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1. Dobashi Y, Suzuki S, Sato E, Hamada Y, Yanagawa T, Ooi A: EGFR-dependent and independent activation of Akt/mTOR cascade in bone and soft tissue tumors. Mod Pathol; 2009 Oct;22(10):1328-40
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  • [Title] EGFR-dependent and independent activation of Akt/mTOR cascade in bone and soft tissue tumors.
  • To gain the insight into the involvement of signaling mediated by the mammalian target of rapamycin (mTOR) in the phenotype and biological profiles of tumors and tumor-like lesions of the bone and soft tissue, we analyzed the expression and phosphorylation (activation) of mTOR and its correlation with the status of upstream and downstream modulator proteins Akt, p70S6-kinase (S6K), and eukaryotic initiation factor 4E-binding protein 1 (4E-BP1), which we refer to collectively as mTOR cassette proteins.
  • Immunohistochemical analysis of 140 cases showed activation of Akt in 55% (61% in malignant and 27% in benign), and mTOR expression in 61% (66% in malignant and 39% in benign).
  • The preponderance of mTOR activation was found in tumors of peripheral nerve sheath (malignant peripheral nerve sheath tumor and schwannoma), skeletal muscle origin (rhabdomyosarcoma), and in those exhibiting epithelial nature (chordoma and synovial sarcoma).
  • We conclude that mTOR-mediated signaling proteins function not only in the proliferation of the tumor cells, but also in the differentiation and/or maintenance of morphological phenotypes in tumors of rhabdomyoblastic and nerve sheath cell origin.
  • Overall, these results suggest that inhibitors of mTOR cassette may be useful as novel components of combined chemotherapy for a defined subset of bone and soft tissue sarcomas.
  • [MeSH-major] Adaptor Proteins, Signal Transducing / analysis. Bone Neoplasms / enzymology. Phosphoproteins / analysis. Protein Kinases / analysis. Proto-Oncogene Proteins c-akt / analysis. Receptor, Epidermal Growth Factor / analysis. Ribosomal Protein S6 Kinases, 70-kDa / analysis. Signal Transduction. Soft Tissue Neoplasms / enzymology
  • [MeSH-minor] Cell Proliferation. Enzyme Activation. Humans. Immunoblotting. Immunohistochemistry. Mutation. Neoplasm Staging. Phosphorylation. Prognosis. TOR Serine-Threonine Kinases

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  • (PMID = 19648884.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adaptor Proteins, Signal Transducing; 0 / EIF4EBP1 protein, human; 0 / Phosphoproteins; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.10.1 / EGFR protein, human; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt; EC 2.7.11.1 / Ribosomal Protein S6 Kinases, 70-kDa
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2. Casanova M, Meazza C, Gronchi A, Fiore M, Zaffignani E, Podda M, Collini P, Gandola L, Ferrari A: Soft-tissue sarcomas of the extremities in patients of pediatric age. J Child Orthop; 2007 Sep;1(3):195-203

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  • [Title] Soft-tissue sarcomas of the extremities in patients of pediatric age.
  • PURPOSE: The extremity site is a peculiar location for soft-tissue sarcomas (STS) of children and adolescents.
  • METHODS: The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma.
  • Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy.
  • RESULTS: For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure.
  • For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors.
  • DISCUSSION: While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results.
  • The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.

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  • (PMID = 19308495.001).
  • [ISSN] 1863-2521
  • [Journal-full-title] Journal of children's orthopaedics
  • [ISO-abbreviation] J Child Orthop
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2656726
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3. Ferrari A, Bisogno G, Macaluso A, Casanova M, D'Angelo P, Pierani P, Zanetti I, Alaggio R, Cecchetto G, Carli M: Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1. Cancer; 2007 Apr 1;109(7):1406-12
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  • [Title] Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1.
  • BACKGROUND: Patients affected by neurofibromatosis type 1 (NF1) are at higher risk of developing soft-tissue sarcomas (STS) than the general population.
  • METHODS: The study included 37 patients with neurogenic sarcomas (36 malignant peripheral nerve sheath tumors [MPNST], 1 triton tumor) and 6 cases of rhabdomyosarcoma (RMS).
  • The prevalence of NF1 observed during the study period was 43% in the MPNST population and 1% in the RMS group.
  • Two of 16 patients with evaluable disease responded to chemotherapy.
  • All 6 RMS patients were </=3 years old and had embryonal subtype, 5 of 6 arising in the genitourinary tract or pelvis (paravesical); 4 were alive in first remission at the time of the analysis, 1 was alive in second remission after a local recurrence, and 1 died of disease.
  • CONCLUSIONS: The occurrence of STS in pediatric patients with NF1 syndrome in Italy is discussed, confirming that NF1 patients have a high risk of developing STS, and particularly MPNST, often with an aggressive clinical presentation and poor outcome.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasms, Second Primary / complications. Neoplasms, Second Primary / diagnosis. Neoplasms, Second Primary / therapy. Nerve Sheath Neoplasms / complications. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / therapy. Neurofibrosarcoma / complications. Neurofibrosarcoma / diagnosis. Neurofibrosarcoma / therapy. Peripheral Nervous System Neoplasms / complications. Peripheral Nervous System Neoplasms / diagnosis. Peripheral Nervous System Neoplasms / therapy. Prognosis. Prospective Studies. Rhabdomyosarcoma / complications. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Risk Factors. Survival Rate

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  • [Copyright] (c) 2007 American Cancer Society.
  • (PMID = 17330850.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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4. 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.
  • 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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5. Dartnell J, Pilling J, Ferner R, Cane P, Lang-Lazdunski L: Malignant triton tumor of the brachial plexus invading the left thoracic inlet: a rare differential diagnosis of pancoast tumor. J Thorac Oncol; 2009 Jan;4(1):135-7

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  • [Title] Malignant triton tumor of the brachial plexus invading the left thoracic inlet: a rare differential diagnosis of pancoast tumor.
  • Malignant triton tumor is a divergent malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation.
  • We report a case of malignant triton tumor arising in the brachial plexus of a 28-year-old women with neurofibromatosis type 1.
  • Fluorodeoxyglucose-positron emission tomography-computed tomography before excision demonstrated a tumor with a maximum standard uptake value of 21 at 4 hours postinjection.
  • The patient underwent complete excision of the tumor through median sternotomy and left supraclavicular approach.
  • Adjuvant radiotherapy and chemotherapy were planned but the patient died of metastatic disease within 3 months of surgical resection.
  • [MeSH-major] Brachial Plexus / pathology. Neurilemmoma / diagnosis. Pancoast Syndrome / diagnosis. Peripheral Nervous System Neoplasms / diagnosis. Thoracic Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Fatal Outcome. Female. Fluorodeoxyglucose F18. Humans. Magnetic Resonance Imaging. Neoplasm Invasiveness. Neurofibromatosis 1 / complications. Positron-Emission Tomography. Radiopharmaceuticals. Tomography, X-Ray Computed

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  • (PMID = 19096322.001).
  • [ISSN] 1556-1380
  • [Journal-full-title] Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
  • [ISO-abbreviation] J Thorac Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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6. Hoshimoto S, Morise Z, Takeura C, Ikeda M, Kagawa T, Tanahashi Y, Okabe Y, Mizoguchi Y, Sugioka A: Malignant Triton tumor in the retroperitoneal space associated with neurofibromatosis type 1: a case study. Rare Tumors; 2009;1(2):e27

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  • [Title] Malignant Triton tumor in the retroperitoneal space associated with neurofibromatosis type 1: a case study.
  • We report an extremely rare case of malignant Triton tumor developing in the retroperitoneal space in a patient with neurofibromatosis type 1.
  • A 21-year old man who had been diagnosed with neurofibromatosis type 1 was admitted to our hospital with the chief complaint of a palpable abdominal mass.
  • Abdominal computed tomography revealed a huge heterogeneous tumor measuring approximately 17 cm in diameter occupying the left retroperitoneal space, and numerous metastatic lesions between the left psoas muscle and the left thigh with dissolution of the left hip joint.
  • After the diagnosis of a retroperitoneal malignant neurogenic tumor, resection of the tumor with reconstruction of the abdominal aorta was conducted, followed by postoperative transarterial infusion chemotherapy.
  • The histopathological diagnosis was malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation, namely malignant Triton tumor.
  • Postoperative chemotherapy was in vain and the patient died 14 months after the surgery as a result of lung metastasis.

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  • (PMID = 21139906.001).
  • [ISSN] 2036-3613
  • [Journal-full-title] Rare tumors
  • [ISO-abbreviation] Rare Tumors
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC2994481
  • [Keywords] NOTNLM ; malignant Triton tumor / malignant peripheral nerve sheath tumor / neurofibromatosis / retroperitoneal tumor.
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7. Jacobs S, Fox E, Krailo M, Hartley G, Navid F, Wexler L, Blaney SM, Goodwin A, Goodspeed W, Balis FM, Adamson PC, Widemann BC: Phase II trial of ixabepilone administered daily for five days in children and young adults with refractory solid tumors: a report from the children's oncology group. Clin Cancer Res; 2010 Jan 15;16(2):750-4
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  • PURPOSE: Ixabepilone is a microtubule-stabilizing agent with activity in adult solid tumors and in pediatric tumor xenograft models that are resistant to paclitaxel.
  • This study aimed to determine the response rate to ixabepilone in six solid tumor strata in children and young adults.
  • EXPERIMENTAL DESIGN: We conducted a phase II trial of ixabepilone (8 mg/m(2)/dose for 5 days every 21 days) using a two-stage design in taxane-naïve children and young adults with treatment-refractory, measurable rhabdomyosarcoma, Ewing sarcoma family tumors, osteosarcoma, synovial sarcoma, or malignant peripheral nerve sheath tumor, neuroblastoma, and Wilms tumor.
  • Seven patients received >or=3 cycles, and two had prolonged stable disease (Wilms' tumor, 38 cycles; synovial sarcoma, 8 cycles).

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  • (PMID = 20068084.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA 98543; United States / Intramural NIH HHS / / ; United States / NCI NIH HHS / CA / U10 CA098543-08; None / None / / U10 CA098543-08; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / U10 CA098413-08; None / None / / U10 CA098413-08
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Epothilones; K27005NP0A / ixabepilone
  • [Other-IDs] NLM/ NIHMS160304; NLM/ PMC3086796
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8. Thoennissen NH, Schliemann C, Brunnberg U, Schmidt E, Staebler A, Stegger L, Bremer C, Schleicher C, Mesters RM, Müller-Tidow C, Berdel WE: Chemotherapy in metastatic malignant triton tumor: report on two cases. Oncol Rep; 2007 Oct;18(4):763-7
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  • [Title] Chemotherapy in metastatic malignant triton tumor: report on two cases.
  • Malignant triton tumor (MTT) is a rare, highly malignant nerve sheath tumor with rhabdomyoblastic differentiation.
  • Initial debulking surgery followed by adjuvant therapy is the current treatment of choice, but has very limited efficacy when optimal cytoreduction is not achieved by surgical procedure.
  • Neoadjuvant therapy for MTT, to potentially facilitate subsequent surgery, eradicate micrometastatic lesions and, therefore, improve the therapeutical outcome, has never before been presented in literature.
  • Treatment modalities involved neoadjuvant and adjuvant chemotherapy, surgical resection, and radiation.
  • In both cases, integrated Positron Emission Tomography/Computed Tomography (PET/CT) emerged as an important diagnostic tool for the reliable assessment of MTT response and metabolic remission.
  • [MeSH-major] Cecal Neoplasms / therapy. Ileal Neoplasms / therapy. Liver Neoplasms / therapy. Neurilemmoma / therapy. Ovarian Neoplasms / drug therapy. Uterine Neoplasms / therapy
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Chemotherapy, Adjuvant. Combined Modality Therapy. Female. Fluorodeoxyglucose F18. Humans. Male. Neoadjuvant Therapy. Neurofibromatosis 1 / pathology. Positron-Emission Tomography. Radiopharmaceuticals. Tomography, X-Ray Computed

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  • (PMID = 17786333.001).
  • [ISSN] 1021-335X
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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9. Kasper B, Lehnert T, Bernd L, Mechtersheimer G, Goldschmidt H, Ho AD, Egerer G: High-dose chemotherapy with autologous peripheral blood stem cell transplantation for bone and soft-tissue sarcomas. Bone Marrow Transplant; 2004 Jul;34(1):37-41
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  • [Title] High-dose chemotherapy with autologous peripheral blood stem cell transplantation for bone and soft-tissue sarcomas.
  • The role of high-dose chemotherapy (HDCT) with PBSCT in the treatment of bone and soft-tissue sarcomas is not established.
  • In total, 27 patients (15 female, median age at TPL 30.6 years (range: 13-59)) were analyzed (Ewing sarcoma family n=8, osteosarcoma n=6, MPNST (malignant peripheral nerve sheath tumor) n=4, synovial sarcoma n=3, liposarcoma n=2, leiomyosarcoma n=2, rhabdomyosarcoma n=1, meningosarcoma n=1).
  • Following chemotherapy and surgery complete remission (CR) (n=9), partial remission (PR) (n=10), stable disease (SD) (n=2) and progressive disease (PD) (n=6) were reached prior HDCT.
  • Although the role of HDCT in the treatment of sarcomas is not defined, a subgroup of patients who achieved CR before HDCT could benefit from this therapy.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Bone Neoplasms / therapy. Peripheral Blood Stem Cell Transplantation / methods. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Adult. Female. Humans. Male. Middle Aged. Remission Induction. Retrospective Studies. Survival Analysis. Transplantation, Autologous. Treatment Outcome


10. Ferrari A, Bisogno G, Carli M: Management of childhood malignant peripheral nerve sheath tumor. Paediatr Drugs; 2007;9(4):239-48
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  • [Title] Management of childhood malignant peripheral nerve sheath tumor.
  • Malignant peripheral nerve sheath tumor (MPNST) is rare, but is one of the most frequent non-rhabdomyosarcoma soft-tissue sarcomas in the pediatric population.
  • They are often associated with neurofibromatosis type 1 (NF-1): the lifetime risk of patients with NF-1 developing MPNST has been estimated at 8-13%, compared with 0.001% in the general population.
  • Because of the rarity of this tumor, little information is available on its clinical management, particularly in the pediatric age group.
  • In a recent report on the clinical findings and treatment outcomes from a large number of children and adolescents with MPNST in an Italian and German series, less satisfactory overall outcomes than those for other pediatric sarcomas were described.
  • Therefore, the approach to the treatment of patients with MPNST should be aggressive and risk adapted, and is necessarily complex.
  • Patients should be referred to selected institutions with adequate experience in treating soft-tissue sarcomas, and with the multidisciplinary skills for enrolling patients in clinical trials.
  • Surgical resection represents the mainstay of treatment, while the role of adjuvant treatment is not yet clear.
  • Although lack of local control is the major cause of treatment failure, MPNST may give rise to distant metastases.
  • These tumors are usually considered as having uncertain chemosensitivity, but recent evidence suggests that there may be a role for chemotherapy in patients with a high-grade histology.
  • For the near future, our hopes lie in the development of novel tailored therapies directed specifically against the molecular targets of the neoplastic cells: soft-tissue sarcomas seem particularly promising candidates for targeted therapy.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Nerve Sheath Neoplasms / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Drug Delivery Systems. Humans. Infant. Infant, Newborn. Neoplasm Staging. Neurofibromatosis 1 / complications. Prognosis. Treatment Outcome

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  • (PMID = 17705563.001).
  • [ISSN] 1174-5878
  • [Journal-full-title] Paediatric drugs
  • [ISO-abbreviation] Paediatr Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 59
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11. Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J: Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol; 2005 Nov 20;23(33):8422-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group.
  • PURPOSE: To assess the value of chemotherapy and radiotherapy in children with malignant peripheral nerve sheath tumors (MPNSTs) and to identify risk factors associated with outcome.
  • Seventeen percent of patients had neurofibromatosis type 1 (NF1).
  • Chemotherapy was administered to 74% of patients; radiotherapy was administered to 38% of patients.
  • The 5-year OS and PFS by Intergroup Rhabdomyosarcoma Study (IRS) groupings were as follows: group I, 82% and 61%; group II, 62% and 37%; group III, 32% and 27%; group IV, 26% and 21%, respectively.
  • Univariate analysis identified IRS groups, size, invasiveness, primary site, age, and presence of NF1 as prognostic factors; multivariate analysis identified absence of NF1, tumor invasiveness T1, IRS groups I to II and extremity of primary site as independent favorable factors for OS.
  • The overall response rate to primary chemotherapy, including minor responses, in group III patients was 45%.
  • CONCLUSION: MPNST is an aggressive tumor for which complete surgical resection is the mainstay of successful treatment.
  • Postoperative radiotherapy may have a role in improving local control in patients with minimal residual tumor.
  • The reported responses to primary chemotherapy suggest that it may be effective in patients with tumor considered unresectable at diagnosis.
  • [MeSH-major] Nerve Sheath Neoplasms / drug therapy. Nerve Sheath Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Germany / epidemiology. Humans. Infant. Infant, Newborn. Italy / epidemiology. Male. Multivariate Analysis. Proportional Hazards Models. Risk Factors. Survival Rate. Treatment Outcome

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  • [ErratumIn] J Clin Oncol. 2006 Feb 1;24(4):724. Koscielniak, Eura [corrected to Koscielniak, Ewa]
  • (PMID = 16293873.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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12. Rekhi B, Jambhekar NA, Puri A, Agrawal M, Chinoy RF: Clinicomorphologic features of a series of 10 cases of malignant triton tumors diagnosed over 10 years at a tertiary cancer hospital in Mumbai, India. Ann Diagn Pathol; 2008 Apr;12(2):90-7
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  • [Title] Clinicomorphologic features of a series of 10 cases of malignant triton tumors diagnosed over 10 years at a tertiary cancer hospital in Mumbai, India.
  • A rhabdomyoblastic differentiation in a malignant peripheral nerve sheath tumor is unusual and is termed as a malignant triton tumor.
  • Distinct rhabdomyoblastic cells were identified in the areas of malignant peripheral nerve sheath tumor.
  • Immunohistochemistry confirmed the neurogenic differentiation with varying S-100 expression and the rhabdomyoblastic differentiation with desmin and myoglobin positivity in all cases.
  • Surgery with adequate margins constituted the treatment mainstay with adjuvant chemotherapy and/or radiotherapy in individual cases.
  • Malignant triton tumor is an uncommon tumor associated with an aggressive behavior.
  • Surgery with clear margins is the treatment mainstay.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Rhabdomyosarcoma / pathology
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / metabolism. Female. Humans. Immunohistochemistry. Male. Middle Aged. Neoplasm Recurrence, Local. S100 Proteins / analysis. Treatment Outcome

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  • (PMID = 18325468.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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13. Coffin CM, Cassity J, Viskochil D, Randall RL, Albritton K: Non-neurogenic sarcomas in four children and young adults with neurofibromatosis type 1. Am J Med Genet A; 2004 May 15;127A(1):40-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Non-neurogenic sarcomas in four children and young adults with neurofibromatosis type 1.
  • It is well known that children and young adults with neurofibromatosis type 1 (NF1) have a higher risk for non-neurogenic sarcomas than the general population, in addition to an increased risk for malignant peripheral nerve sheath tumor.
  • When non-neurogenic sarcomas occur in early childhood, a subsequent malignant peripheral nerve sheath tumor can occur as a second malignant neoplasm, especially after alkylating agent chemotherapy and irradiation.
  • This report includes the clinicopathologic features of non-neurogenic sarcomas and secondary malignant peripheral nerve sheath tumor in the context of four cases of NF1.
  • The purpose is to emphasize that early diagnosis of NF1 and recognition of potential manifestations of non-neurogenic sarcomas are important for clinical care of these patients and their families.
  • [MeSH-major] Neoplasms, Second Primary / diagnosis. Neurofibromatosis 1 / diagnosis. Sarcoma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Child, Preschool. Female. Humans. Male. Nerve Sheath Neoplasms / genetics. Nerve Sheath Neoplasms / secondary. Phyllodes Tumor / diagnosis. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Prognosis. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15103715.001).
  • [ISSN] 1552-4825
  • [Journal-full-title] American journal of medical genetics. Part A
  • [ISO-abbreviation] Am. J. Med. Genet. A
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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14. Chao MM, Levine JE, Ruiz RE, Kohlmann WK, Bower MA, Petty EM, Mody RJ: Malignant triton tumor in a patient with Li-Fraumeni syndrome and a novel TP53 mutation. Pediatr Blood Cancer; 2007 Dec;49(7):1000-4
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  • [Title] Malignant triton tumor in a patient with Li-Fraumeni syndrome and a novel TP53 mutation.
  • We report a 3-year-old boy with a malignant triton tumor (MTT) involving the left masticator space with local invasion and regional lymph node metastasis.
  • Family history and detection of a novel germline TP53 mutation confirmed his diagnosis of Li Fraumeni syndrome (LFS).
  • The tumor could not be resected and he was successfully treated with intensive induction chemotherapy, irradiation, and high-dose chemotherapy with autologous stem cell transplantation.
  • [MeSH-major] Germ-Line Mutation. Li-Fraumeni Syndrome / genetics. Nerve Sheath Neoplasms / genetics. Nose Neoplasms / genetics. Tumor Suppressor Protein p53 / genetics
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Combined Modality Therapy. Follow-Up Studies. Hematopoietic Stem Cell Transplantation. Humans. Male. Pedigree. Tomography, X-Ray Computed. Transplantation, Autologous. Treatment Outcome

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  • [Copyright] 2007 Wiley-Liss, Inc
  • (PMID = 16333835.001).
  • [ISSN] 1545-5009
  • [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] 0 / Tumor Suppressor Protein p53
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15. Häcker FM, von Schweinitz D, Gambazzi F: The relevance of surgical therapy for bilateral and/or multiple pulmonary metastases in children. Eur J Pediatr Surg; 2007 Apr;17(2):84-9
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  • [Title] The relevance of surgical therapy for bilateral and/or multiple pulmonary metastases in children.
  • PURPOSE: Pulmonary surgery is frequently used for the treatment of metastases in children with various malignant diseases.
  • The benefit of an aggressive surgical treatment in children with bilateral and/or multiple pulmonary metastases is still discussed controversially.
  • The primary malignancies were osteosarcoma (n = 4), hepatoblastoma (n = 3), malignant peripheral nerve sheath tumor (n = 1), adrenocortical carcinoma (n = 1) and alveolar rhabdomyosarcoma (n = 1).
  • Preoperative induction chemotherapy with tumor regression and a subsequent decrease in the size and number of pulmonary metastases was mandatory for the surgery of metastases.
  • CONCLUSION: Complete surgical resection of pulmonary metastases after response to induction chemotherapy may increase survival in carefully selected children, even in cases with multiple and recurrent metastatic disease.

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  • (PMID = 17503299.001).
  • [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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16. Morgan JA, George S, Desai J, St Amand M, Horton D, Wilkins E, Manola J, Demetri GD: Phase II study of gemcitabine/vinorelbine (GV) as first or second line chemotherapy in patients with metastatic soft tissue sarcoma (STS). J Clin Oncol; 2004 Jul 15;22(14_suppl):9009

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phase II study of gemcitabine/vinorelbine (GV) as first or second line chemotherapy in patients with metastatic soft tissue sarcoma (STS).
  • : 9009 Background: Both single agent gemcitabine and vinorelbine have efficacy for treatment of STS.
  • Combination GV is active therapy for metastatic carcinoma, with acceptable toxicity.
  • This single institution phase II trial has been undertaken to determine response rates and toxicity of combination GV for treatment of metastatic STS.
  • Gemcitabine at 800mg/m2 was infused over 90 minutes on days 1 and 8 of a 21 day cycle, following vinorelbine at 25mg/m2.
  • Pts with grade 3 or 4 toxicity were subsequently treated at the same doses on days 1 and 15 of a 28 day cycle.
  • Histology was uterine or extremity leiomyosarcoma (LMS) in 9, high grade pleomorphic sarcoma in 2, and 1 each with carcinosarcoma, pleomorphic liposarcoma, malignant peripheral nerve sheath tumor (MPNST), desmoplastic small round cell tumor, rhabdomyosarcoma, and small round cell sarcoma.
  • No treatment related deaths have occurred.
  • Of the remaining 9, 4 have been treatment related, including cough, nausea, vomiting, and SGPT elevation.
  • There have been two confirmed PRs, one high grade uterine LMS progressing after single agent doxorubicin, and one small round cell tumor recurring within 6 months of completion of a 5 drug Ewing's regimen.
  • One metastatic MPNST has exhibited stable disease for greater than 4 months.
  • Median time to progression has been 4.2 months and median survival 6.25 months.
  • CONCLUSIONS: GV appears to be a well-tolerated and potentially effective regimen for first or second line treatment of metastatic STS.

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  • (PMID = 28013692.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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17. Chugh R, Thomas D, Wathen K, Thall PF, Benjamin RS, Maki RS, Samuels BL, Keohan ML, Priebat DA, Baker LH: Imatinib mesylate in soft tissue and bone sarcomas: Interim results of a Sarcoma Alliance for Research thru Collaboration (SARC) phase II trial. J Clin Oncol; 2004 Jul 15;22(14_suppl):9001

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imatinib mesylate in soft tissue and bone sarcomas: Interim results of a Sarcoma Alliance for Research thru Collaboration (SARC) phase II trial.
  • : 9001 Background: The success of imatinib (Gleevec) in gastrointestinal stromal tumor (GIST) is proof that a small oncogene targeted molecule can have significant benefit in a solid tumor.
  • Rules for early termination within each disease type were based on a hierarchical Bayesian probability model accounting for correlation of the responses of the 10 disease types.
  • Tissue specimens were analyzed by immunohistochemistry for c-kit, PDGFRα, PDGFR****223'3f NEEDS TO BE ADDED TO TIMES NEW ROMAN GREEK FONT****, AKT, PTEN, FKHR, and by allelic PCR analysis for PDGFRα exon 18.
  • Patients received prior chemotherapy and all had progressive disease.
  • Four month progression-free survival (PFS) rates follow: all sarcoma subtypes 18% (27/147), angiosarcoma 10% (1/10), Ewing sarcoma 0% (0/13), fibrosarcoma 29% (2/7), liposarcoma 32% (9/28), leiomyosarcoma (LMS) 20% (6/30), malignant fibrous histiocytoma 1/15 (7%), osteosarcoma 18% (3/17), peripheral nerve sheath tumor 20% (1/5), rhabdomyosarcoma 0% (0/2), synovial sarcoma 20% (4/20).
  • CONCLUSIONS: Further investigation of imatinib in the therapy of liposarcoma, LMS, and fibrosarcoma is warranted.

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  • (PMID = 28013622.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Malerba M, Garofalo A: [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor)]. Tumori; 2003 Jul-Aug;89(4 Suppl):246-50

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor)].
  • [Transliterated title] Un raro caso di sarcoma delle guaine nervose a differenziazione rabdomioblastica (malignant Triton tumor).
  • Malignant peripheral nerve sheath tumors (MPNST) are spindle-cell sarcomas that appear in a setting of neurofibroma or schwannoma or are associated with peripheral nerves or demonstrate nerve sheath differentiation.
  • Malignant triton tumor (MTT) is a subtype of MPNST that also contain tissue with skeletal muscle differentiation (embryonal, plemorphic and botryoid rhabdomyosarcoma).
  • Seventeen months before, when the patient underwent surgery at the same Department for both a left-sided paravertebral inferior mediastinal neurofibroma and a right-sided axillary neurofibroma, diagnosis of von Recklinghausen disease (NF1) was made, according to the criteria established by the NIH Consensus Development.
  • A xifopubic laparotomy was performed: the tumor appeared to be localized, well-capsulated and strictly associated to the lumbar and sacral nervous radicles (L4, L5, S1) without evidence of invasion.
  • The tumor was completely resected with sparing of the psoas muscle and the lumbar plexus through a subperineural dissection technique.
  • Postoperative pathologic findings showed evidence for a trition tumor.
  • The popliteal mass was resected too and resulted to be a neurofibroma just like the tumors resected 17 months before when diagnosis of von Recklinghausen disease was made.
  • Sarcoma arising in anatomic site other than extremity and superficial trunk are often more difficult to control because of anatomic constraints, delayed disease presentation, proximity to neurovascular and osseous structures and toxicity for normal adjacent tissues that limits the use of adequate radiation doses.
  • In contrast to the benefit most patients with high grade soft tissue sarcomas of the extremities receive from adjuvant radiation and chemotherapy, these modalities have been of little value for retroperitoneal tumors.
  • Current chemotherapy for retroperitoneal sarcomas is ineffective.
  • Local adjuvant therapy such as intraperitoneal chemotherapy or experimental immunotherapy seems to be attractive in theory, but needs further investigations through prospective randomized multicentric trials.
  • In conclusion, to date aggressive surgical management remains the most effective modality for selected primary and recurrent retroperitoneal soft tissue sarcomas including MPNSTs and the subtype MTT.
  • Patients with incomplete resection and other risk factors such as younger age and high grade tumors may be suitable candidates for investigational adjuvant therapy.
  • [MeSH-major] Neurilemmoma / pathology. Neurofibromatosis 1 / pathology. Peripheral Nervous System Neoplasms / pathology. Retroperitoneal Neoplasms / pathology
  • [MeSH-minor] Adult. Axilla. Cell Differentiation. Humans. Knee. Male. Mediastinal Neoplasms / pathology. Mediastinal Neoplasms / surgery. Muscles / pathology. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery. Spinal Nerve Roots / pathology. Spinal Nerve Roots / surgery

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  • (PMID = 12903608.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] United States
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19. Radovanovic D, Vukotic-Maletic V, Stojanovic D, Lalosevic Dj, Likic I, Stojsic Z, Bacetic D: Retroperitoneal "Triton" tumor. Hepatogastroenterology; 2008 Mar-Apr;55(82-83):527-30

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Retroperitoneal "Triton" tumor.
  • Malignant Triton tumor is a malignant peripheral nerve sheat tumor with rhabdomioblastic differentiation.
  • These tumors are frequently associated with Neurofibromatosis type 1, sporadic cases being exteremly rare.
  • Retroperitoneal localization have the most unfavorable prognois due to delayed diagnosis but also due to relation to adjacant organs.
  • Preoperative diagnosis is inaccurate, but core needle biopsy gives more promising results.
  • Aggressive surgical management remains the most effective modality since adjuvant forms of treatment like irradiation or chemotherapy do not have reproducible results.
  • We present a 60-year-old female patient in whom a retroperitoneal presacral mass was postoperatively diagnosed as Triton tumor.
  • At time of diagnosis, no visible metastases were present.
  • Intended chemotherapy was never started since multiple pulmonary, hepatic and splenic metastases were diagnosed only a month after surgery, with rapid lethal outcome.
  • This case demonstrates the bad prognosis of malignant retroperitoneal tumors.
  • [MeSH-major] Nerve Sheath Neoplasms. Retroperitoneal Neoplasms

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  • (PMID = 18613401.001).
  • [ISSN] 0172-6390
  • [Journal-full-title] Hepato-gastroenterology
  • [ISO-abbreviation] Hepatogastroenterology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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20. Terzic A, Bode B, Gratz KW, Stoeckli SJ: Prognostic factors for the malignant triton tumor of the head and neck. Head Neck; 2009 May;31(5):679-88
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic factors for the malignant triton tumor of the head and neck.
  • BACKGROUND: Malignant triton tumors are rare neoplasias consisting of a malignant peripheral nerve sheath tumor with additional rhabdomyoblastic differentiation.
  • METHODS: From 1993 to 2005, 7 patients with a malignant triton tumor of the head and neck were treated at our institution.
  • Additional radiation or chemotherapy show little effect.
  • CONCLUSION: Location of the primary tumor is a key factor for prognosis.
  • Complete surgical removal is the only treatment associated with survival.
  • [MeSH-major] Head and Neck Neoplasms / mortality. Nerve Sheath Neoplasms / mortality

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  • (PMID = 19283843.001).
  • [ISSN] 1097-0347
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 47
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21. López Alvarez F, Llorente Pendás JL, Coca Pelaz A, Fernández García MS, Cuello Bueno G, Suárez Nieto C: Malignant triton tumor of the infratemporal fossa. J Craniofac Surg; 2009 Jul;20(4):1282-6

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  • [Title] Malignant triton tumor of the infratemporal fossa.
  • Malignant triton tumor is a very aggressive type of sarcoma that comprises rhabdomyoblasts and malignant Schwann cells.
  • It is a different entity from malignant schwannoma, characterized by their aggressiveness and poor prognosis.
  • Head and neck location is frequent, and early diagnosis and complete resection followed by radiation therapy is important for long-term survival.
  • However, the therapeutic plan should be individualized, taking into account the location and size of the primary tumor.
  • The use of adjuvant chemotherapy and molecular therapies should be considered in the treatment of these tumors.
  • We report an unusual presentation of a malignant triton tumor located in the infratemporal fossa, describing its clinical and pathologic features, and we try to update the knowledge in the management of these tumors, including the use of molecular therapies.
  • [MeSH-major] Hamartoma / diagnosis. Skull Base Neoplasms / diagnosis
  • [MeSH-minor] Biopsy. Combined Modality Therapy. Diagnosis, Differential. Endoscopy. Fatal Outcome. Female. Humans. Tomography, X-Ray Computed. Young Adult

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  • (PMID = 19625850.001).
  • [ISSN] 1536-3732
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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22. Malagón HD, Valdez AM, Moran CA, Suster S: Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. Am J Surg Pathol; 2007 Sep;31(9):1356-62
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  • The germ cell component consisted of pure mature or immature teratoma (23 cases), teratoma mixed with other seminomatous or nonseminomatous components (17), pure seminoma (2), intratubular germ cell neoplasia (1), and yolk sac tumor (1).
  • The SC included embryonal rhabdomyosarcoma (29), angiosarcoma (6), leiomyosarcoma (4), undifferentiated sarcoma (3), myxoid liposarcoma (1), malignant peripheral nerve sheath tumor (1), malignant "triton" tumor (1), and epithelioid hemangioendothelioma (1).
  • All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery.
  • Thirty-two of 40 patients either died of tumor (25/40; 62.5%) or were alive with advanced, progressive disease (7/40; 17.5%), and only 8/40 (20%) were alive and free of disease between 5 to 40 months (mean=18 mo).
  • [MeSH-major] Immunohistochemistry. Mediastinal Neoplasms / diagnosis. Neoplasms, Germ Cell and Embryonal / diagnosis. Ovarian Neoplasms / diagnosis. Retroperitoneal Neoplasms / diagnosis. Sarcoma / diagnosis. Testicular Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Antineoplastic Combined Chemotherapy Protocols. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Metastasis. Neoplasm Staging. Orchiectomy. Ovariectomy. Time Factors. Treatment Outcome

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  • (PMID = 17721191.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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23. Terenziani M, D'Angelo P, Bisogno G, Boldrini R, Cecchetto G, Collini P, Conte M, De Laurentis T, Ilari I, Indolfi P, Inserra A, Piva L, Siracusa F, Spreafico F, Tamaro P, Lo Curto M: Teratoma with a malignant somatic component in pediatric patients: the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience. Pediatr Blood Cancer; 2010 Apr;54(4):532-7
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  • [Title] Teratoma with a malignant somatic component in pediatric patients: the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience.
  • BACKGROUND: Teratoma with a malignant somatic component (TMSC) is rare but described in adults, whereas information on pediatric presentation is sparse.
  • PROCEDURE: The Associazione Italiana Ematologia Oncologia Pediatrica identified 14 cases of TMSC.
  • RESULTS: The series (9 female, 5 male) showed the following disease: testis (2), sacrococcygeal (3), ovary (3), retroperitoneum (3), mediastinum (2), and foot soft tissue (1).
  • Distribution of the somatic component was: carcinoma (4), pancreatic neuroendocrine tumor (1), neuroblastoma (3), rhabdomyosarcoma (3), rhabdomyosarcoma plus liposarcoma, chondrosarcoma, neurogenic sarcoma (1), chondrosarcoma plus neuroectodermal sarcoma (1), malignant peripheral nerve sheath tumor (1).
  • The pediatric disease appears to be more heterogeneous in tumor site distribution and MSC histology than in adults.
  • Chemotherapy optimized for histology should include reagents directed to the somatic malignancy, if chemosensitive.
  • Malignant GCT warrants GCT-directed chemotherapy.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Italy. Male. Neoplasm Staging. Prognosis. Retrospective Studies. Treatment Outcome

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  • (PMID = 20049928.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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24. Stachowicz-Stencel T, Bień E, Stefanowicz J, Połczytńska K, Sierota D, Szołkiewicz A, Drozyńska E, Kosiak W, Stankiewicz C, Pietniczka M, Kukwa A, Czauderna P, Balcerska A: [Diagnostic and therapeutic difficulties in soft tissue sarcomas localized in nonparameningeal head and neck region--own experiences]. Med Wieku Rozwoj; 2005 Jul-Sep;9(3 Pt 2):487-94
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  • [Title] [Diagnostic and therapeutic difficulties in soft tissue sarcomas localized in nonparameningeal head and neck region--own experiences].
  • INTRODUCTION: Soft tissue sarcomas (MTM) localized within the nonorbital and non-parameningeal head and neck region in children are associated with favourable prognosis.
  • However in our material we have observed many therapeutic failures in this group of patients.
  • The aim of the study was to analyze the reasons for disappointing results of oncological therapy in children with MTM treated between 1992 and 2004.
  • Five patients were diagnosed with rhabdomyosarcoma, four--with non-rhlabdomyosarcoma, including: angiosarcoma, malignant triton tumour, fibrosarcoma and leiomyosarcoma.
  • The first sign of the neoplastic disease in all children was tumour.
  • In as many as five of nine patients initially a false histopathological diagnosis was made based on material obtained from aspiration biopsy of the tumour performed in non-oncological centres.
  • This resulted in a significant delay of the proper diagnosis of malignant disease ranging from 2 to 15 months (mean 7 months).
  • Therapy was conducted according to the schemes: MMT-89, CWS-91, CWS-96 and CWS-2002.
  • Durable complete remission after the first line therapy was obtained in one child only.
  • Six patients developed MTM relapse and two--progression during chemotherapy.
  • Finally five children remain disease-free after treatment termination with follow-up of 1 to 1,5 years.
  • Four of then had microscopically complete delayed resection of the tumour or the relapse.
  • Four patients died of neoplasm recurrence and progression.
  • In three of them the proper diagnosis was delayed significantly and they were diagnosed in the first or even second relapse of the tumour.
  • CONCLUSION: Unfavourable course and treatment results in MTM located in nonorbital and nonparameningeal head and neck region in our patients result from initial wrong histopathological diagnosis and delayed therapy institution.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / therapy. Sarcoma / diagnosis. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Diagnosis, Differential. Female. Fibrosarcoma / diagnosis. Fibrosarcoma / therapy. Hemangiosarcoma / diagnosis. Hemangiosarcoma / therapy. Humans. Infant. Leiomyosarcoma / diagnosis. Leiomyosarcoma / therapy. Male. Neoplasm Staging. Prognosis. Retrospective Studies. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Treatment Outcome

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  • (PMID = 16719161.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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25. Chugh R, Wathen JK, Maki RG, Benjamin RS, Patel SR, Meyers PA, Priebat DA, Reinke DK, Thomas DG, Keohan ML, Samuels BL, Baker LH: Phase II multicenter trial of imatinib in 10 histologic subtypes of sarcoma using a bayesian hierarchical statistical model. J Clin Oncol; 2009 Jul 1;27(19):3148-53
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  • Rules for early termination within each disease type were based on a Bayesian hierarchical probability model (BHM) accounting for correlation of the responses of the 10 subtypes.
  • Available tissue samples were analyzed for molecules within the KIT/platelet-derived growth factor receptor (PDGFR) signal transduction pathway.
  • A CBR was achieved in 28 patients treated overall and by subtype: two angiosarcomas (n = 16), 0 Ewing (n = 13), one fibrosarcoma (n = 12), six leiomyosarcomas (n = 29), seven liposarcomas (n = 31), three malignant fibrous histiocytomas (n = 30), five osteosarcomas (n = 27), one malignant peripheral-nerve sheath tumor (n = 7), 0 rhabdomyosarcoma (n = 2), and three synovial sarcomas (n = 22).
  • CONCLUSION This is the first phase II study of a new agent in sarcoma to include sufficient patients with each of the common histologic subtypes to permit generalizable conclusions.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Piperazines / therapeutic use. Pyrimidines / therapeutic use. Sarcoma / drug therapy. Sarcoma / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Bayes Theorem. Benzamides. Disease-Free Survival. Female. Humans. Imatinib Mesylate. Immunohistochemistry. Male. Middle Aged. Polymerase Chain Reaction. Treatment Outcome. Young Adult

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  • [ErratumIn] J Clin Oncol. 2009 Sep 20;27(27):4630. Myers, Paul A [corrected to Meyers, Paul A]
  • (PMID = 19451433.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
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