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1. Mantripragada KK, Díaz de Ståhl T, Patridge C, Menzel U, Andersson R, Chuzhanova N, Kluwe L, Guha A, Mautner V, Dumanski JP, Upadhyaya M: Genome-wide high-resolution analysis of DNA copy number alterations in NF1-associated malignant peripheral nerve sheath tumors using 32K BAC array. Genes Chromosomes Cancer; 2009 Oct;48(10):897-907
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  • [Title] Genome-wide high-resolution analysis of DNA copy number alterations in NF1-associated malignant peripheral nerve sheath tumors using 32K BAC array.
  • Neurofibromatosis Type I (NF1) is an autosomal dominant disorder characterized by the development of both benign and malignant tumors.
  • The lifetime risk for developing a malignant peripheral nerve sheath tumor (MPNST) in NF1 patients is approximately 10% with poor survival rates.
  • To date, the molecular basis of MPNST development remains unclear.
  • Here, we report the first genome-wide and high-resolution analysis of DNA copy number alterations in MPNST using the 32K bacterial artificial chromosome microarray on a series of 24 MPNSTs and three neurofibroma samples.
  • The profiles of malignant samples, however, revealed specific loss of chromosomal regions including 1p35-33, 1p21, 9p21.3, 10q25, 11q22-23, 17q11, and 20p12.2 as well as gain of 1q25, 3p26, 3q13, 5p12, 5q11.2-q14, 5q21-23, 5q31-33, 6p23-p21, 6p12, 6q15, 6q23-q24, 7p22, 7p14-p13, 7q21, 7q36, 8q22-q24, 14q22, and 17q21-q25.
  • Copy number gains were more frequent than deletions in the MPNST samples (62% vs. 38%).
  • [MeSH-major] Comparative Genomic Hybridization / methods. Genes, Neurofibromatosis 1. Nerve Sheath Neoplasms / genetics. Neurofibromatosis 1 / genetics. Oligonucleotide Array Sequence Analysis / methods. Skin Neoplasms / genetics
  • [MeSH-minor] Adult. Chromosome Aberrations. Chromosomes, Artificial, Bacterial. Female. Gene Dosage. Genome, Human. Humans. Male. Middle Aged

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  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • (PMID = 19603524.001).
  • [ISSN] 1098-2264
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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2. Thanapaisal C, Koonmee S, Siritunyaporn S: Malignant peripheral nerve sheath tumor of breast in patient without Von Recklinghausen's neurofibromatosis: a case report. J Med Assoc Thai; 2006 Mar;89(3):377-9
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  • [Title] Malignant peripheral nerve sheath tumor of breast in patient without Von Recklinghausen's neurofibromatosis: a case report.
  • Malignant peripheral nerve sheath tumor (MPNST) of the breast without Von Recklinghausen's neurofibromatosis (VRN) is extremely rare.
  • The histologic diagnosis was made with immunohistochemical study in which the tumor showed positivity of vimentin, S-100 protein, neuron-specific protein(NSE), neurofilament protein(NF) and glial fibrillary acidic protein(GFAP).
  • [MeSH-major] Breast Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adult. Biopsy. Female. Glial Fibrillary Acidic Protein / analysis. Humans. Neurofibroma / diagnosis. Prognosis. Rare Diseases. S100 Proteins / analysis. Vimentin / analysis

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  • (PMID = 16696423.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Thailand
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein; 0 / S100 Proteins; 0 / Vimentin
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3. Pusceddu S, Bajetta E, Buzzoni R, Carcangiu ML, Platania M, Del Vecchio M, Ditto A: Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report. Int J Gynecol Pathol; 2008 Oct;27(4):596-600
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  • [Title] Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report.
  • A rare variant of malignant melanoma (MM) of the uterine cervix that mimics a malignant peripheral nerve sheath tumor (MPNST) is described.
  • Neoadjuvant chemotherapy and total abdominal hysterectomy and bilateral salpingo-ovariectomy plus pelvic lymphadenectomy were performed, and the diagnosis was MPNST, FIGO IIB.
  • A pathological review was obtained in our institution by a gynecological pathologist, who defined the primary neoplasm in the cervix as an MM, with a pattern of growth histologically simulating an MPNST, metastatic to the vagina.
  • To our knowledge, this is the first report in literature of MM of the uterine cervix resembling MPNST.
  • Despite its rarity, this variant of MM should be considered when a diagnosis of cervix MPNST is made.
  • [MeSH-major] Melanoma / pathology. Nerve Sheath Neoplasms / pathology. Uterine Cervical Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Histocytochemistry. Humans


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4. Widemann BC: Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep; 2009 Jul;11(4):322-8
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  • [Title] Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive soft tissue sarcomas that rarely occur in the general population but have a lifetime incidence of 8% to 13% in those with neurofibromatosis type 1 (NF1).
  • The response rate of MPNSTs to standard chemotherapeutic agents used to treat pediatric and adult soft tissue sarcomas is unknown and is currently undergoing evaluation in a multi-institutional clinical trial.
  • This knowledge, coupled with the availability of preclinical MPNST models, likely will accelerate the development of effective treatments for this malignancy.
  • [MeSH-major] Nerve Sheath Neoplasms / genetics. Nerve Sheath Neoplasms / therapy. Neurofibromatosis 1 / complications
  • [MeSH-minor] Clinical Trials as Topic. DNA-Binding Proteins / genetics. Genetic Predisposition to Disease. Genome-Wide Association Study. Nuclear Proteins / genetics. Receptor, Epidermal Growth Factor / genetics. Tumor Protein p73. Tumor Suppressor Protein p53 / genetics. Tumor Suppressor Proteins / genetics. Vascular Endothelial Growth Factor A / genetics

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  • (PMID = 19508838.001).
  • [ISSN] 1534-6269
  • [Journal-full-title] Current oncology reports
  • [ISO-abbreviation] Curr Oncol Rep
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / Nuclear Proteins; 0 / TP53 protein, human; 0 / Tumor Protein p73; 0 / Tumor Suppressor Protein p53; 0 / Tumor Suppressor Proteins; 0 / Vascular Endothelial Growth Factor A; EC 2.7.10.1 / Receptor, Epidermal Growth Factor
  • [Number-of-references] 80
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5. Thanakit V, Shuangshoti S, Ruangvejvorachai P: Expression of CyclinD1, p27kp-1, and bcl-2 in plexiform neurofibroma with and without malignant transformation in neurofibromatosis type 1. J Med Assoc Thai; 2006 Sep;89 Suppl 3:S53-7
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  • [Title] Expression of CyclinD1, p27kp-1, and bcl-2 in plexiform neurofibroma with and without malignant transformation in neurofibromatosis type 1.
  • Plexiform neurofibroma (PNF) has a low potential to undergo malignant transformation.
  • Identification of markers associated with tumor progression is important since it may serve as prognostic indicators or adjuncts to standard pathological examination.
  • Six of the cases had progressed into malignant peripheral nerve sheath tumor (MPNST), and the transitional area of each sample was also stained separately in order to identify protein(s) associated with tumor progression.
  • The protein is, thus, likely to play a role in the malignant transformation.
  • There was no significant difference in the expression of p27kip-1 and bcl-2 during the malignant progression of PNF.
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / metabolism. Cell Transformation, Neoplastic. Child. Cyclin-Dependent Kinase Inhibitor p27. Disease Progression. Female. Humans. Immunohistochemistry. Male. Middle Aged


6. Houreih MA, Eyden B, Deolekar M, Banerjee S: A case of fibroblastic low-grade malignant peripheral nerve sheath tumor--a true neurofibrosarcoma. Ultrastruct Pathol; 2007 Sep-Oct;31(5):347-56
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  • [Title] A case of fibroblastic low-grade malignant peripheral nerve sheath tumor--a true neurofibrosarcoma.
  • The authors report a case of low-grade retroperitoneal malignant peripheral nerve sheath tumor (MPNST) showing Schwannian and fibroblastic differentiation in individual tumor cells.
  • The tumor was detected in a 29-year-old male and posed diagnostic difficulty because of the unusual morphologic and immunophenotypic features.
  • Morphologic examination of the H&E sections revealed a rather circumscribed, highly vascular, moderately cellular spindle cell tumor.
  • There were satellite nodules outside the main tumor mass and low mitotic activity but no necrosis.
  • The tumor cells stained strongly and diffusely for both S-100 protein and CD34.
  • Although the capacity of MPNST to exhibit divergent differentiation is well known, fibroblastic differentiation is generally poorly and inconsistently documented.
  • The present case represents an unambiguous demonstration of the co-expression within individual tumor cells of Schwannian and fibroblastic differentiation in a low-grade MPNST.
  • [MeSH-major] Fibroblasts / pathology. Nerve Sheath Neoplasms / pathology. Neurofibrosarcoma / pathology
  • [MeSH-minor] Adult. Antigens, CD34 / analysis. Biomarkers, Tumor / analysis. Cell Nucleus / ultrastructure. Cytoplasm / ultrastructure. Humans. Male. Microscopy, Electron, Transmission. S100 Proteins / analysis. Schwann Cells / ultrastructure

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  • (PMID = 17963184.001).
  • [ISSN] 1521-0758
  • [Journal-full-title] Ultrastructural pathology
  • [ISO-abbreviation] Ultrastruct Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD34; 0 / Biomarkers, Tumor; 0 / S100 Proteins
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7. Subhashraj K, Balanand S, Pajaniammalle S: Ancient schwannoma arising from mental nerve. A case report and review. Med Oral Patol Oral Cir Bucal; 2009 Jan;14(1):E12-4
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  • [Title] Ancient schwannoma arising from mental nerve. A case report and review.
  • Schwannoma is an intraoral rare, benign neoplasm derived from the nerve sheath of peripheral nerves.
  • "Ancient schwannoma" shows histopathological features, such as degenerative changes and atypical nuclei, and may easily be confused with malignant neoplasms.
  • Ancient schwannoma of the head and neck region is relatively uncommon and very few cases had been reported in the oral cavity.
  • We present a case of ancient schwannoma arising from the mental nerve in a 19 year old male which was of eight months duration.
  • Ultrasonography showed that the tumor was closely associated with the mental nerve on the left side, suggestive of a peripheral neural sheath tumor.
  • Complete excision of the lesion was done under local anesthesia, preserving the mental nerve.
  • The histological picture was strongly suggestive of ancient schwannoma (Antoni A type).
  • [MeSH-major] Chin / innervation. Neurilemmoma / pathology. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Humans. Male. Young Adult

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  • (PMID = 19114949.001).
  • [ISSN] 1698-6946
  • [Journal-full-title] Medicina oral, patología oral y cirugía bucal
  • [ISO-abbreviation] Med Oral Patol Oral Cir Bucal
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 11
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8. Karatzoglou P, Karagiannidis A, Kountouras J, Christofiridis CV, Karavalaki M, Zavos C, Gavalas E, Patsiaoura K, Vougiouklis N: Von Recklinghausen's disease associated with malignant peripheral nerve sheath thmor presenting with constipation and urinary retention: a case report and review of the literature. Anticancer Res; 2008 Sep-Oct;28(5B):3107-13
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  • [Title] Von Recklinghausen's disease associated with malignant peripheral nerve sheath thmor presenting with constipation and urinary retention: a case report and review of the literature.
  • A malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm arising from peripheral nerve sheaths.
  • We herein report the first case of MPNST originating from the left gluteal muscle region, diffusely extending into the adjacent small pelvis and perineum.
  • A computed tomography scan revealed a giant tumor which displaced the bladder and segments of the intestine.
  • The histopathological diagnosis was MPNST.
  • [MeSH-major] Constipation / etiology. Nerve Sheath Neoplasms / complications. Neurofibromatosis 1 / complications. Urinary Retention / etiology
  • [MeSH-minor] Adult. Fatal Outcome. Humans. Male

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  • (PMID = 19031965.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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9. Otsuka H, Graham MM, Kubo A, Nishitani H: FDG-PET/CT findings of sarcomatous transformation in neurofibromatosis: a case report. Ann Nucl Med; 2005 Feb;19(1):55-8
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  • About 5% of patients with NF-1 develop sarcomatous transformation of a malignant peripheral nerve sheath tumor which arises from plexiform neurofibromas and is often associated with a poor prognosis.
  • Morphologic imaging techniques such as Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) are the standard methods to define the anatomic extent of the tumor, although tumor heterogeneity prevents reliable differentiation between benign and malignant lesions.
  • The degree of fluoro-deoxyglucose (FDG) uptake correlates with histologic grade in neurogenic tumors in NF-1 patients.
  • Our patient had a huge mass in the left gluteus area with a large nearly circular focus of increased FDG uptake in the tumor.
  • We surmised that the high FDG uptake indicated a high grade sarcoma, which was confirmed histologically.
  • FDG-PET/CT can identify sarcomatous change from benign neurogenic tumor with minimal misregistration, and can also detect metastatic disease.
  • [MeSH-major] Cell Transformation, Neoplastic / pathology. Fluorodeoxyglucose F18. Neurofibromatoses / radiography. Neurofibromatoses / radionuclide imaging. Sarcoma / radiography. Sarcoma / radionuclide imaging. Subtraction Technique
  • [MeSH-minor] Adult. Humans. Male. Positron-Emission Tomography / methods. Radiopharmaceuticals. Tomography, X-Ray Computed / methods

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  • (PMID = 15770975.001).
  • [ISSN] 0914-7187
  • [Journal-full-title] Annals of nuclear medicine
  • [ISO-abbreviation] Ann Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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10. Thorson JA, Weigelin HC, Ruiz RE, Howard JK, Lucas DR: Identification of SYT-SSX transcripts from synovial sarcomas using RT-multiplex PCR and capillary electrophoresis. Mod Pathol; 2006 May;19(5):641-7
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  • Synovial sarcomas are highly malignant tumors of soft tissue which are characterized by the t(X;18) resulting in SYT-SSX fusion transcript production.
  • In a study of 32 formalin-fixed soft tissue tumor specimens, the assay detected chimeric transcripts from 17/22 (77%) synovial sarcomas.
  • Chimeric transcripts were not detected in 9/9 malignant peripheral nerve sheath tumors or 1/1 epithelioid sarcoma.
  • [MeSH-major] Biomarkers, Tumor / genetics. Electrophoresis, Capillary / methods. Oncogene Proteins, Fusion / genetics. Reverse Transcriptase Polymerase Chain Reaction / methods. Sarcoma, Synovial / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Base Sequence. Child. Female. Genotype. Humans. Male. Middle Aged. Molecular Sequence Data. RNA, Neoplasm / genetics. RNA, Neoplasm / metabolism. Reproducibility of Results. Sequence Analysis, DNA. Transcription, Genetic / genetics

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  • (PMID = 16547469.001).
  • [ISSN] 0893-3952
  • [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
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Oncogene Proteins, Fusion; 0 / RNA, Neoplasm; 0 / SYT-SSX fusion protein
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11. Santaella Y, Borrego I, López J, Ortiz MJ, Vázquez R: [18-FDG-PET in a case of recurrent malignant schwannoma]. Rev Esp Med Nucl; 2005 Mar-Apr;24(2):127-30
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  • [Title] [18-FDG-PET in a case of recurrent malignant schwannoma].
  • [Transliterated title] Diagnóstico de recurrencia mediante FDG-PET en un caso clínico de schwannoma maligno.
  • The peripheral nerve sarcoma, also called malignant schwannoma, is originally a soft tissue sarcoma.
  • It is mainly located in the peripheral sheath nerve of the limbs and usually infiltrates the nerve fibres.
  • We present the case of a thirty year old woman with a malignant schwannoma in her left leg sciatic nerve who had been treated on several occasions.
  • PET can be a useful technique to detect recurrence for this kind of tumor, mainly in patients who have been previously radiated when the MRI is insufficient to perform a differential diagnosis between postirradiation fibrosis and tumoral recurrence, allowing for suitable therapeutic management of the patient.
  • [MeSH-major] Fluorodeoxyglucose F18. Neurilemmoma / radionuclide imaging. Peripheral Nervous System Neoplasms / radionuclide imaging. Positron-Emission Tomography. Radiopharmaceuticals. Sciatic Neuropathy / radionuclide imaging
  • [MeSH-minor] Adult. Female. Humans

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  • (PMID = 15745683.001).
  • [ISSN] 0212-6982
  • [Journal-full-title] Revista española de medicina nuclear
  • [ISO-abbreviation] Rev Esp Med Nucl
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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12. Thomas C, Somani N, Owen LG, Malone JC, Billings SD: Cutaneous malignant peripheral nerve sheath tumors. J Cutan Pathol; 2009 Aug;36(8):896-900
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  • [Title] Cutaneous malignant peripheral nerve sheath tumors.
  • Cutaneous malignant peripheral nerve sheath tumors (MPNSTs) are rare entities compared with their deep soft tissue counterparts.
  • The second case presented in an 88-year-old man with no stigmata of neurofibromatosis as a rapidly growing subcutaneous tumor of the right calf.
  • The diagnosis of MPNST was rendered in both cases.
  • [MeSH-minor] Adult. Aged, 80 and over. Female. Humans. Male. Mitosis

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  • (PMID = 19586501.001).
  • [ISSN] 1600-0560
  • [Journal-full-title] Journal of cutaneous pathology
  • [ISO-abbreviation] J. Cutan. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
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13. Keizman D, Issakov J, Meller I, Maimon N, Ish-Shalom M, Sher O, Merimsky O: Expression and significance of EGFR in malignant peripheral nerve sheath tumor. J Neurooncol; 2009 Sep;94(3):383-8
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  • [Title] Expression and significance of EGFR in malignant peripheral nerve sheath tumor.
  • Malignant peripheral nerve sheath tumor (MPNST) is an aggressive sarcoma.
  • The study was aimed at investigating the expression and prognostic influence of EGFR in MPNST.
  • Forty-three percentage of 46 patients with MPNST overexpressed EGFR in the primary tumor, and had a higher prevalence of advanced-stage tumors (>or=IIc, 46% vs. 80%, P = 0.011).
  • EGFR appeared to play a role in MPNST progression.
  • Clinical trials of targeting EGFR in MPNST are warranted.
  • [MeSH-major] Nerve Sheath Neoplasms / metabolism. Receptor, Epidermal Growth Factor / metabolism
  • [MeSH-minor] Adult. Cohort Studies. Female. Humans. Logistic Models. Male. Middle Aged. Multivariate Analysis. Neoplasm Staging. Prognosis. Retrospective Studies. Young Adult

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  • [ErratumIn] J Neurooncol. 2009 Sep;94(3):389. Meimon, Natalie [corrected to Maimon, Natalie]
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  • (PMID = 19330289.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Receptor, Epidermal Growth Factor
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14. Shimada S, Tsuzuki T, Kuroda M, Nagasaka T, Hara K, Takahashi E, Hayakawa S, Ono K, Maeda N, Mori N, Illei PB: Nestin expression as a new marker in malignant peripheral nerve sheath tumors. Pathol Int; 2007 Feb;57(2):60-7
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  • [Title] Nestin expression as a new marker in malignant peripheral nerve sheath tumors.
  • Malignant peripheral nerve sheath tumor (MPNST) can be difficult to diagnose because it lacks specific immunohistochemical markers.
  • S-100, which is a useful marker of MPNST, has limited diagnostic utility.
  • The diagnostic utility of immunostains for nestin and three other neural markers (S-100, CD56 and protein gene product 9.5 (PGP 9.5)) were evaluated in 35 cases of MPNST and in other spindle cell tumors.
  • All MPNST cases were strongly positive for nestin and had cytoplasmic staining.
  • Nestin was negative in 10/10 leiomyomas, and weak nestin expression was seen in 10/10 schwannomas, 3/10 neurofibromas, 2/8 synovial sarcomas, 2/10 liposarcomas, 4/7 carcinosarcomas and 3/7 malignant fibrous histiocytomas.
  • Nestin is more sensitive for MPNST than other neural markers and immunostains for nestin in combination with other markers could be useful in the diagnosis of MPNST.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Intermediate Filament Proteins / metabolism. Nerve Sheath Neoplasms / metabolism. Nerve Tissue Proteins / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Cauda Equina / metabolism. Cauda Equina / pathology. Cell Transformation, Neoplastic / genetics. Cell Transformation, Neoplastic / metabolism. Cell Transformation, Neoplastic / pathology. Child. Female. Gene Expression Regulation, Neoplastic. Humans. Leiomyosarcoma / metabolism. Leiomyosarcoma / pathology. Male. Melanoma / metabolism. Melanoma / pathology. Middle Aged. Nestin. Neurilemmoma / metabolism. Neurilemmoma / pathology. Rhabdomyosarcoma / metabolism. Rhabdomyosarcoma / pathology. Sarcoma / metabolism. Sarcoma / pathology. Schwann Cells / metabolism. Schwann Cells / pathology

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  • (PMID = 17300669.001).
  • [ISSN] 1320-5463
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Intermediate Filament Proteins; 0 / NES protein, human; 0 / Nerve Tissue Proteins; 0 / Nestin
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15. Upadhyaya M, Kluwe L, Spurlock G, Monem B, Majounie E, Mantripragada K, Ruggieri M, Chuzhanova N, Evans DG, Ferner R, Thomas N, Guha A, Mautner V: Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs). Hum Mutat; 2008 Jan;29(1):74-82
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  • [Title] Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs).
  • About 10% of neurofibromatosis type 1 (NF1) patients develop malignant peripheral nerve sheath tumors (MPNSTs) and represent considerable patient morbidity and mortality.
  • Elucidation of the genetic mechanisms by which inherited and acquired NF1 disease gene variants lead to MPNST development is important.
  • The NF1 germline mutation spectrum was similar to that previously identified in adult NF1 patients without MPNST.
  • Somatic NF1 mutations were identified in tumor DNA from 31 out of 34 MPNSTs, of which 28 were large genomic deletions.
  • The high prevalence (>90%) of such deletions in MPNST contrast with the =or<20% found in benign neurofibromas and is indicative of the involvement of different mutational mechanisms in these tumors.
  • [MeSH-major] Germ-Line Mutation. Mutation. Nerve Sheath Neoplasms / genetics. Neurofibromin 1 / genetics. Peripheral Nervous System Neoplasms / genetics
  • [MeSH-minor] Adult. DNA Mutational Analysis. DNA, Neoplasm / metabolism. Humans. Loss of Heterozygosity. Lymphocytes / metabolism. Sequence Deletion. Tumor Suppressor Protein p53 / genetics. Tumor Suppressor Protein p53 / metabolism

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17960768.001).
  • [ISSN] 1098-1004
  • [Journal-full-title] Human mutation
  • [ISO-abbreviation] Hum. Mutat.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Neurofibromin 1; 0 / Tumor Suppressor Protein p53
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16. Shimizu J, Arano Y, Murata T, Ishikawa N, Yachi T, Nomura T, Minato H: A case of intrathoracic giant malignant peripheral nerve sheath tumor in neurofibromatosis type I (von Recklinghausen's disease). Ann Thorac Cardiovasc Surg; 2008 Feb;14(1):42-7
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  • [Title] A case of intrathoracic giant malignant peripheral nerve sheath tumor in neurofibromatosis type I (von Recklinghausen's disease).
  • She underwent surgery to alleviate respiratory and circulatory disorders caused by compression of the right lung and inferior vena cava due to the giant tumor.
  • Intraoperatively, the tumor was found to have originated from the 5th intercostal nerve.
  • The resected tumor was 20x17x15 cm in size and 2,300 g in weight.
  • It was histologically diagnosed as a malignant peripheral nerve sheath tumor.
  • Seven months after surgery, however, a recurrent tumor was found in the right thoracic cavity.
  • She died of rapid growth of recurrent tumor 3 months thereafter.
  • This tumor often complicates neurofibromatosis I and has a high frequency of local recurrence and distant metastasis, resulting in poor prognosis.
  • Neither an optimal extent of resection needed for complete resection of this tumor nor an optimal regimen of chemotherapy, radiotherapy, or other therapy for the tumor has yet been established.
  • [MeSH-major] Nerve Sheath Neoplasms / surgery. Neurofibromatosis 1 / complications. Thoracic Neoplasms / surgery
  • [MeSH-minor] Adult. Diagnosis, Differential. Fatal Outcome. Humans. Magnetic Resonance Imaging. Neoplasm Recurrence, Local. Tomography, X-Ray Computed


17. Murovic JA, Gibbs IC, Chang SD, Mobley BC, Park J, Adler JR Jr: Foraminal nerve sheath tumors: intermediate follow-up after cyberknife radiosurgery. Neurosurgery; 2009 Feb;64(2 Suppl):A33-43
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  • [Title] Foraminal nerve sheath tumors: intermediate follow-up after cyberknife radiosurgery.
  • OBJECTIVE: To conduct a retrospective review of outcomes in 15 patients with 18 foraminal tumors, including 17 benign peripheral nerve sheath tumors and 1 malignant peripheral nerve sheath tumor, who underwent CyberKnife (Accuray, Inc., Sunnyvale, CA) radiosurgery at Stanford University Medical Center from 1999 to 2006.
  • METHODS: Symptoms and findings, neurofibromatosis (NF) association, previous radiation, imaging, dosimetry, tumor volume, central necrosis, and the relation of these factors to outcomes were evaluated.
  • Three patients had NF1-associated neurofibromas, 1 patient with NF2 had a schwannoma, and 1 patient had a schwannomatosis-related lesion.
  • Two likely radiation-induced lesions, a neurofibroma and a malignant peripheral nerve sheath tumor, were observed.
  • Prescribed doses ranging from 16 to 24 Gy, delivered in 1 to 3 fractions of 6 to 20 Gy, resulted in maximum tumor doses ranging from 20.9 to 30 Gy.
  • Tumor volumes decreased in 12 (67%) of 18 tumors and increased in 3 tumors.
  • Tumor volumes decreased in 8 of 10 schwannomas and 3 of 7 neurofibromas.
  • CONCLUSION: CyberKnife radiosurgery resulted in pain relief and functional preservation in selected foraminal peripheral nerve sheath tumors and a malignant peripheral nerve sheath tumor.
  • [MeSH-major] Nerve Sheath Neoplasms / surgery. Radiosurgery. Spinal Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neurofibromatoses / complications. Retrospective Studies. Treatment Outcome

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  • (PMID = 19165072.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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18. Hemalatha AL, Karthikeyan TM, Bharatnur SS, Kumar AS: Malignant peripheral nerve sheath tumor in oral cavity--a rare site. Indian J Pathol Microbiol; 2006 Jul;49(3):397-9
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  • [Title] Malignant peripheral nerve sheath tumor in oral cavity--a rare site.
  • MPNST occurring in oral cavity, which is a rare site for the tumour, in a 35 year old female patient with history of swelling underneath the tongue present since one year diagnosed clinically as ranula is presented here.
  • Histopathological examination of the excised mass showed features of spindle cell sarcoma following which a provisional diagnosis of MPNST was offered.
  • [MeSH-major] Mouth / pathology. Mouth Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans

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  • (PMID = 17001897.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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19. 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.
  • CONCLUSION: Location of the primary tumor is a key factor for prognosis.
  • [MeSH-major] Head and Neck Neoplasms / mortality. Nerve Sheath Neoplasms / mortality
  • [MeSH-minor] Adult. Aged. Female. Humans. Male. Middle Aged. Prognosis. Survival Analysis

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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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20. Levy AD, Patel N, Dow N, Abbott RM, Miettinen M, Sobin LH: From the archives of the AFIP: abdominal neoplasms in patients with neurofibromatosis type 1: radiologic-pathologic correlation. Radiographics; 2005 Mar-Apr;25(2):455-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Mutations of the NF1 gene lead to abnormal tumor suppression.
  • Consequently, patients with NF1 have an increased prevalence of benign and malignant neoplasms throughout the body.
  • There are five categories of NF1 tumors that occur in the abdomen: neurogenic, neuroendocrine, nonneurogenic gastrointestinal mesenchymal, embryonal, and miscellaneous.
  • Malignant peripheral nerve sheath tumor is an aggressive malignancy that is the most common malignant tumor of the abdomen in patients with NF1.
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Nerve Sheath Neoplasms / diagnosis. Neuroendocrine Tumors / diagnosis. Neurofibroma / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 15798063.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 77
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21. Guo H, Xiong Y, Nong L, Zhang S, Li T: [Reassessment of the pathological diagnosis in 33 cases of malignant fibrous histiocytoma]. Beijing Da Xue Xue Bao; 2008 Aug 18;40(4):374-9
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  • [Title] [Reassessment of the pathological diagnosis in 33 cases of malignant fibrous histiocytoma].
  • OBJECTIVE: Since malignant fibrous histiocytoma (MFH) may be taken as an undifferentiated pleomorphic sarcoma (UPS), this study was conducted to reassess 33 previously diagnosed MFH cases in the past 10 years based on the latest WHO concept.
  • METHODS: Thirty-three cases in tissue microarray were studied by immunohistochemistry with panels of neurogenic, myogenic, and lipogenic antibodies.
  • RESULTS: Among the 33 cases, 17 cases (51.5%) of MFH had their diagnoses changed, including 5 leiomyosarcomas, 3 malignant peripheral nerve sheath tumors, 1 fibrosarcoma, 1 inflammatory myofibrosarcoma, 1 giant cell tumor and 1 angiomatoid fibrous histiocytoma.
  • The median tumor size was 6.0 cm (range: 3.0 to 14.0 cm), 8 cases (50%) located in lower limb and 5 cases (31.3%) located in thigh.
  • Eleven cases (68.8%) variously expressed CD68 (KP1) and 7 cases (43.8%) expressed CD68 (PG-M1), which were much higher than leiomyosarcoma, malignant peripheral nerve sheath tumor and liposarcoma with significant difference.
  • CONCLUSION: MFH/UPS often show marked histological pleomorphism, and the diagnosis must be made by exclusion of other definitive sarcomas, especially myogenic and neurogenic sarcoma.
  • [MeSH-minor] Adult. Aged. Diagnosis, Differential. Humans. Immunohistochemistry. Middle Aged

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  • (PMID = 18677383.001).
  • [ISSN] 1671-167X
  • [Journal-full-title] Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
  • [ISO-abbreviation] Beijing Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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22. Brekke HR, Kolberg M, Skotheim RI, Hall KS, Bjerkehagen B, Risberg B, Domanski HA, Mandahl N, Liestøl K, Smeland S, Danielsen HE, Mertens F, Lothe RA: Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors. Neuro Oncol; 2009 Oct;11(5):514-28
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  • [Title] Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors.
  • The purpose of this study was to identify new prognostic biomarkers with clinical impact in malignant peripheral nerve sheath tumor (MPNST), a highly aggressive malignancy for which no consensus therapy exists besides surgery.
  • We have used tissue microarrays (TMAs) to assess in situ expression of 14 cell-cycle-regulating proteins in 64 well-characterized MPNST patients: 36 sporadic and 28 with neurofibromatosis type 1 (NF1).
  • We developed a new software application for evaluation and logistics of the TMA images and performed a literature survey of cell cycle proteins in MPNST.
  • For the total series of MPNSTs, p53 was shown to be an independent predictor of survival, and patients without remission, with tumor size larger than 8 cm, and with positive p53 expression had a 60 times greater risk of dying within the first 5 years compared with the remaining patients (p = 0.000002).
  • This is the most comprehensive study of in situ protein expression in MPNST so far, and expressed p53 was found to be a strong surrogate marker for outcome.
  • Patients in complete remission with a primary p53-positive MPNST diagnosis may be considered in a high-risk subgroup and candidates for adjuvant treatment.
  • [MeSH-major] Biomarkers, Tumor / analysis. Nerve Sheath Neoplasms / mortality. Nerve Sheath Neoplasms / pathology. Tumor Suppressor Protein p53 / biosynthesis
  • [MeSH-minor] Adult. Blotting, Western. Comparative Genomic Hybridization. Disease-Free Survival. Female. Gene Expression. Humans. Image Processing, Computer-Assisted / methods. Immunohistochemistry. Kaplan-Meier Estimate. Male. Middle Aged. Software. Tissue Array Analysis

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  • (PMID = 19182148.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Tumor Suppressor Protein p53
  • [Other-IDs] NLM/ PMC2765341
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23. Kohashi K, Oda Y, Yamamoto H, Tamiya S, Matono H, Iwamoto Y, Taguchi T, Tsuneyoshi M: Reduced expression of SMARCB1/INI1 protein in synovial sarcoma. Mod Pathol; 2010 Jul;23(7):981-90
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  • [Title] Reduced expression of SMARCB1/INI1 protein in synovial sarcoma.
  • Synovial sarcoma is classified as a tumor of uncertain differentiation, and some synovial sarcomas have rhabdoid cells.
  • In previous studies, all malignant rhabdoid tumors and renal medullary carcinomas, some extraskeletal myxoid chondrosarcomas, almost all epithelioid sarcomas and half of epithelioid malignant peripheral nerve sheath tumors showed a loss of SMARCB1/INI1 protein expression in tumor cells and all of these tumors are also known to have rhabdoid cells.
  • We analyzed the immunohistochemical and mRNA expression of SMARCB1/INI1 in 95 synovial sarcomas (73 monophasic fibrous type, 18 biphasic type and 4 poorly differentiated type) and 30 spindle cell sarcomas (3 adult fibrosarcomas, 7 fibrosarcomas arising in dermatofibrosarcoma protuberans, 10 leiomyosarcomas and 10 malignant peripheral nerve sheath tumors) resembling monophasic fibrous synovial sarcoma.
  • The results have shown that 66 of the 95 synovial sarcoma cases (69%) had reduced SMARCB1/INI1 protein expression, whereas the remaining 29 cases (31%) and all 30 spindle cell sarcomas showed preserved this protein expression.
  • The median values of SMARCB1/INI1 mRNA expression in non-tumor skeletal muscle and synovial sarcoma with reduced protein expression were 12.86 and 134.01, respectively, and a statistically significant difference was detected between these two groups (P=0.0000004).
  • However, there was no statistically significant difference of prognosis between the synovial sarcoma group with reduced and that with preserved SMARCB1/INI1 protein expression (P=0.46).
  • Therefore, it was suggested that there is a post-transcriptional SMARCB1/INI1 regulatory mechanism in the tumor cells of synovial sarcoma.
  • [MeSH-major] Chromosomal Proteins, Non-Histone / biosynthesis. DNA-Binding Proteins / biosynthesis. Sarcoma, Synovial / metabolism. Sarcoma, Synovial / pathology. Soft Tissue Neoplasms / metabolism. Soft Tissue Neoplasms / pathology. Transcription Factors / biosynthesis
  • [MeSH-minor] Biomarkers, Tumor / analysis. Blotting, Western. Humans. Immunohistochemistry. RNA, Messenger / analysis. Reverse Transcriptase Polymerase Chain Reaction. Survival Analysis

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  • (PMID = 20305614.001).
  • [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 / Biomarkers, Tumor; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / RNA, Messenger; 0 / SMARCB1 protein, human; 0 / Transcription Factors
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24. Hui P, Li N, Johnson C, De Wever I, Sciot R, Manfioletti G, Tallini G: HMGA proteins in malignant peripheral nerve sheath tumor and synovial sarcoma: preferential expression of HMGA2 in malignant peripheral nerve sheath tumor. Mod Pathol; 2005 Nov;18(11):1519-26
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  • [Title] HMGA proteins in malignant peripheral nerve sheath tumor and synovial sarcoma: preferential expression of HMGA2 in malignant peripheral nerve sheath tumor.
  • Histological separation of synovial sarcomas from malignant peripheral nerve sheath tumors can be difficult and available immunohistochemical markers sometimes give rise to overlapping staining patterns.
  • To this end, we explored diagnostic applications of HMGA (HMGA1 and HMGA2) protein immunohistochemistry in comparable groups of synovial sarcoma and malignant peripheral nerve sheath tumors.
  • The histological diagnosis of these cases was confirmed by the presence or absence of synovial sarcoma specific SYT-SSX fusion transcript analyzed by real-time reverse transcription polymerase chain reaction.
  • In all, 13 malignant peripheral nerve sheath tumors and 15 synovial sarcomas were included in this study.
  • Immunohistochemically, most malignant peripheral nerve sheath tumors expressed both HMGA1 and HMGA2 protein (12/13 and 12/13 cases, respectively) with moderate to strong nuclear staining patterns.
  • However, significant immunoreactivity for HMGA2 was present in the glandular component of a biphasic tumor (1/1) and rarely detected in monophasic synovial sarcomas (1/14).
  • In summary, expression of HMGA2 is a feature of MPNST but not of synovial sarcoma and immunohistochemical staining of HMGA2 may be a useful marker to separate malignant peripheral nerve sheath tumor from synovial sarcoma.
  • [MeSH-major] HMGA Proteins / biosynthesis. Nerve Sheath Neoplasms / diagnosis. Sarcoma, Synovial / diagnosis
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Male. Oncogene Proteins, Fusion / genetics. Reverse Transcriptase Polymerase Chain Reaction

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  • [Copyright] Modern Pathology (2005) 18, 1519-1526. doi:10.1038/modpathol.3800464; published online 29 July 2005.
  • (PMID = 16056249.001).
  • [ISSN] 0893-3952
  • [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
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / HMGA Proteins; 0 / Oncogene Proteins, Fusion; 0 / SYT-SSX fusion protein
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25. Okoshi A, Shiga K, Sasaki K, Asada Y, Nishikawa H, Kobayashi T, Watanabe M: [Two cases of radiation-induced sarcoma after radiation therapy in nasopharyngeal carcinoma]. Nihon Jibiinkoka Gakkai Kaiho; 2008 Jul;111(7):533-6
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  • [Title] [Two cases of radiation-induced sarcoma after radiation therapy in nasopharyngeal carcinoma].
  • We report two cases of radiation-induced sarcoma after chemoradiation therapy in nasopharyngeal carcinoma.
  • Case 1: A 40-year-old man developed a malignant peripheral nerve sheath tumor (MPNST) in the posterior floor of the nasal cavity 10 years after treatment for nasopharyngeal cancer.
  • Case 2: A 64 year-old man developed a malignant fibrous histiocytoma (MFH) of the lower gum 10 years after treatment of nasopharyngeal cancer.
  • Despite radical surgery, the man with MPNST had a recurrent tumor and died of the disease.
  • [MeSH-major] Histiocytoma, Malignant Fibrous / etiology. Nasopharyngeal Neoplasms / etiology. Neoplasms, Radiation-Induced / etiology. Nerve Sheath Neoplasms / etiology. Radiotherapy / adverse effects
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carcinoma, Squamous Cell / therapy. Cisplatin / administration & dosage. Cisplatin / therapeutic use. Combined Modality Therapy. Fatal Outcome. Fluorouracil / administration & dosage. Humans. Male. Middle Aged. Radiotherapy Dosage. Treatment Outcome

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  • (PMID = 18697477.001).
  • [ISSN] 0030-6622
  • [Journal-full-title] Nihon Jibiinkoka Gakkai kaiho
  • [ISO-abbreviation] Nippon Jibiinkoka Gakkai Kaiho
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] Q20Q21Q62J / Cisplatin; U3P01618RT / Fluorouracil; CF regimen
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26. Tawbi H, Thomas D, Lucas DR, Biermann JS, Schuetze SM, Hart AL, Chugh R, Baker LH: Epidermal growth factor receptor expression and mutational analysis in synovial sarcomas and malignant peripheral nerve sheath tumors. Oncologist; 2008 Apr;13(4):459-66
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  • [Title] Epidermal growth factor receptor expression and mutational analysis in synovial sarcomas and malignant peripheral nerve sheath tumors.
  • BACKGROUND: Synovial sarcomas (SnSrcs) and malignant peripheral nerve sheath tumors (MPNSTs) are rare mesenchymal tumors of adolescence and young adulthood.
  • METHODS: Tissue microarrays containing 48 cases of SnSrc and 32 cases of MPNST were stained for EGFR, EGFRvIII, and activated EGFR (pY1068-EGFR).
  • Tumor DNA was extracted from fresh and formalin-fixed, paraffin-embedded tissue blocks and sequenced for exons 17-21 of EGFR and exon 2 of K-ras and b-raf.
  • No K-ras or b-raf mutations were observed in either tumor type.
  • CONCLUSIONS: Expression of EGFR in SnSrcs and MPNSTs with an intact EGFR/mitogen-activated protein kinase pathway has been hypothesized to contribute to the malignant potential of these tumors.
  • [MeSH-major] Mutation. Nerve Sheath Neoplasms / diagnosis. Protein Kinase Inhibitors / therapeutic use. Receptor, Epidermal Growth Factor / genetics. Sarcoma, Synovial / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Child. DNA Mutational Analysis. Female. Gene Expression Regulation, Neoplastic. Genes, ras / genetics. Humans. Immunohistochemistry. Male. Middle Aged. Proto-Oncogene Proteins B-raf / genetics. Translocation, Genetic


27. Kresse SH, Skårn M, Ohnstad HO, Namløs HM, Bjerkehagen B, Myklebost O, Meza-Zepeda LA: DNA copy number changes in high-grade malignant peripheral nerve sheath tumors by array CGH. Mol Cancer; 2008;7:48
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  • [Title] DNA copy number changes in high-grade malignant peripheral nerve sheath tumors by array CGH.
  • BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are rare and highly aggressive soft tissue tumors showing complex chromosomal aberrations.
  • In order to identify recurrent chromosomal regions of gain and loss, and thereby novel gene targets of potential importance for MPNST development and/or progression, we have analyzed DNA copy number changes in seven high-grade MPNSTs using microarray-based comparative genomic hybridization (array CGH).
  • CONCLUSION: Our study shows the potential of using DNA copy number changes obtained by array CGH to predict the prognosis of MPNST patients.
  • [MeSH-major] DNA, Neoplasm / analysis. Gene Dosage. Gene Expression Profiling / methods. Gene Expression Regulation, Neoplastic. Nerve Sheath Neoplasms / genetics. Oligonucleotide Array Sequence Analysis. Soft Tissue Neoplasms / genetics
  • [MeSH-minor] Adenovirus E1A Proteins / genetics. Adult. Aged. Amino Acid Oxidoreductases / genetics. Antigens, Neoplasm / genetics. Chromosomes, Human, Pair 17. Chromosomes, Human, Pair 8. DNA Topoisomerases, Type II / genetics. DNA-Binding Proteins / genetics. Female. Gene Expression Regulation, Enzymologic. Humans. Inhibitor of Apoptosis Proteins. Male. Microtubule-Associated Proteins / genetics. Middle Aged. Neoplasm Proteins / genetics. Prognosis. Proto-Oncogene Proteins / genetics. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 18522746.001).
  • [ISSN] 1476-4598
  • [Journal-full-title] Molecular cancer
  • [ISO-abbreviation] Mol. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Adenovirus E1A Proteins; 0 / Antigens, Neoplasm; 0 / BIRC5 protein, human; 0 / DNA, Neoplasm; 0 / DNA-Binding Proteins; 0 / ETV4 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; 0 / Proto-Oncogene Proteins; EC 1.4.- / Amino Acid Oxidoreductases; EC 1.4.3.- / LOXL2 protein, human; EC 5.99.1.3 / DNA Topoisomerases, Type II; EC 5.99.1.3 / DNA topoisomerase II alpha
  • [Other-IDs] NLM/ PMC2442610
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28. Allison KH, Patel RM, Goldblum JR, Rubin BP: Superficial malignant peripheral nerve sheath tumor: a rare and challenging diagnosis. Am J Clin Pathol; 2005 Nov;124(5):685-92
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  • [Title] Superficial malignant peripheral nerve sheath tumor: a rare and challenging diagnosis.
  • We reviewed the clinicopathologic features of 5 cases of malignant peripheral nerve sheath tumor (MPNST) manifesting in superficial locations associated with cutaneous neurofibromas (4 cases) or superficial peripheral nerve (1 case).
  • However, identification of a benign precursor or origin from a nerve may be the most definitive way to properly classify these rare lesions.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Male. S100 Proteins / analysis

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  • (PMID = 16203275.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / S100 Proteins
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29. Melliou A, Karamouzis J, Helis L, Mouzakiti A, Theocharidis G, Karkavelas G, Kouroussis C: Malignant peripheral nerve-sheath tumor of the left cerebello-pontine angle: case report. J BUON; 2006 Jul-Sep;11(3):367-8
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  • [Title] Malignant peripheral nerve-sheath tumor of the left cerebello-pontine angle: case report.
  • [MeSH-major] Cerebellar Neoplasms. Cerebellopontine Angle. Nerve Sheath Neoplasms
  • [MeSH-minor] Adult. Female. Humans

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  • (PMID = 17309166.001).
  • [ISSN] 1107-0625
  • [Journal-full-title] Journal of B.U.ON. : official journal of the Balkan Union of Oncology
  • [ISO-abbreviation] J BUON
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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30. Kang GH, Kim KM, Noh JH, Sohn TS, Kim S, Park CK, Lee CS, Kang DY: WT-1 expression in gastrointestinal stromal tumours. Pathology; 2010 Jan;42(1):54-7
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  • Leiomyosarcomas, schwannomas, malignant peripheral nerve sheath tumours (MPNSTs) and melanomas showed cytoplasmic staining, whereas leiomyomas and mesenteric fibromatoses were totally negative for WT-1.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Gastrointestinal Stromal Tumors / metabolism. Soft Tissue Neoplasms / metabolism. WT1 Proteins / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Cytoplasm / metabolism. Cytoplasm / pathology. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Prognosis. Receptor, Platelet-Derived Growth Factor alpha / metabolism

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  • (PMID = 20025481.001).
  • [ISSN] 1465-3931
  • [Journal-full-title] Pathology
  • [ISO-abbreviation] Pathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / WT1 Proteins; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha
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31. Karabatsou K, Kiehl TR, Wilson DM, Hendler A, Guha A: Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1. Neurosurgery; 2009 Oct;65(4 Suppl):A160-70
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  • [Title] Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1.
  • OBJECTIVE: Benign plexiform neurofibromas (PNfib), especially those occurring in patients with neurofibromatosis type 1, are at a significant risk of progressing to a malignant peripheral nerve sheath tumor (MPNST).
  • Early diagnosis, followed by radical surgery and adjuvant radiation to maintain local tumor control, is of critical importance to prevent metastasis and subsequent mortality from MPNSTs.
  • However, early diagnosis is hampered by the sensitivity of current imaging modalities such as computed tomography (CT) or magnetic resonance imaging to reliably detect this malignant transformation, which can occur heterogeneously in a PNfib to a MPNST.
  • METHODS: In this prospective study, 9 neurofibromatosis type 1-associated PNfibs suspected to have undergone transformation to an MPNST were preoperatively evaluated by 18FDG-PET/CT and magnetic resonance imaging.
  • A detailed histological evaluation correlated the average and regional standard uptake value (SUV) from the 18FDG-PET/CT to grade of malignancy of the suspected MPNST.
  • Stratification of the maximal SUV to low (<4.0), intermediate (4.0-7.0), or high (>7.0) correlated to the proliferative index (Ki-67) and grade of MPNST.
  • A maximal SUV of more than 7.0 was closely correlated to a focus of malignant transformation.
  • [MeSH-major] Nerve Sheath Neoplasms / radionuclide imaging. Neurofibroma / radionuclide imaging. Neurofibromatosis 1 / radionuclide imaging. Peripheral Nerves / radionuclide imaging. Peripheral Nervous System Neoplasms / radionuclide imaging. Positron-Emission Tomography / methods
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Fluorodeoxyglucose F18. Humans. Male. Middle Aged. Predictive Value of Tests. Prospective Studies. Tomography, X-Ray Computed. Young Adult


32. Matsumine A, Shintani K, Kusuzaki K, Matsubara T, Satonaka H, Wakabayashi T, Iino T, Uchida A: Expression of decorin, a small leucine-rich proteoglycan, as a prognostic factor in soft tissue tumors. J Surg Oncol; 2007 Oct 1;96(5):411-8
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  • RESULTS: Lower levels of decorin were expressed in liposarcoma and malignant peripheral nerve sheath tumor than in lipoma (<0.01) and neurofibroma (P < 0.05), respectively.
  • An immunohistochemical analysis for spindle-cell sarcomas demonstrated decorin protein to be produced by myofibroblastic cells in the peripheral stromal extracellular spaces.
  • CONCLUSIONS: A reduced decorin expression was found to be a useful biomarker of aggressiveness in soft tissue tumor.
  • [MeSH-major] Extracellular Matrix Proteins / metabolism. Neoplasms, Connective and Soft Tissue / metabolism. Nerve Sheath Neoplasms / metabolism. Proteoglycans / metabolism. Soft Tissue Neoplasms / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor. Child. Child, Preschool. DNA, Neoplasm / metabolism. Decorin. Female. Humans. Immunohistochemistry. Male. Middle Aged. Polymerase Chain Reaction. Prognosis. Proportional Hazards Models. RNA, Neoplasm / metabolism. Survival Analysis

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  • (PMID = 17579351.001).
  • [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
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DCN protein, human; 0 / DNA, Neoplasm; 0 / Decorin; 0 / Extracellular Matrix Proteins; 0 / Proteoglycans; 0 / RNA, Neoplasm
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33. Gonzalez LF, Lekovic GP, Eschbacher J, Coons S, Spetzler RF: A true malignant schwannoma of the eighth cranial nerve: case report. Neurosurgery; 2007 Aug;61(2):E421-2; discussion E422
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  • [Title] A true malignant schwannoma of the eighth cranial nerve: case report.
  • OBJECTIVE: The clinical presentation, pathology, treatment, and outcome of a 43-year-old woman with a malignant peripheral nerve sheath tumor arising from a benign schwannoma of the eighth cranial nerve are presented.
  • CLINICAL PRESENTATION: Initially, the tumor was debulked.
  • After finding malignant areas within the benign tumor, it was considered to be a malignant transformation of a previously benign tumor.
  • Postoperatively, the tumor bed was radiated for palliation.
  • [MeSH-major] Cranial Nerve Neoplasms / pathology. Neuroma, Acoustic / secondary. Vestibulocochlear Nerve / pathology
  • [MeSH-minor] Adult. Dura Mater / pathology. Fatal Outcome. Female. Humans. Magnetic Resonance Imaging. Meningeal Neoplasms / secondary

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  • (PMID = 17762727.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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34. Chamoun RB, Whitehead WE, Dauser RC, Luerssen TG, Okcu MF, Adesina AM, Jea A: Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature. Pediatr Neurosurg; 2009;45(3):230-6
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  • [Title] Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature.
  • The authors report a rare case of primary disseminated intradural malignant peripheral nerve sheath tumor (MPNST) of the spine in a 5-year-old child without neurofibromatosis type I (NF-I).
  • MRI revealed a large intradural extramedullary tumor at C4-5 with dissemination to the thoracic spine, cauda equina and leptomeninges.
  • Following a 2-level cervical laminectomy, the tumor was biopsied and debulked.
  • Based on pathological and immunohistological findings, the tumor was diagnosed as an MPNST.
  • The reported clinical outcomes for adult and pediatric patients with intradural MPNST are very poor.
  • We report the first pediatric case--without or with NF-I--of disseminated intradural MPNST primarily localized proximal to the conus medullaris.
  • [MeSH-major] Magnetic Resonance Imaging. Nerve Sheath Neoplasms / pathology. Spinal Cord Neoplasms / pathology

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  • [Copyright] Copyright 2009 S. Karger AG, Basel.
  • (PMID = 19521138.001).
  • [ISSN] 1423-0305
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 38
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35. Trufant JW, Brenn T, Fletcher CD, Virata AR, Cook DL, Bosenberg MW: Melanotic schwannoma arising in association with nevus of Ota: 2 cases suggesting a shared mechanism. Am J Dermatopathol; 2009 Dec;31(8):808-13
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  • [Title] Melanotic schwannoma arising in association with nevus of Ota: 2 cases suggesting a shared mechanism.
  • Melanotic schwannoma is a rare markedly pigmented peripheral nerve sheath tumor comprising cells with prominent melanization and schwannian features.
  • We present the first 2 reported cases of melanotic schwannoma arising in patients with a history of nevus of Ota, a rare dermal melanosis believed to represent a failure of melanocyte migration to the epidermis during embryogenesis.
  • Case 1 involves a 40-year-old woman with a 1.8-cm, deeply pigmented, trigeminal nerve mass and pigmentation of the maxillary sinus mucosa and bone.
  • Microscopically, both masses consist of partially encapsulated epithelioid and spindle cells with abundant melanin pigment, arising in association with peripheral nerves.
  • Morphological, immunohistochemical, and ultrastructural features support a diagnosis of melanotic schwannoma.
  • Melanotic schwannoma is most often benign but has been associated with malignant behavior in some cases.
  • Distinguishing this nerve sheath tumor from malignant melanoma can be difficult but is of great clinical importance due to differences in prognosis and treatment.
  • [MeSH-major] Head and Neck Neoplasms / pathology. Neoplasms, Multiple Primary / pathology. Neurilemmoma / pathology. Nevus of Ota / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Adult. Female. Humans. Immunohistochemistry. Melanins. Middle Aged


36. Landy H, Feun L, Markoe A, Patchen S, Bruce J, Marcus J, Levi A: Extended remission of a recurrent median nerve malignant peripheral nerve sheath tumor after multimodal treatment. Case report. J Neurosurg; 2005 Oct;103(4):760-3
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  • [Title] Extended remission of a recurrent median nerve malignant peripheral nerve sheath tumor after multimodal treatment. Case report.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are difficult to control despite aggressive treatment.
  • In this report the authors describe the treatment and follow-up review of a patient with neurofibromatosis Type I who harbored a recurrent median nerve MPNST.
  • Two courses of preoperative intraarterial cisplatin and intravenous Adriamycin produced significant tumor shrinkage.
  • Gross-total removal of the remaining tumor without amputation of the arm was followed by fractionated radiotherapy (total minimum tumor dose 6485 cGy, maximal dose 6575 cGy).
  • The patient is alive 9.5 years after treatment without evidence of tumor recurrence and with only focal median nerve functional deficits.
  • [MeSH-major] Neoplasm Recurrence, Local / surgery. Nerve Sheath Neoplasms / surgery. Neurofibromatosis 1 / surgery
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cisplatin / administration & dosage. Combined Modality Therapy. Doxorubicin / administration & dosage. Humans. Male. Treatment Outcome


37. Ziadi A, Saliba I: Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. Auris Nasus Larynx; 2010 Oct;37(5):539-45
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  • [Title] Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review.
  • OBJECTIVES: The incidence of malignant peripheral nerve sheath tumor (MPNST) is approximately 0.001%.
  • (1) to review all cases of intracranial MPNST described in the literature, (2) to highlight the suspicion of intracranial MPNST, (3) to identify the gross pathology, the histopathology, the immunohistochemistry, (4) to discuss the differential diagnosis, the treatment, the recurrence rate, the follow-up, the incidence of metastasis and the prognosis.
  • We used the following Keywords: "malignant peripheral nerve sheath tumor", "cranial nerve", "neurosarcoma", "malignant schwannoma", "neurofibroma", "malignant neurofibroma" and "nerve tumor".
  • We considered cases where MPNST involved an intracranial cranial nerve.
  • RESULTS: We identified 32 cases of cranial MPNST including our case.
  • Most cases are developed sporadically (50%), 31% arise from a malignant transformation of schwannoma and 19% from a neurofibroma.
  • The cranial nerve VIII is the most involved (15/32), followed by the Vth (10/32) and the VIIth (5/32).
  • MPNST will strongly express protein S-100 and collagen IV-laminin.
  • 13 cases were treated with radiotherapy for tumor recurrence and metastasis.
  • CONCLUSION: MPNST of cranial nerves are very rare.
  • In neurofibroma, even though MPNST is mainly associated to type 1, we should keep in mind its association to NF2.
  • Inaccessibility of cranial MPNST may explain the subtotal resection and thus the poor prognosis.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Cell Transformation, Neoplastic / pathology. Child. Child, Preschool. Diagnosis, Differential. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / pathology. Neurilemmoma / diagnosis. Neurilemmoma / pathology. Neurilemmoma / radiotherapy. Neurilemmoma / surgery. Neurofibroma / diagnosis. Neurofibroma / pathology. Neurofibroma / radiotherapy. Neurofibroma / surgery. Neurofibromatosis 1 / diagnosis. Neurofibromatosis 1 / pathology. Neurofibromatosis 1 / radiotherapy. Neurofibromatosis 1 / surgery. Neurofibromatosis 2 / diagnosis. Neurofibromatosis 2 / pathology. Neurofibromatosis 2 / radiotherapy. Neurofibromatosis 2 / surgery. Radiotherapy, Adjuvant. Spinal Neoplasms / mortality. Spinal Neoplasms / pathology. Spinal Neoplasms / secondary. Young Adult

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  • [Copyright] Copyright 2010 Elsevier Ireland Ltd. All rights reserved.
  • (PMID = 20399579.001).
  • [ISSN] 1879-1476
  • [Journal-full-title] Auris, nasus, larynx
  • [ISO-abbreviation] Auris Nasus Larynx
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 35
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38. Zhang PJ, Brooks JS, Goldblum JR, Yoder B, Seethala R, Pawel B, Gorman JH, Gorman RC, Huang JH, Acker M, Narula N: Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol; 2008 Sep;39(9):1385-95
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  • Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm.
  • There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas.
  • In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up.
  • Tumor grade appeared to be prognostically important in cardiac sarcoma.
  • [MeSH-major] Heart Neoplasms / pathology. Sarcoma / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Infant. Male. Middle Aged. Survival Analysis

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  • (PMID = 18602663.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / R01 HL063954
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS522251; NLM/ PMC4081532
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39. Spurlock G, Knight SJ, Thomas N, Kiehl TR, Guha A, Upadhyaya M: Molecular evolution of a neurofibroma to malignant peripheral nerve sheath tumor (MPNST) in an NF1 patient: correlation between histopathological, clinical and molecular findings. J Cancer Res Clin Oncol; 2010 Dec;136(12):1869-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular evolution of a neurofibroma to malignant peripheral nerve sheath tumor (MPNST) in an NF1 patient: correlation between histopathological, clinical and molecular findings.
  • OBJECTIVE: Neurofibromatosis type 1 (NF1) patients have a 13% risk of developing a malignant peripheral nerve sheath tumor (MPNST).
  • Many MPNSTs are histopathologically complex, with regions exhibiting features of the original benign plexiform neurofibroma (PNF), of an atypical PNF, or of MPNST showing varying degrees of de-differentiation.
  • This study analyzed the genetic alterations associated with this pathological heterogeneity in order to identify the genetic processes involved in transformation from a benign to an aggressive malignant tumor.
  • METHODS: A histological and molecular analysis of a single MPNST tumor that was subdivided into three histopathologically distinct regions, a benign PNF (region 1), an atypical PNF (region 2), and a high-grade MPNST (region 3), was carried out.
  • Tumor DNA from each region was analyzed in conjunction with the patient's lymphocyte DNA.
  • The NF1-associated LOH analysis found that LOH increased in the three tumor areas, with 9, 42, and 97% LOH evident in regions 1, 2, and 3, respectively.
  • Additional genetic changes, including losses of TP53, RB1, CDKN2A, and of several oncogenes and cell-cycle genes, were found only in the malignant MPNST (region 3).
  • DISCUSSION: This is the first study that correlates the histological and molecular changes associated with MPNST development, confirming the significant cellular and genetic heterogeneity that poses both diagnostic and therapeutic challenges.
  • [MeSH-major] Nerve Sheath Neoplasms / genetics. Neurofibroma / genetics. Neurofibromatosis 1 / genetics. Neurofibromin 1 / genetics
  • [MeSH-minor] Adult. Base Sequence. Comparative Genomic Hybridization. DNA Mutational Analysis. Disease Progression. Germ-Line Mutation. Humans. Loss of Heterozygosity. Male. Molecular Sequence Data


40. Pasmant E, Ortonne N, Rittié L, Laurendeau I, Lévy P, Lazar V, Parfait B, Leroy K, Dessen P, Valeyrie-Allanore L, Perbal B, Wolkenstein P, Vidaud M, Vidaud D, Bièche I: Differential expression of CCN1/CYR61, CCN3/NOV, CCN4/WISP1, and CCN5/WISP2 in neurofibromatosis type 1 tumorigenesis. J Neuropathol Exp Neurol; 2010 Jan;69(1):60-9
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  • Neurofibromatosis type 1 patients with plexiform neurofibromas are at risk of developing malignant peripheral nerve sheath tumors.
  • Several CCN gene family members were dysregulated in neurofibromatosis type 1 tumorigenesis; the angiogenic gene CCN1/CYR61 was specifically upregulated in the plexiform neurofibromas; CCN4/WISP1 was upregulated, and CCN3/NOV and CCN5/WISP2 were downregulated in paired comparisons of plexiform neurofibroma and malignant peripheral nerve sheath tumor from the same patients.
  • [MeSH-minor] Adolescent. Adult. CCN Intercellular Signaling Proteins. Carcinogenicity Tests / methods. Cell Differentiation / genetics. Cells, Cultured. Cysteine-Rich Protein 61 / genetics. Cysteine-Rich Protein 61 / metabolism. Female. Gene Expression Profiling / methods. Humans. Intracellular Signaling Peptides and Proteins / genetics. Intracellular Signaling Peptides and Proteins / metabolism. Male. Middle Aged. Nephroblastoma Overexpressed Protein / genetics. Nephroblastoma Overexpressed Protein / metabolism. Oligonucleotide Array Sequence Analysis / methods. Proto-Oncogene Proteins / genetics. Proto-Oncogene Proteins / metabolism. RNA, Messenger / metabolism. Repressor Proteins. Schwann Cells / physiology. Statistics, Nonparametric. Transcription Factors / genetics. Transcription Factors / metabolism. Young Adult

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  • (PMID = 20010302.001).
  • [ISSN] 1554-6578
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CCN Intercellular Signaling Proteins; 0 / CYR61 protein, human; 0 / Cysteine-Rich Protein 61; 0 / Intercellular Signaling Peptides and Proteins; 0 / Intracellular Signaling Peptides and Proteins; 0 / NOV protein, human; 0 / Nephroblastoma Overexpressed Protein; 0 / Proto-Oncogene Proteins; 0 / RNA, Messenger; 0 / Repressor Proteins; 0 / Transcription Factors; 0 / WISP1 protein, human; 0 / WISP2 protein, human
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41. Wasa J, Nishida Y, Tsukushi S, Shido Y, Sugiura H, Nakashima H, Ishiguro N: MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol; 2010 Jun;194(6):1568-74
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas.
  • OBJECTIVE: The objective of this study was to identify the MRI criteria that best differentiate malignant peripheral nerve sheath tumors from benign neurofibromas.
  • MATERIALS AND METHODS: We retrospectively analyzed MR images obtained for 41 histologically diagnosed cases of malignant peripheral nerve sheath tumor and 20 cases of neurofibroma that had been treated at four tertiary institutions.
  • Twenty of the patients with malignant peripheral nerve sheath tumors and 14 patients with neurofibromas developed the disease in association with neurofibromatosis 1.
  • The MR images were evaluated with regard to tumor size, signal intensity, heterogeneity of T1- and T2-weighted MR images, enhancement pattern, definition of margins, presence of perilesional edemalike zone, and presence of intratumoral cystic lesions.
  • RESULTS: Significant differences between malignant peripheral nerve sheath tumors and neurofibromas were noted for the largest dimension of the mass, peripheral enhancement pattern, perilesional edemalike zone, and intratumoral cystic lesion.
  • In cases associated with neurofibromatosis 1, heterogenicity on T1-weighted images was also significant in differentiating neurofibroma from malignant peripheral nerve sheath tumor.
  • The presence of two or more of the four features suggestive of malignancy indicated malignant peripheral nerve sheath tumor with a sensitivity of 61% and a specificity of 90%.
  • CONCLUSION: The MR features described in this study are useful for distinguishing malignant peripheral nerve sheath tumors from neurofibromas.
  • If a tumor has two or more of the four statistically significant features, it can be considered to be highly suspicious of malignancy and should be subjected to a biopsy for early diagnosis.
  • [MeSH-major] Magnetic Resonance Imaging / methods. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Chi-Square Distribution. Contrast Media. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Neurofibroma / diagnosis. Neurofibroma / pathology. Retrospective Studies. Statistics, Nonparametric

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  • (PMID = 20489098.001).
  • [ISSN] 1546-3141
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media
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42. Dang JD, Cohen PR: Segmental neurofibromatosis and malignancy. Skinmed; 2010 May-Jun;8(3):156-9
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  • The malignancies include malignant peripheral nerve sheath tumor (3), malignant melanoma (2), breast cancer (1), colon cancer (1), gastric cancer (1), lung cancer (1), and Hodgkin lymphoma (1).
  • The most common malignancies in patients with segmental neurofibromatosis are derived from neural crest cells: malignant peripheral nerve sheath tumor and malignant melanoma.
  • [MeSH-minor] Adult. Aged. Cafe-au-Lait Spots / etiology. Cafe-au-Lait Spots / pathology. Female. Humans. Incidence. Male. Middle Aged. Neural Crest / cytology. Neural Crest / pathology. Risk

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  • (PMID = 21137621.001).
  • [ISSN] 1540-9740
  • [Journal-full-title] Skinmed
  • [ISO-abbreviation] Skinmed
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
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43. Rodriguez AO, Truskinovsky AM, Kasrazadeh M, Leiserowitz GS: Case report: Malignant peripheral nerve sheath tumor of the uterine cervix treated with radical vaginal trachelectomy. Gynecol Oncol; 2006 Jan;100(1):201-4
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  • [Title] Case report: Malignant peripheral nerve sheath tumor of the uterine cervix treated with radical vaginal trachelectomy.
  • BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST) are rare tumors which occur primarily in major nerve trunks and most commonly in patients with neurofibromatosis.
  • CASE: We report a 22-year-old woman with MPNST of the uterine cervix which recurred after loop electrocautery excision and had positive margins on cone biopsy.
  • CONCLUSIONS: MPNST of the uterine cervix is a rare, but potentially aggressive neoplasm.
  • This is the first case of such a tumor treated successfully with preservation of the patients fertility.
  • [MeSH-major] Neoplasm Recurrence, Local / surgery. Nerve Sheath Neoplasms / surgery. Uterine Cervical Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Fertility. Gynecologic Surgical Procedures. Humans


44. Friedrich RE, Kluwe L, Fünsterer C, Mautner VF: Malignant peripheral nerve sheath tumors (MPNST) in neurofibromatosis type 1 (NF1): diagnostic findings on magnetic resonance images and mutation analysis of the NF1 gene. Anticancer Res; 2005 May-Jun;25(3A):1699-702
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  • [Title] Malignant peripheral nerve sheath tumors (MPNST) in neurofibromatosis type 1 (NF1): diagnostic findings on magnetic resonance images and mutation analysis of the NF1 gene.
  • About 10% of patients with NF1 develop malignant peripheral nerve sheath tumors (MPNST), usually arising from PNF, and this is the major cause of poor prognosis.
  • Our objective was to establish magnetic resonance imaging (MRI) criteria for MPNST, and to test their usefulness in detecting early malignant changes in PNF and to correlate the findings with the mutations of the NF1 gene.
  • The study was approved by the Institutional Review Board and all patients gave informed consent to analyze clinical records and tumor material for scientific purposes.
  • RESULTS: MRI was performed on 50 patients with NF1 and nerve sheath tumors, of whom 7 had atypical pain, tumor growth or neurological deficits indicative of malignancy; the other 43 were asymptomatic.
  • All 3 asymptomatic patients with inhomogeneous lesions were shown to have MPNST.
  • Analysis of mutations of the NF1 gene of the 10 MPNST patients revealed a variety of mutations.
  • Concerning the correlation of genetic findings and MPNST in NF1, the sample size of this study group was too small to define genotype-phenotype relations.
  • CONCLUSION: This study provides evidence for certain radiographic findings on MRI in PNF of NF1 patients that have to be considered as signs of malignancy, in particular indicating an MPNST.
  • [MeSH-major] Genes, Neurofibromatosis 1. Mutation. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Humans. Magnetic Resonance Imaging. Middle Aged


45. Rahman M, Cook DS, Ellis G, O'keefe PA: Malignant peripheral nerve sheath tumor of the heart. Asian Cardiovasc Thorac Ann; 2006 Oct;14(5):425-7
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  • [Title] Malignant peripheral nerve sheath tumor of the heart.
  • A 32-year-old man presented with dyspnea, chest pain, palpitations and ankle edema and was found to have a tumor involving the heart.
  • This was diagnosed as malignant peripheral nerve sheath tumor, a rare sarcoma of the heart.
  • [MeSH-major] Heart Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis
  • [MeSH-minor] Adult. Fatal Outcome. Humans. Male

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  • (PMID = 17005894.001).
  • [ISSN] 1816-5370
  • [Journal-full-title] Asian cardiovascular & thoracic annals
  • [ISO-abbreviation] Asian Cardiovasc Thorac Ann
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 8
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46. Subramanian S, Thayanithy V, West RB, Lee CH, Beck AH, Zhu S, Downs-Kelly E, Montgomery K, Goldblum JR, Hogendoorn PC, Corless CL, Oliveira AM, Dry SM, Nielsen TO, Rubin BP, Fletcher JA, Fletcher CD, van de Rijn M: Genome-wide transcriptome analyses reveal p53 inactivation mediated loss of miR-34a expression in malignant peripheral nerve sheath tumours. J Pathol; 2010 Jan;220(1):58-70
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  • [Title] Genome-wide transcriptome analyses reveal p53 inactivation mediated loss of miR-34a expression in malignant peripheral nerve sheath tumours.
  • Malignant peripheral nerve sheath tumours (MPNSTs) are aggressive soft tissue tumours that occur either sporadically or in patients with neurofibromatosis type 1.
  • The malignant transformation of the benign neurofibroma to MPNST is incompletely understood at the molecular level.
  • We have determined the gene expression signature for benign and malignant PNSTs and found that the major trend in malignant transformation from neurofibroma to MPNST consists of the loss of expression of a large number of genes, rather than widespread increase in gene expression.
  • Subsequent microRNA profiling of benign and malignant PNSTs indicated a relative down-regulation of miR-34a in most MPNSTs compared to neurofibromas.
  • In vitro studies using the cell lines MPNST-14 (NF1 mutant) and MPNST-724 (from a non-NF1 individual) show that exogenous expression of p53 or miR-34a promotes apoptotic cell death.
  • In addition, exogenous expression of p53 in MPNST cells induces miR-34a and other miRNAs.
  • Our data show that p53 inactivation and subsequent loss of expression of miR-34a may significantly contribute to the MPNST development.
  • Collectively, our findings suggest that deregulation of miRNAs has a potential role in the malignant transformation process in peripheral nerve sheath tumours.
  • [MeSH-major] Genes, p53. MicroRNAs / metabolism. Nerve Sheath Neoplasms / genetics. RNA, Neoplasm / metabolism
  • [MeSH-minor] Adult. Apoptosis / genetics. Cell Adhesion / genetics. Cell Proliferation. Cluster Analysis. Down-Regulation. Female. Gene Expression Profiling / methods. Gene Expression Regulation, Neoplastic. Gene Silencing. Humans. Male. Middle Aged. Neoplasm Proteins / genetics. Neoplasm Proteins / metabolism. Neurofibroma. Oligonucleotide Array Sequence Analysis / methods. Receptor, Epidermal Growth Factor / genetics. Receptor, Epidermal Growth Factor / metabolism. Reverse Transcriptase Polymerase Chain Reaction / methods. Signal Transduction / genetics. Tumor Cells, Cultured. Tumor Suppressor Protein p53 / metabolism


47. Nicklas BJ, Smith AD, Wolff RD, Johnson FA: Malignant peripheral nerve sheath tumor arising in association with the sural nerve. J Foot Ankle Surg; 2006 Jan-Feb;45(1):38-41
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  • [Title] Malignant peripheral nerve sheath tumor arising in association with the sural nerve.
  • Malignant peripheral nerve sheath tumor is a rare sarcoma of peripheral nerves found most often in deep soft tissue.
  • This aggressive tumor is difficult to diagnose clinically and must be surgically excised for therapy.
  • The authors present a case of a 39-year-old African American woman with malignant peripheral nerve sheath tumor in association with the sural nerve.
  • The tumor was surgically removed and sent for pathologic studies.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Soft Tissue Neoplasms / pathology. Sural Nerve / pathology
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging

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  • (PMID = 16399558.001).
  • [ISSN] 1067-2516
  • [Journal-full-title] The Journal of foot and ankle surgery : official publication of the American College of Foot and Ankle Surgeons
  • [ISO-abbreviation] J Foot Ankle Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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48. Baek WS, Pytel P, Undevia SD, Rubeiz H: Spinal cord metastasis of a non-neurofibromatosis type-1 malignant peripheral nerve sheath tumor: an unusual manifestation of a rare tumor. J Neurooncol; 2005 Sep;74(2):183-5
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  • [Title] Spinal cord metastasis of a non-neurofibromatosis type-1 malignant peripheral nerve sheath tumor: an unusual manifestation of a rare tumor.
  • Malignant peripheral nerve sheath tumors are rare spindle-cell sarcomas derived from Schwann cells or pluripotent cells of the neural crest.
  • They arise from the spinal roots, peripheral nerves, brachial and lumbosacral plexi, cranial nerves and terminal nerve fibers within soft tissue, intestine, lung and bone.
  • Spinal cord metastasis from malignant nerve sheath tumors associated with neurofibromatosis type 1 is very rare.
  • We describe a rare case of near-total spinal cord metastasis in a patient with malignant nerve sheath tumor in the absence of neurofibromatosis, and highlight the microscopic findings and natural history of this disease process.
  • [MeSH-major] Lung Neoplasms / secondary. Nerve Sheath Neoplasms / pathology. Neurofibroma / pathology. Spinal Cord Neoplasms / secondary
  • [MeSH-minor] Adult. Benzenesulfonates / therapeutic use. Chemotherapy, Adjuvant. Fatal Outcome. Female. Humans. Magnetic Resonance Imaging. Niacinamide / analogs & derivatives. Phenylurea Compounds. Pyridines / therapeutic use


49. Holtkamp N, Atallah I, Okuducu AF, Mucha J, Hartmann C, Mautner VF, Friedrich RE, Mawrin C, von Deimling A: MMP-13 and p53 in the progression of malignant peripheral nerve sheath tumors. Neoplasia; 2007 Aug;9(8):671-7
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  • [Title] MMP-13 and p53 in the progression of malignant peripheral nerve sheath tumors.
  • Malignant peripheral nerve sheath tumors (MPNST) are sarcomas with poor prognosis and limited treatment options.
  • Factors contributing to tumor progression are largely unknown.
  • We therefore examined MPNST from 22 neurofibromatosis type 1 (NF1) patients, 14 non-NF1 patients, and 14 neurofibroma patients for matrix metalloproteinase 13 (MMP-13) expression.
  • MMP-13 expression was detected in 58% of MPNST and was significantly associated with recurrent MPNST (P = .019).
  • p53 was observed in 78% of MPNST and was found to be strongly associated with MMP-13 expression (P = .005).
  • TP53 mutations were found in only 11% of MPNST and were associated with high tumor grades (P = .029).
  • No significant association between mutant TP53 and MMP-13 was observed, indicating that other factors drive MMP-13 expression in MPNST.
  • In summary, our data suggest that MMP-13 expression in nerve sheath tumors is coupled with malignant progression.
  • [MeSH-major] Biomarkers, Tumor / physiology. Genes, p53 / physiology. Matrix Metalloproteinase 13 / physiology. Nerve Sheath Neoplasms / metabolism. Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Disease Progression. Female. Follow-Up Studies. Gene Expression Regulation, Neoplastic / physiology. Humans. Male. Middle Aged. Mutation. Neurofibroma / genetics. Neurofibroma / metabolism. Neurofibroma / pathology

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  • (PMID = 17786186.001).
  • [ISSN] 1476-5586
  • [Journal-full-title] Neoplasia (New York, N.Y.)
  • [ISO-abbreviation] Neoplasia
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; EC 3.4.24.- / Matrix Metalloproteinase 13
  • [Other-IDs] NLM/ PMC1950437
  • [Keywords] NOTNLM ; Malignant peripheral nerve sheath tumor / TP53 / malignant progression / matrix metalloproteinase 13 / neurofibromatosis type 1
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50. Brekke HR, Ribeiro FR, Kolberg M, Agesen TH, Lind GE, Eknaes M, Hall KS, Bjerkehagen B, van den Berg E, Teixeira MR, Mandahl N, Smeland S, Mertens F, Skotheim RI, Lothe RA: Genomic changes in chromosomes 10, 16, and X in malignant peripheral nerve sheath tumors identify a high-risk patient group. J Clin Oncol; 2010 Mar 20;28(9):1573-82
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  • [Title] Genomic changes in chromosomes 10, 16, and X in malignant peripheral nerve sheath tumors identify a high-risk patient group.
  • PURPOSE: The purpose of this study was to identify genetic aberrations contributing to clinical aggressiveness of malignant peripheral nerve sheath tumors (MPNSTs).
  • RESULTS: Forty-four (92%) MPNSTs displayed DNA copy number changes (median, 18 changes per tumor; range, 2 to 35 changes).
  • Multivariate analyses including NF1 status, tumor location, size, grade, sex, complete remission, and initial metastatic status showed that the genomic high-risk group was the most significant predictor of poor survival.
  • [MeSH-major] Chromosomes, Human, Pair 10 / genetics. Chromosomes, Human, Pair 16 / genetics. Chromosomes, Human, X / genetics. DNA Copy Number Variations. Nerve Sheath Neoplasms / genetics. Nervous System Neoplasms / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Female. Gene Expression. Genome. Humans. Male. Middle Aged. Neurofibromatosis 1 / genetics. Prognosis. Risk Factors. Survival Analysis. Young Adult

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  • (PMID = 20159821.001).
  • [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; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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51. Aoki M, Nabeshima K, Nishio J, Ishiguro M, Fujita C, Koga K, Hamasaki M, Kaneko Y, Iwasaki H: Establishment of three malignant peripheral nerve sheath tumor cell lines, FU-SFT8611, 8710 and 9817: conventional and molecular cytogenetic characterization. Int J Oncol; 2006 Dec;29(6):1421-8
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  • [Title] Establishment of three malignant peripheral nerve sheath tumor cell lines, FU-SFT8611, 8710 and 9817: conventional and molecular cytogenetic characterization.
  • Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant tumor, for which only a few cultured cell lines are available to date.
  • In the present study, we established three new MPNST cell lines, FU-SFT8611, FU-SFT8710 and FU-SFT9817, from a 40-year-old Japanese man without neurofibromatosis 1 (NF1), a 43-year-old Japanese woman with NF1, and a 61-year-old Japanese woman without NF1, respectively.
  • These newly established cell lines provide a valuable resource for biological and pathological investigations into new treatment regimes for MPNST.
  • [MeSH-major] Cell Line, Tumor. Nerve Sheath Neoplasms / genetics
  • [MeSH-minor] Adult. Animals. Cell Growth Processes / physiology. Cytogenetic Analysis / methods. Female. Humans. Immunohistochemistry. Karyotyping. Male. Mice. Mice, Inbred BALB C. Mice, Nude. Mice, SCID. Middle Aged. Neoplasm Transplantation. Neurofibromatosis 1 / genetics. Neurofibromatosis 1 / pathology. Nucleic Acid Hybridization. Transplantation, Heterologous

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  • (PMID = 17088980.001).
  • [ISSN] 1019-6439
  • [Journal-full-title] International journal of oncology
  • [ISO-abbreviation] Int. J. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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52. Stark AM, Mehdorn HM: Multiple intracranial metastases from a malignant peripheral nerve sheath tumor of the extremities. J Neurooncol; 2006 Jun;78(2):209-10
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  • [Title] Multiple intracranial metastases from a malignant peripheral nerve sheath tumor of the extremities.
  • [MeSH-major] Brain Neoplasms / secondary. Lung Neoplasms / secondary. Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adenoma / metabolism. Adenoma / pathology. Adult. Growth Hormone / metabolism. Humans. Male. Pituitary Neoplasms / metabolism. Pituitary Neoplasms / pathology. Thigh. Tomography, X-Ray Computed

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  • (PMID = 16598432.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 9002-72-6 / Growth Hormone
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53. Shimizu K, Okita R, Uchida Y, Hihara J: Long survival after resection for lung metastasis of malignant peripheral nerve sheath tumor in neurofibromatosis 1. Ann Thorac Cardiovasc Surg; 2008 Oct;14(5):322-4
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  • [Title] Long survival after resection for lung metastasis of malignant peripheral nerve sheath tumor in neurofibromatosis 1.
  • A 31-year-old man with neurofibromatosis 1 (NF1) was admitted for the treatment of solitary lung tumor.
  • Nine months earlier he had undergone a large resection for malignant peripheral nerve sheath tumors (MPNSTs) in his back.
  • Surgical resection of the right lower lobe was performed, and the tumor was pathologically diagnosed as a metastasis of MPNST.
  • The survival of patients with pulmonary metastasis of MPNST is extremely poor, especially of those with NF1, but this patient has survived 5 years without recurrence.
  • [MeSH-major] Lung Neoplasms / surgery. Nerve Sheath Neoplasms / surgery. Neurofibromatosis 1 / surgery. Pneumonectomy
  • [MeSH-minor] Adult. Humans. Male. Time Factors. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 18989250.001).
  • [ISSN] 2186-1005
  • [Journal-full-title] Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
  • [ISO-abbreviation] Ann Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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54. Miyake M, Tateishi U, Maeda T, Arai Y, Seki K, Hasegawa T, Sugimura K: A case of ganglioneuroma presenting abnormal FDG uptake. Ann Nucl Med; 2006 Jun;20(5):357-60
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  • A 26-year-old woman presented to the hospital with a slowly growing abdominal tumor without symptoms.
  • However, a second primary malignant tumor, such as malignant peripheral nerve sheath tumor arising in ganglioneuroma, could not be ruled out.
  • Pathological investigation may be needed to differentiate ganglioneuroma from other malignant tumors and, therefore, FDG-PET/CT findings can be helpful for biopsy planning.
  • [MeSH-minor] Adult. Female. Humans. Radiopharmaceuticals / pharmacokinetics

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  • (PMID = 16878708.001).
  • [ISSN] 0914-7187
  • [Journal-full-title] Annals of nuclear medicine
  • [ISO-abbreviation] Ann Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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55. Lee CC, Yen YS, Pan DH, Chung WY, Wu HM, Guo WY, Chen MT, Liu KD, Shih YH: Delayed microsurgery for vestibular schwannoma after gamma knife radiosurgery. J Neurooncol; 2010 Jun;98(2):203-12
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  • [Title] Delayed microsurgery for vestibular schwannoma after gamma knife radiosurgery.
  • However, on rare occasions, some cases have needed traditional microsurgery to remove the tumor several months or years after radiosurgery.
  • The mean size of the tumor during GKS was 10.4 ml (range 2.3-23.5 ml).
  • The indications of microsurgery included adverse radiation effect with peri-focal edema, tumor enlargement, and cyst enlargement.
  • Although the perifocal edema could lead to more difficulty in surgery than in typically performed operations for schwannoma, subtotal resection was achieved in all patients.
  • The histology showed benign tumor in five patients, malignant peripheral nerve sheath tumor in one, and necrotic tissue in one.
  • [MeSH-minor] Adult. Aged. Female. Humans. Image Processing, Computer-Assisted / methods. Ki-67 Antigen / metabolism. Longitudinal Studies. Magnetic Resonance Imaging / methods. Male. Middle Aged. Retrospective Studies. S100 Proteins / metabolism. Severity of Illness Index. Time Factors. Treatment Outcome

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  • (PMID = 20405307.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 0 / S100 Proteins
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56. Maki RG, D'Adamo DR, Keohan ML, Saulle M, Schuetze SM, Undevia SD, Livingston MB, Cooney MM, Hensley ML, Mita MM, Takimoto CH, Kraft AS, Elias AD, Brockstein B, Blachère NE, Edgar MA, Schwartz LH, Qin LX, Antonescu CR, Schwartz GK: Phase II study of sorafenib in patients with metastatic or recurrent sarcomas. J Clin Oncol; 2009 Jul 01;27(19):3133-40
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  • PURPOSE Since activity of sorafenib was observed in sarcoma patients in a phase I study, we performed a multicenter phase II study of daily oral sorafenib in patients with recurrent or metastatic sarcoma.
  • PATIENTS AND METHODS We employed a multiarm study design, each representing a sarcoma subtype with its own Simon optimal two-stage design.
  • In each arm, 12 patients who received 0 to 1 prior lines of therapy were treated (0 to 3 for angiosarcoma and malignant peripheral-nerve sheath tumor).
  • If at least one Response Evaluation Criteria in Solid Tumors (RECIST) was observed, 25 further patients with that sarcoma subtype were accrued.
  • Further evaluation of sorafenib in these and possibly other sarcoma subtypes appears warranted, presumably in combination with cytotoxic or kinase-specific agents.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Benzenesulfonates / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Pyridines / therapeutic use. Sarcoma / drug therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Disease-Free Survival. Female. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Niacinamide / analogs & derivatives. Phenylurea Compounds. Young Adult

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  • (PMID = 19451436.001).
  • [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
  • [Grant] United States / NCI NIH HHS / CA / N01CM62202; United States / NCI NIH HHS / CA / P01 CA047179
  • [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 / Benzenesulfonates; 0 / Phenylurea Compounds; 0 / Pyridines; 25X51I8RD4 / Niacinamide; 9ZOQ3TZI87 / sorafenib
  • [Other-IDs] NLM/ PMC2716936
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57. Oda Y, Saito T, Tateishi N, Ohishi Y, Tamiya S, Yamamoto H, Yokoyama R, Uchiumi T, Iwamoto Y, Kuwano M, Tsuneyoshi M: ATP-binding cassette superfamily transporter gene expression in human soft tissue sarcomas. Int J Cancer; 2005 May 10;114(6):854-62
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  • The phenomenon of multidrug resistance (MDR) in various malignant neoplasms has been reported as being caused by one or multiple expressions of ATP-binding cassette (ABC) superfamily protein, including P-glycoprotein/multidrug resistance (MDR) 1 and the MDR protein (MRP) family.
  • In 86 cases of surgically resected soft tissue sarcoma, intrinsic mRNA levels of MDR1, MRP1, MRP2 and MRP3 were assessed using a quantitative reverse transcriptase-PCR (RT-PCR) method.
  • Among the various histologic types, malignant peripheral nerve sheath tumor (MPNST) showed significantly high levels of MDR1 (p=0.017) and MRP3 (p=0.0384) mRNA expression, compared to the other tumor types.
  • P-gp expression was significantly correlated with large tumor size (> or =5 cm, p=0.041) and high AJCC stage (stages III and IV) (p=0.0365).
  • Our results suggest that MDR1/P-gp expression may have an important role to play in tumor progression in the cases of soft tissue sarcoma, and p53 may be one of the active regulators of the MDR1 transcript.
  • In addition, the high levels of both MDR1 and MRP3 mRNA expression in MPNST may help to explain the poor response of this tumor to anticancer-drugs.
  • [MeSH-major] ATP-Binding Cassette Transporters / biosynthesis. ATP-Binding Cassette Transporters / genetics. Drug Resistance, Multiple. Gene Expression Regulation, Neoplastic. Genes, p53. Sarcoma / drug therapy. Sarcoma / genetics
  • [MeSH-minor] Adolescent. Adult. Disease Progression. Drug Resistance, Neoplasm / genetics. Female. Gene Expression Profiling. Humans. Immunohistochemistry. Male. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15609299.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ATP-Binding Cassette Transporters
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58. Rawal A, Yin Q, Roebuck M, Sinopidis C, Kalogrianitis S, Helliwell TR, Frostick S: Atypical and malignant peripheral nerve-sheath tumors of the brachial plexus: report of three cases and review of the literature. Microsurgery; 2006;26(2):80-6
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  • [Title] Atypical and malignant peripheral nerve-sheath tumors of the brachial plexus: report of three cases and review of the literature.
  • Tumor involvement of the brachial plexus is uncommon.
  • Malignant peripheral nerve-sheath tumors (MPNST) are rare at this site, arising spontaneously or in the context of NF-1.
  • MPNST are intermediate or high-grade sarcomas with a high risk of local and distant spread.
  • Approximately 50% of MPNST arise in patients with NF-1, and therefore these patients should be thoroughly investigated for any new symptoms or masses.
  • MPNST of the brachial plexus should be treated with an adequate wide local excision, with adjuvant high-dose radiotherapy pre- or postoperatively.
  • The role of chemotherapy in the treatment of MPNST is not clearly defined, but it may have some benefit in salvaging treatment failures.
  • [MeSH-major] Brachial Plexus. Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Humans. Male. Neurofibromatosis 1 / complications

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  • (PMID = 16538633.001).
  • [ISSN] 0738-1085
  • [Journal-full-title] Microsurgery
  • [ISO-abbreviation] Microsurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 24
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59. Steigen SE, Schaeffer DF, West RB, Nielsen TO: Expression of insulin-like growth factor 2 in mesenchymal neoplasms. Mod Pathol; 2009 Jul;22(7):914-21
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  • The insulin-like growth factor (IGF) system plays an important role in the growth and development of cells and has been implicated in oncogenesis and tumor progression.
  • Of 20 tumor types represented by more than 10 cases, synovial sarcomas, myxoid liposarcomas, GISTs, malignant peripheral nerve sheath tumors, chondrosarcomas, undifferentiated pleomorphic sarcomas (MFH), Ewing's sarcomas and tenosynovial giant cell tumors showed high levels of expression in more than 20% of cases.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Biomarkers, Tumor. Female. Gastrointestinal Stromal Tumors / metabolism. Gastrointestinal Stromal Tumors / mortality. Gastrointestinal Stromal Tumors / pathology. Humans. Immunohistochemistry. Male. Mesenchymoma / metabolism. Mesenchymoma / pathology. Middle Aged. Nerve Sheath Neoplasms / metabolism. Nerve Sheath Neoplasms / pathology. Norway / epidemiology. Sarcoma / metabolism. Sarcoma / pathology. Survival Rate. Tissue Array Analysis. Young Adult

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  • (PMID = 19407853.001).
  • [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
  • [Grant] United States / NCI NIH HHS / CA / CA 112270
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / IGF2 protein, human; 67763-97-7 / Insulin-Like Growth Factor II
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60. Walker JB, Harkey HL, Buciuc R: Percutaneous placement of an external drain of the cisterna magna using interventional magnetic resonance imaging in a patient with a persistent cerebrospinal fluid fistula: technical case report. Neurosurgery; 2008 Aug;63(2):E375; discussion E375
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  • PRESENTATION: A 28-year-old woman with a previously diagnosed malignant peripheral nerve sheath tumor of the thoracic spine presented with a refractory postoperative cerebrospinal fluid leak complicated by diffuse meningeal carcinomatosis.
  • External lumbar drainage was unsuccessful because of complete tumor obliteration.
  • Ventricular drainage was deferred because of concern for tumor seeding, thus necessitating a more aggressive approach.
  • [MeSH-minor] Adult. Catheterization / instrumentation. Catheterization / methods. Female. Humans. Neurosurgical Procedures / instrumentation. Neurosurgical Procedures / methods. Postoperative Complications / diagnosis. Postoperative Complications / surgery

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  • (PMID = 18797320.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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61. 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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62. Kushida Y, Haba R, Kobayashi S, Ishikawa M, Doi T, Kadota K: Ectopic hamartomatous thymoma: a case report with immunohistochemical study and review of the literature. J Cutan Pathol; 2006 May;33(5):369-72
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  • Ectopic hamartomatous thymoma (EHT) is a rare benign tumor.
  • The tumor consisted of spindle cells, epithelial cells, adipose cells, and a small amount of lymphocytes, as described previously.
  • Recognition of EHT is important and needs to be differentiated from high-grade sarcomas such as synovial sarcoma or glandular malignant peripheral nerve sheath tumor.
  • [MeSH-minor] Adult. Antigens, CD / metabolism. Antigens, CD1 / metabolism. Antigens, CD20 / metabolism. Antigens, CD34 / metabolism. Antigens, CD45 / metabolism. Cell Adhesion Molecules / metabolism. Diagnosis, Differential. Humans. Keratins / metabolism. Male. Nerve Sheath Neoplasms / pathology. Sarcoma, Synovial / metabolism. Sarcoma, Synovial / pathology. Vimentin / metabolism

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  • (PMID = 16640545.001).
  • [ISSN] 0303-6987
  • [Journal-full-title] Journal of cutaneous pathology
  • [ISO-abbreviation] J. Cutan. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD1; 0 / Antigens, CD20; 0 / Antigens, CD34; 0 / CD1a antigen; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Vimentin; 68238-35-7 / Keratins; EC 3.1.3.48 / Antigens, CD45
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63. Zou C, Smith KD, Liu J, Lahat G, Myers S, Wang WL, Zhang W, McCutcheon IE, Slopis JM, Lazar AJ, Pollock RE, Lev D: Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg; 2009 Jun;249(6):1014-22
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  • [Title] Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome.
  • OBJECTIVE: Improved staging systems for malignant peripheral nerve sheath tumor (MPNST) prognostication and management are needed.
  • Consequently, we sought to identify clinical, pathologic, and molecular predictors of outcome in patients with/without neurofibromatosis type 1 (NF-1) associated MPNST.
  • METHODS: MPNST patients treated from 1986 to 2006 (n = 140) were identified; 72 had NF-1 syndrome and 68 did not.
  • Paraffin-embedded neurofibroma or MPNST blocks were assembled in a tissue microarray; marker expression was evaluated immunohistochemically.
  • The 5 years DSS for localized tumor patients was 35% for NF-1 patients and 50% for sporadic patients.
  • MPNST >or=10 cm at diagnosis, partial resection, and metastasis development were significant negative predictors of DSS; completely resected tumors that lacked S-100 immunoreactivity had a nearly 5-fold increased risk of developing distant metastasis.
  • Ki67, vascular endothelial growth factor, p53, and pMEK were over-expressed in MPNST compared with benign neurofibromas.
  • Only tumor size and nuclear p53 expression were found to be independent prognosticators for MPNST DSS in a multivariable analysis.
  • CONCLUSIONS: MPSNT is a markedly metastatic and aggressive poor prognosis tumor.
  • Multiple clinical, pathologic, and molecular markers identified in this study, coupled with findings from previous series, should be considered for an improved MPNST staging system useful for prognostic assessment and management decisions.
  • [MeSH-major] Neoplasm Recurrence, Local / epidemiology. Nerve Sheath Neoplasms / metabolism. Nerve Sheath Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers / metabolism. Child. Child, Preschool. Cohort Studies. Disease-Free Survival. Female. Humans. Infant. Male. Middle Aged. Neurofibromatosis 1 / complications. Retrospective Studies. Risk Factors. Survival Rate. Treatment Outcome. Young Adult

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  • (PMID = 19474676.001).
  • [ISSN] 1528-1140
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers
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64. Snyder LA, Linder KE, Neel JA: Malignant peripheral nerve sheath tumor in a hamster. J Am Assoc Lab Anim Sci; 2007 Nov;46(6):55-7
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  • [Title] Malignant peripheral nerve sheath tumor in a hamster.
  • An adult female golden hamster (Mesocricetus auratus) developed a firm subcutaneous mass on the lateral distal right forelimb that progressed to diffuse limb enlargement accompanied by extensive cutaneous ulceration and drainage and axillary lymph node metastasis.
  • On histology, the invasive neoplasm merged with a large subcutaneous nerve and was composed of spindle cells with a high mitotic index, and lymph node metastasis was confirmed.
  • Histologic morphology and positive immunohistochemical staining of neoplastic cells for vimentin, S100, and neuron-specific enolase were consistent with a malignant peripheral nerve sheath tumor.
  • Although relatively common in dogs, peripheral nerve sheath tumors had not been reported previously in hamsters.
  • [MeSH-major] Mesocricetus. Nerve Sheath Neoplasms / veterinary. Soft Tissue Neoplasms / veterinary

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  • (PMID = 17994674.001).
  • [ISSN] 1559-6109
  • [Journal-full-title] Journal of the American Association for Laboratory Animal Science : JAALAS
  • [ISO-abbreviation] J. Am. Assoc. Lab. Anim. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / S100 Proteins; 0 / Vimentin; EC 4.2.1.11 / Phosphopyruvate Hydratase
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65. Grimm S, Chamberlain MC: Adult primary spinal cord tumors. Expert Rev Neurother; 2009 Oct;9(10):1487-95
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  • [Title] Adult primary spinal cord tumors.
  • Resective surgery can usually be accomplished with spinal ependymomas owing to separation of tumor from spinal cord and, when complete, require no further therapy.
  • Intradural extramedullary tumors are either peripheral nerve sheath tumors (neurofibromas or schwanommas) or meningiomas.
  • Radiotherapy is reserved for rare malignant variants and for patients in whom surgery is contraindicated.
  • Primary treatment is surgery in essentially all spinal cord tumors, and predictors of outcome include preoperative functional status, histological grade of tumor and extent of surgical resection.
  • [MeSH-minor] Adult. Combined Modality Therapy. Humans. Neurosurgical Procedures. Young Adult

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  • (PMID = 19831838.001).
  • [ISSN] 1744-8360
  • [Journal-full-title] Expert review of neurotherapeutics
  • [ISO-abbreviation] Expert Rev Neurother
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 82
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66. Martinez Devesa P, Mitchell TE, Scott I, Moffat DA: Malignant peripheral nerve sheath tumors of the head and neck: two cases and a review of the literature. Ear Nose Throat J; 2006 Jun;85(6):392-6
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  • [Title] Malignant peripheral nerve sheath tumors of the head and neck: two cases and a review of the literature.
  • Malignant peripheral nerve sheath tumors are uncommon lesions that occasionally affect the head and neck.
  • One tumor involved the parotid gland and resulted in erosion of the temporal bone, and the other affected the lower lip.
  • A rapid diagnosis has significant implications for management because of the tumor's potential for aggressive behavior and its high rate of recurrence.
  • [MeSH-major] Lip Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis. Parotid Neoplasms / diagnosis
  • [MeSH-minor] Adult. Angiography. Hearing Loss, Sensorineural / etiology. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male. Middle Aged. Parotid Gland / surgery. Tomography, X-Ray Computed

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  • (PMID = 16866118.001).
  • [ISSN] 0145-5613
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 23
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67. Katenkamp K, Katenkamp D: [Low-malignant peripheral nerve sheath tumors of nasal and sinonasal mucous membranes]. Pathologe; 2005 Mar;26(2):90-5
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  • [Title] [Low-malignant peripheral nerve sheath tumors of nasal and sinonasal mucous membranes].
  • Sinonasal malignant peripheral nerve sheath tumors (MPNST) are infrequent neoplasms.
  • 16 cases of low-malignant MPNST in this localization were retrieved from the files of soft tissue tumors established in Jena.
  • The importance of an only partial immunostaining by S100 protein antibodies for diagnosis and differential diagnostic discrimination to benign peripheral nerve sheath tumors (schwannomas and neurofibromas) is explained.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Paranasal Sinus Neoplasms / pathology. S100 Proteins / analysis. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Biomarkers, Tumor / analysis. Diagnosis, Differential. Female. Humans. Male. Middle Aged

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  • (PMID = 15657686.001).
  • [ISSN] 0172-8113
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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68. Galanis E, Okuno SH, Nascimento AG, Lewis BD, Lee RA, Oliveira AM, Sloan JA, Atherton P, Edmonson JH, Erlichman C, Randlev B, Wang Q, Freeman S, Rubin J: Phase I-II trial of ONYX-015 in combination with MAP chemotherapy in patients with advanced sarcomas. Gene Ther; 2005 Mar;12(5):437-45
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  • We, therefore, undertook a phase I/II study of ONYX-015, days 1-5 every month administered intratumorally under radiographic guidance, in combination with MAP (mitomycin-C, doxorubicin, cisplatin) chemotherapy in patients with advanced sarcoma.
  • Sarcoma histologies were gastrointestinal stromal tumors (GIST, two patients), leiomyosarcoma (two patients), liposarcoma (one patient), and malignant peripheral nerve sheath tumor (1 patient).
  • ONYX-015 viral DNA was detected by quantitative PCR in the plasma of 5/6 patients on day 5 of the first cycle, and up to day 12 (7 days after the last viral dose) in one patient who had extended sampling for viral kinetics performed, suggesting viral replication in sarcoma tissue.
  • Detection of viral DNA in post treatment tumor specimens by ISH and detection of the ONYX-015 genome in the peripheral blood by quantitative PCR, up to 7 days after the last viral dose provide evidence for adenoviral replication.
  • [MeSH-major] Adenoviridae. Antineoplastic Agents / administration & dosage. Genetic Therapy / methods. Sarcoma / therapy
  • [MeSH-minor] Adult. Aged. Antibodies, Viral / blood. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Cisplatin / administration & dosage. Combined Modality Therapy. DNA, Viral / analysis. DNA, Viral / blood. Doxorubicin / administration & dosage. Female. Humans. In Situ Hybridization. Injections, Intralesional. Male. Middle Aged. Mitomycin / administration & dosage. Viral Vaccines. Virus Replication

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  • (PMID = 15647767.001).
  • [ISSN] 0969-7128
  • [Journal-full-title] Gene therapy
  • [ISO-abbreviation] Gene Ther.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 84388; United States / NCI NIH HHS / CA / U01CA 69912
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibodies, Viral; 0 / Antineoplastic Agents; 0 / DNA, Viral; 0 / ONYX015; 0 / Viral Vaccines; 50SG953SK6 / Mitomycin; 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin; MAP protocol
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69. Bredella MA, Torriani M, Hornicek F, Ouellette HA, Plamer WE, Williams Z, Fischman AJ, Plotkin SR: Value of PET in the assessment of patients with neurofibromatosis type 1. AJR Am J Roentgenol; 2007 Oct;189(4):928-35
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: The objective of our study was to investigate the use of PET in the detection of malignant peripheral nerve sheath tumors (MPNSTs) in patients with neurofibromatosis type 1 (NF1).
  • MATERIALS AND METHODS: Forty-five patients with NF1 who underwent whole-body PET for suspected MPNST based on clinical symptoms or radiologic examinations were retrospectively evaluated.
  • PET may improve preoperative tumor staging by detecting metastases or second primary tumors, which often are present in patients with NF1.
  • [MeSH-major] Fluorodeoxyglucose F18. Methionine / deficiency. Neurofibromatosis 1 / radionuclide imaging. Peripheral Nervous System Neoplasms / radionuclide imaging. Positron-Emission Tomography / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Carbon Isotopes. Female. Humans. Male. Middle Aged. Radiopharmaceuticals. Reproducibility of Results. Sensitivity and Specificity. Whole Body Imaging / methods

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  • (PMID = 17885067.001).
  • [ISSN] 1546-3141
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Carbon Isotopes; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; AE28F7PNPL / Methionine
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70. Mott RT, Goodman BK, Burchette JL, Cummings TJ: Loss of chromosome 13 in a case of soft tissue perineurioma. Clin Neuropathol; 2005 Mar-Apr;24(2):69-76
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  • Soft tissue perineuriomas are rare mesenchymal tumors that are derived from perineurial cells of the peripheral nerve sheath.
  • Histologically, the tumor was composed of a diffuse to fascicular arrangement of spindle cells with bland, elongated nuclei with long, thin, tapering cytoplasmic processes.
  • Although never described in this group of neoplasms, loss of chromosome 13 has been identified in a large number of other soft tissue tumors, particularly sarcomas and malignant peripheral nerve sheath tumors.
  • [MeSH-major] Chromosome Deletion. Chromosomes, Human, Pair 13 / genetics. Nerve Sheath Neoplasms / genetics. Soft Tissue Neoplasms / genetics. Thigh
  • [MeSH-minor] Adult. Female. Humans

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  • (PMID = 15803806.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 65
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71. Chirife AM, Bello L, Celeste F, Giménez L, Gorostidy S: [Primary sarcomas of the breast]. Medicina (B Aires); 2006;66(2):135-8
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  • Primary sarcomas of the breast are extremely rare with less than 1% of all malignant tumours of the breast reported in literature.
  • At our Institution 1315 malignant tumours of the breast were diagnosed between 1999-2004; nine of them corresponded to primary sarcomas: angiosarcoma (3), leiomyosarcoma (1), low-grade fibromyxoid sarcoma (1), dematofibrosarcoma protuberans (1), liposarcoma (1), osteosarcoma (1), malignant peripheral nerve sheath tumour (1).
  • Most of the tumours (67%) showed p53 (mayor or equal to 20% of nuclear staining) over-expression but this did not show a direct relationship with the evolution of each neoplasm.
  • [MeSH-major] Breast Neoplasms. Sarcoma
  • [MeSH-minor] Adult. Argentina / epidemiology. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Female. Humans. Incidence. Middle Aged. Phenotype. Prevalence. Prognosis. Retrospective Studies. Tumor Suppressor Protein p53 / genetics. Tumor Suppressor Protein p53 / metabolism


72. Benz MR, Czernin J, Dry SM, Tap WD, Allen-Auerbach MS, Elashoff D, Phelps ME, Weber WA, Eilber FC: Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer; 2010 Jan 15;116(2):451-8
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  • [Title] Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign.
  • BACKGROUND: Correct pretreatment classification is critical for optimizing diagnosis and treatment of patients with peripheral nerve sheath tumors (PNSTs).
  • The aim of this study was to evaluate whether F18-fluorodeoxyglucose positron emission tomography (FDG PET) can differentiate malignant (MPNST) from benign PNSTs.
  • METHODS: Thirty-four adult patients presenting with PNST who underwent a presurgical FDG PET/computed tomography (CT) scan between February 2005 and November 2008 were included in the study.
  • Tumors were characterized histologically, by FDG maximum standardized uptake value (SUV(max) [g/mL]), and by CT size (tumor maximal diameter [cm]).
  • The accuracy of FDG PET for differentiating MPNSTs from benign PNSTs (neurofibroma and schwannoma) was evaluated by receiver operating characteristic (ROC) curve analysis.
  • SUV(max) was significantly higher in MPNST compared with benign PNST (12.0 +/- 7.1 vs 3.4 +/- 1.8; P < .001).
  • By ROC curve analysis, SUV(max) reliably differentiated between benign and malignant PNSTs (area under the ROC curve of 0.97).
  • Given the difficulties in clinically evaluating PNST and in distinguishing benign PNST from MPNST, FDG PET imaging should be used for diagnostic intervention planning and for optimizing treatment strategies.

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  • (PMID = 19924789.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P50 CA086306; United States / NCI NIH HHS / CA / 5 P50 CA086306
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
  • [Other-IDs] NLM/ NIHMS324300; NLM/ PMC3188986
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73. Al Akloby O, Bukhari IA, El-Shawarby M, Al Mulhim F: Malignant peripheral nerve sheath tumor of the skin: case report. Am J Clin Dermatol; 2006;7(3):201-3
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  • [Title] Malignant peripheral nerve sheath tumor of the skin: case report.
  • Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors derived from Schwann cells or pluripotent cells of the neural crest.
  • In this report, we describe a 21-year-old Saudi woman who presented with an asymptomatic, solid, round, protruding tumor on the right upper back which was diagnosed as an MPNST with no stigmata of neurofibromatosis.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Skin Neoplasms / diagnosis
  • [MeSH-minor] Adult. Back. Female. Humans. Magnetic Resonance Imaging

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  • (PMID = 16734508.001).
  • [ISSN] 1175-0561
  • [Journal-full-title] American journal of clinical dermatology
  • [ISO-abbreviation] Am J Clin Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] New Zealand
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74. Friedrich RE, Derlin T, Hagel C: Atypical plexiform neurofibroma in NF1 with high standardised uptake value (SUV) in positron-emission tomography (PET) expressing podoplanin. In Vivo; 2010 Nov-Dec;24(6):871-6
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  • A 19-year-old female with established neurofibromatosis type 1 (NF1) diagnosis and history of malignant peripheral nerve sheath tumour (MPNST) of the lower extremities showed a tumour of her right upper extremity with a maximum standardised uptake value (SUV) of 5.7 on positron emission/computerised tomography (PET/CT) scan.
  • Scattered nerve fibres were labelled with neurofilament antibodies within the tumour.
  • This atypical neurofibroma showed a high SUV on PET that is indicative for MPNST.
  • [MeSH-minor] Biomarkers, Tumor / biosynthesis. Female. Humans. Positron-Emission Tomography. Young Adult

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  • (PMID = 21164047.001).
  • [ISSN] 1791-7549
  • [Journal-full-title] In vivo (Athens, Greece)
  • [ISO-abbreviation] In Vivo
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Membrane Glycoproteins; 0 / PDPN protein, human
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75. Maldonado AR, Klanke C, Jegga AG, Aronow BJ, Mahller YY, Cripe TP, Crombleholme TM: Molecular engineering and validation of an oncolytic herpes simplex virus type 1 transcriptionally targeted to midkine-positive tumors. J Gene Med; 2010 Jul;12(7):613-23
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  • BACKGROUND: Expression profile analyses of midkine (MDK), a multifunctional protein important in development but repressed postnataly, indicate that it is highly expressed in approximately 80% of adult carcinomas and many childhood cancers including malignant peripheral nerve sheath tumors (MPNST).
  • RESULTS: Tissue-specific MDK promoter activity in human tumor cells and transgene biological activity was confirmed in human MPNST tumor cells.
  • In vitro replication and cytotoxicity in human fibroblasts and MPNST cells by plaque and MTT assays showed that oHSV-MDK-34.5 increased replication and cytotoxicity compared to oHSV-MDK-Luc.
  • By contrast, no significant difference in cytotoxicity was detected between these viruses in normal human fibroblasts. oHSV-MDK-34.5 impaired in vivo tumor growth and increased median survival of MPNST tumor-bearing nude mice.
  • CONCLUSIONS: The transcriptional targeting of HSV lytic infection to MDK-expressing tumor cells is feasible. oHSV-MDK-34.5 shows enhanced anti-tumor effects both in vitro and in vivo.

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  • [Copyright] Copyright (c) 2010 John Wiley & Sons, Ltd.
  • (PMID = 20603890.001).
  • [ISSN] 1521-2254
  • [Journal-full-title] The journal of gene medicine
  • [ISO-abbreviation] J Gene Med
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R21 CA133663; United States / NCI NIH HHS / CA / F31 CA132613-01; United States / NIDDK NIH HHS / DK / R01 DK072446; United States / NCI NIH HHS / CA / R01 CA114004; United States / NCI NIH HHS / CA / F31 CA132613; United States / NIDDK NIH HHS / DK / R01 DK074055
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Validation Studies
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MDK protein, human; 0 / Nerve Growth Factors; 0 / RNA, Small Interfering; EC 1.13.12.- / Luciferases
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76. Muehling BM, Toelkes S, Schelzig H, Barth TF, Sunder-Plassmann L: Tyrosine kinase expression in pulmonary metastases and paired primary tumors. Interact Cardiovasc Thorac Surg; 2010 Feb;10(2):228-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Tissue specimen from 35 lung metastases of 33 patients with renal cell carcinoma (n=8), sarcoma (n=10), colorectal carcinoma (n=6), otolaryngologic carcinoma (OLC, n=4), testicular and endometrial cancer (n=1 each), malignant melanoma (n=1), adrenal cancer (n=2), malignant fibrous histiocytoma and malignant peripheral nerve sheath tumor (n=1 each) have been immunohistochemically tested for the expression of PDGFR alpha/beta, VEGFR and EGFR.
  • Our investigation of a pilot character represents a 'biomarker-based' analysis of pulmonary metastases of different primary tumors; we conclude that an immediate 'tumor profiling' at initial diagnosis should be considered in order to guide tumor therapy individually.
  • [MeSH-major] Biomarkers, Tumor / analysis. Lung Neoplasms / enzymology. Lung Neoplasms / secondary. Protein-Tyrosine Kinases / analysis
  • [MeSH-minor] Adolescent. Adult. Aged. Angiogenesis Inhibitors / therapeutic use. Female. Humans. Immunohistochemistry. Male. Middle Aged. Protein Kinase Inhibitors / therapeutic use. Receptor, Epidermal Growth Factor / analysis. Receptor, Platelet-Derived Growth Factor alpha / analysis. Receptor, Platelet-Derived Growth Factor beta / analysis. Receptors, Vascular Endothelial Growth Factor / analysis. Young Adult

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  • (PMID = 19948538.001).
  • [ISSN] 1569-9285
  • [Journal-full-title] Interactive cardiovascular and thoracic surgery
  • [ISO-abbreviation] Interact Cardiovasc Thorac Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Biomarkers, Tumor; 0 / Protein Kinase Inhibitors; EC 2.7.10.1 / EGFR protein, human; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor beta; EC 2.7.10.1 / Receptors, Vascular Endothelial Growth Factor
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77. Liang YM, Li XH, Lü YL, Zhong M: [Morphology and immunohistochemical characteristics of hepatic primary and metastatic malignant spindle cell tumors]. Zhonghua Yi Xue Za Zhi; 2005 Jan 12;85(2):96-100
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Morphology and immunohistochemical characteristics of hepatic primary and metastatic malignant spindle cell tumors].
  • OBJECTIVE: To investigate the morphology and immunohistochemical characteristics of hepatic primary and metastatic malignant spindle cell tumors, and to conclude the diagnostic and differential diagnostic criteria for these morphologically similar tumors.
  • 20 primary tumors (43.4%), including 3 cases of sarcomatoid carcinoma (6.5%), 11 of angiosarcoma (23.9%), 2 of epithelioid hemangioendothelioma (5%), 1 of spindle cell carcinoid (2.2%), and 3 of undifferentiated sarcoma (6.5%).
  • and 26 metastatic malignant tumors (56.5%), including 20 cases of gastrointestinal stromal tumors (GIST, 43.4%), 3 of leiomyosarcoma (6.5%), 2 of malignant peripheral never sheath tumor (4.3%), and 1 of meningeal hemangiopericytoma (2.2%), resected during operation or collected during imaging-mediated liver puncture underwent hematoxylin-eosin staining, SP staining, and EnVision immunohistochemical staining.
  • Most stromal tumor cases were CD117 positive, and existed the condition that the primary tumor was positive and the metastatic tumor was negative or vice versa or one part of specimen was positive but other part was negative.
  • Leiomyosarcoma was immunoreactive to smooth muscle specific antigen (SMA), malignant peripheral nerve sheath tumor was immunoreactive to S-100 protein and neurofilament (NF), and both were CD117 negative.
  • CONCLUSION: Primary angiosarcoma is the most common form of primary spindle cell tumor in liver, and metastatic GIST is predominant in hepatic metastatic spindle cell tumors.
  • [MeSH-minor] Adolescent. Adult. Aged. Antigens, CD31 / biosynthesis. Antigens, CD34 / biosynthesis. Biomarkers, Tumor. Child. Female. Humans. Immunohistochemistry. Male. Middle Aged

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  • (PMID = 15774214.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Antigens, CD31; 0 / Antigens, CD34; 0 / Biomarkers, Tumor
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78. Levi AD, Ross AL, Cuartas E, Qadir R, Temple HT: The surgical management of symptomatic peripheral nerve sheath tumors. Neurosurgery; 2010 Apr;66(4):833-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The surgical management of symptomatic peripheral nerve sheath tumors.
  • OBJECTIVE: To determine the clinical presentation and morbidity of the surgical management of peripheral nerve sheath tumors (PNSTs).
  • RESULTS: There were a total of 140 cases, including 87 schwannomas, 34 neurofibromas, and 19 malignant peripheral nerve sheath tumors (MPNSTs).
  • Tumor size was the best predictor of adverse outcome, as all MPNST mortalities occurred in patients with a tumor size of more than 7 cm.
  • [MeSH-major] Nerve Sheath Neoplasms / surgery. Neurilemmoma / surgery. Neurofibroma / surgery. Neurosurgery / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Retrospective Studies. Treatment Outcome. Young Adult

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  • (PMID = 20190660.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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79. Moon SJ, Lee JK, Seo BR, Kim JH, Kim SH, Lee KH, Lee MC: An intraosseous malignant peripheral nerve sheath tumor of the cervical spine: a case report and review of the literature. Spine (Phila Pa 1976); 2008 Sep 1;33(19):E712-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] An intraosseous malignant peripheral nerve sheath tumor of the cervical spine: a case report and review of the literature.
  • OBJECTIVES: To report a rare case of intraosseous malignant peripheral nerve sheath tumors (MPNST), and review the pertinent medical literature.
  • SUMMARY OF BACKGROUND DATA: The spinal MPNST that develops from spinal nerve roots and secondary bony erosion is well-known entity.
  • Complete excision of the tumor and posterior stabilization were performed through a posterior approach.
  • The tumor was noted to originate from the posterior element of C7.
  • RESULTS: The histopathology was diagnostic for a MPNST.
  • CONCLUSION: We report an intraosseous MPNST of the cervical spine.
  • MPNST should be added to the differential diagnosis of primary bone tumors causing spinal cord compression.
  • [MeSH-major] Cervical Vertebrae / pathology. Nerve Sheath Neoplasms / pathology. Spinal Neoplasms / pathology
  • [MeSH-minor] Adult. Antigens, CD / metabolism. Antigens, CD34 / metabolism. Biomarkers, Tumor / metabolism. Cell Adhesion Molecules / metabolism. Chemotherapy, Adjuvant. Combined Modality Therapy. Disease-Free Survival. Humans. Ki-67 Antigen / metabolism. Magnetic Resonance Imaging. Male. S100 Proteins / metabolism. Spinal Cord Compression / etiology. Spinal Cord Compression / pathology. Vimentin / metabolism

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  • (PMID = 18758353.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD34; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Ki-67 Antigen; 0 / S100 Proteins; 0 / Vimentin
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80. Dawamneh MF, Amra NK, Amr SS: Low grade fibromyxoid sarcoma: report of a case with fine needle aspiration cytology and histologic correlation. Acta Cytol; 2006 Mar-Apr;50(2):208-12
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  • [Title] Low grade fibromyxoid sarcoma: report of a case with fine needle aspiration cytology and histologic correlation.
  • BACKGROUND: Low grade fibromyxoid sarcoma has been fully described histologically; however, the fine needle aspiration (FNA) cytologic findings are scantily defined, and the distinction from other benign and malignant soft tissue tumors can be difficult.
  • The excised tumor was well circumscribed, focally infiltrating the surrounding muscles.
  • The tumor cells were spindly, with fusiform, uniform nuclei.
  • The tumor cells were positive for vimentin, alpha-1-antitrypsin and lysozyme and negative for S-100, actin, desmin and CD34.
  • CONCLUSION: Although low grade fibromyxoid sarcoma is a rare neoplasm, it should be recognized and distinguished from other soft tissue tumors because of its low malignant potential.
  • The definitive FNA cytologic diagnosis can be challenging but is possible if the tumor is adequately sampled, with multiple passes from different areas.
  • All spindle cell tumors with myxoid changes, such as myxoid liposarcoma, myxofibrosarcoma, cellular myxoma, myxoid leiomyosarcoma and peripheral nerve sheath tumors, should be considered in the differential diagnosis.
  • [MeSH-minor] Adult. Biopsy, Fine-Needle / methods. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Sensitivity and Specificity. Treatment Outcome

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  • (PMID = 16610692.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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81. Hagel C, Zils U, Peiper M, Kluwe L, Gotthard S, Friedrich RE, Zurakowski D, von Deimling A, Mautner VF: Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors. J Neurooncol; 2007 Apr;82(2):187-92
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors.
  • The differences in the clinical course and histopathology of sporadic and neurofibromatosis type 1 (NF1)-associated malignant peripheral nerve sheath tumors (MPNST) were investigated retrospectively.
  • In patients with the original histopathological data available (22 NF1 patients, 14 sporadic cases), NF1-associated MPNST showed a significantly higher cellularity compared to sporadic tumors (p<0.001) whereas sporadic MPNST featured a significantly higher pleomorphism (p<0.01).
  • Most importantly, while histopathological variables correlated with French Fédération Nationale des Centres de Lutte Contre le Cancer grading in sporadic MPNST, this was not the case for NF1-associated tumors.
  • The differences between NF1-associated and sporadic MPNST in regard to the clinical course and histopathology may reflect some fundamental differences in biology and pathomechanism of the two tumor groups.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / pathology. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Female. Humans. Incidence. Male. Middle Aged. Survival Rate

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  • (PMID = 17111191.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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82. James G, Crocker M, King A, Bodi I, Ibrahim A, Chitnavis BP: Malignant triton tumors of the spine. J Neurosurg Spine; 2008 Jun;8(6):567-73
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  • [Title] Malignant triton tumors of the spine.
  • Malignant triton tumors (MTTs) are malignant peripheral nerve sheath tumors with rhabdomyosarcomatous differentiation.
  • Malignant triton tumors affecting the spine are rare but present special challenges to the neurosurgeon.
  • Nine patients presented with symptoms related to the spinal cord, cauda equina, or nerve root compression.
  • Seven patients had intradural extension of tumor.
  • Malignant triton tumors are rare but should be included in the differential diagnosis of spinal tumors, particularly in patients who have undergone previous radiotherapy or who have neurofibromatosis.
  • [MeSH-major] Neurilemmoma / diagnosis. Spinal Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Desmin / analysis. Diagnosis, Differential. Female. Humans. Ki-67 Antigen / analysis. Lumbar Vertebrae / pathology. Male. Neoplasm Recurrence, Local / diagnosis. S100 Proteins / analysis. Sacrum / pathology. Spinal Canal / pathology. Spinal Cord Compression / diagnosis. Thoracic Vertebrae / pathology. Vimentin / analysis

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  • (PMID = 18518679.001).
  • [ISSN] 1547-5654
  • [Journal-full-title] Journal of neurosurgery. Spine
  • [ISO-abbreviation] J Neurosurg Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Desmin; 0 / Ki-67 Antigen; 0 / S100 Proteins; 0 / Vimentin
  • [Number-of-references] 13
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83. Albayrak BS, Gorgulu A, Kose T: A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1. J Neurooncol; 2006 Jun;78(2):187-90
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1.
  • Histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST), a rare sarcoma with a dismal prognosis.
  • Tumor recurred in its previous site with an adjacent apical mass in the left lung 7 weeks following initial surgery and repeat surgery was performed with complete removal of intra-dural tumor.
  • We report the first patient with intra-dural MPNST localized proximal to conus medullaris; in upper thoracic spine.
  • It must always be considered the possibility of a rare but a devastating tumor, MPNST beside schwannomas and neurofibromas in patients with NF1 when an intra-spinal mass is diagnosed.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / pathology. Spinal Cord Neoplasms / pathology
  • [MeSH-minor] Adult. Dura Mater / pathology. Humans. Male. Neoplasm Recurrence, Local / surgery. Thoracic Vertebrae. Treatment Outcome


84. Isefuku S, Seki M, Tajino T, Hakozaki M, Asano S, Hojo H, Hatori M: Ewing's sarcoma in the spinal nerve root: a case report and review of the literature. Tohoku J Exp Med; 2006 Aug;209(4):369-77
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  • [Title] Ewing's sarcoma in the spinal nerve root: a case report and review of the literature.
  • Ewing's sarcoma (ES) is a highly malignant tumor composed of uniform small round cells.
  • Recently, a single biologic entity, Ewing's sarcoma family of tumors (ESFT) has been accepted.
  • The entity includes ES, extraskeletal Ewing's sarcoma (EES) and primitive neuroectodermal tumor (PNET).
  • We present an extremely unusual case with ESFT in a spinal nerve root mimicking a neurogenic dumbbell tumor.
  • Surgery was performed with a presumptive diagnosis of a nerve sheath tumor.
  • At surgery, the tumor was located in the right L5 nerve root sleeve.
  • [MeSH-major] Peripheral Nervous System Neoplasms / physiopathology. Sarcoma, Ewing / physiopathology. Spinal Nerve Roots / physiopathology
  • [MeSH-minor] Adult. Humans. Male. Spine / physiopathology. Spine / radiography

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  • (PMID = 16864960.001).
  • [ISSN] 0040-8727
  • [Journal-full-title] The Tohoku journal of experimental medicine
  • [ISO-abbreviation] Tohoku J. Exp. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
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85. Mautner VF, Brenner W, Fünsterer C, Hagel C, Gawad K, Friedrich RE: Clinical relevance of positron emission tomography and magnetic resonance imaging in the progression of internal plexiform neurofibroma in NF1. Anticancer Res; 2007 Jul-Aug;27(4A):1819-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Neurofibromatosis type 1 (NF1) is a frequent and inherited disease with a predisposition for malignant peripheral nerve sheath tumor (MPNST) development.
  • MPNST are soft tissue sarcomas that arise from peripheral nerves, being one of the most aggressive malignancies in humans with extremely poor prognosis.
  • MPNST frequently arise from a previously undetected plexiform neurofibroma (PNF).
  • The malignant transformation of an internal PNF to an MPNST is difficult to assess and requires advanced imaging techniques like magnetic resonance imaging or positron emission tomography.
  • Despite the high quality of current diagnostics, the changing tumor biology inside a plexiform neurofibroma cannot currently be visualized accurately.
  • We report 4 cases of NF1 patients with PNF who showed imaging findings suspicious for malignant degeneration, but proved to have MPNST in only one case.
  • Three tumors might represent an intermediate type between PNF and MPNST.
  • Ablative surgery and complete histological work-up of specimens is the only way to clarify tumor status, thereby enabling provision of adequate local treatment.
  • [MeSH-major] Nerve Sheath Neoplasms / diagnosis. Neurofibroma, Plexiform / diagnosis. Neurofibromatosis 1 / complications
  • [MeSH-minor] Adult. Child. Diagnosis, Differential. Disease Progression. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Positron-Emission Tomography

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  • (PMID = 17649778.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
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86. Aoki M, Nabeshima K, Koga K, Hamasaki M, Suzumiya J, Tamura K, Iwasaki H: Imatinib mesylate inhibits cell invasion of malignant peripheral nerve sheath tumor induced by platelet-derived growth factor-BB. Lab Invest; 2007 Aug;87(8):767-79
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imatinib mesylate inhibits cell invasion of malignant peripheral nerve sheath tumor induced by platelet-derived growth factor-BB.
  • Malignant peripheral nerve sheath tumor (MPNST) is rare, highly aggressive, resistant to radiochemotherapy, and associated with poor prognosis.
  • This study was designed to identify motogenic factor(s) involved in MPNST cell invasion and inhibitor(s) of such invasive activity.
  • We profiled the invasion-inducing activities of eight motogenic growth factors on two human MPNST cell lines, FU-SFT8611 and 9817, using in vitro Matrigel invasion assays.
  • Platelet-derived growth factor-BB (PDGF-BB) was identified as the most effective MPNST cell invasion-inducing factor.
  • Epidermal growth factor (EGF) and hepatocyte growth factor (HGF) also stimulated invasion in one MPNST cell line.
  • Expressions of PDGF-BB and EGF receptors (PDGFR-beta and EGFR) mRNAs were detected more frequently and their proteins were expressed at higher levels in MPNST tissues than benign peripheral nerve sheath tumors (schwannomas and neurofibromas).
  • In both MPNST cell lines, PDGF-BB induced tyrosine phosphorylation of PDGFR-beta but not of PDGFR-alpha, and specific PDGFR-beta inhibition by small interfering RNA to the receptor inhibited PDGF-BB-stimulated MPNST cell invasion, suggesting the predominant role of PDGFR-beta.
  • No mutations were present in exons 12 and 18 of PDGFR-beta in both MPNST cell lines and 10 human MPNST tissues examined.
  • Our results indicated that PDGF-BB enhanced the invasive activity of MPNST cells through PDGFR phosphorylation and that imatinib inhibited such activity.
  • The results provide the ground for further assessment of the therapeutic potential of imatinib in suppressing the invasion and growth of MPNST.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Nerve Sheath Neoplasms / metabolism. Piperazines / pharmacology. Platelet-Derived Growth Factor / pharmacology. Pyrimidines / pharmacology
  • [MeSH-minor] Adult. Benzamides. Cell Line, Tumor. Female. Humans. Imatinib Mesylate. Intercellular Signaling Peptides and Proteins / pharmacology. Intercellular Signaling Peptides and Proteins / physiology. Male. Middle Aged. Mutation. Neoplasm Invasiveness. Phosphorylation. Proto-Oncogene Proteins c-sis. RNA, Messenger / metabolism. Receptor, Platelet-Derived Growth Factor beta / antagonists & inhibitors. Receptor, Platelet-Derived Growth Factor beta / genetics. Receptor, Platelet-Derived Growth Factor beta / metabolism. Receptors, Growth Factor / genetics. Receptors, Growth Factor / metabolism

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  • (PMID = 17558420.001).
  • [ISSN] 0023-6837
  • [Journal-full-title] Laboratory investigation; a journal of technical methods and pathology
  • [ISO-abbreviation] Lab. Invest.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Intercellular Signaling Peptides and Proteins; 0 / Piperazines; 0 / Platelet-Derived Growth Factor; 0 / Proto-Oncogene Proteins c-sis; 0 / Pyrimidines; 0 / RNA, Messenger; 0 / Receptors, Growth Factor; 0 / platelet-derived growth factor BB; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor beta
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87. Yildirim G, Gillenwater AM, Ordonez NG, Garden AS, El-Naggar AK: Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease. Head Neck; 2008 May;30(5):675-9
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  • [Title] Concurrent epithelioid malignant peripheral nerve sheath tumor and papillary thyroid carcinoma in the treated field of Hodgkin's disease.
  • A 1.5-cm papillary thyroid carcinoma was identified in thyroidectomy and an initial diagnosis of undifferentiated malignant neoplasm was rendered on the neck mass biopsy.
  • Subsequent surgical excision of the neck mass and immunohistochemical analysis revealed malignant peripheral nerve sheath tumor.
  • Rare malignancies including malignant peripheral nerve sheath tumor may be encountered along with the more common papillary thyroid carcinoma.
  • [MeSH-major] Carcinoma, Papillary / pathology. Neoplasms, Second Primary / pathology. Nerve Sheath Neoplasms / pathology. Peripheral Nervous System Neoplasms / pathology. Thyroid Neoplasms / pathology
  • [MeSH-minor] Adult. Epithelioid Cells / pathology. Female. Hodgkin Disease / radiotherapy. Humans. Neoplasms, Radiation-Induced / pathology. Neoplasms, Radiation-Induced / surgery. Thyroidectomy

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  • (PMID = 17972308.001).
  • [ISSN] 1043-3074
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA016672
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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88. Ferrone M, Perrone F, Tamborini E, Paneni MS, Fermeglia M, Suardi S, Pastore E, Delia D, Pierotti MA, Pricl S, Pilotti S: Functional analysis and molecular modeling show a preserved wild-type activity of p53(C238Y). Mol Cancer Ther; 2006 Jun;5(6):1467-73
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  • In a previous TP53 analysis carried out on sporadic and NF1-related malignant peripheral nerve sheath tumors, in two cases, we observed the occurrence of C238Y missense mutation, leading to p53 stabilization unexpectedly coupled with immunophenotypic MDM2 overexpression.
  • [MeSH-major] Models, Molecular. Mutation, Missense. Proto-Oncogene Proteins c-mdm2 / genetics. Tumor Suppressor Protein p53 / physiology
  • [MeSH-minor] Adult. Blotting, Southern. Cyclin-Dependent Kinase Inhibitor p21 / metabolism. Female. Gene Amplification / physiology. Humans. Lymphoma, Non-Hodgkin / metabolism. Lymphoma, Non-Hodgkin / pathology. Lymphoma, Non-Hodgkin / therapy. Male. Middle Aged. Mutagenesis, Site-Directed. Nerve Sheath Neoplasms / metabolism. Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / therapy. Phosphorylation. Protein Conformation

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  • (PMID = 16818505.001).
  • [ISSN] 1535-7163
  • [Journal-full-title] Molecular cancer therapeutics
  • [ISO-abbreviation] Mol. Cancer Ther.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclin-Dependent Kinase Inhibitor p21; 0 / Tumor Suppressor Protein p53; EC 6.3.2.19 / MDM2 protein, human; EC 6.3.2.19 / Proto-Oncogene Proteins c-mdm2
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89. Kim JG, Sung WJ, Kim DH, Kim YH, Sohn SK, Lee KB: Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass. Korean J Intern Med; 2005 Mar;20(1):100-4
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  • [Title] Malignant peripheral nerve sheath tumor in neurofibromatosis type I: unusual presentation of intraabdominal or intrathoracic mass.
  • A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population.
  • On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease).
  • This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / complications. Thoracic Neoplasms / diagnosis
  • [MeSH-minor] Adult. Female. Humans


90. Thway K, Fisher C: Diffuse ganglioneuromatosis in small intestine associated with neurofibromatosis type 1. Ann Diagn Pathol; 2009 Feb;13(1):50-4
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  • We report a case of diffuse ganglioneuromatosis of the small bowel, found incidentally during surgery for a malignant peripheral nerve sheath tumor arising in the retroperitoneum in a 32-year-old man with neurofibromatosis type 1, and review previously reported cases.
  • [MeSH-major] Ganglioneuroma / pathology. Intestinal Neoplasms / pathology. Neoplasms, Multiple Primary / pathology. Nerve Sheath Neoplasms / pathology. Neurofibromatosis 1 / pathology. Retroperitoneal Neoplasms / pathology
  • [MeSH-minor] Adult. Fatal Outcome. Humans. Male

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  • (PMID = 19118783.001).
  • [ISSN] 1532-8198
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 22
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91. Zhang J, Sun Y, Peng ZL: Malignant peripheral nerve sheath tumor of the vagina. Saudi Med J; 2009 May;30(5):705-7
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  • [Title] Malignant peripheral nerve sheath tumor of the vagina.
  • Malignant peripheral nerve sheath tumors (MPNSTs) usually develop in major nerve trunks, giving rise to tumors in the proximal portions of the upper and lower extremities and trunk.
  • We describe a case of MPNST of the vagina and discuss its diagnosis and treatment.
  • [MeSH-major] Peripheral Nerves / pathology. Vaginal Neoplasms / diagnosis
  • [MeSH-minor] Adult. Female. Humans. Treatment Outcome

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  • (PMID = 19417975.001).
  • [ISSN] 0379-5284
  • [Journal-full-title] Saudi medical journal
  • [ISO-abbreviation] Saudi Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Saudi Arabia
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92. Lehnhardt M, Daigeler A, Homann HH, Hauser J, Langer S, Steinsträsser L, Soimaru C, Puls A, Steinau HU: [Importance of specialized centers in diagnosis and treatment of extremity-soft tissue sarcomas. Review of 603 cases]. Chirurg; 2009 Apr;80(4):341-7
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  • The records of 603 patients with soft tissue tumors of the extremities were reviewed concerning mismatches in primary and definite diagnoses relating to entity, evaluation of primary or recurrent tumor specimens, and the diagnosing pathology institution.
  • Liposarcoma and malignant fibrous histiocytoma were the most often diagnosed subgroups at 24% and 22.6%, respectively.
  • In the eight most frequent sarcoma types, malignant peripheral nerve sheath tumors and leiomyosarcoma had the highest rates of false primary diagnosis, 78.4% and 74.2% of cases, respectively.
  • [MeSH-major] Cancer Care Facilities. Extremities / surgery. Hospitals, Special. Hospitals, University. Sarcoma / diagnosis. Sarcoma / surgery. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Chemotherapy, Adjuvant. Child. Combined Modality Therapy. Diagnostic Errors. Female. Germany. Histiocytoma, Benign Fibrous / diagnosis. Histiocytoma, Benign Fibrous / pathology. Histiocytoma, Benign Fibrous / surgery. Humans. Leiomyosarcoma / diagnosis. Leiomyosarcoma / pathology. Leiomyosarcoma / surgery. Liposarcoma / diagnosis. Liposarcoma / pathology. Liposarcoma / surgery. Male. Middle Aged. Neoplasm Staging. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / pathology. Nerve Sheath Neoplasms / surgery. Radiotherapy, Adjuvant. Referral and Consultation. Retrospective Studies. Young Adult

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  • (PMID = 18523742.001).
  • [ISSN] 1433-0385
  • [Journal-full-title] Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
  • [ISO-abbreviation] Chirurg
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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93. Shimada S, Ishizawa T, Ishizawa K, Matsumura T, Hasegawa T, Hirose T: The value of MDM2 and CDK4 amplification levels using real-time polymerase chain reaction for the differential diagnosis of liposarcomas and their histologic mimickers. Hum Pathol; 2006 Sep;37(9):1123-9
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  • Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL) and dedifferentiated liposarcoma (DDL) are reported to have murine double-minute type 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) amplification as a characteristic genetic alteration.
  • To evaluate the diagnostic utility of this gene abnormality, we analyzed 19 liposarcomas, 21 malignant fibrous histiocytomas, 3 leiomyosarcomas, 5 malignant peripheral nerve sheath tumors, 23 lipomas, and 28 nonneoplastic fat tissues using real-time polymerase chain reaction (PCR) and fluorescence in situ hybridization (FISH).
  • In FISH, all ALT/WDLs and DDLs had both MDM2 and CDK4 amplifications, and all of the myxoid/round cell liposarcomas, leiomyosarcomas, malignant peripheral nerve sheath tumors, and all but one of the malignant fibrous histiocytomas did not have the amplifications.
  • [MeSH-major] Biomarkers, Tumor / genetics. Cyclin-Dependent Kinase 4 / genetics. Liposarcoma / diagnosis. Liposarcoma / genetics. Proto-Oncogene Proteins c-mdm2 / genetics
  • [MeSH-minor] Adipose Tissue / pathology. Adipose Tissue / physiology. Adolescent. Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Female. Gene Amplification. Histiocytoma, Malignant Fibrous / diagnosis. Histiocytoma, Malignant Fibrous / genetics. Humans. In Situ Hybridization, Fluorescence. Infant. Leiomyosarcoma / diagnosis. Leiomyosarcoma / genetics. Lipoma / diagnosis. Lipoma / genetics. Male. Middle Aged. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / genetics. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 16938516.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; EC 2.7.11.22 / Cyclin-Dependent Kinase 4; EC 6.3.2.19 / MDM2 protein, human; EC 6.3.2.19 / Proto-Oncogene Proteins c-mdm2
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94. Kobayashi C, Oda Y, Takahira T, Izumi T, Kawaguchi K, Yamamoto H, Tamiya S, Yamada T, Iwamoto Y, Tsuneyoshi M: Aberrant expression of CHFR in malignant peripheral nerve sheath tumors. Mod Pathol; 2006 Apr;19(4):524-32
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  • [Title] Aberrant expression of CHFR in malignant peripheral nerve sheath tumors.
  • In MPNST, complex karyotypes showing numerical and structural aberrations have been described.
  • We examined the expression of CHFR in 96 cases of MPNST by immunohistochemical and molecular methods.
  • We found reduced (score, < or = 3) expression of CHFR in 63 out of 96 (66%) cases of MPNST, and such alteration was significantly correlated with a high mitotic count, a high Ki-67-labeling index, and a poor prognosis.
  • In addition, MPNST with normal karyotype showed a strong (score, =5) expression of CHFR.
  • Our results support the assertion that CHFR functions as an inhibitor of tumor proliferation.
  • [MeSH-major] Cell Cycle Proteins / genetics. Neoplasm Proteins / genetics. Nerve Sheath Neoplasms / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Cell Line, Tumor. Child. Child, Preschool. Female. Gene Expression Regulation, Neoplastic. Humans. Immunohistochemistry. Infant. Ki-67 Antigen / analysis. Male. Middle Aged. Multivariate Analysis. Prognosis. RNA, Messenger / genetics. RNA, Messenger / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Survival Analysis

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  • (PMID = 16554732.001).
  • [ISSN] 0893-3952
  • [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 / CHFR protein, human; 0 / Cell Cycle Proteins; 0 / Ki-67 Antigen; 0 / Neoplasm Proteins; 0 / RNA, Messenger
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95. Batra PS, Lanza DC: Endoscopic power-assisted orbital exenteration. Am J Rhinol; 2005 May-Jun;19(3):297-301
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  • One patient with persistent cavernous sinus malignant peripheral nerve sheath tumor died 4 months after resection despite proton beam therapy.
  • [MeSH-minor] Adult. Carcinoma, Squamous Cell / surgery. Child. Eyelids / surgery. Female. Humans. Male. Middle Aged. Mucormycosis / surgery. Nerve Sheath Neoplasms / surgery. Paranasal Sinus Neoplasms / surgery. Retrospective Studies. Sinusitis / microbiology. Sinusitis / surgery. Time Factors. Tomography, X-Ray Computed. Wound Healing

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  • (PMID = 16011138.001).
  • [ISSN] 1050-6586
  • [Journal-full-title] American journal of rhinology
  • [ISO-abbreviation] Am J Rhinol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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96. Fukushima S, Kageshita T, Wakasugi S, Matsushita S, Kaguchi A, Ishihara T, Ono T: Giant malignant peripheral nerve sheath tumor of the scalp. J Dermatol; 2006 Dec;33(12):865-8
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  • [Title] Giant malignant peripheral nerve sheath tumor of the scalp.
  • Herein, we describe a rare case of giant malignant peripheral nerve sheath tumor of the head in a 38-year-old Japanese man.
  • The tumor measured 210 mm at its largest diameter and was ulcerated, hemorrhagic, multilocular and non-mobile.
  • It should be noted that the patient stubbornly refused to see a doctor for a long time, resulting in the extreme growth of the tumor.
  • Dermatohistopathological findings of the biopsy indicated ancient schwannoma and total excision was therefore performed.
  • Post-mortem skin biopsy revealed features of malignant peripheral nerve sheath tumor.
  • We propose that the expressions of Ki67 and p16 should be checked for all lesions of peripheral nerve sheath tumor for distinguishing benign from malignant forms.
  • [MeSH-major] Neurilemmoma / diagnosis. Scalp / pathology. Skin Neoplasms / diagnosis
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Cyclin-Dependent Kinase Inhibitor p16 / analysis. Fatal Outcome. Hemorrhage / pathology. Humans. Ki-67 Antigen / analysis. Male. Skin Ulcer / pathology

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  • (PMID = 17169091.001).
  • [ISSN] 0385-2407
  • [Journal-full-title] The Journal of dermatology
  • [ISO-abbreviation] J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Ki-67 Antigen
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97. Shah H, Pervez S: Immunophenotypic characterization of high grade pleomorphic sarcomas: a demographic and immunohistochemical study in a major referral center of Pakistan. J Pak Med Assoc; 2005 Mar;55(3):101-4
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  • RESULTS: Of the 134 cases which were characterized, 38.1% were pleomorphic leiomyosarcoma, followed by pleomorphic rhabdomyosarcoma 14.9%, Malignant Peripheral Nerve Sheath Tumour 9%, pleomorphic liposarcoma 3.7% and pleomorphic storiform Malignant Fibrous Histiocytoma 0.7%.
  • Mean/ median age for Leiomyosarcoma was 50/50, for Rhabdomyosarcomas 33/22, for MPNST 42/41, for Liposarcoma 52/50 and for Malignant Fibrous Histiocytoma 46/46 respectively.
  • CONCLUSION: It was concluded that the most common pleomorphic sarcoma occurring in our adult population was Leiomyosarcoma, and that immunohistochemical stains are essential in most cases for further characterization of pleomorphic high grade sarcoma.
  • [MeSH-major] Sarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Age Distribution. Aged. Antigens, Neoplasm / immunology. Biomarkers, Tumor / analysis. Cross-Sectional Studies. Humans. Immunoenzyme Techniques. Immunohistochemistry. Leiomyosarcoma / pathology. Liposarcoma / genetics. Liposarcoma / pathology. Middle Aged. Nerve Sheath Neoplasms / genetics. Nerve Sheath Neoplasms / pathology. Pakistan. Phenotype. Rhabdomyosarcoma / genetics. Rhabdomyosarcoma / pathology. Surveys and Questionnaires

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  • (PMID = 15852744.001).
  • [ISSN] 0030-9982
  • [Journal-full-title] JPMA. The Journal of the Pakistan Medical Association
  • [ISO-abbreviation] J Pak Med Assoc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Pakistan
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor
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98. Saint-Blancard P, Harket A, Bonnichon A, Jancovici R: [Neurogenic spindle-cell tumors of the mediastinum: two cases]. Presse Med; 2008 Feb;37(2 Pt 1):229-34
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  • [Title] [Neurogenic spindle-cell tumors of the mediastinum: two cases].
  • INTRODUCTION: Neurogenic tumors can develop from neural cells in any location.
  • Neurogenic tumors can be benign or malignant.
  • CASES: We report one case of a malignant peripheral nerve sheath tumor in the posterior mediastinum of a 29-year-old man and another of a schwannoma of the anterior mediastinum, in an 82-year-old woman.
  • DISCUSSION: Neurogenic tumors of spindle-shaped cells in the mediastinum are generally benign, but can be malignant.
  • [MeSH-minor] Adult. Aged, 80 and over. Female. Humans. Male

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  • (PMID = 17988829.001).
  • [ISSN] 2213-0276
  • [Journal-full-title] Presse medicale (Paris, France : 1983)
  • [ISO-abbreviation] Presse Med
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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99. Matsuda E, Okabe K, Matsuoka T, Hirazawa K, Azuma T, Murakami T, Sugi K: [Lung metastasis of malignant peripheral nerve sheath tumor: report of a case]. Kyobu Geka; 2007 Sep;60(10):950-3
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  • [Title] [Lung metastasis of malignant peripheral nerve sheath tumor: report of a case].
  • He had underwent extended radical tumorectomy for malignant peripheral nerve sheath tumor (MPNST) in left lower limb 33 months before.
  • Chest X-ray and computed tomography (CT) scan revealed solitary tumor on right S10.
  • Tumor was resected under thoracoscopic surgery.
  • Histological diagnosis was metastasis of MPNST.
  • MPNST with lung metastasis showing very poor prognosis.
  • Careful follow up is important for MPNST.
  • [MeSH-major] Lung Neoplasms / secondary. Nerve Sheath Neoplasms / secondary. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Adult. Humans. Male. Neurofibromatosis 1 / complications. Prognosis. Survivors. Thoracoscopy. Tomography, X-Ray Computed

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  • (PMID = 17877020.001).
  • [ISSN] 0021-5252
  • [Journal-full-title] Kyobu geka. The Japanese journal of thoracic surgery
  • [ISO-abbreviation] Kyobu Geka
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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100. Dozois EJ, Wall JC, Spinner RJ, Jacofsky DJ, Yaszemski MJ, Sim FH, Moran SL, Cima RR, Larson DR, Haddock MG, Okuno SH, Larson DW: Neurogenic tumors of the pelvis: clinicopathologic features and surgical outcomes using a multidisciplinary team. Ann Surg Oncol; 2009 Apr;16(4):1010-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Neurogenic tumors of the pelvis: clinicopathologic features and surgical outcomes using a multidisciplinary team.
  • BACKGROUND: Few data exist regarding the outcomes in patients undergoing surgery for pelvic tumors of neurogenic origin.
  • Our aim was to characterize the clinical and pathologic features of pelvic neurogenic tumors and assess surgical outcomes.
  • METHODS: All patients who underwent operations for pelvic neurogenic tumors at our institution between 1956 and 2004 were identified.
  • Malignant lesions were found in 43 patients (48%).
  • Schwannomas were the most common benign tumor (61%) and malignant peripheral nerve sheath tumors the most common malignant lesion (81%).
  • Median tumor size was 9.5 cm (range 0.8-32 cm).
  • Malignant tumors had histopathologic evidence of infiltration of surrounding structures in 49% of cases.
  • Intralesional resection was the most common surgical technique for both benign and malignant tumors.
  • Adjuvant therapy was given to 91% of the patients with malignant disease.
  • Five-year local recurrence rates for benign and malignant lesions were 35.9% and 35.0%, respectively.
  • Distant recurrence for malignant lesions was 65.1% at 5 years.
  • Five-year disease-free survival for malignant tumors was 25.9%.
  • CONCLUSION: Pelvic neurogenic tumors occurring in young patients may be large when detected and present with nonspecific symptoms.
  • Benign and malignant tumors had a high local recurrence rate and survival for malignant tumors was poor.
  • [MeSH-major] Neoplasms, Nerve Tissue / pathology. Neoplasms, Nerve Tissue / surgery. Pelvic Neoplasms / pathology. Pelvic Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Humans. Male. Patient Care Team. Survival Analysis. Treatment Outcome

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  • (PMID = 19194756.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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