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[PMID]:29044531
[Au] Autor:Tsuda Y; Ogura K; Hakozaki M; Kikuta K; Ae K; Tsuchiya H; Iwata S; Ueda T; Kawano H; Kawai A
[Ad] Endereço:Department of Orthopedic Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
[Ti] Título:Mesenchymal chondrosarcoma: A Japanese Musculoskeletal Oncology Group (JMOG) study on 57 patients.
[So] Source:J Surg Oncol;115(6):760-767, 2017 May.
[Is] ISSN:1096-9098
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: This study aimed to elucidate the clinical features and prognostic factors of mesenchymal chondrosarcoma (MCS) and investigate optimal treatment strategies. METHODS: Data from 57 patients with MCS were collected from a Japanese Musculoskeletal Oncology Group (JMOG) and retrospectively analyzed. RESULTS: Data from 29 males and 28 females were collected. Primary tumor sites were the head and neck (7 patients), trunk (35 patients), and extremities (15 patients). The tumors originating in the trunk were significantly associated with a worse OS compared with those originating at the other sites in all patients and those with localized disease (P = 0.020 and P = 0.019, respectively). In patients with localized disease, the tumors originating in the head and neck were significantly associated with better OS and MFS compared with those originating in the trunk (P = 0.024 and P = 0.014, respectively). Positive surgical margin was significantly correlated with the worse LRFS (P = 0.018). Adjuvant chemotherapy exhibited a clear trend toward improved OS when MCS was localized in the trunk or extremities (P = 0.057). CONCLUSIONS: Adequate surgery is considered to be the mainstay of treatment for localized MCS. Prognosis was different depending on the site of tumor origin.
[Mh] Termos MeSH primário: Condrossarcoma Mesenquimal/patologia
Condrossarcoma Mesenquimal/terapia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
Criança
Condrossarcoma Mesenquimal/tratamento farmacológico
Condrossarcoma Mesenquimal/radioterapia
Feminino
Seres Humanos
Japão
Masculino
Meia-Idade
Prognóstico
Estudos Retrospectivos
Inquéritos e Questionários
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171026
[Lr] Data última revisão:
171026
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171019
[St] Status:MEDLINE
[do] DOI:10.1002/jso.24567


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[PMID]:28352960
[Au] Autor:Kumar R; Duran C; Amini B; Araujo DM; Wang WL
[Ad] Endereço:Department of Diagnostic Imaging, The University of Texas M.D. Anderson Cancer Center, Unit 1475, 1515 Holcombe Blvd, Unit 1475, Houston, Texas, 77030, USA. rajkumar@mdanderson.org.
[Ti] Título:Periosteal mesenchymal chondrosarcoma of the tibia with multifocal bone metastases: a case report.
[So] Source:Skeletal Radiol;46(7):995-1000, 2017 Jul.
[Is] ISSN:1432-2161
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:Mesenchymal chondrosarcoma of bone is a rare high-grade variant of chondrosarcoma, which typically has central intramedullary location. The tumor is characterized by admixture of highly anaplastic small round malignant cells and islands of mineralized low-grade hyaline cartilage. It is most unusual for this tumor to arise on the surface of a long bone. We describe a patient with periosteal mesenchymal chondrosarcoma that arose at the surface of the right tibia with multifocal bone metastases. Radiographic, CT, MRI, and PET-CT features of this unusual tumor are presented.
[Mh] Termos MeSH primário: Neoplasias Ósseas/diagnóstico por imagem
Neoplasias Ósseas/patologia
Condrossarcoma Mesenquimal/diagnóstico por imagem
Condrossarcoma Mesenquimal/patologia
[Mh] Termos MeSH secundário: Adulto
Meios de Contraste
Evolução Fatal
Seres Humanos
Masculino
Neoplasias da Coluna Vertebral/diagnóstico por imagem
Neoplasias da Coluna Vertebral/secundário
Tíbia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Contrast Media)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171106
[Lr] Data última revisão:
171106
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170330
[St] Status:MEDLINE
[do] DOI:10.1007/s00256-017-2626-5


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[PMID]:28258179
[Au] Autor:Jakhetiya A; Shukla NK; Muduly D; Kale SS
[Ad] Endereço:Department of Surgical Oncology, All India Institute of Medical Sciences, NewDelhi, India.
[Ti] Título:Extraskeletal orbital mesenchymal chondrosarcoma: surgical approach and mini review.
[So] Source:BMJ Case Rep;2017, 2017 Mar 03.
[Is] ISSN:1757-790X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Extraskeletal orbital mesenchymal chondrosarcoma (MC) is an extremely rare and highly aggressive tumour. It has characteristic radiological features and pathognomic histological biphasic pattern. Radical resection with negative margins is the mainstay of treatment; role of adjuvant chemotherapy and radiotherapy is yet not well defined. We report a rare case of 18-year-old man who was diagnosed to have orbital MC. He presented with locally advanced disease with no vision in the affected eye. He underwent right orbital exenteration; a transcranial intradural approach was used to divide the optic nerve, and the temporalis muscle flap was utilised to fill the exenterated orbit. Though optic nerve involvement is rare in orbital MCs, a transcranial approach may be used effectively to avoid traction on optic chiasma and ensure margin-free resection in case of optic nerve involvement up to orbital apex. Unfortunately, prognosis remains dismal in MCs despite treatment.
[Mh] Termos MeSH primário: Condrossarcoma Mesenquimal/cirurgia
Neoplasias Orbitárias/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Condrossarcoma Mesenquimal/diagnóstico
Condrossarcoma Mesenquimal/radioterapia
Craniotomia/métodos
Seres Humanos
Masculino
Neoplasias Orbitárias/diagnóstico
Neoplasias Orbitárias/radioterapia
Radioterapia Adjuvante/métodos
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170313
[Lr] Data última revisão:
170313
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170305
[St] Status:MEDLINE


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[PMID]:28042016
[Au] Autor:Laghari AA; Javed G; Khan MF; Ahmed SI; Nathani KR; Ahmed R
[Ad] Endereço:Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan. Electronic address: altaf.alilaghari@aku.edu.
[Ti] Título:Spontaneous Intraventricular Hemorrhage: A Rare Presentation of a Skull Base Mesenchymal Chondrosarcoma.
[So] Source:World Neurosurg;99:811.e1-811.e5, 2017 Mar.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Chondrosarcomas are very rare malignant, slow-growing tumors that develop in or near the petroclival region of the brain. We report a very rare case in which the tumor originated from left petrous bone and induced intraventricular hemorrhage leading to an acute comatose presentation. CASE DESCRIPTION: A 28-year-old man initially presented to the outpatient department with a 1-month history of headache, vomiting, vertigo, and left facial numbness. A lesion at the cerebellopontine angle with extension into the middle cranial fossa was demonstrated on computed tomography and magnetic resonance imaging. The following night his condition worsened, and he presented to the emergency department with intraventricular hemorrhage with hydrocephalus. An external ventricular drain was placed in the emergency department to relieve hydrocephalus, and definitive surgical resection of the tumor was subsequently. Postoperatively, his Glasgow Coma Scale score improved, and he was transferred to the surgical intensive care unit where he remained for 3 days. He was subsequently stepped down to a special care unit and then to a ward room. The patient is currently awake and has grade II facial palsy (House-Brackmann), demonstrates spontaneous purposeful eye opening, inconsistently obeys single-step orders, demonstrates no meaningful phonation or vocalization, and has at least grade 4 power in all 4 extremities. He is currently fed through a nasogastric tube and is in rehabilitation. CONCLUSIONS: Our experience of petroclival junction chondrosarcoma causing intraventricular hemorrhage may be the first to be documented. Preferred treatment of this highly malignant lesion is radical removal with postoperative radiotherapy.
[Mh] Termos MeSH primário: Hemorragia Cerebral/diagnóstico por imagem
Ventrículos Cerebrais/diagnóstico por imagem
Condrossarcoma Mesenquimal/diagnóstico por imagem
Hidrocefalia/diagnóstico por imagem
Neoplasias da Base do Crânio/diagnóstico por imagem
[Mh] Termos MeSH secundário: Adulto
Ângulo Cerebelopontino/diagnóstico por imagem
Hemorragia Cerebral/etiologia
Condrossarcoma Mesenquimal/complicações
Condrossarcoma Mesenquimal/cirurgia
Fossa Craniana Média/diagnóstico por imagem
Seres Humanos
Hidrocefalia/etiologia
Imagem por Ressonância Magnética
Masculino
Osso Petroso/diagnóstico por imagem
Neoplasias da Base do Crânio/complicações
Neoplasias da Base do Crânio/cirurgia
Tomografia Computadorizada por Raios X
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170918
[Lr] Data última revisão:
170918
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170103
[St] Status:MEDLINE


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[PMID]:27048221
[Au] Autor:Miller BJ
[Ad] Endereço:University of Iowa, 200 Hawkins Dr., 01025 JPP, Iowa City, IA, 52242, USA. benjamin-j-miller@uiowa.edu.
[Ti] Título:CORR Insights : Survival in Mesenchymal Chondrosarcoma Varies Based on Age and Tumor Location: A Survival Analysis of the SEER Database.
[So] Source:Clin Orthop Relat Res;475(3):806-807, 2017 03.
[Is] ISSN:1528-1132
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Condrossarcoma Mesenquimal
Análise de Sobrevida
[Mh] Termos MeSH secundário: Neoplasias Ósseas
Condrossarcoma
Seres Humanos
Programa de SEER
[Pt] Tipo de publicação:JOURNAL ARTICLE; COMMENT
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:160407
[St] Status:MEDLINE
[do] DOI:10.1007/s11999-016-4818-z


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[PMID]:26975384
[Au] Autor:Schneiderman BA; Kliethermes SA; Nystrom LM
[Ad] Endereço:Harbor-UCLA Medical Center, Los Angeles, CA, USA.
[Ti] Título:Survival in Mesenchymal Chondrosarcoma Varies Based on Age and Tumor Location: A Survival Analysis of the SEER Database.
[So] Source:Clin Orthop Relat Res;475(3):799-805, 2017 Mar.
[Is] ISSN:1528-1132
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Studies suggest that mesenchymal chondrosarcoma is associated with a poorer prognosis and a higher proportion of extraskeletal tumors than conventional chondrosarcoma. However, these investigations have been small heterogeneous cohorts, limiting analysis of prognostic factors. QUESTIONS/PURPOSES: (1) What is the 5- and 10-year survival rate of patients diagnosed with mesenchymal chondrosarcoma? (2) What is the effect of demographic and tumor characteristics on survival in patients with mesenchymal chondrosarcoma? METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify all patients diagnosed with mesenchymal chondrosarcoma from 1973 to 2011. SEER reports survival data on over 8.2 million patients with cancer and has attained 98% completeness in reporting. Using variables within the database, this study designated each patient's tumor as skeletal or extraskeletal and cranial, axial, or appendicular, respectively. Overall survival (OS) was determined for the entire series as well as each group. Median survival was calculated using Kaplan-Meier methods. Cox proportional hazards regression was used to determine whether demographic and tumor variables affected survival. Two hundred five patients with mesenchymal chondrosarcoma were identified, including 82 (40%) skeletal and 123 (60%) extraskeletal. RESULTS: OS for the entire series was 51% (95% confidence interval [CI], 43%-58%) and 43% (95% CI, 35%-51%) at 5 and 10 years, respectively. No difference in OS was detected between extraskeletal and skeletal tumors. Kaplan-Meier analyses showed OS was worse for tumors in axial locations compared with appendicular and cranial locations. Appendicular tumors demonstrated an OS of 50% (95% CI, 36%-63%) at 5 years and 39% (95% CI, 26%-52%) at 10 years. OS for axial tumors was 37% (95% CI, 25%-49%) and 31% (95% CI, 20%-43%), whereas it was 74% (95% CI, 59%-84%) and 67% (95% CI, 50%-79%) for cranial tumors at 5 and 10 years, respectively. When controlling for age, sex, tumor origin, and tumor location, the presence of metastasis (hazard ratio [HR], 12.38; 95% CI, 5.75-26.65; p < 0.001) and 1-cm size increase (HR, 1.16; 95% CI, 1.09-1.23; p < 0.001) were both independently associated with an increased risk of death. Tumor location showed different behaviors depending on patient age. In comparison to cranial tumors at age 20 years, the HR was 5.56 (95% CI, 1.47-21.05; p = 0.01) for axial tumors and 6.26 (95% CI, 1.54-25.42; p = 0.01) for appendicular tumors. At age 60 years, those ratios were 0.10 (95% CI, 0.02-0.55; p = 0.01) and 0.14 (95% CI, 0.04-0.58; p = 0.01), respectively. CONCLUSIONS: Our data suggest that extraskeletal tumors are more common than previously reported; however, this factor does not have clear prognostic value. Presence of metastatic disease and increased tumor size are the main predictors of poor survival outcome. Cranial tumors appear to have a different clinical behavior with our data suggesting better overall survival in young patients (compared with axial and appendicular locations) and a worse survival outcome in older patients. LEVEL OF EVIDENCE: Level IV, prognostic study.
[Mh] Termos MeSH primário: Neoplasias Ósseas/epidemiologia
Condrossarcoma Mesenquimal/epidemiologia
[Mh] Termos MeSH secundário: Adulto
Fatores Etários
Neoplasias Ósseas/mortalidade
Neoplasias Ósseas/patologia
Condrossarcoma Mesenquimal/mortalidade
Condrossarcoma Mesenquimal/secundário
Bases de Dados Factuais
Feminino
Seres Humanos
Estimativa de Kaplan-Meier
Masculino
Meia-Idade
Análise Multivariada
Prognóstico
Modelos de Riscos Proporcionais
Estudos Retrospectivos
Medição de Risco
Fatores de Risco
Programa de SEER
Fatores de Tempo
Carga Tumoral
Estados Unidos/epidemiologia
Adulto Jovem
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:160316
[St] Status:MEDLINE
[do] DOI:10.1007/s11999-016-4779-2


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[PMID]:28111733
[Au] Autor:Pirbudak L; Sevinç A; Kervancioglu S; Kervancioglu P; Ates D
[Ad] Endereço:Department of Anesthesiology and Algology, Gaziantep University Faculty of Medicine, Gaziantep, Turkey. lutfiyep@hotmail.com.
[Ti] Título:Pulsed radiofrequency treatment of piriformis syndrome in a pregnant patient with malignant mesenchymal tumor.
[So] Source:Agri;28(4):194-198, 2016 Oct.
[Is] ISSN:1300-0012
[Cp] País de publicação:Turkey
[La] Idioma:eng
[Ab] Resumo:Cancer is frequently seen in women of reproductive age. Diagnosis, management of treatment, and safety of the therapeutic approach are particularly important for these patients. Presently described is pain management in a case of pregnancy with malignant mesenchymal tumor. A 23-year-old woman in 30th gestational week presented with severe pain in right hip and back of the right thigh. Piriformis block successfully decreased pain and was followed by pulsed radiofrequency (PRF) to the piriformis muscle. PRF, as a non-neurodestructive method, is a safe and effective method to treat cancer pain in a pregnant patient.
[Mh] Termos MeSH primário: Condrossarcoma Mesenquimal/complicações
Dor Intratável/terapia
Síndrome do Músculo Piriforme/terapia
Complicações Neoplásicas na Gravidez
[Mh] Termos MeSH secundário: Ablação por Cateter
Feminino
Seres Humanos
Recém-Nascido
Dor Intratável/etiologia
Síndrome do Músculo Piriforme/etiologia
Gravidez
Terceiro Trimestre da Gravidez
Coxa da Perna/inervação
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170619
[Lr] Data última revisão:
170619
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170124
[St] Status:MEDLINE
[do] DOI:10.5505/agri.2015.32657


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[PMID]:27790706
[Au] Autor:De Amorim Bernstein K; Liebsch N; Chen YL; Niemierko A; Schwab JH; Raskin K; Lozano-Calderon SA; Cote G; Harmon DC; Choy E; Haynes A; Mullen J; Hornicek FJ; DeLaney TF
[Ad] Endereço:Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
[Ti] Título:Clinical outcomes for patients after surgery and radiation therapy for mesenchymal chondrosarcomas.
[So] Source:J Surg Oncol;114(8):982-986, 2016 Dec.
[Is] ISSN:1096-9098
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: We report the outcome of 23 patients with mesenchymal chondrosarcomas treated with surgery and radiation therapy +/- chemotherapy. The intent of the project was to review the impact of patient and treatment variables on treatment outcome, in particular with regard to extent of surgery and radiation dose. PATIENTS AND METHODS: Twenty-three patients with mesenchymal chondrosarcomas were treated with surgery and radiation therapy (min. dose 44 Gy; max. dose 78 Gy; median dose 60 Gy; mean dose 61 Gy). RESULTS: The median survival for the entire cohort of patients was 21.65 years (95% confidence interval ± 4.25). The 5- and 10-year OS was 78.6%. Median disease-free survival for the 23 patients was 7.2 years. Disease-free survival (DFS) at 3 and 5 years was 70.7% and 57.8%, respectively. The local control rate at 5 and 10 years was 89.5% (95%CI 64.1-97.3%). Only three patients experienced local failure, three patients had regional failure, and eight developed distant metastases. CONCLUSIONS: In this cohort of patients local tumor control was high when using a combination of surgery and radiation. There was not a clear relationship between radiation dose and local tumor control. J. Surg. Oncol. 2016;114:982-986. © 2016 Wiley Periodicals, Inc.
[Mh] Termos MeSH primário: Neoplasias Ósseas/radioterapia
Neoplasias Ósseas/cirurgia
Condrossarcoma Mesenquimal/radioterapia
Condrossarcoma Mesenquimal/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Protocolos de Quimioterapia Combinada Antineoplásica
Neoplasias Ósseas/mortalidade
Quimioterapia Adjuvante
Criança
Pré-Escolar
Condrossarcoma Mesenquimal/tratamento farmacológico
Condrossarcoma Mesenquimal/mortalidade
Feminino
Seres Humanos
Masculino
Meia-Idade
Dosagem Radioterapêutica
Radioterapia Adjuvante
Estudos Retrospectivos
Análise de Sobrevida
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170213
[Lr] Data última revisão:
170213
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161030
[St] Status:MEDLINE
[do] DOI:10.1002/jso.24435


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[PMID]:27565470
[Au] Autor:Sadashiva N; Sharma A; Shukla D; Rajalakshmi P; Mahadevan A; Devi BI
[Ad] Endereço:Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India.
[Ti] Título:Intracranial Extraskeletal Mesenchymal Chondrosarcoma.
[So] Source:World Neurosurg;95:618.e1-618.e6, 2016 Nov.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Intracranial extraskeletal mesenchymal chondrosarcomas (MCSs) are rare tumors accounting for <0.16% of intracranial tumors. They are usually described as occurring in the age group of 20-30 years and are commonly found in the frontoparietal region arising from the falx and surrounding dura. We describe 3 cases at varying ages, locations, and outcomes. CASE DESCRIPTION: A 42-year-old woman with anterior one third falx-based lesion; a 7-year-old boy with mid one third falcine lesion with hyperostosis of bone, presenting in comatose stage; and a 52-year-old woman with left posterior lateral frontal dural-based lesion are presented. Histopathology of all cases was confirmed with immunohistochemistry. All patients underwent complete excision but had varying outcomes. CONCLUSIONS: Intracranial MCSs are rare malignant tumors with poor prognosis. Because of their rarity, it may not be considered as a differential diagnosis and it is often misdiagnosed radiologically, but the importance lies in the need of radical excision. The potential effect of postoperative radiotherapy and chemotherapy is controversial. This article sheds some light on variable prognosis of this tumor.
[Mh] Termos MeSH primário: Neoplasias Encefálicas/diagnóstico por imagem
Condrossarcoma Mesenquimal/diagnóstico por imagem
Dura-Máter/diagnóstico por imagem
Neoplasias Meníngeas/diagnóstico por imagem
[Mh] Termos MeSH secundário: Antígeno 12E7/metabolismo
Adulto
Neoplasias Encefálicas/metabolismo
Neoplasias Encefálicas/patologia
Neoplasias Encefálicas/cirurgia
Criança
Condrossarcoma Mesenquimal/metabolismo
Condrossarcoma Mesenquimal/patologia
Condrossarcoma Mesenquimal/cirurgia
Dura-Máter/metabolismo
Dura-Máter/patologia
Dura-Máter/cirurgia
Feminino
Seres Humanos
Imuno-Histoquímica
Imagem por Ressonância Magnética
Masculino
Neoplasias Meníngeas/metabolismo
Neoplasias Meníngeas/patologia
Neoplasias Meníngeas/cirurgia
Meia-Idade
Mucina-1/metabolismo
Proteínas S100/metabolismo
Sinaptofisina/metabolismo
Tomografia Computadorizada por Raios X
Vimentina/metabolismo
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (12E7 Antigen); 0 (Mucin-1); 0 (S100 Proteins); 0 (Synaptophysin); 0 (Vimentin)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160828
[St] Status:MEDLINE


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[PMID]:27544802
[Au] Autor:Cohen JN; Solomon DA; Horvai AE; Kakar S
[Ad] Endereço:Department of Pathology, University of California, San Francisco, San Francisco, CA 94143.
[Ti] Título:Pancreatic involvement by mesenchymal chondrosarcoma harboring the HEY1-NCOA2 gene fusion.
[So] Source:Hum Pathol;58:35-40, 2016 Dec.
[Is] ISSN:1532-8392
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Mesenchymal chondrosarcoma (MC) is an aggressive small, round, blue cell tumor with chondrogenic differentiation that typically arises in bony sites. Approximately, a third of these tumors develop in extraskeletal sites such as the meninges, and somatic soft tissue. The MCs are well-circumscribed, lobulated masses, with focal calcification. Histologically, 2 distinct populations of neoplastic cells characterize MC: sheets of primitive small, round, blue cells surrounding islands of well-developed hyaline cartilage with mature chondrocytes in lacunae. Involvement of the gastrointestinal tract and pancreas by primary or metastatic MC is a relatively rare occurrence. We identified 8 patients with MC in our departmental archives from 1990 to 2015, two of which had pancreatic involvement. The patients were young women who developed masses in the distal pancreas. Molecular testing demonstrated that both tumors harbored the recently described HEY1-NCOA2 gene fusion. These cases illustrate that pancreatic involvement can occur in MC, and the demonstration of HEY1-NCOA2 fusion can be helpful to confirm the diagnosis.
[Mh] Termos MeSH primário: Fatores de Transcrição Hélice-Alça-Hélice Básicos/genética
Biomarcadores Tumorais/genética
Neoplasias Ósseas/genética
Proteínas de Ciclo Celular/genética
Condrossarcoma Mesenquimal/genética
Fusão Gênica
Coativador 2 de Receptor Nuclear/genética
Neoplasias Pancreáticas/genética
[Mh] Termos MeSH secundário: Adulto
Neoplasias Ósseas/patologia
Neoplasias Ósseas/terapia
Condrossarcoma Mesenquimal/patologia
Condrossarcoma Mesenquimal/terapia
Feminino
Predisposição Genética para Doença
Seres Humanos
Técnicas de Diagnóstico Molecular
Neoplasias Pancreáticas/patologia
Neoplasias Pancreáticas/terapia
Fenótipo
Valor Preditivo dos Testes
Reação em Cadeia da Polimerase Via Transcriptase Reversa
Tomografia Computadorizada por Raios X
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Basic Helix-Loop-Helix Transcription Factors); 0 (Biomarkers, Tumor); 0 (Cell Cycle Proteins); 0 (HEY1 protein, human); 0 (NCOA2 protein, human); 0 (Nuclear Receptor Coactivator 2)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170828
[Lr] Data última revisão:
170828
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160822
[St] Status:MEDLINE



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