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1. Abramovitch R, Itzik A, Harel H, Nagler A, Vlodavsky I, Siegal T: Halofuginone inhibits angiogenesis and growth in implanted metastatic rat brain tumor model--an MRI study. Neoplasia; 2004 Sep-Oct;6(5):480-9
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  • [Title] Halofuginone inhibits angiogenesis and growth in implanted metastatic rat brain tumor model--an MRI study.
  • Halofuginone (HF) is a potent inhibitor of collagen type alpha1(I).
  • By employing magnetic resonance imaging (MRI), we monitored the effect of HF on tumor progression and vascularization by utilizing an implanted malignant fibrous histiocytoma metastatic rat brain tumor model.
  • Here we demonstrate that treatment with HF effectively and dose-dependently reduced tumor growth and angiogenesis.
  • Treatment with HF significantly prolonged survival of treated animals (142%; P = .001).
  • Additionally, HF treatment inhibited vessel maturation (P = .03).
  • We therefore conclude that HF is effective for treatment of metastatic brain tumors.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Antineoplastic Agents / therapeutic use. Brain Neoplasms / drug therapy. Magnetic Resonance Imaging. Neovascularization, Pathologic / drug therapy. Quinazolines / therapeutic use
  • [MeSH-minor] Animals. Cell Line, Tumor. Disease Models, Animal. Histiocytoma, Benign Fibrous / pathology. Neoplasm Metastasis. Neoplasm Transplantation. Piperidines. Quinazolinones. Rats

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  • (PMID = 15548356.001).
  • [ISSN] 1522-8002
  • [Journal-full-title] Neoplasia (New York, N.Y.)
  • [ISO-abbreviation] Neoplasia
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antineoplastic Agents; 0 / Piperidines; 0 / Quinazolines; 0 / Quinazolinones; L31MM1385E / halofuginone
  • [Other-IDs] NLM/ PMC1635242
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2. Jain A, Sajeevan KV, Babu KG, Lakshmaiah KC: Chemotherapy in adult soft tissue sarcoma. Indian J Cancer; 2009 Oct-Dec;46(4):274-87
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  • [Title] Chemotherapy in adult soft tissue sarcoma.
  • Soft tissue sarcomas (STSs) are rare and histologically diverse neoplasms.
  • Recent results of various meta-analyses and development of newer drugs have changed the medical management of soft tissue sarcoma.
  • This review gives an outline of chemotherapy and the newer targeted therapies for the same.
  • We have carried out an extensive search in PubMed, Medline for almost all relevant articles concerning chemotherapy of soft tissue sarcoma.
  • The role of neoadjuvant and adjuvant chemotherapy has been found to be controversial.
  • The recent meta-analysis for adjuvant therapy in STSs has shown an increase in the overall survival with combination of ifosfamide and adriamycin.
  • In locally advanced and metastatic STSs, single agent adriamycin remains the basic standard of medication.
  • Newer combinations of docetaxel and gemcitabine appear promising in selected subgroups, especially in leiomyosarcoma and malignant fibrous histiocytoma.
  • Some recent developments include the European Union's approval of trabectedin for advanced STSs patients who had progressed on adriamycin and ifosfamide therapy.
  • The future of mTOR inhibitors, insulin like growth factor receptor inhibitors and anti-angiogenic drugs appear quite promising.
  • Newer methodologies such as, Bayesian adaptive randomization and inclusion of newer end points like progression-free rate, time of progression rate, and tumor growth rate will improve the results of sarcoma trials.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Clinical Trials as Topic. Combined Modality Therapy. Humans. Neoadjuvant Therapy


3. Karavasilis V, Seddon BM, Ashley S, Al-Muderis O, Fisher C, Judson I: Significant clinical benefit of first-line palliative chemotherapy in advanced soft-tissue sarcoma: retrospective analysis and identification of prognostic factors in 488 patients. Cancer; 2008 Apr 1;112(7):1585-91
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  • [Title] Significant clinical benefit of first-line palliative chemotherapy in advanced soft-tissue sarcoma: retrospective analysis and identification of prognostic factors in 488 patients.
  • BACKGROUND: The efficacy of palliative chemotherapy was investigated in a large group of patients with advanced soft-tissue sarcomas (STS) treated on routine palliative protocols.
  • METHODS: Patients with STS who had first-line chemotherapy for advanced and/or metastatic disease between 1991 and 2005 were identified from the Royal Marsden Hospital's sarcoma database.
  • Patients with Ewing sarcoma, rhabdomyosarcoma, desmoplastic small round cell tumor, and gastrointestinal stromal tumors were excluded from the study.
  • The median age was 49 years and the majority (83%) received chemotherapy for metastatic disease.
  • The most common histologic subtypes were leiomyosarcoma (35%) synovial sarcoma (13%), liposarcoma (10%), and malignant fibrous histiocytoma (10%).
  • In all, 61% received single-agent chemotherapy, usually doxorubicin.
  • An objective response was reported in 33% of patients (53% in those with synovial sarcoma); 22% had stable disease and 45% derived 'clinical benefit' (objective responses + stable disease for >or= 6 months).
  • Patients treated with combination chemotherapy experienced longer OS than those treated with a single agent.
  • CONCLUSIONS: Palliative chemotherapy may be beneficial in approximately half of patients with advanced STS.
  • Synovial sarcoma and liposarcoma subtypes have a better prognosis.
  • [MeSH-major] Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Antineoplastic Combined Chemotherapy Protocols. Bone Neoplasms / drug therapy. Bone Neoplasms / secondary. Chemotherapy, Adjuvant. Female. Follow-Up Studies. Humans. Liver Neoplasms / drug therapy. Liver Neoplasms / secondary. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Male. Middle Aged. Neoplasm Staging. Palliative Care. Prognosis. Prospective Studies. Retrospective Studies. Survival Rate

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  • (PMID = 18278813.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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4. Asavamongkolkul A, Waikakul S, Phimolsarnti R, Kiatisevi P, Wangsaturaka P: Endoprosthetic reconstruction for malignant bone and soft-tissue tumors. J Med Assoc Thai; 2007 Apr;90(4):706-17
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  • [Title] Endoprosthetic reconstruction for malignant bone and soft-tissue tumors.
  • BACKGROUND: Nowadays, the results of the management of malignant bone and soft-tissue tumors have been dramatically improved because of the advance in imaging, chemotherapy, radiation therapy, and surgical techniques.
  • Patients can have longer survival times with limb-salvage surgery.
  • Several techniques of reconstruction have been advocated and gained more popularity following malignant tumor resection by using allograft, tumor prostheses, composite allograft prosthesis, or arthrodesis.
  • OBJECTIVE: To report the preliminary results of 32 endoprosthetic reconstructions following malignant bone and soft-tissue tumor resection.
  • MATERIAL AND METHOD: Since September 1988, the authors have performed 188 limb-salvage surgical operations for the treatment of musculoskeletal tumors at Siriraj Hospital.
  • From March 1994 to July 2006, 32 endoprosthetic reconstructions were performed on 30 patients following malignant bone or soft-tissue tumor removal.
  • The diagnosis was conventional osteosarcoma in 16 patients, parosteal osteosarcoma in two patients, chondrosarcoma in two patients, leiomyosarcoma in two patients, failed allograft in two patients and one patient each of periosteal osteosarcoma, Ewing's sarcoma, Gorham's disease, synovial sarcoma, malignant fibrous histiocytoma, metastatic renal cell carcinoma, and prosthetic loosening.
  • Five proximal femurs, 17 distal femurs, 1 total femur 3 proximal tibias, 1 intercalary tibia, 4 proximal humerus and 1 distal humerus were used for reconstruction.
  • RESULTS: The mean follow-up time was 26 months (range 6-128.7).
  • CONCLUSION: Endoprosthetic reconstruction could yield satisfactory results as a wide excision and limb-salvage for patients with malignant bone and soft-tissue tumors.
  • [MeSH-major] Bone Neoplasms / surgery. Limb Salvage. Osteosarcoma / surgery. Sarcoma, Ewing / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 17487125.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Thailand
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5. Abe T, Yamanaka K, Nakata W, Mori N, Sekii K, Yoshioka T, Itatani H: [A case of retroperitoneal malignant fibrous histiocytoma with marked response to concurrent cisplatin and radiation therapy: a case report]. Hinyokika Kiyo; 2007 Apr;53(4):241-6
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  • [Title] [A case of retroperitoneal malignant fibrous histiocytoma with marked response to concurrent cisplatin and radiation therapy: a case report].
  • Abdominal computed tomography and magnetic resonance imaging revealed a large retroperitoneal tumor adjacent to the aorta and the renal vessels.
  • Histopathological examination showed a malignant fibrous histiocytoma (MFH).
  • Two cycles of systemic chemotherapy with pirarubicin, vincritine and cyclophosphamide were ineffective, then we tried concurrent cisplatin and radiation therapy.
  • Chemoradiation therapy showed a marked decrease in the size of the tumor, and the patient also recovered from right lumbar pain without serious side effects.
  • After chemoradiation therapy, we performed tumorectomy.
  • He suffered from repeated recurrence and metastases of MFH afterwards.
  • We performed repeatedly concurrent cisplatin and radiation therapy for the recurrent and metastatic tumor sites, and chemoradiation therapy led to regression of the tumors every time.
  • Concurrent cisplatin and radiation therapy might be an effective treatment for unresectable MFH.
  • [MeSH-major] Cisplatin / therapeutic use. Histiocytoma, Malignant Fibrous / drug therapy. Histiocytoma, Malignant Fibrous / radiotherapy. Radiation-Sensitizing Agents / therapeutic use. Retroperitoneal Neoplasms / drug therapy. Retroperitoneal Neoplasms / radiotherapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Humans. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed

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  • (PMID = 17515074.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Radiation-Sensitizing Agents; Q20Q21Q62J / Cisplatin
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6. Maki RG: Gemcitabine and docetaxel in metastatic sarcoma: past, present, and future. Oncologist; 2007 Aug;12(8):999-1006
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  • [Title] Gemcitabine and docetaxel in metastatic sarcoma: past, present, and future.
  • Objective. In the era of oral molecular kinase inhibitors, cytotoxic chemotherapy agents are somewhat overlooked, but remain the backbone of treatment for most cancers.
  • Patients with non-gastrointestinal stromal tumor sarcomas, such as leiomyosarcoma, liposarcoma, and undifferentiated high-grade pleomorphic sarcoma (formerly called malignant fibrous histiocytoma), have received doxorubicin and ifosfamide as the backbone of their treatment for over 15 years or more.
  • The goal of this article is to review the data that have led to the use of gemcitabine and docetaxel as a useful combination for patients with metastatic sarcomas, and to comment on possible synergy of the combination.
  • The literature regarding the use of gemcitabine, docetaxel, or both, is reviewed, with emphasis on patients with metastatic sarcoma.
  • Results. Activity of gemcitabine and docetaxel is observed in leiomyosarcoma and undifferentiated high-grade pleomorphic sarcoma.
  • Conclusions. The combination of gemcitabine and docetaxel is an effective option for patients with metastatic sarcoma, increasing the armamentarium for the practicing oncologist in treating this heterogeneous group of diseases.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Deoxycytidine / analogs & derivatives. Sarcoma / drug therapy. Sarcoma / pathology. Taxoids / therapeutic use
  • [MeSH-minor] Drug Synergism. Humans


7. Fu DL, Yang F, Maskay A, Long J, Jin C, Yu XJ, Xu J, Zhou ZW, Ni QX: Primary intestinal malignant fibrous histiocytoma: two case reports. World J Gastroenterol; 2007 Feb 28;13(8):1299-302
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  • [Title] Primary intestinal malignant fibrous histiocytoma: two case reports.
  • Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities and trunk, but rarely in the intestine.
  • Here we report two cases of primary intestinal MFH.
  • At laparotomy, a tumor was found originating from the cecum, with a suspicious metastatic nodule on the surface of the right lobe of the liver.
  • Though the symptoms were not typical, based on histological and immunohistochemical studies, the patients were diagnosed as MFH of the intestine.
  • They were not treated with chemotherapy or radiotherapy and both died within 3 mo.
  • MFH of the intestine is an extremely rare neoplasm with an aggressive biological behavior.
  • Complete surgical excision is preferred, adjuvant chemotherapy or radiotherapy may be advisable.
  • [MeSH-major] Histiocytoma, Malignant Fibrous / diagnosis. Intestinal Neoplasms / diagnosis

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  • (PMID = 17451221.001).
  • [ISSN] 1007-9327
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Other-IDs] NLM/ PMC4147015
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8. Sugihara T, Fujimura T, Kume H, Homma Y: Successful treatment of metastatic malignant fibrous histiocytoma of the kidney. Urol Int; 2010;85(1):118-20
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  • [Title] Successful treatment of metastatic malignant fibrous histiocytoma of the kidney.
  • Malignant fibrous histiocytoma (MFH) of the kidney is a rare sarcoma that often undergoes local recurrence and/or distant metastasis.
  • However, little is known about the outcome of metastatic diseases.
  • We present the case of a 46-year-old male suffering from renal MFH with pulmonary metastasis, who has undergone complete response for 3 years after surgical resection and MAID chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Histiocytoma, Malignant Fibrous / therapy. Kidney Neoplasms / therapy. Lung Neoplasms / therapy. Nephrectomy. Thoracoscopy
  • [MeSH-minor] Biopsy. Chemotherapy, Adjuvant. Dacarbazine / administration & dosage. Doxorubicin / administration & dosage. Humans. Ifosfamide / administration & dosage. Male. Mesna / administration & dosage. Middle Aged. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright (c) 2010 S. Karger AG, Basel.
  • (PMID = 20516674.001).
  • [ISSN] 1423-0399
  • [Journal-full-title] Urologia internationalis
  • [ISO-abbreviation] Urol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; NR7O1405Q9 / Mesna; UM20QQM95Y / Ifosfamide; MAID protocol
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9. Hoshi M, Takami M, Ieguchi M: Pleomorphic malignant fibrous histiocytoma: response of bone, lung, and brain metastases to chemotherapy. Radiat Med; 2008 Oct;26(8):499-503
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  • [Title] Pleomorphic malignant fibrous histiocytoma: response of bone, lung, and brain metastases to chemotherapy.
  • We present a case of pleomorphic malignant fibrous histiocytoma arising from the left forearm in a 45-year-old man who had undergone resection and radiotherapy for a tumor 3 years previously.
  • Although these metastases responded well to systemic chemotherapy, brain metastases newly appeared and caused the death of the patient.
  • These findings demonstrate that individual sarcomatous metastatic organs exhibit different sensitivities to chemotherapy.
  • It is suggested that the blood-brain barrier may play an important role in sensitivity to chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Brain Neoplasms / drug therapy. Histiocytoma, Malignant Fibrous / drug therapy. Lung Neoplasms / drug therapy. Neoplasm Recurrence, Local
  • [MeSH-minor] Acetabulum. Bone and Bones / drug effects. Brain / drug effects. Doxorubicin / administration & dosage. Forearm. Humans. Ifosfamide / administration & dosage. Lung / drug effects. Male. Middle Aged


10. Shields JA, Husson M, Shields CL, Krema H, Eagle RC Jr, Singh AD: Orbital malignant fibrous histiocytoma following irradiation for retinoblastoma. Ophthal Plast Reconstr Surg; 2001 Jan;17(1):58-61
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  • [Title] Orbital malignant fibrous histiocytoma following irradiation for retinoblastoma.
  • PURPOSE: An unusual case is reported of orbital malignant fibrous histiocytoma that developed after irradiation for retinoblastoma.
  • RESULTS: A 5-month-old girl underwent enucleation of the left eye, external beam irradiation of the right eye, and systemic chemotherapy for bilateral sporadic retinoblastoma.
  • At age 17 years, a malignant fibrous histiocytoma developed in the medial aspect of the orbit and nasal cavity.
  • The patient was alive 12 months postoperatively without local or metastatic disease.
  • CONCLUSIONS: Although orbital fibrous histiocytoma occurs usually as a primary tumor of adulthood, it can also develop as a secondary tumor after irradiation for retinoblastoma.
  • [MeSH-major] Histiocytoma, Benign Fibrous / etiology. Neoplasms, Radiation-Induced / etiology. Orbital Neoplasms / etiology. Retinal Neoplasms / radiotherapy. Retinoblastoma / radiotherapy

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  • (PMID = 11206748.001).
  • [ISSN] 0740-9303
  • [Journal-full-title] Ophthalmic plastic and reconstructive surgery
  • [ISO-abbreviation] Ophthal Plast Reconstr Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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11. Nooij MA, Whelan J, Bramwell VH, Taminiau AT, Cannon S, Hogendoorn PC, Pringle J, Uscinska BM, Weeden S, Kirkpatrick A, Glabbeke Mv, Craft AW, European Osteosarcoma Intergroup: Doxorubicin and cisplatin chemotherapy in high-grade spindle cell sarcomas of the bone, other than osteosarcoma or malignant fibrous histiocytoma: a European Osteosarcoma Intergroup Study. Eur J Cancer; 2005 Jan;41(2):225-30
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  • [Title] Doxorubicin and cisplatin chemotherapy in high-grade spindle cell sarcomas of the bone, other than osteosarcoma or malignant fibrous histiocytoma: a European Osteosarcoma Intergroup Study.
  • There are limited data that define the role of chemotherapy in the treatment of high-grade spindle cell sarcomas of bone, other than osteosarcoma or malignant fibrous histiocytoma (MFH-B).
  • Thirty-seven patients, age 65 years, with spindle cell sarcoma of bone, except osteosarcoma or MFH-B, were included.
  • Chemotherapy consisted of doxorubicin and cisplatin every 3 weeks for six cycles.
  • Median time to progression was 30 months (95% Confidence Interval (CI), 8-51 months) for the operable non-metastatic patients; median survival 41 months (95% CI, 16-82 months).
  • Median time to progression in the metastatic group was 10 months (95% CI, 0-18 months) and median survival was 14 months (95% CI, 4-45 months).
  • This study suggests a limited role for doxorubicin and cisplatin in metastatic high-grade spindle cell sarcoma of bone, other than osteosarcoma or MFH-B cases.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Rare Diseases / drug therapy. Sarcoma / drug therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Cisplatin / administration & dosage. Cisplatin / adverse effects. Combined Modality Therapy. Disease Progression. Doxorubicin / administration & dosage. Doxorubicin / adverse effects. Female. Humans. Infusions, Intravenous. Lymphatic Metastasis. Male. Middle Aged. Prospective Studies. Survival Analysis. Treatment Outcome


12. Kusuzaki K, Shinjo H, Murata H, Takeshita H, Hashiguchi S, Nozaki T, Emoto K, Ashihara T, Hirasawa Y: Relationship between doxorubicin binding ability and tumor volume decrease after chemotherapy in adult malignant soft tissue tumors in the extremities. Anticancer Res; 2000 Sep-Oct;20(5C):3813-6
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  • [Title] Relationship between doxorubicin binding ability and tumor volume decrease after chemotherapy in adult malignant soft tissue tumors in the extremities.
  • We performed the present study to clarify the relationship between the DOX binding ability (%DB) and the histologic response, rate of decrease in tumor volume of malignant soft tissue tumors after preoperative chemotherapy and prognosis.
  • Nine malignant soft tissue tumors (4 liposarcomas, 3 synovial sarcomas, one malignant fibrous histiocytoma (MFH) and one extraskeletal osteosarcoma (EOS)) which arose at the extremities of adult patients were analyzed by the DOX binding assay using freshly biopsied specimens.
  • After preoperative chemotherapy including DOX or pirarubicin (THP), the rate of decrease in tumor volume was measured using magnetic resonance imaging, and the histologic response expressed as tumor necrosis to chemotherapy was also investigated.
  • All the patients, apart for one, were continuously disease-free after treatment.
  • One patient with EOS died of metastatic disease before surgery.
  • These results suggest that in malignant soft tissue tumors, the rate of decrease in tumor volume after chemotherapy might be a better indicator for chemosensitivity than the histologic response and also that the DOX binding ability might be a good predictor for chemosensitivity before chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Doxorubicin / analogs & derivatives. Doxorubicin / pharmacokinetics. Soft Tissue Neoplasms / drug therapy. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Agents / pharmacokinetics. Antineoplastic Agents / therapeutic use. Arm. Carboplatin / administration & dosage. Chemotherapy, Adjuvant. Female. Humans. Liposarcoma / drug therapy. Liposarcoma / pathology. Magnetic Resonance Imaging. Male. Middle Aged. Osteosarcoma / drug therapy. Osteosarcoma / pathology. Sarcoma, Synovial / drug therapy. Sarcoma, Synovial / pathology. Thigh

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  • (PMID = 11268459.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 80168379AG / Doxorubicin; BG3F62OND5 / Carboplatin; D58G680W0G / pirarubicin
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13. Bodner K, Bodner-Adler B, Mayerhofer S, Grünberger W, Wierrani F, Czerwenka K, Leodolter S, Mayerhofer K: Malignant fibrous histiocytoma (MFH) of the mesentery: a case report. Anticancer Res; 2002 Mar-Apr;22(2B):1169-70
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  • [Title] Malignant fibrous histiocytoma (MFH) of the mesentery: a case report.
  • BACKGROUND: Primary tumors of the mesentery are rare; only a few cases of malignant fibrous histiocytoma have been reported in the literature.
  • This case report presents the management of a patient with malignantfibrous histiocytoma of the mesentery.
  • The tumor was histologically classified as a malignant fibrous histiocytoma, showing a heterologeous picture consisting of large, multinucleated cells and spindle-shaped cells forming a storiform-like growth pattern.
  • A radical excision of the tumor and the lymphnodes was performed and the patient received adjuvant irradiation therapy.
  • CONCLUSION: Malignant fibrous histiocytoma of the mesentery is an extremely rare, highly malignant neoplasm with early metastatic spread.
  • The treatment of choice is wide surgical excision, while the role of adjuvant chemotherapy and irradiation still remains controversiaL
  • [MeSH-major] Histiocytoma, Benign Fibrous / pathology. Mesentery / pathology

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  • (PMID = 12168919.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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14. Salemis NS, Gourgiotis S, Tsiambas E, Panagiotopoulos N, Karameris A, Tsohataridis E: Primary intra-abdominal malignant fibrous histiocytoma: a highly aggressive tumor. J Gastrointest Cancer; 2010 Dec;41(4):238-42
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  • [Title] Primary intra-abdominal malignant fibrous histiocytoma: a highly aggressive tumor.
  • BACKGROUND AND PURPOSE: Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma of late adult life occurring predominantly in the extremities.
  • Primary intra-abdominal MFH is a very rare occurrence.
  • The aim of this study is to describe a very rare case of an intra-abdominal MFH with a highly aggressive clinical course.
  • Computed tomography (CT) scan demonstrated a mass in the right iliac fossa.
  • Histological and immunohistochemical examinations showed a MFH.
  • One month after surgery, while on adjuvant chemotherapy, the patient was readmitted with dyspnea and a slightly palpable mass in the area of the previous radical resection.
  • CT scan revealed local tumor recurrence along with multiple pulmonary metastatic deposits.
  • Unfortunately, despite treatment, the patient died of progressive disease 5 weeks later.
  • CONCLUSIONS: Primary intra-abdominal MFH is a very rare but aggressive malignancy with a high tendency of local recurrence and metastatic spread.
  • Early detection and complete surgical excision with clear margins is the treatment of choice.
  • In some cases, however, the tumor can exhibit a highly aggressive clinical course despite radical surgery and adjuvant therapy.
  • [MeSH-major] Abdomen / pathology. Histiocytoma, Malignant Fibrous / secondary. Soft Tissue Neoplasms / pathology

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  • (PMID = 20419356.001).
  • [ISSN] 1941-6636
  • [Journal-full-title] Journal of gastrointestinal cancer
  • [ISO-abbreviation] J Gastrointest Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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15. Zlowodzki M, Allen B, Schreibman KL, Vance RB, Kregor PJ: CASE REPORTS: malignant fibrous histiocytoma of bone arising in chronic osteomyelitis. Clin Orthop Relat Res; 2005 Oct;439:269-73
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  • [Title] CASE REPORTS: malignant fibrous histiocytoma of bone arising in chronic osteomyelitis.
  • According to published reports, a coincidence of malignant fibrous histiocytoma of bone and post-fracture osteomyelitis has occurred in only four patients.
  • Our report details the treatment of 51-year-old man with a fracture 15 years previously and subsequent chronic osteomyelitis of the left distal femur.
  • The original treatment was open reduction and casting.
  • Fifteen years after the injury, the patient presented to the emergency room with increasing pain, erythema, swelling, and increased purulent discharge from the distal femur.
  • The left distal femur was radically resected for treatment of osteomyelitis.
  • Histologic samples of the specimen revealed malignant fibrous histiocytoma of bone.
  • A metastatic workup was negative.
  • One of two inguinal lymph nodes removed at that time was positive for malignant fibrous histiocytoma.
  • The patient had additional chemotherapy.
  • [MeSH-major] Bone Neoplasms / complications. Histiocytoma, Malignant Fibrous / complications. Osteomyelitis / complications
  • [MeSH-minor] Chronic Disease. Femur / pathology. Femur / surgery. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Tomography, X-Ray Computed

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  • (PMID = 16205169.001).
  • [ISSN] 0009-921X
  • [Journal-full-title] Clinical orthopaedics and related research
  • [ISO-abbreviation] Clin. Orthop. Relat. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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16. Belal A, Kandil A, Allam A, Khafaga Y, El-Husseiny G, El-Enbaby A, Memon M, Younge D, Moreau P, Gray A, Schultz H: Malignant fibrous histiocytoma: a retrospective study of 109 cases. Am J Clin Oncol; 2002 Feb;25(1):16-22
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  • [Title] Malignant fibrous histiocytoma: a retrospective study of 109 cases.
  • The purpose of this report is to assess the prognostic factors that could influence management and clinical outcome of malignant fibrous histiocytoma (MFH) of soft tissues.
  • Between 1975 and 1998, 109 patients diagnosed with MFH of the soft tissues, seen at King Faisal Specialist Hospital and Research Center, have been reviewed.
  • The remaining 92 patients had localized disease and had surgery as the primary treatment modality with or without radiotherapy and/or chemotherapy.
  • Isolated local recurrence occurred in 20 patients (22%), isolated metastatic disease without local recurrence in 9 patients (10%), and combined local and metastatic disease occurred in 20 patients (22%).
  • Complete surgical resection at the time of primary tumor presentation is likely to afford the best chance for RFS and OS.
  • Radiation therapy plays an important role, in combination with surgery for better local control, particularly in high-grade lesions, and in cases with positive surgical margins after wide complete gross excision.
  • The role of adjuvant chemotherapy remains investigational.
  • [MeSH-major] Histiocytoma, Benign Fibrous. Soft Tissue Neoplasms

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  • (PMID = 11823689.001).
  • [ISSN] 0277-3732
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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17. Klopfenstein KJ, Scott S, Schuller DE, Ruymann FB: Prolonged survival with continuous infusion topotecan: a report of 2 cases. J Pediatr Hematol Oncol; 2008 Jun;30(6):468-70
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  • Two patients with solid tumors were treated with 21-day continuous infusion topotecan as palliation therapy.
  • Case 1: A 10-year-old girl was diagnosed with progressive, metastatic hepatocellular carcinoma.
  • Case 2: A 17-year-old girl developed a malignant fibrous histiocytoma as a second malignant neoplasm.
  • After partial resection and failure of multiagent chemotherapy, she started continuous infusion topotecan and was disease-free for 58 months when she died of pneumonia.
  • [MeSH-major] Antineoplastic Agents / administration & dosage. Carcinoma, Hepatocellular / drug therapy. Head and Neck Neoplasms / drug therapy. Histiocytoma, Malignant Fibrous / drug therapy. Liver Neoplasms / drug therapy. Topotecan / administration & dosage
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infusions, Intravenous. Neoplasms, Second Primary / drug therapy. Neoplasms, Second Primary / mortality. Palliative Care. Survival

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  • (PMID = 18525467.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 7M7YKX2N15 / Topotecan
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18. Staals EL, Bacchini P, Bertoni F: Dedifferentiated central chondrosarcoma. Cancer; 2006 Jun 15;106(12):2682-91
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  • METHODS: In this retrospective study, the clinical, radiographic, and histologic features and the treatments in 123 patients from the Rizzoli Institute were reviewed in an attempt to define which factors may be related to outcome in patients with dedifferentiated central chondrosarcoma.
  • Radiographically, a soft tissue mass was present in 87% of patients, and a bimorphic pattern was appreciated in 53% of patients.
  • In most patients, the dedifferentiated component showed the features of an osteosarcoma (92 patients), followed by fibrosarcoma (19 patients), and malignant fibrous histiocytoma (9 patients).
  • For 101 patients, surgery was a component of their definitive management.
  • In 25 patients, surgery was combined with chemotherapy.
  • CONCLUSIONS: Metastatic disease at diagnosis, malignant fibrous histiocytoma dedifferentiation, and a high percentage of dedifferentiated component were related to poorer outcomes.
  • There was no statistical evidence of any beneficial effect from adjuvant chemotherapy.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Cell Differentiation. Combined Modality Therapy. Drug Therapy. Female. Femur / pathology. Histiocytoma, Malignant Fibrous / pathology. Histiocytoma, Malignant Fibrous / radiography. Histiocytoma, Malignant Fibrous / therapy. Humans. Humerus / pathology. Male. Middle Aged. Neoplasm Metastasis. Pelvis / pathology. Prognosis. Retrospective Studies. Survival Analysis. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright 2006 American Cancer Society.
  • (PMID = 16691621.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. Check JH, Dix E, Cohen R, Check D, Wilson C: Efficacy of the progesterone receptor antagonist mifepristone for palliative therapy of patients with a variety of advanced cancer types. Anticancer Res; 2010 Feb;30(2):623-8
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  • [Title] Efficacy of the progesterone receptor antagonist mifepristone for palliative therapy of patients with a variety of advanced cancer types.
  • The present study evaluated the palliative effect of mifepristone in a variety of different types of human cancer.
  • PATIENTS AND METHODS: Mifepristone was given at 200 mg daily orally with permission from the Food and Drug Administration to people with widely metastatic human cancer no longer responsive to other chemotherapy regimens.
  • RESULTS: Improvement in pain and energy and/or length of life was found in thymic epithelial cell carcinoma, transitional cell carcinoma of the renal pelvis, leiomyosarcoma, pancreatic carcinoma, malignant fibrous histiocytoma and another case of adenocarcinoma of the colon.
  • CONCLUSION: Our data demonstrate a palliative role for the use of mifepristone in cancer therapy.
  • Progesterone receptor antagonists should be given a therapeutic trial in larger controlled studies of various malignancies in humans.
  • [MeSH-major] Hormone Antagonists / therapeutic use. Mifepristone / therapeutic use. Neoplasms / drug therapy. Palliative Care. Receptors, Progesterone / antagonists & inhibitors
  • [MeSH-minor] Adult. Aged. Carcinoma, Transitional Cell / drug therapy. Carcinoma, Transitional Cell / secondary. Colonic Neoplasms / drug therapy. Colonic Neoplasms / pathology. Female. Histiocytoma, Malignant Fibrous / drug therapy. Histiocytoma, Malignant Fibrous / pathology. Humans. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Leiomyosarcoma / drug therapy. Leiomyosarcoma / secondary. Male. Middle Aged. Pancreatic Neoplasms / drug therapy. Pancreatic Neoplasms / pathology. Survival Rate. Thymus Neoplasms / drug therapy. Thymus Neoplasms / pathology. Treatment Outcome. Young Adult

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  • (PMID = 20332480.001).
  • [ISSN] 1791-7530
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Hormone Antagonists; 0 / Receptors, Progesterone; 320T6RNW1F / Mifepristone
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20. Minard-Colin V, Kalifa C, Guinebretiere JM, Brugieres L, Dubousset J, Habrand JL, Vassal G, Hartmann O: Outcome of flat bone sarcomas (other than Ewing's) in children and adolescents: a study of 25 cases. Br J Cancer; 2004 Feb 9;90(3):613-9
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  • We analysed the clinical features and outcome of young patients with non-Ewing's flat bone sarcoma treated during the era of contemporary chemotherapy.
  • The characteristics and outcome of 25 patients (15 males and 10 females) with primary or radiation-related flat bone sarcoma treated in the Pediatrics Department at the Institut Gustave Roussy from 1981 to 1999 were reviewed.
  • In all, 20 patients had osteosarcoma, four chondrosarcoma and one malignant fibrous histiocytoma.
  • Four patients had metastatic disease at diagnosis.
  • The EFS rate of patients with second bone sarcoma was similar to that of patients with de novo flat bone sarcoma (P=0.1).
  • The aim of treatment was curative for 24 patients, 23 of whom were treated with intensive chemotherapy regimens and 19 with surgery.
  • Significant adverse prognostic factors on survival included incomplete surgical resection (P=0.001) and use of regimens without pre- and postoperative chemotherapy (P=0.007).
  • Nine of the 25 patients were treated with pre- and postoperative chemotherapy and complete surgical resection.
  • Nevertheless, our results suggest a more favourable outcome since the advent of intensive chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Bone Neoplasms / pathology. Chondrosarcoma / drug therapy. Chondrosarcoma / pathology. Histiocytoma, Benign Fibrous / drug therapy. Histiocytoma, Benign Fibrous / pathology. Neoplasms, Radiation-Induced / drug therapy. Neoplasms, Radiation-Induced / pathology. Osteosarcoma / drug therapy. Osteosarcoma / pathology
  • [MeSH-minor] Adolescent. Adult. Age of Onset. Child. Child, Preschool. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Prognosis. Treatment Outcome

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  • (PMID = 14760373.001).
  • [ISSN] 0007-0920
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2409588
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21. Rajan DK, Soulen MC, Clark TW, Baum RA, Haskal ZJ, Shlansky-Goldberg RD, Freiman DB: Sarcomas metastatic to the liver: response and survival after cisplatin, doxorubicin, mitomycin-C, Ethiodol, and polyvinyl alcohol chemoembolization. J Vasc Interv Radiol; 2001 Feb;12(2):187-93
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  • [Title] Sarcomas metastatic to the liver: response and survival after cisplatin, doxorubicin, mitomycin-C, Ethiodol, and polyvinyl alcohol chemoembolization.
  • PURPOSE: To evaluate the response to and survival after chemoembolization with cisplatin, doxorubicin, mitomycin-C, Ethiodol, and polyvinyl alcohol for patients with sarcomas metastatic to the liver that are surgically unresectable.
  • Primary tumors included 11 gastrointestinal leiomyosarcomas, two splenic angiosarcomas, one leiomyosarcoma of the broad ligament, one leiomyosarcoma of the inferior vena cava, and one malignant fibrous histiocytoma of the colon.
  • Chemoembolization with cisplatin, doxorubicin, mitomycin-C, Ethiodol, and polyvinyl alcohol particles was performed 1-5 times at approximately monthly intervals (mean, 2.8).
  • Pre- and posttreatment cross-sectional imaging was performed 1 month after completion of treatment and then every 3 months.
  • RESULTS: Two patients (13%) exhibited partial morphologic response, 11 patients (69%) were morphologically stable, and three (19%) demonstrated progression of disease 30 days after completion of treatment.
  • Among the 13 responders, two underwent partial hepatectomy after initial treatment.
  • Seven developed intrahepatic progression at a mean of 10 months and a median time of 8 months.
  • The remaining four patients had no documented intrahepatic progression at the time of last imaging follow-up.
  • Nine patients developed extrahepatic progression at a mean time of 6.3 months and a median time of 6 months, of whom four underwent additional surgical resection.
  • Response to therapy was based on time of first intervention.
  • Cumulative survival from time of diagnosis with use of Kaplan-Meier analysis was 81% at 1 year, 54% at 2 years, and 40% at 3 years.
  • Median survival time was 20 months.
  • Cumulative survival from initial chemoembolization was 67% at 1 year, 50% at 2 years, and 40% at 3 years, with a median survival time of 13 months.
  • CONCLUSION: Durable tumor response with chemoembolization is possible in this form of metastatic disease, which is highly resistant to systemic chemotherapy.
  • [MeSH-major] Chemoembolization, Therapeutic. Leiomyosarcoma / secondary. Leiomyosarcoma / therapy. Liver Neoplasms / secondary. Liver Neoplasms / therapy
  • [MeSH-minor] Cisplatin / administration & dosage. Disease Progression. Doxorubicin / administration & dosage. Ethiodized Oil / administration & dosage. Female. Follow-Up Studies. Humans. Male. Mitomycin / administration & dosage. Polyvinyl Alcohol / administration & dosage. Survival Rate. Time Factors

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  • (PMID = 11265882.001).
  • [ISSN] 1051-0443
  • [Journal-full-title] Journal of vascular and interventional radiology : JVIR
  • [ISO-abbreviation] J Vasc Interv Radiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 50SG953SK6 / Mitomycin; 8008-53-5 / Ethiodized Oil; 80168379AG / Doxorubicin; 9002-89-5 / Polyvinyl Alcohol; Q20Q21Q62J / Cisplatin
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22. Neragi-Miandoab S, Kim J, Vlahakes GJ: Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol); 2007 Dec;19(10):748-56
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  • [Title] Malignant tumours of the heart: a review of tumour type, diagnosis and therapy.
  • Primary cardiac neoplasms are rare and occur less commonly than metastatic disease of the heart.
  • In this overview, current published studies concerning malignant neoplasms of the heart are reviewed, together with some insights into their aetiology, diagnosis and management.
  • Sarcomas are the most common cardiac tumours and include myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, neurofibrosarcoma, malignant fibrous histiocytoma and undifferentiated sarcoma.
  • Computed tomography and magnetic resonance imaging studies have improved the diagnostic approach in recent decades.
  • Successful treatment for benign cardiac tumours is usually achieved by surgical resection.
  • The prognosis after surgery is usually excellent in the case of benign tumours, but the prognosis of malignant tumours remains dismal.
  • Surgery is the cornerstone of therapy.
  • However, a multi-treatment approach, including chemotherapy, radiation as well as evolving approaches such as gene therapy, might provide a better palliative and curative result.
  • [MeSH-major] Heart Neoplasms. Sarcoma
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Cardiovascular Surgical Procedures. Echocardiography, Three-Dimensional. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 17693068.001).
  • [ISSN] 0936-6555
  • [Journal-full-title] Clinical oncology (Royal College of Radiologists (Great Britain))
  • [ISO-abbreviation] Clin Oncol (R Coll Radiol)
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 77
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23. Ravi V, Benjamin RS: Systemic therapy for cardiac sarcomas. Methodist Debakey Cardiovasc J; 2010 Jul-Sep;6(3):57-60
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  • [Title] Systemic therapy for cardiac sarcomas.
  • Cardiac sarcomas create 2 risks: local problems and metastatic disease.
  • Most frequently, the histologies are angiosarcoma and high-grade pleomorphic unclassified sarcoma (formerly called MFH or malignant fibrous histiocytoma).
  • Conventional wisdom indicates that soft-tissue sarcomas are poorly responsive to chemotherapy.
  • Attempts to concentrate on the local problem only with therapies up to and including cardiac transplantation have been unsuccessful due to the high rate of fatal metastatic disease.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Heart Neoplasms / drug therapy. Sarcoma / drug therapy
  • [MeSH-minor] Humans. Treatment Outcome

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  • (PMID = 20834213.001).
  • [ISSN] 1947-6094
  • [Journal-full-title] Methodist DeBakey cardiovascular journal
  • [ISO-abbreviation] Methodist Debakey Cardiovasc J
  • [Language] eng
  • [Publication-type] Journal Article; Portraits
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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24. Tsukahara T, Kawaguchi S, Torigoe T, Asanuma H, Nakazawa E, Shimozawa K, Nabeta Y, Kimura S, Kaya M, Nagoya S, Wada T, Yamashita T, Sato N: Prognostic significance of HLA class I expression in osteosarcoma defined by anti-pan HLA class I monoclonal antibody, EMR8-5. Cancer Sci; 2006 Dec;97(12):1374-80
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  • With the goal of establishing efficacious peptide-based immunotherapy for patients with bone and soft tissue sarcomas, we previously identified the cytotoxic T lymphocyte-defined osteosarcoma antigenic gene Papillomavirus binding factor.
  • The present study was designed to determine the status of HLA class I expression in osteosarcoma and other bone and soft tissue sarcomas.
  • Seventy-four formalin-fixed paraffin-embedded specimens of various bone and soft tissue sarcomas, including 33 osteosarcomas, were stained with the anti-HLA class I monoclonal antibody EMR8-5, which we recently generated.
  • With respect to osteosarcoma, loss or downregulation of HLA class I expression was seen in 13 (52%) of 25 primary tumors and seven (88%) of eight metastatic tumors.
  • Subsequently the prognostic significance of HLA class I expression was analyzed in 21 patients with osteosarcoma who had completed multidrug neoadjuvant chemotherapy and undergone adequate surgery.
  • In contrast, such prognostic significance of HLA class I expression was not found in 15 patients with malignant fibrous histiocytoma of soft tissue.
  • [MeSH-minor] Adolescent. Adult. Child. Female. Histiocytoma, Malignant Fibrous / immunology. Histiocytoma, Malignant Fibrous / pathology. Histiocytoma, Malignant Fibrous / secondary. Humans. Immunoenzyme Techniques. Lymphocytes, Tumor-Infiltrating / immunology. Male. Middle Aged. Prognosis. RNA, Messenger / genetics. RNA, Messenger / metabolism. RNA, Neoplasm / genetics. RNA, Neoplasm / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Sarcoma / immunology. Sarcoma / metabolism. Sarcoma / secondary. Soft Tissue Neoplasms / immunology. Soft Tissue Neoplasms / metabolism. Soft Tissue Neoplasms / pathology. Survival Rate. T-Lymphocytes, Cytotoxic / immunology. beta 2-Microglobulin / genetics. beta 2-Microglobulin / immunology. beta 2-Microglobulin / metabolism

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  • (PMID = 16995877.001).
  • [ISSN] 1347-9032
  • [Journal-full-title] Cancer science
  • [ISO-abbreviation] Cancer Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Histocompatibility Antigens Class I; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 0 / beta 2-Microglobulin
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25. McGrory JE, Pritchard DJ, Arndt CA, Nascimento AG, Remstein ED, Rowland CM: Nonrhabdomyosarcoma soft tissue sarcomas in children. The Mayo Clinic experience. Clin Orthop Relat Res; 2000 May;(374):247-58
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  • [Title] Nonrhabdomyosarcoma soft tissue sarcomas in children. The Mayo Clinic experience.
  • Eighty-six children to 18 years of age were treated for nonrhabdomyosarcoma soft tissue sarcomas of the trunk and extremities.
  • Synovial sarcoma (31), fibrosarcoma (13), malignant fibrous histiocytoma (11), epithelioid sarcoma (10), and clear cell sarcoma (7) were the most common diagnoses.
  • Four patients presented with metastatic disease.
  • Patients were treated with wide removal of the tumor when possible, with judicious use of adjuvant radiation, or with chemotherapy in selected cases.
  • When compared with published data in adults, the prognosis of primary, localized nonrhabdomyosarcoma soft tissue sarcomas in children appears to be more favorable.
  • [MeSH-major] Fibrosarcoma / pathology. Fibrosarcoma / therapy. Histiocytoma, Benign Fibrous / pathology. Histiocytoma, Benign Fibrous / surgery. Sarcoma / pathology. Sarcoma / surgery. Sarcoma, Clear Cell / pathology. Sarcoma, Clear Cell / surgery. Sarcoma, Synovial / pathology. Sarcoma, Synovial / surgery. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Age Factors. Biopsy. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant. Survival Analysis. Treatment Outcome

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  • (PMID = 10818984.001).
  • [ISSN] 0009-921X
  • [Journal-full-title] Clinical orthopaedics and related research
  • [ISO-abbreviation] Clin. Orthop. Relat. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
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26. Domson GF, Shahlaee A, Reith JD, Bush CH, Gibbs CP: Infarct-associated bone sarcomas. Clin Orthop Relat Res; 2009 Jul;467(7):1820-5
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  • Sarcoma associated with bone infarct is a rare condition sparsely reported in the literature.
  • Sixty percent of cases arise about the knee and most are malignant fibrous histiocytomas.
  • Treatment was limb salvage in seven patients, amputation in six, and biopsy alone in two.
  • For patients without metastatic disease at presentation, the 2-year disease-free survival rate was 63% (seven of 11).
  • Two patients received chemotherapy and both were continuously disease-free at last followup.
  • Of 13 patients who received chemotherapy, eight (62%) were continuously disease-free at 24 months compared with 24% (13 of 54) of those who did not receive chemotherapy.
  • Overall, prognosis for these patients is poor, but survival in patients without metastatic disease at diagnosis approaches that of other bone sarcomas.
  • There is a trend suggesting adjuvant chemotherapy combined with appropriate surgery may improve patient outcomes.
  • LEVEL OF EVIDENCE: Level IV, therapeutic study.
  • [MeSH-major] Bone Neoplasms / complications. Histiocytoma, Malignant Fibrous / complications. Infarction / complications. Sarcoma / complications

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  • (PMID = 19229663.001).
  • [ISSN] 1528-1132
  • [Journal-full-title] Clinical orthopaedics and related research
  • [ISO-abbreviation] Clin. Orthop. Relat. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2690751
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27. Collini P, Sorensen PH, Patel S, Blay JY, Issels RD, Maki RG, Eriksson M, del Muro XG: Sarcomas with spindle cell morphology. Semin Oncol; 2009 Aug;36(4):324-37
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  • In the days before the term "high-grade undifferentiated pleomorphic sarcoma" came into use, one of the most common sarcoma diagnoses was "malignant fibrous histiocytoma," and before that, in an era before immunohistochemistry, "fibrosarcoma" was used to describe most sarcomas.
  • As a very broad generalization, sarcomas with a spindle cell microscopic morphology occur in adults and are treated primarily with surgery and often adjuvant or neoadjuvant radiation as primary therapy.
  • In comparison to small round cell sarcomas such as Ewing sarcoma, the use of adjuvant chemotherapy remains controversial, and the sensitivity of these tumors to chemotherapy in the metastatic setting is highly variable.
  • [MeSH-major] Sarcoma / pathology
  • [MeSH-minor] Chemotherapy, Adjuvant. Fibrosarcoma / genetics. Fibrosarcoma / pathology. Gene Fusion. Heat-Shock Response. Humans. Hyperthermia, Induced. Protein Kinase Inhibitors / therapeutic use. Sarcoma, Synovial / genetics. Sarcoma, Synovial / pathology. Translocation, Genetic

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  • (PMID = 19664493.001).
  • [ISSN] 0093-7754
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA47179
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors
  • [Number-of-references] 62
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28. Tejwani A, Kobayashi W, Chen YL, Rosenberg AE, Yoon S, Raskin KA, Rosenthal DI, Nielsen GP, Hornicek FJ, Delaney TF: Management of acral myxoinflammatory fibroblastic sarcoma. Cancer; 2010 Dec 15;116(24):5733-9
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  • [Title] Management of acral myxoinflammatory fibroblastic sarcoma.
  • BACKGROUND: Acral myxoinflammatory fibroblastic sarcoma (AMFS) is a rare, low-grade sarcoma that commonly affects the distal extremities.
  • From the published cases, therapy for AMFS to date has been comprised of excision or amputation, with limited use of radiotherapy (RT) or chemotherapy.
  • METHODS: A retrospective review of all cases of AMFS identified in the Sarcoma Database in the Department of Radiation Oncology at the study institution was conducted.
  • Treatment records and data from follow-up visits of patients were reviewed.
  • The average total dose was 56.4 Gray (Gy).
  • He was free of disease 23 months after his last treatment.
  • No metastatic disease was observed in any of the patients.
  • CONCLUSIONS: Data were consistent with local control of distal extremity sarcomas with resection and RT, suggesting that limb-sparing surgery with this treatment combination is an appropriate option in the limb-sparing control of patients with AMFS, even those with positive surgical margins.
  • [MeSH-major] Extremities. Histiocytoma, Malignant Fibrous / radiotherapy. Histiocytoma, Malignant Fibrous / surgery. Sarcoma / radiotherapy. Sarcoma / surgery

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  • [Copyright] Copyright © 2010 American Cancer Society.
  • (PMID = 20737559.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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29. Skubitz KM, Pambuccian S, Manivel JC, Skubitz AP: Identification of heterogeneity among soft tissue sarcomas by gene expression profiles from different tumors. J Transl Med; 2008 May 06;6:23
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  • [Title] Identification of heterogeneity among soft tissue sarcomas by gene expression profiles from different tumors.
  • The heterogeneity that soft tissue sarcomas (STS) exhibit in their clinical behavior, even within histological subtypes, complicates patient care.
  • Morphologic features are generally good predictors of biologic behavior, however, metastatic propensity, tumor growth, and response to chemotherapy may be determined by gene expression patterns that do not correlate well with morphology.
  • In this study, gene expression in 53 samples of STS and AF [including 16 malignant fibrous histiocytoma (MFH), 9 leiomyosarcoma, 12 liposarcoma, 4 synovial sarcoma, and 12 samples of AF] was determined at Gene Logic Inc. (Gaithersburg, MD) using Affymetrix GeneChip U_133 arrays containing approximately 40,000 genes/ESTs.
  • Hierarchical clustering of the STS using our three previously reported gene sets, each generated subgroups within the STS that for some subtypes correlated with histology, and also suggested the existence of subsets of MFH.
  • In addition, several genes that are targets of some anti-tumor drugs were found to be differentially expressed in particular subsets of STS.

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  • (PMID = 18460215.001).
  • [ISSN] 1479-5876
  • [Journal-full-title] Journal of translational medicine
  • [ISO-abbreviation] J Transl Med
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA106878-03; United States / NCI NIH HHS / CA / R01 CA106878; United States / NCI NIH HHS / CA / R01 CA106878-03; United States / NCI NIH HHS / CA / R01CA106878
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / RNA, Neoplasm
  • [Other-IDs] NLM/ PMC2412854
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30. Chidiac T, Budd GT, Pelley R, Sandstrom K, McLain D, Elson P, Crownover R, Marks K, Muschler G, Joyce M, Zehr R, Bukowski R: Phase II trial of liposomal doxorubicin (Doxil) in advanced soft tissue sarcomas. Invest New Drugs; 2000 Aug;18(3):253-9
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  • [Title] Phase II trial of liposomal doxorubicin (Doxil) in advanced soft tissue sarcomas.
  • PURPOSE: To assess the objective response rate, toxicity experienced, progression-free survival, and overall survival of patients with previously untreated advanced soft tissue sarcomas treated with a liposomal doxorubicin formulation (Doxil).
  • METHODS: Patients with metastatic or recurrent soft tissue sarcoma who had received no prior chemotherapy for advanced disease were treated with liposomal doxorubicin (Doxil) according to a two stage accrual design.
  • Leiomyosarcoma (7/15) and malignant fibrous histiocytoma (2/15) were the most common histologic diagnoses.
  • The median time to progression was 1.9 months (range 0.9-6.2).
  • CONCLUSION: Though well-tolerated, Doxil given according to this dose and schedule to patients with advanced soft tissue sarcoma had no significant therapeutic activity.
  • Future investigations of Doxil in soft tissue sarcomas should use a different schedule and dose.
  • [MeSH-major] Doxorubicin / administration & dosage. Sarcoma / drug therapy
  • [MeSH-minor] Adult. Aged. Drug Carriers. Female. Follow-Up Studies. Humans. Liposomes. Male. Middle Aged

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  • (PMID = 10958594.001).
  • [ISSN] 0167-6997
  • [Journal-full-title] Investigational new drugs
  • [ISO-abbreviation] Invest New Drugs
  • [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 / Drug Carriers; 0 / Liposomes; 80168379AG / Doxorubicin
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31. Vander Salm TJ: Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg; 2000 Apr;12(2):89-100
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  • Primary tumors of the heart, with the exception of atrial myxomas, occur rarely; tumors metastatic to or directly invasive of the heart are far more common.
  • The last 3 may also be malignant.
  • The malignant tumors consist of various sarcomas: myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma, reticulum cell sarcoma, neurofibrosarcoma, and malignant fibrous histiocytoma.
  • The echocardiographic appearance may also allow quite accurate prediction of the tumor type and whether it is malignant or benign.
  • Magnetic resonance imaging serves as the next most important test where the density of T1 and T2 images may allow tumor cell type identification.
  • Many of the malignant tumors cannot be resected completely, either because of the extent of local spread and invasion or because of the frequent distant metastases.
  • For patients with unresectable sarcomas, radiation and chemotherapy may be used, but without great expectation of successful results.

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  • (PMID = 10807431.001).
  • [ISSN] 1043-0679
  • [Journal-full-title] Seminars in thoracic and cardiovascular surgery
  • [ISO-abbreviation] Semin. Thorac. Cardiovasc. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 69
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32. Antoniello L, Cecchetto G, Carli M, Dall'Igna P, Bisogno G, Lo Piccolo R, Gigante C, Zanetti I, Guglielmi M: [Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88]. Pediatr Med Chir; 2003 Jul-Aug;25(4):255-60
MedlinePlus Health Information. consumer health - Soft Tissue Sarcoma.

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  • [Title] [Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88].
  • Aim of the study was to evaluate the role of mutilating surgery in the patients with non chemosensitive soft tissue sarcomas (STS) registered in the Italian Studies.
  • HISTOLOGY: fibrosarcoma 29, Malignant Perpheral Nerve Sheath Tumors (MPNST) 40, malignant fibrous histiocytoma 5, hemangiopericytoma 6, leiomyosarcoma 4, others 20, STS nos 10.
  • The mutilating procedure was carried out at diagnosis in 6 cases (4 in RMS-79 and 2 in RMS-and 88) and achieved radicality in 5/6 cases.
  • It was performed after ineffective chemotherapy (CT) in 5 pts (1 in RMS-79 and 4 in RMS-88).
  • III pts 1 is alive NED and 4 died (3 of metastatic spread and 1 of 2nd tumor); the pt amputated after repeated local relapses (Gr.
  • CONCLUSIONS: In the RMS-79 study the mutilations were frequent and were performed at diagnosis in several cases; this trend decreased in the 2nd study in which chemotherapy was attempted in most of the patients.
  • [MeSH-major] Sarcoma / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 15070267.001).
  • [ISSN] 0391-5387
  • [Journal-full-title] La Pediatria medica e chirurgica : Medical and surgical pediatrics
  • [ISO-abbreviation] Pediatr Med Chir
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
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33. Ferraresi V, Ciccarese M, Cercato MC, Nuzzo C, Zeuli M, Di Filippo F, Giannarelli D, Cognetti F: Gemcitabine at fixed dose-rate in patients with advanced soft-tissue sarcomas: a mono-institutional phase II study. Cancer Chemother Pharmacol; 2008 Dec;63(1):149-55
MedlinePlus Health Information. consumer health - Soft Tissue Sarcoma.

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  • [Title] Gemcitabine at fixed dose-rate in patients with advanced soft-tissue sarcomas: a mono-institutional phase II study.
  • PURPOSE: To explore the clinical activity and toxicity of gemcitabine infused at the fixed dose of 10 mg/m(2)/min over 100 min in patients with soft tissue sarcomas (STSs).
  • PATIENTS AND METHODS: Fourteen patients with advanced locally unresectable and/or metastatic, pretreated STSs (seven leiomyosarcoma, three malignant schwannoma, one synovialsarcoma, one malignant fibrous histiocytoma, one endometrial stromal cell sarcoma, one undifferentiated) were treated with gemcitabine 10 mg/m(2)/min/week over 100 min given for 3 weeks out of 4.
  • The median number of previous medical treatments for advanced disease was 1 (range 1-2).
  • Treatment was well tolerated and the main causes of dose-reduction or omission/delay were hematological and liver toxicities.
  • One patient (7%; 95% confidence interval: 0.2-33.9%) with a metastatic uterine leiomyosarcoma obtained a partial response that lasted for 6.5 months.
  • The median time to progression was 3.1 months (range 1.0-9.5).
  • [MeSH-major] Antimetabolites, Antineoplastic / therapeutic use. Deoxycytidine / analogs & derivatives. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Combined Modality Therapy. Disease Progression. Dose-Response Relationship, Drug. Female. Gastrointestinal Diseases / chemically induced. Hematologic Diseases / chemically induced. Humans. Male. Middle Aged

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  • (PMID = 18351342.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine
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34. Stoeckle E, Coindre JM, Bonvalot S, Kantor G, Terrier P, Bonichon F, Nguyen Bui B, French Federation of Cancer Centers Sarcoma Group: Prognostic factors in retroperitoneal sarcoma: a multivariate analysis of a series of 165 patients of the French Cancer Center Federation Sarcoma Group. Cancer; 2001 Jul 15;92(2):359-68
MedlinePlus Health Information. consumer health - Soft Tissue Sarcoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic factors in retroperitoneal sarcoma: a multivariate analysis of a series of 165 patients of the French Cancer Center Federation Sarcoma Group.
  • BACKGROUND: Surgery is the main prognostic factor in retroperitoneal sarcoma.
  • However, despite progress, surgery alone is rarely curative, and analysis of the causes of failures and of other prognostic factors are warranted to ascertain treatment orientations.
  • METHODS: Data of patients treated from 1.80 to 12.94 for primary retroperitoneal sarcoma were extracted from the French Federation of Cancer Centers Sarcoma Group registry.
  • Liposarcoma, leiomyosarcoma, and malignant fibrous histiocytoma represented 66% of the tumors.
  • Multimodality treatment included surgery (150 patients), radiotherapy (92 patients), and chemotherapy (77 patients).
  • The main prognostic factors for survival were initial metastases and surgery, which represented the major treatment-linked factor.
  • High-grade of tumors affected local recurrence, metastatic recurrence, and survival.
  • CONCLUSIONS: Aggressive surgery remains mandatory in retroperitoneal sarcoma, but a randomized trial is needed to evaluate the place of radiotherapy for local control.
  • [MeSH-major] Neoplasm Recurrence, Local. Retroperitoneal Neoplasms / pathology. Sarcoma / pathology

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  • [Copyright] Copyright 2001 American Cancer Society.
  • (PMID = 11466691.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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35. Bafaloukos D, Papadimitriou C, Linardou H, Aravantinos G, Papakostas P, Skarlos D, Kosmidis P, Fountzilas G, Gogas H, Kalofonos C, Dimopoulos AM: Combination of pegylated liposomal doxorubicin (PLD) and paclitaxel in patients with advanced soft tissue sarcoma: a phase II study of the Hellenic Cooperative Oncology Group. Br J Cancer; 2004 Nov 1;91(9):1639-44
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  • [Title] Combination of pegylated liposomal doxorubicin (PLD) and paclitaxel in patients with advanced soft tissue sarcoma: a phase II study of the Hellenic Cooperative Oncology Group.
  • Patients with soft tissue sarcoma (STS), even after complete local disease control, often relapse locally or with distant metastases.
  • This multicenter phase II study was conducted to evaluate the safety and efficacy of the combination of pegylated liposomal doxorubicin (PLD) and paclitaxel, as first-line treatment in patients with advanced STS.
  • In all, 42 patients with locally advanced or metastatic STS, median age 54 years and median Eastern Cooperative Oncology Group performance status (PS) 1 were treated with PLD 45 mg m(-2) and paclitaxel 150 mg m(-2), every 28 days for a total of six cycles.
  • Histological types included mainly leiomyosarcomas (43%), malignant fibrous histiocytomas (14%) and liposarcomas (12%).
  • At median follow-up 41.5 months, median time to progression (TTP) was 5.7 months with median overall survival (OS) 13.2 months.
  • There were no treatment-related deaths.
  • The combination of PLD and paclitaxel is a safe and well-tolerated regimen demonstrating modest efficacy as first-line treatment in patients with advanced STS.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Histiocytoma, Benign Fibrous / drug therapy. Leiomyosarcoma / drug therapy. Liposarcoma / drug therapy. Neoplasm Recurrence, Local / drug therapy. Sarcoma / drug therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Disease Progression. Doxorubicin / administration & dosage. Female. Humans. Liposomes. Liver Neoplasms / drug therapy. Liver Neoplasms / secondary. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Lymphatic Metastasis / pathology. Male. Middle Aged. Paclitaxel / administration & dosage. Safety. Survival Rate. Time Factors. Treatment Outcome

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  • (PMID = 15494721.001).
  • [ISSN] 0007-0920
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Liposomes; 80168379AG / Doxorubicin; P88XT4IS4D / Paclitaxel
  • [Other-IDs] NLM/ PMC2409958
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36. Gebhard S, Coindre JM, Michels JJ, Terrier P, Bertrand G, Trassard M, Taylor S, Château MC, Marquès B, Picot V, Guillou L: Pleomorphic liposarcoma: clinicopathologic, immunohistochemical, and follow-up analysis of 63 cases: a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol; 2002 May;26(5):601-16
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  • [Title] Pleomorphic liposarcoma: clinicopathologic, immunohistochemical, and follow-up analysis of 63 cases: a study from the French Federation of Cancer Centers Sarcoma Group.
  • The clinicopathologic and immunohistochemical features of 63 pleomorphic liposarcomas are presented.
  • Histologically, lesions show a varying combination of lipogenic and nonlipogenic areas characterized by malignant fibrous histiocytoma-like, round cell liposarcoma-like, and/or epithelioid/carcinoma-like features.
  • Treatment procedures in 51 patients consisted of simple tumorectomy (16) and wide excision (33).
  • Five and 31 patients received neoadjuvant and adjuvant chemotherapy and/or radiation therapy, respectively.
  • Seventeen patients (35%) died of disease, of whom none was metastatic at diagnosis.
  • In conclusion, pleomorphic liposarcoma is a rare, often deep-seated and limb-based aggressive and metastasizing neoplasm of late adulthood.
  • [MeSH-major] Liposarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / analysis. Disease-Free Survival. Female. Humans. Immunohistochemistry. Male. Middle Aged. Neoplasm Proteins / analysis. Survival Rate. Treatment Outcome

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  • (PMID = 11979090.001).
  • [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
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins
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