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1. Grunewald TG, von Luettichau I, Weirich G, Wawer A, Behrends U, Prodinger PM, Jundt G, Bielack SS, Gradinger R, Burdach S: Sclerosing epithelioid fibrosarcoma of the bone: a case report of high resistance to chemotherapy and a survey of the literature. Sarcoma; 2010;2010:431627
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  • [Title] Sclerosing epithelioid fibrosarcoma of the bone: a case report of high resistance to chemotherapy and a survey of the literature.
  • Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue sarcoma mostly occurring in extraosseous sites.
  • SEF represents a clinically challenging entity especially because no standardized treatment regimens are available.
  • The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction.
  • Histopathological analysis of the resected tumor showed >90% vital tumor cells suggesting no response to chemotherapy.
  • Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma.
  • Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF.

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  • (PMID = 20396630.001).
  • [ISSN] 1369-1643
  • [Journal-full-title] Sarcoma
  • [ISO-abbreviation] Sarcoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2853979
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2. Cecchetto G, Alaggio R, Dall'Igna P, Bisogno G, Ferrari A, Gigante C, Casanova M, Sotti G, Zanetti I, Carli M: Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies. Cancer; 2005 Nov 1;104(9):2006-12
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  • [Title] Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.
  • BACKGROUND: Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity.
  • The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24-year period.
  • Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy.
  • Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5.
  • Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1.
  • Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors.
  • Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor.
  • CONCLUSIONS: Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns-NRSTS.
  • [MeSH-major] Extremities. Sarcoma / diagnosis
  • [MeSH-minor] Adolescent. Age Factors. Chemotherapy, Adjuvant. Child. Female. Humans. Italy. Male. Neoadjuvant Therapy. Survival Analysis

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  • [Copyright] (c) 2005 American Cancer Society.
  • (PMID = 16161038.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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3. Bisogno G, Sotti G, Nowicki Y, Ferrari A, Garaventa A, Zanetti I, Favre C, Schiavetti A, Tamaro P, Carli M: Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group. Cancer; 2004 Apr 15;100(8):1758-65
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  • [Title] Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group.
  • BACKGROUND: Survivors of childhood malignancies have an increased risk of developing second malignant neoplasms (SMN) due to their prior treatment and/or genetic susceptibility.
  • A small proportion of SMNs are soft tissue sarcomas (STS), whose prognosis is generally thought to be poor, though publications on such patients' treatment and outcome is limited.
  • METHODS: The authors analyzed 25 patients who were registered for the Italian Cooperative Group protocols for pediatric STS from 1979 to 2000.
  • The primary tumor was STS in five patients; Hodgkin disease in five patients; leukemia in four patients; retinoblastoma, neuroblastoma, and Wilms tumor in two patients each; and other tumor types in five patients.
  • SMNs occurred after a median of 8 years (range, 1.9-15.0 years) and included rhabdomyosarcoma (RMS) in 4 patients, malignant peripheral nerve sheath tumor in 4 patients, extraosseous Ewing family tumor (EFT) in 4 patients, leiomyosarcoma in 3 patients, fibrosarcoma in 2 patients, synovial sarcoma in 2 patients, and other tumor types in 6 patients.
  • Treatment generally was administered according to the guidelines for primary STS.
  • RESULTS: Seven non-RMS patients with STS underwent surgery alone, whereas 18 patients received chemotherapy and 8 patients received radiotherapy.
  • Fifteen patients were alive in complete remission of their SMN at the time of last follow-up.
  • Responses to chemotherapy and survival were satisfactory for patients with tumors such as RMS and EFT.
  • Complete tumor resection was correlated with a favorable prognosis in patients with other types of STS and in patients with postirradiation sarcoma.
  • Two patients developed a third malignancy.
  • CONCLUSIONS: Although prior treatment may hinder the management of these patients, pediatric STS second malignancies can be cured using the same strategies used for de novo pediatric sarcomas.
  • Long-term follow-up is mandatory given the risks of further malignancies and more severe, treatment-related side effects.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neoplasms, Second Primary. Sarcoma / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Prognosis. Survival Analysis. Treatment Outcome

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  • [Copyright] Copyright 2004 American Cancer Society.
  • (PMID = 15073867.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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4. Gosau M, Draenert FG, Winter WA, Mueller-Hoecker J, Driemel O: Fibrosarcoma of the childhood mandible. Head Face Med; 2008;4:21
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  • [Title] Fibrosarcoma of the childhood mandible.
  • A case of low-grade intraosseous fibrosarcoma of the mandible in a 9-year-old girl is described.
  • The patient underwent pre-surgical chemotherapy which was abandoned as unsuccessful after two cycles.
  • No adjuvant therapy was given and lymph node dissection was not performed.
  • No signs of recurrences or metastasis have been observed after a follow up time of 3 years so far.
  • This article is presented to document the rarity of fibrosarcomas in the jaws of children and emphasizes the possible changes in the appearance of radiological imaging under tumour progression.
  • [MeSH-major] Fibrosarcoma / pathology. Fibrosarcoma / surgery. Mandibular Neoplasms / pathology. Mandibular Neoplasms / surgery
  • [MeSH-minor] Biopsy, Needle. Child. Female. Follow-Up Studies. Humans. Immunohistochemistry. Neoplasm Staging. Radiography, Panoramic. Risk Assessment. Surgical Procedures, Operative / methods. Tomography, X-Ray Computed. Treatment Outcome

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  • [Journal-full-title] Head & face medicine
  • [ISO-abbreviation] Head Face Med
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  • [Other-IDs] NLM/ PMC2556660
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5. Cecchetto G, Carli M, Alaggio R, Dall'Igna P, Bisogno G, Scarzello G, Zanetti I, Durante G, Inserra A, Siracusa F, Guglielmi M, Italian Cooperative Group: Fibrosarcoma in pediatric patients: results of the Italian Cooperative Group studies (1979-1995). J Surg Oncol; 2001 Dec;78(4):225-31
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  • [Title] Fibrosarcoma in pediatric patients: results of the Italian Cooperative Group studies (1979-1995).
  • BACKGROUND AND OBJECTIVES: Fibrosarcoma is a rare soft tissue sarcoma (STS) that has two peaks of incidence in pediatric patients: one in infants and young children (infantile fibrosarcoma), another in older children ("adult type" fibrosarcoma).
  • The purpose of this study was to describe the clinical features and the treatment results in patients affected by fibrosarcoma enrolled in two consecutive studies run by the STS-Italian Cooperative Group (ICG) between 1979 and 1995.
  • The cut-off point between the two forms was considered the age of 2 years: 14 patients were affected by infantile and 11 by adult type of fibrosarcoma.
  • The therapeutic guidelines were not strict and not different for the two forms: patients with initial macroscopic excision (Gr I-II) were given IVA or VAC; Gr III had VAC or VAIA and subsequent excision, if feasible.
  • Radiation therapy (RT) was delivered in patients > 3 years with microscopic (42 Gy) and macroscopic (54 Gy) residuals.
  • CONCLUSIONS: The complete excision at diagnosis was the treatment of choice and was related to the best outcome.
  • Microscopical residuals were difficult to treat with chemo-radiotherapy in both forms of fibrosarcoma.
  • Neoadjuvant chemotherapy (CT) obtained a partial remission (PR) only in three of eight cases, while no conclusions concerning the efficacy of CT for infantile forms are possible.
  • [MeSH-major] Fibrosarcoma / radiotherapy. Soft Tissue Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Age Factors. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Radiotherapy Dosage. Sarcoma / radiotherapy. Sarcoma / surgery. Survival Analysis. Treatment Outcome

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  • [Copyright] Copyright 2001 Wiley-Liss, Inc.
  • (PMID = 11745814.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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6. Russell H, Hicks MJ, Bertuch AA, Chintagumpala M: Infantile fibrosarcoma: clinical and histologic responses to cytotoxic chemotherapy. Pediatr Blood Cancer; 2009 Jul;53(1):23-7
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  • [Title] Infantile fibrosarcoma: clinical and histologic responses to cytotoxic chemotherapy.
  • BACKGROUND: Infantile fibrosarcoma (IF) is a rare soft tissue sarcoma that presents either at birth or in the first year of life.
  • Complete surgical resection is usually curative but chemotherapy may shrink the tumor to facilitate complete resection.
  • This report describes the histologic changes and outcomes in four patients with IF treated with chemotherapy and surgical resection.
  • PROCEDURE: A retrospective review was performed of patients treated between 2000 and 2007.
  • Two lower extremity tumors had only modest changes in dimensions but upon resection, the tumor bed contained fibrous tissue with exaggerated small caliber vessels.
  • The fourth infant developed metastatic lesions in the central nervous system, orbits, lungs, and kidney after complete removal of the primary tumor.
  • The metastatic lesions responded to chemotherapy and have remained stable for over 3 years.
  • In patients where a clinical response is not apparent, cytoreduction of the tumor and replacement with fibrotic and fibrovascular tissue may facilitate gross-total resection.
  • The chemotherapy-responsiveness of this tumor may abrogate unfavorable features such as metastatic or residual tumor.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibrosarcoma / drug therapy. Fibrosarcoma / pathology
  • [MeSH-minor] Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Female. Head and Neck Neoplasms / diagnosis. Head and Neck Neoplasms / drug therapy. Head and Neck Neoplasms / pathology. Head and Neck Neoplasms / surgery. Humans. Infant. Infant, Newborn. Leg. Lymphatic Metastasis. Male. Retrospective Studies. Treatment Outcome. Vincristine / administration & dosage

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  • [Copyright] Copyright 2009 Wiley-Liss, Inc.
  • (PMID = 19340853.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide
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7. Demir HA, Akyüz C, Varan A, Ergen FB, Büyükpamukçu M: Right foot congenital infantile fibrosarcoma treated only with chemotherapy. Pediatr Blood Cancer; 2010 Apr;54(4):618-20
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  • [Title] Right foot congenital infantile fibrosarcoma treated only with chemotherapy.
  • Congenital infantile fibrosarcoma (CIF) is a rare tumor in childhood.
  • The patient was treated only with VAC chemotherapy and is able to walk normally.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibrosarcoma / congenital. Fibrosarcoma / drug therapy. Foot / pathology. Soft Tissue Neoplasms / congenital. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Cyclophosphamide / therapeutic use. Dactinomycin / therapeutic use. Humans. Infant, Newborn. Male. Vincristine / therapeutic use

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  • [CommentIn] Pediatr Blood Cancer. 2010 Oct;55(4):770; author reply 771 [20533521.001]
  • (PMID = 19998472.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide; VAC protocol
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8. DeComas AM, Heinrich SD, Craver R: Infantile fibrosarcoma successfully treated with chemotherapy, with occurrence of calcifying aponeurotic fibroma and pleomorphic/spindled celled lipoma at the site 12 years later. J Pediatr Hematol Oncol; 2009 Jun;31(6):448-52
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  • [Title] Infantile fibrosarcoma successfully treated with chemotherapy, with occurrence of calcifying aponeurotic fibroma and pleomorphic/spindled celled lipoma at the site 12 years later.
  • The treatment of infantile fibrosarcoma has traditionally been wide resection.
  • Chemotherapy has been investigated as an adjuvant and primary treatment in cases in which surgery would cause unacceptable morbidity.
  • Recurrences normally occur within a year of completion of the chemotherapy and display the same histology.
  • We present a child with an infantile fibrosarcoma of the elbow, successfully treated with chemotherapy alone, who developed a calcifying aponeurotic fibroma and a spindle cell/pleomorphic lipoma at the tumor site 12 years later.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Fibroma / etiology. Fibrosarcoma / drug therapy. Lipoma / etiology. Neoplasms, Second Primary / etiology. Soft Tissue Neoplasms / drug therapy

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  • (PMID = 19648795.001).
  • [ISSN] 1536-3678
  • [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
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9. Kaminski JM, Yang CC, Yagmai F, Movsas B, Lee M, Barrett JT: Intracranial fibrosarcoma arising 5 years after chemotherapy alone for glioblastoma multiforme in a child. Pediatr Neurosurg; 2000 Nov;33(5):257-260
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  • [Title] Intracranial fibrosarcoma arising 5 years after chemotherapy alone for glioblastoma multiforme in a child.
  • We present a child diagnosed with glioblastoma multiforme during infancy and successfully treated with the 'eight-in-one' chemotherapy regimen, who developed an intracranial fibrosarcoma 5 years later.
  • After resection of the fibrosarcoma, she received cranial radiation therapy and high dose chemotherapy with bone marrow transplant.
  • [MeSH-major] Brain Neoplasms / drug therapy. Brain Neoplasms / pathology. Fibrosarcoma / pathology. Frontal Lobe / pathology. Glioblastoma / drug therapy. Neoplasms, Second Primary / pathology. Occipital Lobe / pathology
  • [MeSH-minor] Bone Marrow Transplantation. Combined Modality Therapy. Female. Humans. Infant. Magnetic Resonance Imaging. Time Factors

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  • [Copyright] Copyright 2001 S. Karger AG, Basel.
  • (PMID = 11155063.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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10. Loh ML, Ahn P, Perez-Atayde AR, Gebhardt MC, Shamberger RC, Grier HE: Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children's Hospital, Boston. J Pediatr Hematol Oncol; 2002 Dec;24(9):722-6
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  • [Title] Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children's Hospital, Boston.
  • PURPOSE: To retrospectively evaluate the treatment and outcome of patients diagnosed with infantile fibrosarcoma at the Dana-Farber Cancer Institute and Children's Hospital, Boston.
  • PATIENTS AND METHODS: Between 1982 and 1998, a total of 11 infants were diagnosed pathologically with infantile fibrosarcoma.
  • A retrospective chart review was conducted to determine the extent of surgical therapy and chemotherapy required for a favorable clinical outcome.
  • Two patients received chemotherapy only after limited biopsy or subtotal resection and are alive with no evidence of disease 8 and 18 years from diagnosis.
  • Four patients had limited biopsies followed by chemotherapy with delayed resection.
  • One of these four patients had negative margins and received no further chemotherapy.
  • The other three of these patients had positive microscopic margins; two of them received postoperative chemotherapy while the third did not.
  • Two patients had progressive disease within 7 and 10 months of diagnosis while on chemotherapy after subtotal resections.
  • Seven patients had archived or frozen tissue available for molecular analysis.
  • Six patients had characteristic trisomies previously reported to be associated with infantile fibrosarcoma.
  • CONCLUSIONS: Previously reported series of treatment outcomes in infantile fibrosarcoma have been limited to very few patients due to the rare occurrence of this tumor.
  • In our experience, initial chemotherapy combined with surgery has been successful for most cases.
  • The role of additional chemotherapy for microscopic margins after local control is not clear.
  • We propose a uniform approach to treatment to gather clinical and biologic information about this rare and curable disease.
  • [MeSH-major] Fibrosarcoma / drug therapy. Fibrosarcoma / surgery
  • [MeSH-minor] Boston. Child. Combined Modality Therapy. Disease Progression. Disease-Free Survival. Female. Follow-Up Studies. Humans. Infant. Infant, Newborn. Male. Registries. Retrospective Studies. Time Factors

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  • (PMID = 12468912.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Grant] United States / NICHD NIH HHS / HD / HD 28825; United States / NCI NIH HHS / CA / K23 CA 80915
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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11. Loeb DM, Hill DA, Dome JS: Complete response of recurrent cellular congenital mesoblastic nephroma to chemotherapy. J Pediatr Hematol Oncol; 2002 Aug-Sep;24(6):478-81
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  • [Title] Complete response of recurrent cellular congenital mesoblastic nephroma to chemotherapy.
  • The sensitivity of this tumor to chemotherapy is unknown.
  • The recent description of a t(12;15)(p13;q25) chromosomal translocation in both cellular CMN and congenital infantile fibrosarcoma suggests that these entities have a common pathogenesis, and that cellular CMN might respond to chemotherapy like congenital infantile fibrosarcoma does.
  • The authors describe three patients with recurrent cellular CMN who showed a complete response to chemotherapy.
  • Based on these patients and a review of the literature, the authors suggest that chemotherapy be considered as a part of the therapy for recurrent or unresectable cellular CMN.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Neoplasm Recurrence, Local / drug therapy. Nephroma, Mesoblastic / drug therapy


12. Akyüz C, Sari N, Vargel I, Gedikoglu G, Haliloglu M, Büyükpamukçu M: A newborn with infantile fibrosarcoma of foot: treatment with chemotherapy and extremity-sparing surgery. J Perinatol; 2010 Jan;30(1):63-5
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  • [Title] A newborn with infantile fibrosarcoma of foot: treatment with chemotherapy and extremity-sparing surgery.
  • Infantile fibrosarcoma represents less than 1% of all childhood cancers, but it is the most common soft-tissue sarcoma in those under 1 year of age.
  • We report an infant with congenital infantile fibrosarcoma diagnosed as hemangiopericytoma.
  • He was treated with chemotherapy and extremity-sparing surgery.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols. Fibrosarcoma / drug therapy. Fibrosarcoma / surgery. Foot Diseases / congenital. Hemangiopericytoma / drug therapy. Hemangiopericytoma / surgery

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  • (PMID = 20038940.001).
  • [ISSN] 1476-5543
  • [Journal-full-title] Journal of perinatology : official journal of the California Perinatal Association
  • [ISO-abbreviation] J Perinatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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13. Kurkchubasche AG, Halvorson EG, Forman EN, Terek RM, Ferguson WS: The role of preoperative chemotherapy in the treatment of infantile fibrosarcoma. J Pediatr Surg; 2000 Jun;35(6):880-3
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  • [Title] The role of preoperative chemotherapy in the treatment of infantile fibrosarcoma.
  • Infantile fibrosarcoma (IFS) is a rare tumor most often affecting the extremities of infants and young children.
  • The dramatic response in 2 recent cases to preoperative chemotherapy, given in an attempt to avoid amputation, prompted this report and a review of the literature.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Arm. Fibrosarcoma / congenital. Fibrosarcoma / surgery
  • [MeSH-minor] Combined Modality Therapy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Humans. Infant, Newborn. Male. Vincristine / administration & dosage

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  • (PMID = 10873030.001).
  • [ISSN] 0022-3468
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide; VAC protocol
  • [Number-of-references] 15
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14. Bisogno G, Roganovic J, Carli M, Scarzello G, Calderone M, Faggin R, Perilongo G: Primary intracranial fibrosarcoma. Childs Nerv Syst; 2002 Nov;18(11):648-51
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  • [Title] Primary intracranial fibrosarcoma.
  • INTRODUCTION: Primary fibrosarcomas of the brain are very rare tumors, so that information regarding the treatment is scarce.
  • We report the contributions that different therapeutic options made to the treatment of a child with one of these aggressive tumors.
  • CASE REPORT: A 13-year-old boy underwent a complete resection of a left temporo-parietal mass that had been diagnosed as a fibrosarcoma by two independent pathologists.
  • Adjuvant chemotherapy with vincristine, actinomycin-D, ifosfamide and Adriamycin was started, but after 3 months tumor relapse was evident.
  • The boy subsequently received radiation therapy during which there was evidence of progressive tumor shrinkage.
  • CONCLUSION: Our experience supports the importance of total resection followed by radiation therapy, and radiotherapy should be started as soon as possible after surgical resection, rather than administering chemotherapy first.
  • [MeSH-major] Brain Neoplasms / therapy. Fibrosarcoma / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Gliosis / surgery. Humans. Male. Neoplasm Recurrence, Local. Treatment Outcome

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  • (PMID = 12420128.001).
  • [ISSN] 0256-7040
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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15. Ferguson WS: Advances in the adjuvant treatment of infantile fibrosarcoma. Expert Rev Anticancer Ther; 2003 Apr;3(2):185-91
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  • [Title] Advances in the adjuvant treatment of infantile fibrosarcoma.
  • Infantile fibrosarcoma is a rare soft tissue tumor, predominately affecting young infants.
  • Neoadjuvant chemotherapy will cause many tumors to shrink significantly, allowing less mutilating surgical resections to be performed--this is the current recommendation where immediate surgical removal cannot be accomplished without unacceptable morbidity.
  • In contrast, there is no defined role for adjuvant chemotherapy or radiation following complete surgical resection.
  • [MeSH-major] Fibrosarcoma / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Chemotherapy, Adjuvant. Combined Modality Therapy. Humans. Infant

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  • (PMID = 12722878.001).
  • [ISSN] 1473-7140
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 36
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16. Van Winkle P, Angiolillo A, Krailo M, Cheung YK, Anderson B, Davenport V, Reaman G, Cairo MS: Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children's Cancer Group (CCG) experience. Pediatr Blood Cancer; 2005 Apr;44(4):338-47
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  • [Title] Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children's Cancer Group (CCG) experience.
  • BACKGROUND: The prognosis for children with recurrent/refractory sarcomas is poor.
  • We determined the overall response rate (ORR) and overall survival (OS) of children with recurrent/refractory sarcomas who were given ifosfamide, carboplatin, and etoposide (ICE) in three Children's Cancer Group (CCG) phase I/II trials.
  • PROCEDURE: Children with recurrent/refractory sarcoma were treated with ifosfamide (1,800 mg/m2/day on day 0-4), carboplatin (400 mg/m2/day on day 0-1), etoposide (100 mg/m2/day on day 0-4) and either rhG-CSF (10 microg/kg/day vs. 5 microg/kg/day, CCG-0894, 71 patients), PIXY321 (500-1,000 microg/m2/day, CCG-0924, 14 patients), or rhG-CSF (5 microg/kg/day) and IL-6 (2.5-5 microg/kg/day, CCG-0931, 12 patients).
  • Tumor types were osteosarcoma (OTS) (n = 34), rhabdomyosarcoma (n = 27), Ewing sarcoma (EWS) (n = 21), soft tissue sarcoma-not otherwise specified (n = 5), undifferentiated sarcoma (n = 6), fibrosarcoma (n = 2), peripheral primitive neuroectodermal tumor (n = 1), and extraosseous Ewing (n = 1).
  • CONCLUSIONS: The ORR to ICE reinduction chemotherapy in children with recurrent/refractory sarcoma was 51%.
  • OS of 1 and 2 years appeared significantly improved in patients who had CR or PR following ICE reinduction therapy or who had rhabdomyosarcoma with embryonal histology.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Sarcoma / drug therapy
  • [MeSH-minor] Adolescent. Adult. Carboplatin / administration & dosage. Child. Child, Preschool. Colony-Stimulating Factors / administration & dosage. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Male. Multivariate Analysis. Proportional Hazards Models. Recurrence. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / mortality. Survival Rate

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  • (PMID = 15503297.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Colony-Stimulating Factors; 6PLQ3CP4P3 / Etoposide; BG3F62OND5 / Carboplatin; UM20QQM95Y / Ifosfamide
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17. Yalçin B, Leblebicioğlu G, Güler E, Gedikoğlu G, Kutluk MT: Congenital infantile fibrosarcoma of the thigh in a newborn. Tumori; 2001 Nov-Dec;87(6):436-8
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  • [Title] Congenital infantile fibrosarcoma of the thigh in a newborn.
  • Congenital/infantile fibrosarcoma occurs frequently in the first year of life and differs from fibrosarcoma in adulthood.
  • The clinical course of congenital/infantile fibrosarcoma is more favorable and metastatic spread is rare.
  • While adult fibrosarcomas are common in the thigh, congenital/infantile fibrosarcomas affect chiefly the distal portions of the extremities.
  • Standard treatment is primarily wide surgical excision.
  • Chemotherapy may be given as neoadjuvant or adjuvant treatment in order to avoid the morbidity associated with wide excision.
  • In this case report we present our experience of a newborn affected by congenital/infantile fibrosarcoma of the left thigh.
  • [MeSH-major] Fibrosarcoma / congenital. Fibrosarcoma / diagnosis. Thigh
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Diagnosis, Differential. Fatal Outcome. Humans. Infant, Newborn. Male. Neoadjuvant Therapy

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  • (PMID = 11989601.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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18. Ramphal R, Manson D, Viero S, Zielenska M, Gerstle T, Pappo A: Retroperitoneal infantile fibrosarcoma: clinical, molecular, and therapeutic aspects of an unusual tumor. Pediatr Hematol Oncol; 2003 Dec;20(8):635-42
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  • [Title] Retroperitoneal infantile fibrosarcoma: clinical, molecular, and therapeutic aspects of an unusual tumor.
  • The authors describe a patient with a large retroperitoneal infantile fibrosarcoma that responded well to preoperative chemotherapy, which subsequently facilitated the complete surgical resection of the mass.
  • [MeSH-major] Fibrosarcoma / diagnosis. Retroperitoneal Neoplasms / diagnosis

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  • (PMID = 14578034.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / ETV6-NTRK3 fusion protein, human; 0 / Oncogene Proteins, Fusion
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19. Savas C, Candir O, Ozguner F: Acute respiratory distress due to fibrosarcoma of the carina in a child. Pediatr Pulmonol; 2004 Oct;38(4):355-7
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  • [Title] Acute respiratory distress due to fibrosarcoma of the carina in a child.
  • We report a 12-year-old boy with primary bronchopulmonary fibrosarcoma (PBPF).
  • Successful treatment was achieved with surgical resection of the lesion followed by radiotherapy and chemotherapy.
  • [MeSH-major] Fibrosarcoma / pathology. Lung Diseases / etiology. Lung Neoplasms / pathology
  • [MeSH-minor] Acute Disease. Bronchi / pathology. Bronchoscopy. Child. Cough / etiology. Dyspnea / etiology. Hemoptysis / etiology. Humans. Lung / pathology. Lung / radiography. Male. Radiography. Treatment Outcome

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  • (PMID = 15334517.001).
  • [ISSN] 8755-6863
  • [Journal-full-title] Pediatric pulmonology
  • [ISO-abbreviation] Pediatr. Pulmonol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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20. McCahon E, Sorensen PH, Davis JH, Rogers PC, Schultz KR: Non-resectable congenital tumors with the ETV6-NTRK3 gene fusion are highly responsive to chemotherapy. Med Pediatr Oncol; 2003 May;40(5):288-92
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  • [Title] Non-resectable congenital tumors with the ETV6-NTRK3 gene fusion are highly responsive to chemotherapy.
  • BACKGROUND: Recently, the ETV6-NTRK3 gene fusion has been identified in both infantile fibrosarcoma and cellular mesoblastic nephroma.
  • For both these tumors standard curative treatment has been primarily surgical with wide local excision.
  • PROCEDURE: This report discusses three infants with congenital tumors, two congenital fibrosarcomas, and one atypical congenital mesoblastic nephroma, not easily amenable to surgical intervention.
  • RESULTS: All three were treated with pre-operative chemotherapy with excellent responses negating the need for amputation in two patients.
  • In this group of patients pre-operative chemotherapy may abrogate the need for morbid surgical procedures.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. DNA-Binding Proteins / genetics. Fibrosarcoma / congenital. Kidney Neoplasms / congenital. Nephroma, Mesoblastic / congenital. Receptor, trkC / genetics. Repressor Proteins / genetics. Soft Tissue Neoplasms / congenital

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 12652616.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / ETS translocation variant 6 protein; 0 / Genetic Markers; 0 / Proto-Oncogene Proteins c-ets; 0 / Repressor Proteins; EC 2.7.10.1 / Receptor, trkC
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21. Cosetti M, Wexler LH, Calleja E, Trippett T, LaQuaglia M, Huvos AG, Gerald W, Healey JH, Meyers PA, Gorlick R: Irinotecan for pediatric solid tumors: the Memorial Sloan-Kettering experience. J Pediatr Hematol Oncol; 2002 Feb;24(2):101-5
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  • [Title] Irinotecan for pediatric solid tumors: the Memorial Sloan-Kettering experience.
  • This report describes an institutional experience with irinotecan for the treatment of pediatric solid tumors.
  • RESULTS: Of the 19 patients evaluable for response, four achieved an objective response, including two complete responses and one partial response among four patients with rhabdomyosarcoma and one additional patient with an undifferentiated sarcoma with rhabdomyoblastic features, and one patient with a fibrosarcoma had stable disease.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / therapeutic use. Camptothecin / therapeutic use. Enzyme Inhibitors / therapeutic use. Neoplasm Proteins / antagonists & inhibitors. Neoplasms / drug therapy. Topoisomerase I Inhibitors
  • [MeSH-minor] Adolescent. Adult. Bone Marrow Diseases / chemically induced. Bone Neoplasms / drug therapy. Child. Child, Preschool. Diarrhea / chemically induced. Drug Evaluation. Female. Humans. Lymphoma, Non-Hodgkin / drug therapy. Male. Neuroectodermal Tumors, Primitive / drug therapy. New York City / epidemiology. Osteosarcoma / drug therapy. Retrospective Studies. Rhabdomyosarcoma / drug therapy. Sarcoma, Ewing / drug therapy. Treatment Outcome

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  • [CommentIn] J Pediatr Hematol Oncol. 2002 Feb;24(2):84-5 [11990709.001]
  • (PMID = 11990694.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA-83132
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 0 / Enzyme Inhibitors; 0 / Neoplasm Proteins; 0 / Topoisomerase I Inhibitors; 7673326042 / irinotecan; XT3Z54Z28A / Camptothecin
  • [Number-of-references] 19
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22. 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
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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.
  • Twelve out of 114 pts (7%), 5/33 (14%) in the first study and 7/81 (8%) in the second, underwent mutilating surgery: 8 pts (of whom 3 were < 2 y of age) had a fibrosarcoma and 4 a MPNST.
  • 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).
  • OUTCOME: At present 6/12 pts, 5 with fibrosarcoma and 1 with MPNST, are alive with no evidence of disease (NED), 4 of the first and 2 of the second study.
  • 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.
  • Only fibrosarcomas and MPNST probably requires a more aggressive surgical behaviour.
  • [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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23. Alaggio R, Ninfo V, Rosolen A, Coffin CM: Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases. Am J Surg Pathol; 2006 Mar;30(3):388-94

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma.
  • In this study, we report 6 cases of a primitive myxoid mesenchymal tumor of infancy (PMMTI), which previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma or infantile fibromatosis.
  • PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass.
  • Three patients had recurrences or metastasis treated with a combination of surgery and chemotherapy.
  • The morphologic appearance combined with the ultrastructural features and absence of the typical gene rearrangement of congenital-infantile fibrosarcoma are unique, and we propose that PMMTI represents a new category of pediatric fibroblastic-myofibroblastic tumor.
  • [MeSH-major] Fibrosarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 16538060.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ETV6-NTRK3 fusion protein, human; 0 / Oncogene Proteins, Fusion
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24. Daw NC, Mahmoud HH, Meyer WH, Jenkins JJ, Kaste SC, Poquette CA, Kun LE, Pratt CB, Rao BN: Bone sarcomas of the head and neck in children: the St Jude Children's Research Hospital experience. Cancer; 2000 May 1;88(9):2172-80
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  • [Title] Bone sarcomas of the head and neck in children: the St Jude Children's Research Hospital experience.
  • BACKGROUND: Bone sarcomas of the head and neck are difficult to resect.
  • METHODS: The records of the 28 patients with bone sarcomas originating in the head and neck treated at St. Jude Children's Research Hospital between March 1962 and January 1998 were reviewed.
  • RESULTS: There were 10 males and 18 females (median age, 12.6 years) each with a single sarcoma: osteosarcoma (18), Ewing sarcoma (7), malignant fibrous histiocytoma (MFH) (2), and fibrosarcoma (1).
  • All but one patient with Ewing sarcoma had localized disease at the time of diagnosis.
  • All patients underwent surgery: complete resection, 8; gross total resection, 4; incomplete resection, 14; and biopsy only, 2; 22 also received chemotherapy.
  • Radiotherapy was given to all patients with Ewing sarcoma and to four patients with primary osteosarcoma.
  • Local disease progression was evident in 12 patients (9 with osteosarcoma, 2 with MFH, and 1 with Ewing sarcoma) who died of disease, 9 of whom had the initial treatment of biopsy alone or incomplete resection.
  • Patients with osteosarcoma who had the initial treatment of incomplete resection or biopsy alone were more likely to experience local failure (P = 0.001) and had poorer survival (P = 0.014) than those who underwent complete or gross total resection.
  • CONCLUSIONS: Bone sarcomas of the head and neck are rare among children and most often are localized at the time of diagnosis.
  • Although aggressive surgery is essential for the cure of osteosarcoma, its necessity in the treatment of Ewing sarcomas remains controversial.
  • [MeSH-major] Sarcoma / surgery. Skull Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Cause of Death. Chemotherapy, Adjuvant. Child. Child, Preschool. Disease Progression. Female. Fibrosarcoma / surgery. Histiocytoma, Benign Fibrous / surgery. Hospitals, Pediatric. Humans. Infant. Male. Mandibular Neoplasms / surgery. Maxillary Neoplasms / surgery. Neoplasm Recurrence, Local / pathology. Osteosarcoma / surgery. Radiotherapy, Adjuvant. Retrospective Studies. Sarcoma, Ewing / surgery. Survival Rate. Tennessee. Treatment Outcome

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  • (PMID = 10813731.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-21765; United States / NCI NIH HHS / CA / CA-23099
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] UNITED STATES
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25. Kebudi R, Görgün O, Ayan I: Oral etoposide for recurrent/progressive sarcomas of childhood. Pediatr Blood Cancer; 2004 Apr;42(4):320-4
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  • [Title] Oral etoposide for recurrent/progressive sarcomas of childhood.
  • BACKGROUND: Etoposide (VP-16) is a topoisomerase II inhibitor that is effective in a broad spectrum of pediatric and adult malignancies.
  • The aim of this prospective, single institution study is to assess the efficacy and toxicity of oral VP-16 in children with progressive or recurrent (P/R) sarcomas.
  • PROCEDURE: Twenty-one children (10 girls and 11 boys) with R/P sarcomas and a median age of 11 years (range 3-16 years) were enrolled in this study.
  • The diagnosis was Ewing sarcoma family tumor (ESFT) in seven, osteosarcoma in eight, rhabdomyosarcoma in four, clear cell sarcoma of soft tissue in one, fibrosarcoma in one patient.
  • They are alive with no evidence of disease (NED) 79 and 94 months from time of relapse/progressive disease (PD).
  • A patient developed acute myeloid leukemia and died.
  • CONCLUSIONS: Oral VP-16 therapy is simple, relatively nontoxic, and does not necessitate hospitalization.
  • Given the risk of second malignancy, especially in children with previous exposure to topoisomerase II inhibitors and alkylating agents, this regimen may be used as a palliative treatment or in patients with poor prognosis.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / administration & dosage. Etoposide / administration & dosage. Sarcoma / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Child. Child, Preschool. Disease Progression. Female. Humans. Male. Neoplasms, Second Primary / chemically induced. Palliative Care. Recurrence. Remission Induction. Survival Rate. Treatment Outcome

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 14966827.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 6PLQ3CP4P3 / Etoposide
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26. Pham TH, Iqbal CW, Zarroug AE, Donohue JH, Moir C: Retroperitoneal sarcomas in children: outcomes from an institution. J Pediatr Surg; 2007 May;42(5):829-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Retroperitoneal sarcomas in children: outcomes from an institution.
  • BACKGROUND: Retroperitoneal sarcomas are uncommon in children and tend to present as large tumors with advanced locoregional involvement of abdominopelvic structures.
  • We reviewed our institutional experience with retroperitoneal sarcomas in children.
  • MATERIALS AND METHODS: In a retrospective review of charts dating between 1975 and 2005, we identified patients younger than 18 years who were diagnosed with a histologically confirmed retroperitoneal sarcoma.
  • The common histologic types were rhabdomyosarcoma (33%) and fibrosarcoma (33%).
  • Seventy-six percent of the patients underwent primary or secondary resection, 71% received neoadjuvant and/or adjuvant chemotherapy therapy, and 38% received radiation therapy.
  • For all patients, the mean survival time was 103 +/- 16 months and the 5-year disease-specific survival rate was 62%.
  • Survival was significantly better for patients with low-grade sarcomas than for those with high-grade sarcomas (90% vs 36%, P = .008).
  • Among those who underwent an initial complete resection, 50% had a recurrence at a mean time of 88 +/- 52 months (range = 3-261 months).
  • CONCLUSIONS: Resection of retroperitoneal sarcomas can be performed safely with minimal morbidity and mortality.
  • [MeSH-major] Retroperitoneal Neoplasms / surgery. Sarcoma / surgery
  • [MeSH-minor] Child. Female. Humans. Male. Postoperative Complications. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 17502193.001).
  • [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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27. Méndez R, Arnáiz S, Montero M, Tellado M, País E, Ríos J, Vela D: [Clinical patterns of soft-tissue sarcoma in children]. Cir Pediatr; 2001 Jan;14(1):14-20
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  • [Title] [Clinical patterns of soft-tissue sarcoma in children].
  • [Transliterated title] Patrones clínicos de comportamiento en sarcomas pediátricos de partes blandas.
  • INTRODUCTION AND OBJECTIVES: Soft tissue sarcomas are rare mesenchymal neoplasms that constitute less than 10% of all pediatric malignancies.
  • Half of these are rhabdomyosarcomas, the remaining 50% have a varied and heterogenous histologic and clinical patterns (fibrosarcoma, synovial cell sarcoma, extraskeletal Ewing's sarcoma, angiosarcoma, liposarcoma, leiomyosarcoma, ...).
  • The purpose of this work is to evaluate our clinical experience with soft tissue sarcomas in uncommon sites over the past 10 years in order to delimitate the prognostic factors in survival and modalities of treatment.
  • MATERIAL AND METHODS: Between 1989 and 1998, 10 patients were diagnosed with soft tissue sarcomas in uncommon sites and treated by us over a total number of 139 pediatric neoplasms (7.19%).
  • Variables investigated included histologic findings, tumor size, age at presentation, primary site, clinical group, radiologic test performed, surgical treatment, radiotherapy and adjuvant chemotherapy, complications and survival rates.
  • RESULTS: The following histologic types of these 10 tumors were identified: 1 hemangiopericytoma in oral cavity, 2 extraosseous Ewing's sarcoma, 1 botryoid rhabdomyosarcoma of the bladder, 1 mediastinal fibrosarcoma, 1 retroperitoneal rhabdomyosarcoma, 1 paratesticular rhabdomyosarcoma, 1 cervical condrosarcoma, 1 alveolar rhabdomyosarcoma and 1 deltoid rhabdomyosarcoma.
  • Adjuvant chemotherapy with IVA was followed by radiotherapy only in four patients.
  • All the children classified in clinical groups II, III or IV needed 2nd. line regimens of chemotherapy.
  • Three patients died in the follow-up instead of the multimodal treatment.
  • 2) radiotherapy will only be necessary if margins of resection cannot control the local disease, and 3) chemotherapy have not clearly demonstrated his benefits as adjuvant therapy in clinical group I lesions but his employ is recommended in all cases because of the poor prognosis due to local recurrence.
  • [MeSH-major] Sarcoma / therapy. Soft Tissue Neoplasms / therapy

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  • (PMID = 11339112.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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28. Howman-Giles R, London K, McCowage G, Graf N, Harvey J: Pulmonary inflammatory myofibroblastic tumor after Hodgkin's lymphoma and application of PET imaging. Pediatr Surg Int; 2008 Aug;24(8):947-51
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  • This case report is of a 16-year-old male who had Hodgkin's disease (stage IVA) and who after chemotherapy and radiotherapy developed IMT, 16 months post completion of therapy.
  • [MeSH-major] Fibrosarcoma / diagnosis. Hodgkin Disease / complications. Lung Neoplasms / diagnosis. Neoplasms, Muscle Tissue / diagnosis. Positron-Emission Tomography / methods
  • [MeSH-minor] Adolescent. Diagnosis, Differential. Follow-Up Studies. Humans. Male. Pneumonectomy. Tomography, X-Ray Computed


29. Germann G, Waag KL, Selle B, Jester A: Extremity salvage with a free musculocutaneous latissimus dorsi flap and free tendon transfer after resection of a large congenital fibro sarcoma in a 15-week-old infant. A case report. Microsurgery; 2006;26(6):429-31
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  • [Title] Extremity salvage with a free musculocutaneous latissimus dorsi flap and free tendon transfer after resection of a large congenital fibro sarcoma in a 15-week-old infant. A case report.
  • Biopsy showed a congenital malignant fibro sarcoma.
  • After initial chemotherapy a radical excision of the tumor at the age of 14 weeks was followed.
  • The case shows that Plastic surgery can play an important role in pediatric oncology and should routinely be integrated into the multi-modal treatment concepts.
  • [MeSH-major] Fibrosarcoma / surgery. Foot. Limb Salvage / methods. Muscle Neoplasms / surgery. Surgical Flaps. Tendon Transfer / methods

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  • [Copyright] (c) 2006 Wiley-Liss, Inc. Microsurgery, 2006.
  • (PMID = 16924620.001).
  • [ISSN] 0738-1085
  • [Journal-full-title] Microsurgery
  • [ISO-abbreviation] Microsurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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30. Karnak I, Emin Senocak M, Kutluk T, Tanyel FC, Büyükpamukçu N: Pulmonary metastases in children: an analysis of surgical spectrum. Eur J Pediatr Surg; 2002 Jun;12(3):151-8
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  • Pulmonary surgery is frequently used for the treatment of metastasis or nodules in children with various types of malignancies.
  • The primaries were osteosarcoma (n = 2), synovial sarcoma (n = 1), fibrosarcoma (n = 1), Ewing's sarcoma (n = 2), mesenchymal chondrosarcoma (n = 1), Wilms' tumour (n = 4), clear-cell sarcoma (n = 1), Hodgkin lymphoma (n = 3), hepatoblastoma (n = 1), hepatocellular carcinoma (n = 1) and haemangioendotheliosarcoma (n = 1).
  • Pulmonary metastases were encountered either at the time of initial diagnosis (22 %) or occurred within 6 months to 5 years.
  • The nodules contained tumour cells in most cases (n = 14) (78 %), mature nephrogenic elements (6 %) and no tumour tissue (16 %) in the remaining cases.
  • However, synovial sarcoma was encountered in metastasis in one case with fibrosarcoma primary.
  • Therefore combined therapies such as chemotherapy and/or radiotherapy should be continued in the postoperative period.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Pneumonectomy. Retrospective Studies. Thoracotomy. Time Factors. Treatment Outcome

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  • (PMID = 12101495.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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31. Hadley GP, Govender D, Landers G: Malignant solid tumours in neonates: an African perspective. Pediatr Surg Int; 2002 Dec;18(8):653-7

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  • Neuroblastoma (NB) was the commonest tumour seen (11), but the soft-tissue sarcomas were the dominant group (14).
  • Chemotherapy, despite appropriate dose reduction, had significant morbidity and mortality.
  • Whilst the outcome for congenital fibrosarcoma was good (6/7, 86%), there were no survivors amongst 5 patients with rhabdomyosarcoma.
  • [MeSH-minor] Female. Humans. Infant, Newborn. Male. Neoplasm Staging. South Africa / epidemiology. Survival Analysis. Treatment Outcome

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  • (PMID = 12598957.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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32. Sirvent N, Maire G, Pedeutour F: Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment. Genes Chromosomes Cancer; 2003 May;37(1):1-19
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  • [Title] Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment.
  • The preferred treatment is wide surgical excision with pathologically negative margins.
  • Although rings have been mainly observed in adults, translocations have been reported in all pediatric cases.
  • Recent studies have determined the molecular identity of "classical" DP, giant cell fibroblastoma, Bednar tumor, adult superficial fibrosarcoma, and the granular cell variant of DP.
  • It is encouraging that inhibitory effects of the PDGF receptor tyrosine kinase antagonist imatinib mesylate have been demonstrated in vivo; such targeted therapies might be warranted in the near future for treatment of the few DP cases not manageable by surgery.
  • [MeSH-major] Dermatofibrosarcoma / drug therapy. Dermatofibrosarcoma / genetics. Enzyme Inhibitors / therapeutic use. Ring Chromosomes. Skin Neoplasms / drug therapy. Skin Neoplasms / genetics. src-Family Kinases / antagonists & inhibitors

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 12661001.001).
  • [ISSN] 1045-2257
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; EC 2.7.10.2 / src-Family Kinases
  • [Number-of-references] 109
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33. Meco D, Colombo T, Ubezio P, Zucchetti M, Zaffaroni M, Riccardi A, Faircloth G, Jose J, D'Incalci M, Riccardi R: Effective combination of ET-743 and doxorubicin in sarcoma: preclinical studies. Cancer Chemother Pharmacol; 2003 Aug;52(2):131-8
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  • [Title] Effective combination of ET-743 and doxorubicin in sarcoma: preclinical studies.
  • PURPOSE: To investigate the cytotoxic and antitumor effects of the combination of the novel anticancer drug ET-743 and doxorubicin (Dx) and to determine whether any pharmacokinetic interaction occurs in sarcoma-bearing mice.
  • METHODS: The cytotoxicity of each drug and of their combinations was assessed in the rhabdomyosarcoma cell line TE-671 by a clonogenic assay, and isobologram analysis was performed to detect any synergistic, additive or antagonistic effects.
  • The antitumor activities of each drug and of the combinations were also evaluated in nude mice transplanted subcutaneously with human TE-671 rhabdomyosarcoma and in C3H female mice injected intravenously with UV2237 M fibrosarcoma or with the Dx-resistant subline UV2237 M-ADM which overexpresses Pgp.
  • Antitumor activity was evaluated by monitoring the TE-671 tumor volume over time and, in the case of the murine fibrosarcomas, by evaluation of lung deposits at autopsy quantified by determining lung weight.
  • ET-743 was determined in plasma by an HPLC-MS method and Dx in plasma and tissue by an HPLC method with fluorescence detection.
  • Giving ET-743 1 h before Dx slightly enhanced the effect (LCK 1.12) compared with giving the drugs simultaneously (LCK 0.85) or in the opposite sequence (LCK 0.92).
  • In UV2237 M fibrosarcoma, both Dx and ET-743 showed an effect in reducing the weight of lung metastases, although the combination of the two drugs was not superior to each drug alone.
  • In UV2237 M-ADM tumors neither of the two drugs was active, whereas the combination, particularly when the two drugs were given simultaneously, produced a significant effect.
  • Plasma levels of ET-743 and Dx were not significantly different when the drugs were given alone or in combination.
  • The concentrations of Dx in tissues including tumor, liver, heart and kidney were found to be the same whether the drug was given alone or in combination with ET-743.
  • CONCLUSIONS: These results indicate that ET-743 and Dx in combination produce an additive effect against human sarcoma cells, reinforcing the idea that they act by a different mechanism of action.
  • In mice no pharmacokinetic interaction between the two drugs was found.
  • The observed activity in UV2237 M-ADM and in human TE-671 sarcoma suggests that the combination of the two drugs could be effective for tumors displaying low sensitivity to each drug given alone.
  • Based on these findings a phase I study on the combination of the two drugs was recently initiated.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Rhabdomyosarcoma / drug therapy
  • [MeSH-minor] Animals. Cell Survival / drug effects. Dioxoles / administration & dosage. Dioxoles / pharmacology. Doxorubicin / administration & dosage. Doxorubicin / pharmacology. Drug Screening Assays, Antitumor. Drug Synergism. Female. Humans. Inhibitory Concentration 50. Isoquinolines / administration & dosage. Isoquinolines / pharmacology. Mice. Mice, Inbred C3H. Mice, Nude. Neoplasm Transplantation. Tetrahydroisoquinolines. Tissue Distribution. Tumor Cells, Cultured

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  • (PMID = 12783202.001).
  • [ISSN] 0344-5704
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Dioxoles; 0 / Isoquinolines; 0 / Tetrahydroisoquinolines; 114899-77-3 / trabectedin; 80168379AG / Doxorubicin
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34. Spicer RD: Right foot congenital infantile fibrosarcoma treated only with chemotherapy. Pediatr Blood Cancer; 2010 Oct;55(4):770; author reply 771
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  • [Title] Right foot congenital infantile fibrosarcoma treated only with chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibrosarcoma / congenital. Fibrosarcoma / drug therapy. Foot / pathology. Soft Tissue Neoplasms / congenital. Soft Tissue Neoplasms / drug therapy

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  • [CommentOn] Pediatr Blood Cancer. 2010 Apr;54(4):618-20 [19998472.001]
  • (PMID = 20533521.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] United States
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35. Surico G, Muggeo P, Daniele RM, Novielli C, Rigillo N, Minervini C: Chemotherapy alone for the treatment of congenital fibrosarcoma: is surgery always needed? Med Pediatr Oncol; 2003 Apr;40(4):268-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chemotherapy alone for the treatment of congenital fibrosarcoma: is surgery always needed?
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibrosarcoma / congenital. Fibrosarcoma / drug therapy. Hand / pathology
  • [MeSH-minor] Dactinomycin / administration & dosage. Humans. Ifosfamide / administration & dosage. Infant. Magnetic Resonance Imaging. Male. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 12555264.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; UM20QQM95Y / Ifosfamide
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