver Neoplasms / diagnosis. Liver Neoplasms / secondary. Liver Neoplasms / therapy. Lung Neoplasms / diagnosis. Lung Neoplasms / secondary. Lung Neoplasms / therapy. Neoplasm Recurrence, Local / surgery. Prognosis. Radiotherapy / methods. Retroperitoneal Space / pathology. Retroperitoneal Space / surgery

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  • (PMID = 15668651.001).
  • [ISSN] 1073-2748
  • [Journal-full-title] Cancer control : journal of the Moffitt Cancer Center
  • [ISO-abbreviation] Cancer Control
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 71
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2. Krstić V, Smoljanić Z, Djurović J, Marković V, Kosanović-Jaković N: [Gigantic ovarian and suprarenal cysts ten years after enucleation of unilateral retinoblastoma]. Srp Arh Celok Lek; 2008 Nov-Dec;136(11-12):658-61
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  • Patients with hereditary RB have increased risk of developing additional tumours, predominantly sarcomas.
  • The published results on the treatment of bilateral RB have shown that 10-15% of patients develop second primary tumours (SPT).
  • In the postoperative period, she did not undergo irradiation, nor did she receive chemotherapy.
  • She had no pains or other complaints.
  • Abdominal echosonography confirmed the presence of a huge multilocular cystic formation, 19 x 18 cm in diameter, spreading from the pelvis to the epigastrium, and pressing the liver and spleen.
  • CONCLUSION: We report a patient who developed second non-ocular tumours (ovarian and suprarenal cysts) after successful treatment of unilateral RB.
  • Early recognition of tumours, treatment can prevent possible complications.

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  • (PMID = 19177832.001).
  • [ISSN] 0370-8179
  • [Journal-full-title] Srpski arhiv za celokupno lekarstvo
  • [ISO-abbreviation] Srp Arh Celok Lek
  • [Language] srp
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Serbia
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3. Patel SR, Beach J, Papadopoulos N, Burgess MA, Trent J, Jenkins J, Benjamin RS: Results of a 2-arm Phase II study of 9-nitrocamptothecin in patients with advanced soft-tissue sarcomas. Cancer; 2003 Jun 1;97(11):2848-52
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  • [Title] Results of a 2-arm Phase II study of 9-nitrocamptothecin in patients with advanced soft-tissue sarcomas.
  • BACKGROUND: The authors conducted a 2-arm Phase II trial of 9-nitrocamptothecin (9-NC), an oral topoisomerase I inhibitor, to define response rates in patients with gastrointestinal (GI) leiomyosarcomas and other soft-tissue sarcomas (STS).
  • Response evaluation was performed at 8 weeks, and those with stable or responding disease continued treatment until maximal response was achieved.
  • Seventeen patients were enrolled on the GI leiomyosarcoma arm; only 1 minor response, lasting < 8 weeks in a patient with liver metastases, was noted, and so this arm was terminated.
  • Thirty-nine patients were entered on the other STS arm.
  • Four other patients required hospitalization for nausea, vomiting, and dehydration.
  • Other toxicities included diarrhea (36 patients, 5 with Grade 3 toxicity); fatigue (42 patients, 11 with Grade 3 toxicity); anorexia (32 patients, 1 with Grade 3 toxicity); nausea (37 patients, 2 with Grade 3 toxicity); vomiting (24 patients, 3 with Grade 3 toxicity); neutropenia (14 patients, 5 with Grade 3 toxicity); and thrombocytopenia (16 patients, 5 with Grade 3 or 4 toxicity).
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Camptothecin / analogs & derivatives. Camptothecin / therapeutic use. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Administration, Oral. Adult. Aged. Female. Gastrointestinal Neoplasms / drug therapy. Humans. Leiomyosarcoma / drug therapy. Male. Middle Aged. Topoisomerase I Inhibitors. Treatment Outcome

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  • [Copyright] Copyright 2003 American Cancer Society.
  • (PMID = 12767099.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Topoisomerase I Inhibitors; H19C446XXB / rubitecan; XT3Z54Z28A / Camptothecin
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4. Eggermont AM, ten Hagen TL: Isolated limb perfusion for extremity soft-tissue sarcomas, in-transit metastases, and other unresectable tumors: credits, debits, and future perspectives. Curr Oncol Rep; 2001 Jul;3(4):359-67
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  • [Title] Isolated limb perfusion for extremity soft-tissue sarcomas, in-transit metastases, and other unresectable tumors: credits, debits, and future perspectives.
  • Isolated limb perfusion (ILP) with melphalan is effective against melanoma in-transit metastases but has failed in the treatment of limb-threatening extremity sarcomas.
  • Now, ILP with TNF + melphalan is a very successful treatment to prevent amputation.
  • In a multicenter European trial, ILP with TNF + melphalan resulted in a 76% response rate and a 71% limb salvage rate in patients with limb-threatening soft-tissue sarcomas, deemed unresectable by independent review committees, leading to approval of TNF in Europe.
  • We have also reported on the success of this regimen against bulky melanomas, multifocal skin cancers, and drug-resistant bony sarcomas.
  • High-dose TNF destructs tumor vasculature, and, most importantly, it enhances tumor-selective drug uptake (ie, melphalan and doxorubicin) by threefold to sixfold.
  • Similar synergy is observed in well-vascularized liver metastases after isolated hepatic perfusion with TNF and melphalan.
  • New (vasoactive) drugs and mechanisms of action and interaction with chemotherapy are in development.
  • ILP is also a promising treatment modality for adenoviral vector-mediated gene therapy.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Chemotherapy, Cancer, Regional Perfusion / methods. Extremities. Forecasting. Melphalan / therapeutic use. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy. Tumor Necrosis Factor-alpha / therapeutic use

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  • (PMID = 11389822.001).
  • [ISSN] 1523-3790
  • [Journal-full-title] Current oncology reports
  • [ISO-abbreviation] Curr Oncol Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Tumor Necrosis Factor-alpha; Q41OR9510P / Melphalan
  • [Number-of-references] 88
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5. Horn B, Reiss U, Matthay K, McMillan A, Cowan M: Veno-occlusive disease of the liver in children with solid tumors undergoing autologous hematopoietic progenitor cell transplantation: a high incidence in patients with neuroblastoma. Bone Marrow Transplant; 2002 Mar;29(5):409-15
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  • [Title] Veno-occlusive disease of the liver in children with solid tumors undergoing autologous hematopoietic progenitor cell transplantation: a high incidence in patients with neuroblastoma.
  • Forty-one patients were diagnosed with neuroblastoma and 42 had another solid tumor (Ewing's sarcoma, soft tissue sarcomas, germ cell tumors, etc).
  • Patients with neuroblastoma were more likely than patients with other solid tumors (ST) to be < or =7 years of age, to have a decreased serum albumin level, and to have received abdominal radiation and surgery prior to transplant.
  • Twenty patients (24%) developed VOD.
  • Disease status at transplant, intensity of previous chemotherapy, conditioning regimen, progenitor cell source, ALT, AST, albumin level, renal function prior to transplant, or use of amphotericin, growth-factor or heparin during transplant, did not affect the incidence of VOD.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Humans. Incidence. Infant. Multivariate Analysis. Neoplasms / complications. Neoplasms / epidemiology. Neoplasms / therapy. Retrospective Studies. Risk Factors. Transplantation, Autologous. Treatment Outcome


6. Eilber FC, Rosen G, Forscher C, Nelson SD, Dorey F, Eilber FR: Recurrent gastrointestinal stromal sarcomas. Surg Oncol; 2000 Aug;9(2):71-5
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  • [Title] Recurrent gastrointestinal stromal sarcomas.
  • Gastrointestinal stromal sarcomas, formerly categorized as leiomyosarcomas of gastrointestinal origin, have a common pattern of intraperitoneal dissemination.
  • Despite surgical resection with or without adjuvant systemic chemotherapy the vast majority of these patients succumb to intraperitoneal sarcomatosis and/or hepatic metastases.
  • In an attempt to improve upon the morbidity and mortality associated with this disease we and several other centers have begun treating these patients with intraperitoneal chemotherapy.
  • We have found that aggressive surgical resection with postoperative intraperitoneal chemotherapy has significantly lowered the peritoneal recurrence rate in patients with recurrent gastrointestinal stromal sarcomas as compared to those who have undergone surgical resection alone.
  • However, this treatment approach has proven to be ineffective in preventing hepatic metastases, and thus has had little effect upon overall survival.
  • With the treatment of primary rather than recurrent disease we hope to interrupt the disease process at an earlier stage further decreasing peritoneal recurrences and potentially improving survival.
  • [MeSH-major] Gastrointestinal Neoplasms / drug therapy. Mitoxantrone / administration & dosage. Neoplasm Recurrence, Local / prevention & control. Sarcoma / drug therapy
  • [MeSH-minor] Antineoplastic Agents / administration & dosage. Chemotherapy, Adjuvant. Female. Humans. Injections, Intraperitoneal. Liver Neoplasms / secondary. Male. Neoplasm Staging. Prognosis. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 11094326.001).
  • [ISSN] 0960-7404
  • [Journal-full-title] Surgical oncology
  • [ISO-abbreviation] Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] NETHERLANDS
  • [Chemical-registry-number] 0 / Antineoplastic Agents; BZ114NVM5P / Mitoxantrone
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7. Finegold MJ, Egler RA, Goss JA, Guillerman RP, Karpen SJ, Krishnamurthy R, O'Mahony CA: Liver tumors: pediatric population. Liver Transpl; 2008 Nov;14(11):1545-56
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  • [Title] Liver tumors: pediatric population.
  • Liver tumors in childhood are rare and are typically not detected clinically until they reach a large size and often spread within the organ or metastasize.
  • With very effective chemotherapy for hepatoblastoma and to some extent for sarcomas, many cancers can be shrunk to permit partial hepatectomy, but for most hepatocarcinomas, some of the other malignancies, and even some benign proliferations, their location at the hilum and multiplicity of masses in multiple lobes make transplantation the treatment of choice.
  • Major advances in diagnostic imaging, especially enhanced computed tomography and magnetic resonance imaging, permit a preoperative choice of resection versus transplantation to be achieved in almost all instances, and for the remainder, intraoperative ultrasonography can further help to determine the most desirable approach.
  • In this review, transplantation for liver tumors in children is considered from all aspects, including the importance of screening for tumors whenever possible to avoid the need for transplantation.
  • [MeSH-major] Adenoma / therapy. Carcinoma / therapy. Liver Neoplasms / therapy. Liver Transplantation / methods. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Agents / pharmacology. Child. Child, Preschool. Female. Humans. Infant. Liver / pathology. Male. Medical Oncology / methods. Pediatrics / methods

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  • (PMID = 18975283.001).
  • [ISSN] 1527-6473
  • [Journal-full-title] Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • [ISO-abbreviation] Liver Transpl.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 78
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8. Pham TH, Iqbal CW, Grams JM, Zarroug AE, Wall JC, Ishitani MB, Nagorney DM, Moir C: Outcomes of primary liver cancer in children: an appraisal of experience. J Pediatr Surg; 2007 May;42(5):834-9
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  • [Title] Outcomes of primary liver cancer in children: an appraisal of experience.
  • INTRODUCTION: Hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common primary liver cancers in children.
  • Recent advances in management of pediatric liver cancer have improved disease-specific survival (DSS).
  • This is a review of our experience with childhood liver malignancy over the past 3 decades.
  • MATERIALS AND METHODS: A retrospective chart review from 1975 to 2005 identified patients who were 18 years old or younger with a histologically confirmed diagnosis of primary liver cancer.
  • RESULTS: Fifty-two patients were confirmed to have primary liver cancers, where 24 (46%) patients had HB, 22 (42%) had HCC, 3 (6%) had sarcomas, and 3 (6%) had other histologies.
  • Most patients underwent major liver resection (n = 45, 87%), including: lobectomy (n = 25, 48%), and trisegmentectomy (n = 11, 21%).
  • Three patients underwent liver transplantation (n = 3, 6%) for advanced local disease.
  • Forty-five (87%) received primary or neoadjuvant and/or adjuvant chemotherapy.
  • CONCLUSION: Complete resection of the pediatric primary liver tumors remains the cornerstone of treatment to achieve cure.
  • Major liver resection can be performed with minimal perioperative mortality and morbidity.
  • Liver transplantation in conjunction with chemotherapy may have an increasing role in the management of locally advanced primary liver cancers.
  • [MeSH-major] Carcinoma, Hepatocellular / therapy. Hepatoblastoma / therapy. Liver Neoplasms / therapy
  • [MeSH-minor] Adolescent. Analysis of Variance. Child. Child, Preschool. Combined Modality Therapy. Hepatectomy. Humans. Liver Transplantation. Registries. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 17502194.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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9. Berretta M, Rupolo M, Buonadonna A, Canzonieri V, Brollo A, Morra A, Berretta S, Bearz A, Tirelli U, Frustaci S: Metastatic angiosarcoma of the kidney: a case report with treatment approach and review of the literature. J Chemother; 2006 Apr;18(2):221-4
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  • [Title] Metastatic angiosarcoma of the kidney: a case report with treatment approach and review of the literature.
  • Angiosarcomas are rare soft tissue malignancies.
  • Typically they originate from the skin of the scalp or face, whereas visceral sarcomas are very rare.
  • After surgical removal, a rapid peritoneal, visceral and cutaneous diffusion developed.
  • Palliative chemotherapy, based on anthracycline and ifosfamide, which are normally used to treat all other high-grade spindle cell sarcomas, was totally inactive.
  • On the basis of these results and of the biological characteristics of these rare neoplasms it is mandatory to develop other therapeutic approaches.
  • Antiangiogenetic agents are of interest for this disease due to the peculiar origin of the cells of these sarcomas.
  • [MeSH-major] Hemangiosarcoma / drug therapy. Hemangiosarcoma / surgery. Kidney Neoplasms / drug therapy. Kidney Neoplasms / surgery
  • [MeSH-minor] Aged. Anthracyclines / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Fatal Outcome. Humans. Ifosfamide / administration & dosage. Liver Neoplasms / secondary. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Male. Nephrectomy. Palliative Care. Peritoneal Neoplasms / drug therapy. Peritoneal Neoplasms / secondary. Splenic Neoplasms / drug therapy. Splenic Neoplasms / secondary. Treatment Outcome

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  • (PMID = 16736893.001).
  • [ISSN] 1120-009X
  • [Journal-full-title] Journal of chemotherapy (Florence, Italy)
  • [ISO-abbreviation] J Chemother
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Anthracyclines; UM20QQM95Y / Ifosfamide
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10. Bergman L, Beelen ML, Gallee MP, Hollema H, Benraadt J, van Leeuwen FE: Risk and prognosis of endometrial cancer after tamoxifen for breast cancer. Comprehensive Cancer Centres' ALERT Group. Assessment of Liver and Endometrial cancer Risk following Tamoxifen. Lancet; 2000 Sep 9;356(9233):881-7
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  • [Title] Risk and prognosis of endometrial cancer after tamoxifen for breast cancer. Comprehensive Cancer Centres' ALERT Group. Assessment of Liver and Endometrial cancer Risk following Tamoxifen.
  • Information on tamoxifen use and other risk factors for endometrial cancer was obtained from 309 women with endometrial cancer after breast cancer (cases), and 860 matched controls with breast cancer but without endometrial cancer.
  • For 276 cases, we obtained tissue blocks of endometrial cancer to review the diagnosis, and used immunohistochemistry to examine hormone-receptor status and overexpression of p53.
  • Long-term users were more likely than non-users to have had malignant mixed mesodermal tumours or sarcomas of the endometrium (15.4% vs 2.9%, p < or = 0.02), p53-positive tumours (31.4% vs 18.2%, p=0.05), and negative oestrogen-receptor concentrations (60.8% vs 26.2%, p < or = 0.001).
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Breast Neoplasms / drug therapy. Endometrial Neoplasms / chemically induced. Estrogen Antagonists / therapeutic use. Tamoxifen / therapeutic use
  • [MeSH-minor] Aged. Case-Control Studies. Female. Gene Expression Regulation, Neoplastic. Humans. Middle Aged. Neoplasm Staging. Prognosis. Proportional Hazards Models. Receptors, Estrogen / analysis. Risk Assessment. Risk Factors. Survival Rate. Time Factors. Tumor Suppressor Protein p53 / genetics

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  • [CommentIn] Lancet. 2001 Jan 6;357(9249):65-6; author reply 67 [11197376.001]
  • [CommentIn] Lancet. 2001 Jan 6;357(9249):66-7 [11197379.001]
  • [CommentIn] Lancet. 2001 Jan 6;357(9249):68 [11197381.001]
  • [CommentIn] Lancet. 2001 Jan 6;357(9249):68 [11197382.001]
  • [CommentIn] Lancet. 2000 Sep 9;356(9233):868-9 [11036885.001]
  • [CommentIn] Lancet. 2001 Jan 6;357(9249):67-8 [11197380.001]
  • (PMID = 11036892.001).
  • [ISSN] 0140-6736
  • [Journal-full-title] Lancet (London, England)
  • [ISO-abbreviation] Lancet
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Estrogen Antagonists; 0 / Receptors, Estrogen; 0 / Tumor Suppressor Protein p53; 094ZI81Y45 / Tamoxifen
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11. Losa R, Fra J, López-Pousa A, Sierra M, Goitia A, Uña E, Nadal R, Del Muro JG, Gión M, Maurel J, Escudero P, Esteban E, Buesa JM: Phase II study with the combination of gemcitabine and DTIC in patients with advanced soft tissue sarcomas. Cancer Chemother Pharmacol; 2007 Feb;59(2):251-9
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  • [Title] Phase II study with the combination of gemcitabine and DTIC in patients with advanced soft tissue sarcomas.
  • PURPOSE: Based on the promising results of a Phase I study with a combination of gemcitabine and DTIC performed in advanced soft tissue sarcoma (ASTS) patients, and due to the limited efficacy of second or third line therapies in those patients, we designed a Phase II study to determine the activity of this new regimen.
  • METHODS: Patients with ASTS, measurable disease, pretreated with chemotherapy, received gemcitabine 1,800 mg/m2 infused over 180 min followed by DTIC 500 mg/m2 (one cycle), every 2 weeks.
  • The influence of the sequence of administration on those parameters was examined to exclude potential drug interactions.
  • It should be compared with DTIC, or other gemcitabine-containing combinations, in patients with LMS or MFH, to determine whether this combination offers advantages in PFR or in overall activity.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Sarcoma / drug therapy
  • [MeSH-minor] Adult. Aged. Alanine Transaminase / blood. Area Under Curve. Aspartate Aminotransferases / blood. Dacarbazine / administration & dosage. Dacarbazine / adverse effects. Dacarbazine / analogs & derivatives. Dacarbazine / pharmacokinetics. Deoxycytidine / administration & dosage. Deoxycytidine / adverse effects. Deoxycytidine / analogs & derivatives. Deoxycytidine / pharmacokinetics. Disease Progression. Disease-Free Survival. Doxorubicin / administration & dosage. Doxorubicin / adverse effects. Doxorubicin / pharmacokinetics. Female. Hematologic Diseases / chemically induced. Humans. Ifosfamide / administration & dosage. Ifosfamide / adverse effects. Ifosfamide / pharmacokinetics. Infusions, Intravenous. Liver / drug effects. Liver / enzymology. Lung / drug effects. Lung / pathology. Male. Middle Aged. Remission Induction. Tomography, X-Ray Computed / methods. Treatment Outcome

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  • (PMID = 16736150.001).
  • [ISSN] 0344-5704
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0W860991D6 / Deoxycytidine; 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; 85622-93-1 / temozolomide; B76N6SBZ8R / gemcitabine; EC 2.6.1.1 / Aspartate Aminotransferases; EC 2.6.1.2 / Alanine Transaminase; UM20QQM95Y / Ifosfamide
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12. Kravtsov VG, Zaĭrat'iants OV: [Clinical and morphological characteristics of gastrointestinal stromal tumors]. Arkh Patol; 2007 Sep-Oct;69(5):54-61
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  • GIST are stromal tumors and the gastrointestinal tract (GIT) and some other organs of spindle-cell or epithelioid-cell structure expressing CD117 (C-kit, KIT), as well as those at different rates and in different combinations, CD34, smooth muscle and/or neurogenic differentiation antigens.
  • It should be taken into account that CD117 are also expressed by melanomas, vascular, and some other tumors.
  • This is supported by successful chemotherapy for GIST with a KIT receptor inhibitor.
  • Many GISTs behave like sarcomas and they are characterized by an infiltrating growth, hematogenic (mainly into the liver) and implantational (along the peritoneum) cancer spread.
  • [MeSH-minor] Antigens, CD34 / biosynthesis. Antigens, CD34 / genetics. Apoptosis / genetics. Enzyme Activation / genetics. Epithelioid Cells / metabolism. Epithelioid Cells / pathology. Gene Expression Regulation, Neoplastic / genetics. Humans. Liver Neoplasms / drug therapy. Liver Neoplasms / genetics. Liver Neoplasms / metabolism. Liver Neoplasms / pathology. Liver Neoplasms / secondary. Melanoma / genetics. Melanoma / metabolism. Melanoma / pathology. Mitosis / genetics. Mutation. Myocytes, Smooth Muscle / metabolism. Myocytes, Smooth Muscle / pathology. Neoplasm Metastasis. Neoplastic Stem Cells / metabolism. Neoplastic Stem Cells / pathology. Peritoneal Neoplasms / drug therapy. Peritoneal Neoplasms / genetics. Peritoneal Neoplasms / pathology. Peritoneal Neoplasms / secondary. Sarcoma / drug therapy. Sarcoma / genetics. Sarcoma / metabolism. Sarcoma / pathology

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  • (PMID = 18074824.001).
  • [ISSN] 0004-1955
  • [Journal-full-title] Arkhiv patologii
  • [ISO-abbreviation] Arkh. Patol.
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Russia (Federation)
  • [Chemical-registry-number] 0 / Antigens, CD34; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
  • [Number-of-references] 44
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13. Steinert DM, Blakely LJ, Patel SR, Burgess MA, Chen LL, Trent JC, Raymond AK, Benjamin RS: Outcomes of gastrointestinal stromal tumors (GIST) and other intra-abdominal sarcomas (IAS) in the era of imatinib therapy. J Clin Oncol; 2004 Jul 15;22(14_suppl):9047

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  • [Title] Outcomes of gastrointestinal stromal tumors (GIST) and other intra-abdominal sarcomas (IAS) in the era of imatinib therapy.
  • In the era of kit immunohistochemistry and imatinib mesylate therapy the outcome of IASs is unknown.
  • Patients diagnosed with GIST were treated with imatinib mesylate, and patients diagnosed with other IAS were treated with standard sarcoma chemotherapy.
  • A single sarcoma pathologist reviewed all patients' tumor blocks.
  • RESULTS: Of 268 patients, 4 patients were excluded because of diagnoses other than sarcoma.
  • Another 46 patients were excluded because no data were available at the time of this abstract.
  • Of the remaining 218 patients, 159 (72.9%) were GIST and 59 (27.1%) were IAS specifically: 31 leiomyosarcoma, 10 spindle cell tumors, 4 unclassified sarcomas, and 14 other types of sarcoma.
  • The median age of patients diagnosed with GIST was 54 (25 -90) and was 52 (20 -87) for patients diagnosed with other IAS.
  • The most common primary tumor sites for patients with GIST were stomach (37.1%), small bowel (34%), and colon (6.3%); whereas, patients with other intra-abdominal sarcomas occurred in the retroperitoneum (25.4%), abdominal viscera (18.6%), and pelvis (11.9%).
  • The most common metastatic sites seen in patients with GIST were liver (68.6%) and peritoneum (51.6%); whereas other IASs metastasized to the liver (39%) and peritoneum (30.5%).
  • While median survival from the time of diagnosis has not been reached in patients with GIST, in other IAS median survival is 63.8 months.
  • Time to progression in patients with GIST was 16.4 months after imatinib and 5.1 months in patients with IAS treated with standard sarcoma chemotherapy.
  • The 5 year survival rate for GIST is 0.80 (95%CI 0.74,0.86), while for other IAS it is 0.53 (95% CI 0.41,0.67).
  • CONCLUSIONS: Survival and time to progression are worse for IAS compared to GISTs.
  • New therapies for these tumors are needed.

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  • (PMID = 28014121.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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14. Michael H, Lucia J, Foster RS, Ulbright TM: The pathology of late recurrence of testicular germ cell tumors. Am J Surg Pathol; 2000 Feb;24(2):257-73
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  • A total of 91 men had histologically documented late recurrences of testicular germ cell tumors characterized by a complete response to treatment with a subsequent disease-free interval of at least 2 years and no evidence of a second primary lesion.
  • Ninety percent of the patients for whom information was available received chemotherapy shortly after their initial diagnosis of testicular germ cell tumors; most of the other patients were known to have stage I disease initially.
  • Thus, teratoma was the most common type of neoplasm in late recurrences.
  • Excluding teratoma coexisting with other types of neoplasms, yolk sac tumor was the most frequent type of tumor in patients with late recurrence.
  • It occurred in 47% of patients, either alone or with teratoma, another nonteratomatous germ cell tumor type, or a "nongerm cell malignant tumor."
  • Unusual types of yolk sac tumor, including glandular, parietal, clear cell, and pleomorphic patterns, were seen frequently in late recurrences and often raised differential diagnostic problems with "nongerm cell" carcinomas.
  • A smaller number of late recurrences consisted of other types of neoplasms.
  • Twenty percent of patients with late recurrence had a nonteratomatous germ cell tumor other than yolk sac tumor, either alone, with yolk sac tumor, or with a "nongerm cell malignant tumor."
  • Most of these nonteratomatous germ cell tumors other than yolk sac tumor were embryonal carcinoma, although rarely seminoma and choriocarcinoma were encountered.
  • "Nongerm cell malignant tumors," including both sarcomas and carcinomas of various types, occurred in 23% of late-recurrence patients, either alone or with a nonteratomatous germ cell tumor.
  • Late recurrences were seen in many different sites in these patients, including the retroperitoneum, abdomen, pelvis, liver, mediastinum, lung, bone (femur, vertebra, and rib), lymph nodes outside the retroperitoneum and mediastinum (supraclavicular, neck, and axillary regions), scrotum and inguinal regions, adrenal gland, chest wall, and buttocks.
  • Patients whose late recurrences consisted of pure "nongerm cell malignant tumor" or pure germ cell tumor (yolk sac tumor or other types) had a much worse prognosis: Only 36% to 37% were alive with no evidence of disease.
  • Patients with two different types of nonteratomatous malignancies in their late recurrences had a dismal clinical course: Only 17% with both yolk sac tumor and other nonteratomatous germ cell tumor had no evidence of disease, whereas no patient with both nonteratomatous germ cell tumor and "nongerm cell malignant tumor" was disease free.
  • Late recurrences consisting of teratoma alone often have a favorable outcome, but the prognosis in all other patients is poor.
  • Furthermore, late recurrence is not likely to respond to chemotherapy and is best treated by surgical excision when possible.
  • [MeSH-minor] Adolescent. Adult. Carcinoma, Embryonal / complications. Carcinoma, Embryonal / pathology. Carcinoma, Embryonal / therapy. Choriocarcinoma / complications. Choriocarcinoma / pathology. Choriocarcinoma / therapy. Endodermal Sinus Tumor / complications. Endodermal Sinus Tumor / pathology. Endodermal Sinus Tumor / therapy. Fluorescent Antibody Technique, Direct. Humans. Male. Neoplasm Staging. Neoplasms, Second Primary / pathology. Neoplasms, Second Primary / therapy. Sarcoma / complications. Sarcoma / pathology. Sarcoma / therapy. Seminoma / complications. Seminoma / pathology. Seminoma / therapy. Teratoma / complications. Teratoma / pathology. Teratoma / therapy

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  • (PMID = 10680894.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
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15. Carvalho C, Santos RX, Cardoso S, Correia S, Oliveira PJ, Santos MS, Moreira PI: Doxorubicin: the good, the bad and the ugly effect. Curr Med Chem; 2009;16(25):3267-85
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  • The anthracycline doxorubicin (DOX) is widely used in chemotherapy due to its efficacy in fighting a wide range of cancers such as carcinomas, sarcomas and hematological cancers.
  • A growing body of evidence supports the view that this drug can be a double-edge sword.
  • Indeed, injury to nontargeted tissues often complicates cancer treatment by limiting therapeutic dosages of DOX and diminishing the quality of patients' life during and after DOX treatment.
  • However, this anticancer drug also affects other organs like the brain, kidney and liver.
  • Additionally, clinical studies focusing the therapeutic efficacy and side effects of DOX treatment will be discussed.
  • [MeSH-major] Antibiotics, Antineoplastic / adverse effects. Antibiotics, Antineoplastic / therapeutic use. Doxorubicin / adverse effects. Doxorubicin / therapeutic use. Neoplasms / drug therapy

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  • (PMID = 19548866.001).
  • [ISSN] 1875-533X
  • [Journal-full-title] Current medicinal chemistry
  • [ISO-abbreviation] Curr. Med. Chem.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 80168379AG / Doxorubicin
  • [Number-of-references] 253
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16. Vasanawala MS, Wang Y, Quon A, Gambhir SS: F-18 fluorodeoxyglucose PET/CT as an imaging tool for staging and restaging cutaneous angiosarcoma of the scalp. Clin Nucl Med; 2006 Sep;31(9):534-7
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  • Distant metastases favor lung, liver, lymph nodes, and skin.
  • It differs from other soft tissue sarcomas in that the size of the lesion at presentation instead of tumor grade is the important prognostic factor.
  • Optimal treatment is yet to be determined.
  • Wide-margin complete excision with postoperative radiotherapy has been the most effective therapy.
  • Chemotherapy and gene therapy have been used with some success.
  • PET/CT imaging after chemotherapy and before radiation therapy showed complete resolution of FDG uptake in the scalp and osseous lesions.
  • Evaluation of more cases of this subset of soft tissue sarcoma with FDG PET/CT may suggest a possible role in not only staging angiosarcomas to determine the extent of local as well as distant disease, but also to potentially help determine response to therapy and early recognition of local or distant recurrence.
  • [MeSH-major] Fluorodeoxyglucose F18. Hemangiosarcoma / diagnosis. Hemangiosarcoma / radionuclide imaging. Neoplasm Staging / methods. Positron-Emission Tomography / methods. Radiopharmaceuticals. Scalp / pathology. Skin Neoplasms / diagnosis. Skin Neoplasms / radionuclide imaging. Tomography, X-Ray Computed / methods

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  • (PMID = 16921276.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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17. McClain KL, Natkunam Y, Swerdlow SH: Atypical cellular disorders. Hematology Am Soc Hematol Educ Program; 2004;:283-96
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  • The atypical cellular disorders discussed here all bear some similarities in that abnormal proliferations of lymphocytes and macrophages or dendritic cells result in lymphadenopathy, skin rashes, bone lesions and infiltrations of nearly any other organ system.
  • Kenneth McClain examines the unanswered question of whether LCH is a malignant clonal disorder or an inflammatory response triggered by aberrant cytokine expression or a virus.
  • Advocates of the malignant proliferation theory rest their case primarily on the following two points: Clonality of the CD1a+ Langerhans cells was demonstrated by analysis of the human androgen receptor in patients with single bone lesions (Low Risk) or multisystem disease including spleen, liver, bone marrow, or lung (High Risk).
  • While the basic scientific arguments continue, important advances in the treatment of LCH have been made by international collaborations of the Histiocyte Society.
  • Risk groups have been clearly defined and the response to therapy after the initial 6 weeks is known to be the strongest prognostic variable for outcome.
  • Therapy for SHML varies greatly in reported case series.
  • Others with systemic involvement may benefit from chemotherapy, but no clinical trials have been done.
  • Steven Swerdlow clarifies key features of the four types of CD.
  • Localized cases are divided into the hyaline vascular type and plasma cell type.
  • The two types are distinguished largely by the nature of the follicles and the number of interfollicular plasma cells.
  • Interleukin (IL)-6 expression is increased in the plasma cell type.
  • Multicentric CD of the plasmablastic type is most often found in HIV-positive patients with coincident HHV-8 infection.
  • Many have lymphomas or Kaposi sarcomas.
  • Other cases of multicentric CD are also most like the plasma cell type, however, with disseminated disease and constitutional symptoms.
  • A wide variety of anti-neoplastic drugs, radiation therapy, anti-IL-6 and rituximab or atlizumab have been used with varying success in patients with multicentric CD.

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  • (PMID = 15561688.001).
  • [ISSN] 1520-4391
  • [Journal-full-title] Hematology. American Society of Hematology. Education Program
  • [ISO-abbreviation] Hematology Am Soc Hematol Educ Program
  • [Language] ENG
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 77
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18. Várady E, Deák B, Molnár ZS, Rosta A, Schneider T, Esik O, Eckhardt S: Second malignancies after treatment for Hodgkin's disease. Leuk Lymphoma; 2001 Nov-Dec;42(6):1275-81
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  • [Title] Second malignancies after treatment for Hodgkin's disease.
  • The occurrence of treatment-related second malignancy following Hodgkin's disease (HD) has now been recognized as a major problem.
  • Second neoplasm developed in 32 cases (4.8%).
  • Five patients received chemo- and radiotherapy and in two cases chemotherapy was used.
  • Twenty-five patients have had solid tumors, affecting lung (5), breast (3), colon (3), stomach (2), urinary bladder (2), head-and-neck (1), thyroid gland (1), esophagus (1), liver (1), pancreas (1), furthermore, three sarcomas and two malignant melanomas were observed.
  • Chemotherapy was applied to nine patients, 16 patients received both chemo- and radiotherapy.
  • Since alkylating agents increase the risk of leukemia and irradiation contributes mainly to other malignancies, future treatment protocols should attempt to reduce the most serious consequence of therapy without compromising the survival.
  • [MeSH-major] Hodgkin Disease / therapy. Neoplasms, Second Primary / epidemiology
  • [MeSH-minor] Adult. Aged. Antineoplastic Agents / adverse effects. Female. Humans. Male. Middle Aged. Radiotherapy / adverse effects. Time Factors

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  • (PMID = 11911408.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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19. Poggio JL, Nagorney DM, Nascimento AG, Rowland C, Kay P, Young RM, Donohue JH: Surgical treatment of adult primary hepatic sarcoma. Br J Surg; 2000 Nov;87(11):1500-5
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  • [Title] Surgical treatment of adult primary hepatic sarcoma.
  • BACKGROUND: Primary sarcomas of the liver are extremely rare in adults.
  • Optimal therapeutic approaches remain unclear.
  • METHODS: Twenty consecutive adult patients who had surgical treatment for primary hepatic sarcomas were reviewed.
  • Other than one patient with primary hepatic angiosarcoma who had a history of thorium dioxide colloid (Thorotrast) exposure 23 years before diagnosis, no predisposing causes were apparent.
  • Nineteen patients had hepatic resection and one patient had an orthotopic liver transplant.
  • No patient received neoadjuvant chemotherapy or radiotherapy but radiotherapy was delivered intraoperatively in one patient.
  • RESULTS: Leiomyosarcoma was the most common histological type of sarcoma diagnosed (five of 20 patients), followed by malignant solitary fibrous tumour (four) and epithelioid haemangioendothelioma (three).
  • Fourteen tumours were high-grade sarcomas and six were low grade malignancies.
  • Thirteen patients developed a recurrence.
  • Distant metastases (ten patients) and intrahepatic recurrence (six) were the predominant sites of initial treatment failure.
  • Six patients received salvage chemotherapy.
  • CONCLUSION: Surgical resection is the only effective therapy for primary hepatic sarcomas at present.
  • Better adjuvant therapy is necessary, especially for high-grade malignancies, owing to the high failure rate with operation alone.
  • [MeSH-major] Liver Neoplasms / surgery. Sarcoma / surgery
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Preoperative Care / methods. Retrospective Studies

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  • (PMID = 11091236.001).
  • [ISSN] 0007-1323
  • [Journal-full-title] The British journal of surgery
  • [ISO-abbreviation] Br J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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20. Blanchard DK, Budde JM, Hatch GF 3rd, Wertheimer-Hatch L, Hatch KF, Davis GB, Foster RS Jr, Skandalakis JE: Tumors of the small intestine. World J Surg; 2000 Apr;24(4):421-9

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  • Computed tomography was found to detect LM and LMS most successfully and had the additional advantage of locating metastatic disease.
  • The overall rate of metastatic spread of LMS ranged from 24% to 50%, with the liver being most commonly involved.
  • Unlike other sarcomas, both hematogenous and lymphatic spread were common.
  • For both benign and malignant smooth muscle tumors of the small intestine, surgery remains the treatment of choice, with little efficacy reported for irradiation, chemotherapy, or both.
  • [MeSH-minor] Age Factors. Female. Gastrointestinal Hemorrhage / physiopathology. Humans. Incidence. Lymphatic Metastasis. Male. Middle Aged. Sex Factors. Survival Rate. Tomography, X-Ray Computed

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  • (PMID = 10706914.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 60
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21. Perek D, Brozyna A, Dembowska-Baginska B, Stypinska M, Sojka M, Bacewicz L, Polnik D, Kalicinski P: [Tumours in newborns and infants up to three months of life. One institution experience]. Med Wieku Rozwoj; 2006 Jul-Sep;10(3 Pt 1):711-23
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  • INTRODUCTION: Newborns and infants up to three months of life are a specific group of population in paediatric oncology due to immaturity of tissues and organs and rarity of neoplastic diseases in this group of patients (pts).
  • There are no strict therapeutic procedures established for these children.
  • THE AIM of our study was to examine distribution of tumours in newborns and infants up to 3 months of age treated in our institution and to present our own experience in the treatment of these patients.
  • Distribution of tumour types in newborns and babies from 1 to 3 months of age was analyzed separately.
  • Due to similar growth pattern, response to treatment and it's side effects in newborns and small infants, treatment results were evaluated for the whole group.
  • There were also 3 cases of soft tissue sarcomas (STS), 2 central nervous system tumours (CNS), 2 retinoblastoma (RB), 2 hepatoblastoma (HB).
  • Eleven pts underwent combined treatment of chemotherapy and surgery: 5 with stage III and IV NBL, 6 with other tumours.
  • Four pts are alive, 4 with NBL and 5 with other tumours.
  • Chemotherapy alone was administered to 7 pts in whom local advancement of disease enabled surgery and to pts with RBL.
  • Four pts with NBL (2 stage IV and 2 stage IVS) were treated with irradiation to the liver only.
  • One pt, critically ill, died before any treatment.
  • Fourteen pts died (20%), 8 from disease and 6 of other reasons.
  • 2. Newborns and small infants with advanced neoplastic disease, similarly to older children can be cured with chemotherapy.
  • 3. Individual approach is warranted in newborns and small infants and treatment should be carried out in specialized centres.
  • 4. All patients who completed treatment of any tumour type should be followed up by a pediatric oncologist.
  • [MeSH-major] Infant Welfare / statistics & numerical data. Neoplasms / epidemiology. Neoplasms / therapy

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  • (PMID = 17317902.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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22. Puchalski TA, Ryan DP, Garcia-Carbonero R, Demetri GD, Butkiewicz L, Harmon D, Seiden MV, Maki RG, Lopez-Lazaro L, Jimeno J, Guzman C, Supko JG: Pharmacokinetics of ecteinascidin 743 administered as a 24-h continuous intravenous infusion to adult patients with soft tissue sarcomas: associations with clinical characteristics, pathophysiological variables and toxicity. Cancer Chemother Pharmacol; 2002 Oct;50(4):309-19
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  • [Title] Pharmacokinetics of ecteinascidin 743 administered as a 24-h continuous intravenous infusion to adult patients with soft tissue sarcomas: associations with clinical characteristics, pathophysiological variables and toxicity.
  • In the study reported here, the influence of clinical characteristics and pretreatment pathophysiological variables on the pharmacokinetics of ET-743 and their associations with drug-related toxicity was examined in sarcoma patients treated in three phase II clinical trials.
  • METHODS: Adult patients with various histological subtypes of soft tissue sarcoma received 1.5 mg/m(2) of ET-743 by 24-h continuous i.v. infusion once every 3 weeks.
  • Eligibility criteria were similar for each study, except for the histological subtype of the tumor or the extent of prior treatment with other anticancer agents, and all patients had normal or near-normal liver and renal function.
  • The maximum plasma concentration (C(max)) and area under the plasma profile from time zero to infinity (AUC) of the drug were determined during the first cycle of therapy.
  • The 15 patients with any baseline liver function test exceeding the upper limit of the normal ranges had a significantly greater (P=0.02) incidence of severe toxicity (80% vs 44%).
  • Although the mean AUC of ET-743 in patients with elevated serum levels of hepatic enzymes was 17% greater than that in patients with normal pretreatment liver function tests, the difference was not significant ( P=0.22).
  • In addition, there was no distinct relationship between the grade of the most severe drug-related toxicity that occurred during the first cycle of therapy and the AUC for the entire cohort.
  • There were no significant associations between CL (liters per hour) and body surface area or any other variable related to body size.
  • Dexamethasone cotreatment appeared to decrease the incidence of severe toxicity as well as the AUC of the drug.
  • Delivering a fixed amount of drug without adjustment for the height or weight of the patient may be more appropriate than dose normalization due to the absence of an association between CL and body surface area.
  • Optimizing dosing strategies to further enhance the therapeutic index of ET-743 may depend upon obtaining a better understanding of the metabolic fate of the drug in humans.
  • [MeSH-major] Antineoplastic Agents / pharmacokinetics. Dioxoles / pharmacokinetics. Isoquinolines / pharmacokinetics. Sarcoma / drug therapy
  • [MeSH-minor] Adult. Dexamethasone / pharmacology. Humans. Infusions, Intravenous. Liver Function Tests. Liver Neoplasms / secondary. Male. Middle Aged. Neutropenia / chemically induced. Tetrahydroisoquinolines. Transaminases / blood

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  • (PMID = 12357306.001).
  • [ISSN] 0344-5704
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Dioxoles; 0 / Isoquinolines; 0 / Tetrahydroisoquinolines; 114899-77-3 / trabectedin; 7S5I7G3JQL / Dexamethasone; EC 2.6.1.- / Transaminases
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23. Casali PG, Sanfilippo R, D'Incalci M: Trabectedin therapy for sarcomas. Curr Opin Oncol; 2010 Jul;22(4):342-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Trabectedin therapy for sarcomas.
  • PURPOSE OF REVIEW: The therapeutic armamentarium of adult soft-tissue sarcomas (STS) has widened in recent years.
  • RECENT FINDINGS: Trabectedin has proven efficacy in STS, mainly in leiomyosarcomas, liposarcomas, and other translocation-related sarcomas.
  • Occasional major myelosuppression is possible but proper patient selection (with a focus on liver tests) and possibly steroid premedication are of help.
  • SUMMARY: Trabectedin is a new marine-derived drug with a definite role in the 'histology-driven' medical therapy of STS.
  • [MeSH-major] Dioxoles / therapeutic use. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy. Tetrahydroisoquinolines / therapeutic use
  • [MeSH-minor] Adult. Antineoplastic Agents, Alkylating / therapeutic use. Disease-Free Survival. Humans. Treatment Outcome

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  • (PMID = 20489618.001).
  • [ISSN] 1531-703X
  • [Journal-full-title] Current opinion in oncology
  • [ISO-abbreviation] Curr Opin 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, Alkylating; 0 / Dioxoles; 0 / Tetrahydroisoquinolines; 114899-77-3 / trabectedin
  • [Number-of-references] 43
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24. Tannuri AC, Tannuri U, Gibelli NE, Romão RL: Surgical treatment of hepatic tumors in children: lessons learned from liver transplantation. J Pediatr Surg; 2009 Nov;44(11):2083-7
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  • [Title] Surgical treatment of hepatic tumors in children: lessons learned from liver transplantation.
  • The objective of the present study was to describe our experience in liver resections, in the light of liver transplantation, emphasizing the indications for surgery, surgical techniques, complications, and results.
  • METHODS: The medical records of 53 children who underwent liver resection for primary or metastatic hepatic tumors were reviewed.
  • After neoadjuvant chemotherapy, tumor resectability was evaluated by another CT scan.
  • Surgery was performed by surgeons competent in liver transplantation.
  • As in liver living donor operation, vascular anomalies were investigated.
  • Hilar structures were dissected very close to liver parenchyma.
  • The hepatic artery and portal vein were dissected and ligated near their entrance to the liver parenchyma to avoid damaging the hilar vessels of the other lobe.
  • RESULTS: Fifty-three children with hepatic tumors underwent surgical treatment, 47 patients underwent liver resections, and in 6 cases, liver transplantation was performed because the tumor was considered unresectable.
  • Ten children presented with other malignant tumors-3 undifferentiated sarcomas, 2 hepatocellular carcinomas, 2 fibrolamellar hepatocellular carcinomas, a rhabdomyosarcoma, an immature ovarian teratoma, and a single neuroblastoma.
  • The mortality rate of hepatoblastoma patients was less than other malignant tumors (P = .04).
  • CONCLUSION: The resection of hepatic tumors in children requires expertise in pediatric surgical practice, and many lessons learned from liver transplantation can be applied to hepatectomies.
  • [MeSH-major] Liver Neoplasms / surgery. Liver Transplantation / methods
  • [MeSH-minor] Age Factors. Blood Loss, Surgical. Carcinoma, Hepatocellular / mortality. Carcinoma, Hepatocellular / surgery. Follow-Up Studies. Hepatectomy / methods. Hepatoblastoma / mortality. Hepatoblastoma / surgery. Humans. Infant. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / surgery. Postoperative Complications / etiology. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 19944212.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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25. Sleijfer S, Ray-Coquard I, Papai Z, Le Cesne A, Scurr M, Schöffski P, Collin F, Pandite L, Marreaud S, De Brauwer A, van Glabbeke M, Verweij J, Blay JY: Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer-soft tissue and bone sarcoma group (EORTC study 62043). J Clin Oncol; 2009 Jul 1;27(19):3126-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer-soft tissue and bone sarcoma group (EORTC study 62043).
  • PURPOSE Given the importance of angiogenesis in soft tissue sarcoma (STS), pazopanib, an oral angiogenesis inhibitor that targets vascular endothelial growth factor receptor and platelet-derived growth factor receptor, was explored in patients with advanced STS.
  • PATIENTS AND METHODS Patients with intermediate- or high-grade advanced STS who were ineligible for chemotherapy or who had received no more than two prior cytotoxic agents for advanced disease, who had documented progression, who had adequate performance status, and who had good organ function were eligible.
  • Four different strata were studied: adipocytic STS, leiomyosarcomas, synovial sarcomas, and other STS types.
  • PFR(12 weeks) was 18 (44%) of 41 patients in the leiomyosarcoma cohort, 18 (49%) of 37 in the synovial sarcomas, and 16 (39%) of 41 in the other STS types.
  • Compared with historical controls who were treated with second-line chemotherapy, progression-free and overall survivals were prolonged in the three cohorts in which the primary end point was reached.
  • The most frequent drug-related toxicities were hypertension, fatigue, hypopigmentation, and nausea.
  • Other toxicities included liver enzyme elevations, myelosuppression, and proteinuria, all of which were mostly grades 1 to 2.
  • CONCLUSION Pazopanib is well tolerated in patients with relapsed, advanced STS and demonstrates interesting activity that warrants additional study in patients with leiomyosarcomas, synovial sarcomas, and other STS types.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Pyrimidines / therapeutic use. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy. Sulfonamides / therapeutic use
  • [MeSH-minor] Adult. Aged. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / drug therapy. Neoplasm Recurrence, Local / mortality

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  • (PMID = 19451427.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Pyrimidines; 0 / Sulfonamides; 7RN5DR86CK / pazopanib
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26. Schleyer V, Meyer S, Landthaler M, Szeimies RM: ["Smoldering systemic mastocytosis. "Successful therapy with cladribine]. Hautarzt; 2004 Jul;55(7):658-62
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  • [Title] ["Smoldering systemic mastocytosis. "Successful therapy with cladribine].
  • [Transliterated title] "Smouldering systemic mastocytosis". Erfolgreiche Therapie mit Cladribin.
  • Mastocytoses are a heterogenous group of diseases characterized by proliferation and accumulation of mast cells in the skin and other organs.
  • They are subdivided into cutaneous mastocytoses; systemic forms, which may appear with or without skin lesions; mast cell sarcomas and extracutaneous, localized, benign mastocytomas.
  • Systemic mastocytoses apart from the skin mainly involve bone marrow, gastrointestinal tract, bones, lymph nodes, spleen and liver.
  • Whereas indolent forms of systemic mastocytosis are mainly treated with antihistamines, glucocorticosteroids and PUVA therapy, the more aggressive forms, including mast cell leukemia, often require cytostatic chemotherapy.
  • Treatment with cladribine led to an impressive improvement of skin lesions, a significant decrease in tryptase serum levels and stabilization of bone marrow infiltrates.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Cladribine / therapeutic use. Immunosuppressive Agents / therapeutic use. Mastocytosis, Systemic / drug therapy
  • [MeSH-minor] Biopsy. Follow-Up Studies. Humans. Infusions, Intravenous. Male. Middle Aged. Skin / pathology. Treatment Outcome

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  • (PMID = 15241520.001).
  • [ISSN] 0017-8470
  • [Journal-full-title] Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
  • [ISO-abbreviation] Hautarzt
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Immunosuppressive Agents; 47M74X9YT5 / Cladribine
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27. Lauro VD, Spazzapan S, Lombardi D, Paolello C, Scuderi C, Crivellari D, Magri MD, Veronesi A: Fourteen-day infusion of ifosfamide in the management of advanced breast cancer refractory to protracted continuous infusion of 5-fluorouracil. Tumori; 2001 Jan-Feb;87(1):27-9
Hazardous Substances Data Bank. FLUOROURACIL .

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  • AIMS AND BACKGROUND: Ifosfamide is an active drug in advanced breast cancer.
  • The aim of the study was to evaluate in patients with advanced breast cancer a 14-day infusion schedule previously tested at our center in soft tissue sarcomas.
  • All patients were pretreated with anthracyclines or taxanes; the median number of chemotherapy lines in the metastatic phase was 2 (range, 1-4).
  • Predominant metastatic sites wen soft tissues in 5 patients, lung in 6, liver in 7 and serosal cavities in 3.
  • Other toxicities included nausea in 14 patients (grade 3 in 2) and grade 1-2 vomiting in 2 patients.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Breast Neoplasms / drug therapy. Ifosfamide / administration & dosage
  • [MeSH-minor] Adult. Aged. Antimetabolites, Antineoplastic / administration & dosage. Disease Progression. Drug Administration Schedule. Female. Fluorouracil / administration & dosage. Humans. Infusions, Intravenous. Middle Aged. Retrospective Studies. Treatment Failure. Treatment Outcome

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  • (PMID = 11669554.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / Antineoplastic Agents, Alkylating; U3P01618RT / Fluorouracil; UM20QQM95Y / Ifosfamide
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28. Christinat A, Leyvraz S: Role of trabectedin in the treatment of soft tissue sarcoma. Onco Targets Ther; 2009;2:105-13

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Role of trabectedin in the treatment of soft tissue sarcoma.
  • Interest in marine natural products has allowed the discovery of new drugs and trabectedin (ET-743, Yondelis), derived from the marine tunicate Ecteinascidia turbinata, was approved for clinical use in 2007.
  • In vitro antitumor activity was demonstrated against various cancer cell lines and soft tissue sarcoma cell lines.
  • In phase I studies tumor responses were observed also in osteosarcomas and different soft tissue sarcoma subtypes.
  • The most common toxicities were myelosuppression and transient elevation of liver function tests, which could be reduced by dexamethasone premedication.
  • Combination with other agents are currently studied with promising results.
  • In summary trabectedin is an active new chemotherapeutic agents that has demonstrated its role in the armamentarium of treatments for patients with sarcomas.

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  • (PMID = 20616899.001).
  • [ISSN] 1178-6930
  • [Journal-full-title] OncoTargets and therapy
  • [ISO-abbreviation] Onco Targets Ther
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] New Zealand
  • [Other-IDs] NLM/ PMC2886331
  • [Keywords] NOTNLM ; DNA-minor groove binder / chemotherapy / soft tissue sarcoma / trabectedin
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29. Hauck ML, LaRue SM, Petros WP, Poulson JM, Yu D, Spasojevic I, Pruitt AF, Klein A, Case B, Thrall DE, Needham D, Dewhirst MW: Phase I trial of doxorubicin-containing low temperature sensitive liposomes in spontaneous canine tumors. Clin Cancer Res; 2006 Jul 1;12(13):4004-10
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  • EXPERIMENTAL DESIGN: Privately owned dogs with solid tumors (carcinomas or sarcomas) were treated.
  • Three treatments, given 3 weeks apart, were scheduled.
  • Pharmacokinetics were evaluated during the first treatment cycle.
  • RESULTS: Twenty-one patients were enrolled: 18 with sarcomas and 3 with carcinomas.
  • Grade 4 neutropenia and acute death secondary to liver failure, possibly drug related, were the dose-limiting toxicities.
  • Other toxicities, with the possible exception of renal damage, were consistent with those observed following free doxorubicin administration.
  • Of the 20 dogs that received > or = 2 doses of LTSL-doxorubicin, 12 had stable disease, and 6 had a partial response to treatment.
  • Tumor drug concentrations at a dose of 1.0 mg/kg averaged 9.12 +/- 6.17 ng/mg tissue.
  • CONCLUSION: LTSL-doxorubicin offers a novel approach to improving drug delivery to solid tumors.

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  • (PMID = 16818699.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA042745; United States / NCI NIH HHS / CA / P01CA42745
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Liposomes; 80168379AG / Doxorubicin
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30. Fiorentini G, Rossi S, Lanzanova G, Biancalani M, Palomba A, Bernardeschi P, Dentico P, De Giorgi U: Tyrosine kinase inhibitor imatinib mesylate as anticancer agent for advanced ocular melanoma expressing immunoistochemical C-KIT (CD 117): preliminary results of a compassionate use clinical trial. J Exp Clin Cancer Res; 2003 Dec;22(4 Suppl):17-20
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  • Several other human cancers, as small-cell lung carcinoma, melanoma, seminoma, some sarcomas, and adenoid cystic carcinomas may over-express KIT or PDGF-R, and clinical trials to evaluate the role of IM in the treatment of such cancers are currently ongoing.
  • We obtained in expressing positive immuno-reactivity for CD 117 patients: a reduction of malignant ascites in one, a partial remission in the neck nodes in another, and progression of liver metastases in the third.
  • Evidences of progression has been reported in the other two patients expressing negative immuno-reactivity for CD 117.
  • IM might be a potential therapeutic strategy for these patients.
  • [MeSH-major] Eye Neoplasms / drug therapy. Melanoma / drug therapy. Piperazines / therapeutic use. Protein Kinase Inhibitors / therapeutic use. Proto-Oncogene Proteins c-kit / drug effects. Pyrimidines / therapeutic use

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  • (PMID = 16767900.001).
  • [ISSN] 0392-9078
  • [Journal-full-title] Journal of experimental & clinical cancer research : CR
  • [ISO-abbreviation] J. Exp. Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Clinical Trial; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Benzamides; 0 / Biomarkers, Tumor; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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31. Bramwell VH, Morris D, Ernst DS, Hings I, Blackstein M, Venner PM, Ette EI, Harding MW, Waxman A, Demetri GD: Safety and efficacy of the multidrug-resistance inhibitor biricodar (VX-710) with concurrent doxorubicin in patients with anthracycline-resistant advanced soft tissue sarcoma. Clin Cancer Res; 2002 Feb;8(2):383-93
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  • [Title] Safety and efficacy of the multidrug-resistance inhibitor biricodar (VX-710) with concurrent doxorubicin in patients with anthracycline-resistant advanced soft tissue sarcoma.
  • PURPOSE: Incel (biricodar, VX-710) restores drug sensitivity to P-glycoprotein and multidrug resistance-associated protein-1-expressing cells.
  • This Phase I/II study evaluated the safety/tolerability, pharmacokinetics, and efficacy of VX-710 plus doxorubicin in patients with inoperable, locally advanced or metastatic, anthracycline-resistant/refractory, soft tissue sarcoma.
  • For efficacy evaluations in Phase II, eligible patients had inoperable, locally advanced or metastatic, anthracycline-resistant/refractory soft tissue sarcoma; < or =225 mg/m(2) cumulative prior doxorubicin; and adequate hematological, liver, and kidney function.
  • Among 26 evaluable patients, minimal activity was noted among 11 patients with gastrointestinal stromal tumors (GISTs); however, in 15 patients with anthracycline-resistant sarcomas of other histologies, 2 achieved partial responses and 7 patients had disease stabilization with an overall median progression-free interval of 3.4 months.
  • However, the combination of VX-710 and doxorubicin resulted in objective responses or disease stabilization in patients with strictly defined anthracycline-refractory non-GIST sarcomas, which warrants further evaluation.
  • [MeSH-major] Antibiotics, Antineoplastic / pharmacology. Antineoplastic Agents / pharmacology. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Doxorubicin / therapeutic use. Drug Resistance, Neoplasm. Piperidines / pharmacokinetics. Piperidines / therapeutic use. Pyridines / pharmacokinetics. Pyridines / therapeutic use. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Disease Progression. Disease-Free Survival. Female. Humans. Male. Middle Aged. P-Glycoprotein / metabolism. Time Factors

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  • (PMID = 11839653.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents; 0 / P-Glycoprotein; 0 / Piperidines; 0 / Pyridines; 3KG76X4KJK / biricodar; 80168379AG / Doxorubicin
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32. Shukla S, Wu G, Chatterjee M, Yang W, Sekido M, Diop LA, Müller R, Sudimack JJ, Lee RJ, Barth RF, Tjarks W: Synthesis and biological evaluation of folate receptor-targeted boronated PAMAM dendrimers as potential agents for neutron capture therapy. Bioconjug Chem; 2003 Jan-Feb;14(1):158-67
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  • [Title] Synthesis and biological evaluation of folate receptor-targeted boronated PAMAM dendrimers as potential agents for neutron capture therapy.
  • Successful treatment of cancer by boron neutron capture therapy (BNCT) requires the selective delivery of (10)B to constituent cells within a tumor.
  • Among all prepared combinations, boronated dendrimers with 1-1.5 PEG(2000) units exhibited the lowest hepatic uptake in C57BL/6 mice (7.2-7.7% injected dose (ID)/g liver).
  • Thus, two folate receptor-targeted boronated 3rd generation polyamidoamine dendrimers were prepared, one containing approximately 15 decaborate clusters and approximately 1 PEG(2000) unit with folic acid attached to the distal end, the other containing approximately 13 decaborate clusters, approximately 1 PEG(2000) unit, and approximately 1 PEG(800) unit with folic acid attached to the distal end.
  • Biodistribution studies with this conjugate in C57BL/6 mice bearing folate receptor (+) murine 24JK-FBP sarcomas resulted in selective tumor uptake (6.0% ID/g tumor), but also high hepatic (38.8% ID/g) and renal (62.8% ID/g) uptake, indicating that attachment of a second PEG unit and/or folic acid may adversely affect the pharmacodynamics of this conjugate.
  • [MeSH-major] Boron Compounds / chemistry. Boron Compounds / pharmacokinetics. Boron Neutron Capture Therapy / methods. Carrier Proteins / metabolism. Receptors, Cell Surface
  • [MeSH-minor] Animals. Biocompatible Materials / chemistry. Biocompatible Materials / pharmacokinetics. Dendrimers. Drug Carriers / chemistry. Drug Carriers / pharmacokinetics. Folate Receptors, GPI-Anchored. Folic Acid / chemistry. Folic Acid / pharmacokinetics. Kidney / metabolism. Liver / metabolism. Mice. Mice, Inbred C57BL. Neoplasm Proteins / metabolism. Neoplasms, Experimental / therapy. Polyamines / chemistry. Polyamines / pharmacokinetics. Polyethylene Glycols / chemistry. Structure-Activity Relationship. Tissue Distribution

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  • (PMID = 12526705.001).
  • [ISSN] 1043-1802
  • [Journal-full-title] Bioconjugate chemistry
  • [ISO-abbreviation] Bioconjug. Chem.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01CA79758
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biocompatible Materials; 0 / Boron Compounds; 0 / Carrier Proteins; 0 / Dendrimers; 0 / Drug Carriers; 0 / Folate Receptors, GPI-Anchored; 0 / Neoplasm Proteins; 0 / PAMAM Starburst; 0 / Polyamines; 0 / Receptors, Cell Surface; 30IQX730WE / Polyethylene Glycols; 935E97BOY8 / Folic Acid
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33. Dimitrakopoulou-Strauss A, Hohenberger P, Ströbel P, Marx A, Strauss LG: A recent application of fluoro-18-deoxyglucose positron emission tomography, treatment monitoring with a mammalian target of rapamycin inhibitor: an example of a patient with a desmoplastic small round cell tumor. Hell J Nucl Med; 2007 May-Aug;10(2):77-9
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  • [Title] A recent application of fluoro-18-deoxyglucose positron emission tomography, treatment monitoring with a mammalian target of rapamycin inhibitor: an example of a patient with a desmoplastic small round cell tumor.
  • It has been demonstrated in patients with gastrointestinal stromal tumors (GIST) and other sarcomas who received treatment with imatinib that PET with 18F-FDG is appropriate for treatment monitoring.
  • Data suggest that 18F-FDG monitoring may be used for monitoring not only imatinib but also other kinase inhibitors.
  • A baseline 18F-FDG-PET demonstrated an enhanced FDG uptake in three large liver metastases and in another metastatic lesion in the pelvic area.
  • In conclusion, 18F-FDG-PET seems to be a promising method for monitoring the therapeutic effect of mTOR-inhibitors.
  • [MeSH-major] Abdominal Neoplasms / drug therapy. Abdominal Neoplasms / radionuclide imaging. Fluorodeoxyglucose F18. Peritoneal Neoplasms / drug therapy. Peritoneal Neoplasms / radionuclide imaging. Positron-Emission Tomography / methods. Radiopharmaceuticals


34. Nanni P, Nicoletti G, Landuzzi L, Croci S, Murgo A, Palladini A, Antognoli A, Ianzano ML, Stivani V, Grosso V, Maira SM, García-Echeverría C, Scotlandi K, De Giovanni C, Lollini PL: High metastatic efficiency of human sarcoma cells in Rag2/gammac double knockout mice provides a powerful test system for antimetastatic targeted therapy. Eur J Cancer; 2010 Feb;46(3):659-68
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  • [Title] High metastatic efficiency of human sarcoma cells in Rag2/gammac double knockout mice provides a powerful test system for antimetastatic targeted therapy.
  • Metastatic spread of a panel of human sarcoma cell lines was studied in double knockout Rag2(-/-);gammac(-/-) mice in comparison with NK-depleted nude mice.
  • Rag2(-/-);gammac(-/-) mice receiving intravenous (i.v.) or subcutaneous (s.c.) human sarcoma cell lines developed extensive multiorgan metastases.
  • Most human sarcomas metastasised in the liver of Rag2(-/-);gammac(-/-) mice, a kind of organ preference undetectable in nude mice and specific of sarcomas, as several carcinoma cell lines failed to colonise the liver of Rag2(-/-);gammac(-/-) mice, independently of their metastatic spread to other sites.
  • In vitro analysis of the molecular mechanisms of liver metastasis of sarcomas implicated liver-produced growth and motility factors, in particular the insulin-like growth factor (IGF) axis.
  • NVP-BEZ235, a specific inhibitor of downstream signal transduction targeting PI3K and mTOR, strongly inhibited liver metastasis of human sarcoma cells.
  • In conclusion, the Rag2(-/-);gammac(-/-) mouse model allowed the expression of human metastatic phenotypes inapparent in conventional immunodeficient mice and the preclinical testing of appropriate targeted therapies.
  • [MeSH-major] DNA-Binding Proteins / deficiency. Disease Models, Animal. Sarcoma / secondary
  • [MeSH-minor] Animals. Antineoplastic Agents / therapeutic use. Chemotaxis / immunology. Culture Media, Conditioned. Enzyme Inhibitors / therapeutic use. Female. Humans. Imidazoles / therapeutic use. Immune Tolerance. Liver Neoplasms / drug therapy. Liver Neoplasms / immunology. Liver Neoplasms / pathology. Liver Neoplasms / secondary. Lung Neoplasms / immunology. Lung Neoplasms / pathology. Lung Neoplasms / secondary. Mice. Mice, Knockout. Neoplasm Transplantation. Phosphatidylinositol 3-Kinases / antagonists & inhibitors. Quinolines / therapeutic use. Transplantation, Heterologous. Treatment Outcome. Tumor Cells, Cultured. Xenograft Model Antitumor Assays / methods

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  • [Copyright] Copyright (c) 2009 Elsevier Ltd. All rights reserved.
  • (PMID = 20031388.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Culture Media, Conditioned; 0 / DNA-Binding Proteins; 0 / Enzyme Inhibitors; 0 / Imidazoles; 0 / Quinolines; 0 / Rag2 protein, mouse; EC 2.7.1.- / Phosphatidylinositol 3-Kinases; RUJ6Z9Y0DT / dactolisib
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