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[PMID]:29390485
[Au] Autor:Li Q; Liu Y; Yu Y
[Ad] Endereço:Department of Oncology, Xuzhou Central Hospital Affiliated to Dongnan University, Xuzhou, Jiangsu, China.
[Ti] Título:Antiangiogenic therapy for primitive neuroectodermal tumor with thalidomide: A case report and review of literature.
[So] Source:Medicine (Baltimore);96(51):e9272, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Peripheral primitive neuroectodermal tumor (PNET) is a kind of small round cell tumor derived from primitive neuroectodermal tumor. PATIENT CONCERNS: PNET is a highly malignant tumor that is subordinated to Ewing sarcoma. It occurs predominantly in soft tissue and bone and rarely in the bronchi and lung. Traditional surgery, radiotherapy, and chemotherapy are used for the treatment of PNET, but are usually ineffective. DIAGNOSES: There was a rare case of a 17 year-old man diagnoses with primary pulmonary PNET. INTERVENTIONS: The patient was treated by the remedy treatment with thalidomide after the poor effect of conventional radiotherapy and chemotherapy. OUTCOMES: The patient survived without disease progression for 15 months and was in stable condition. LESSONS: Thalidomide provides a choice for maintenance therapy in PNET.
[Mh] Termos MeSH primário: Inibidores da Angiogênese/uso terapêutico
Neoplasias Pulmonares/tratamento farmacológico
Neoplasias Pulmonares/patologia
Tumores Neuroectodérmicos Primitivos Periféricos/tratamento farmacológico
Tumores Neuroectodérmicos Primitivos Periféricos/patologia
Talidomida/uso terapêutico
[Mh] Termos MeSH secundário: Adolescente
Biópsia por Agulha
Broncoscopia/métodos
Seguimentos
Seres Humanos
Imuno-Histoquímica
Neoplasias Pulmonares/diagnóstico por imagem
Masculino
Invasividade Neoplásica/patologia
Estadiamento de Neoplasias
Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico por imagem
Medição de Risco
Tomografia Computadorizada por Raios X/métodos
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Angiogenesis Inhibitors); 4Z8R6ORS6L (Thalidomide)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180214
[Lr] Data última revisão:
180214
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180203
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009272


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[PMID]:28296680
[Au] Autor:Chiang S; Snuderl M; Kojiro-Sanada S; Quer Pi-Sunyer A; Daya D; Hayashi T; Bosincu L; Ogawa F; Rosenberg AE; Horn LC; Wang L; Iafrate AJ; Oliva E
[Ad] Endereço:*Department of Pathology, Memorial Sloan Kettering Cancer Center †Department of Pathology, New York University Langone Medical Center, New York, NY ‡Department of Pathology, Kurume University School of Medicine, Fukuoka ¶Department of Pathology, Junwakai Memorial Hospital, Miyazaki **Department of Diagnostic Pathology, Sainokuni Higashiomiya Medical Center, Saitama, Japan §Department of Anatomic Pathology, Germans Trias I Pujol Hospital, Badalona, Spain ∥Department of Pathology, McMaster University, Juravinski Hospital, Hamilton, ON, Canada #Department of Pathology, University of Sassari, Sassari, Italy ††Department of Pathology, Miller School of Medicine, University of Miami, Miami, FL ‡‡Division of Gynecologic, Breast and Perinatal Pathology, University Hospital Leipzig, Leipzig, Germany §§Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA.
[Ti] Título:Primitive Neuroectodermal Tumors of the Female Genital Tract: A Morphologic, Immunohistochemical, and Molecular Study of 19 Cases.
[So] Source:Am J Surg Pathol;41(6):761-772, 2017 Jun.
[Is] ISSN:1532-0979
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Primary primitive neuroectodermal tumor (PNET) of the female genital tract is rare, and its proper classification remains unclear. The clinical, histologic, and immunophenotypic features as well as EWSR1 rearrangement status of 19 gynecologic PNETs, including 10 ovarian, 8 uterine, and 1 vulvar tumors, are herein reported. Patient age ranged from 12 to 68 years, with a median age of 20 and 51 years among those with ovarian and uterine PNETs, respectively. Morphologic features of central nervous system (CNS) tumors were seen in 15 PNETs, including 9 medulloblastomas, 3 ependymomas, 2 medulloepitheliomas, and 1 glioblastoma, consistent with central PNET. The remaining 4 PNETs were composed entirely of undifferentiated small round blue cells and were classified as Ewing sarcoma/peripheral PNET. Eight PNETs were associated with another tumor type, including 5 ovarian mature cystic teratomas, 2 endometrial low-grade endometrioid carcinomas, and a uterine carcinosarcoma. By immunohistochemistry, 17 PNETs expressed at least 1 marker of neuronal differentiation, including synaptophysin, NSE, CD56, S100, and chromogranin in 10, 8, 14, 8, and 1 tumors, respectively. GFAP was positive in 4 PNETs, all of which were of central type. Membranous CD99 and nuclear Fli-1 staining was seen in 10 and 16 tumors, respectively, and concurrent expression of both markers was seen in both central and Ewing sarcoma/peripheral PNETs. All tumors expressed vimentin, whereas keratin cocktail (CAM5.2, AE1/AE3) staining was only focally present in 4 PNETs. Fluorescence in situ hybridization was successful in all cases and confirmed EWSR1 rearrangement in 2 of 4 tumors demonstrating morphologic features of Ewing sarcoma/peripheral PNET and concurrent CD99 and Fli-1 expression. In conclusion, central and Ewing sarcoma/peripheral PNETs may be encountered in the female genital tract with central PNETs being more common. Central PNETs show a spectrum of morphologic features that overlaps with CNS tumors but lack EWSR1 rearrangements. GFAP expression supports a morphologic impression of central PNET and is absent in Ewing sarcoma/peripheral PNET. Ewing sarcoma/peripheral PNETs lack morphologic features of CNS tumors.
[Mh] Termos MeSH primário: Neoplasias dos Genitais Femininos/patologia
Tumores Neuroectodérmicos Primitivos Periféricos/patologia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Biomarcadores Tumorais/genética
Biomarcadores Tumorais/metabolismo
Proteínas de Ligação a Calmodulina/genética
Criança
Feminino
Seguimentos
Regulação Neoplásica da Expressão Gênica
Rearranjo Gênico
Neoplasias dos Genitais Femininos/diagnóstico
Neoplasias dos Genitais Femininos/genética
Neoplasias dos Genitais Femininos/metabolismo
Seres Humanos
Imuno-Histoquímica
Hibridização in Situ Fluorescente
Meia-Idade
Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico
Tumores Neuroectodérmicos Primitivos Periféricos/genética
Tumores Neuroectodérmicos Primitivos Periféricos/metabolismo
Prognóstico
Proteína EWS de Ligação a RNA
Proteínas de Ligação a RNA/genética
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers, Tumor); 0 (Calmodulin-Binding Proteins); 0 (EWSR1 protein, human); 0 (RNA-Binding Protein EWS); 0 (RNA-Binding Proteins)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170316
[St] Status:MEDLINE
[do] DOI:10.1097/PAS.0000000000000831


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[PMID]:28052947
[Au] Autor:Ahmad I; Chufal KS; Bhargava A; Bashir I
[Ad] Endereço:Department of Radiation Oncology, Batra Hospital & Medical Research Centre, New Delhi, Delhi, India.
[Ti] Título:Primitive neuroectodermal tumour of the cervix: a rare diagnosis.
[So] Source:BMJ Case Rep;2017, 2017 Jan 04.
[Is] ISSN:1757-790X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:A 48-year-old woman presented with symptoms of lower abdominal pain and vaginal discharge for 6 months. Clinical examination and pelvic ultrasound scan suggested a diagnosis of infected Gartner's cyst, for which she underwent vaginal cystectomy. However, histopathology and immunohistochemistry revealed a diagnosis of primitive neuroectodermal tumour of the cervix. Further investigations revealed the stage to be FIGO IIIB, which was inoperable. She received neoadjuvant chemotherapy (vincristine, adriamycin, cyclophosphamide alternating with ifosfamide, cisplatin and etoposide, every 21 days), but the tumour did not respond to treatment and she was started on radiotherapy with definitive intent (55.8 Gray in 31 fractions over 6.2 weeks). A PET-CT performed 2 months after completion of radiotherapy showed complete response, and she is now receiving adjuvant chemotherapy.
[Mh] Termos MeSH primário: Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico
Neoplasias do Colo do Útero/diagnóstico
[Mh] Termos MeSH secundário: Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
Quimioterapia Adjuvante
Terapia Combinada
Feminino
Seres Humanos
Imagem por Ressonância Magnética
Meia-Idade
Tumores Neuroectodérmicos Primitivos Periféricos/tratamento farmacológico
Tumores Neuroectodérmicos Primitivos Periféricos/radioterapia
Doenças Raras/diagnóstico
Doenças Raras/tratamento farmacológico
Doenças Raras/radioterapia
Neoplasias do Colo do Útero/tratamento farmacológico
Neoplasias do Colo do Útero/radioterapia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170306
[Lr] Data última revisão:
170306
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170106
[St] Status:MEDLINE


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[PMID]:27984122
[Au] Autor:Magro G; Salvatorelli L; Alaggio R; D'Agata V; Nicoletti F; Di Cataldo A; Parenti R
[Ad] Endereço:Department of Medical and Surgical Sciences and Advanced Technologies, GF Ingrassia, Azienda Ospedaliero-Universitaria "Policlinico-Vittorio Emanuele," Anatomic Pathology Section, School of Medicine, University of Catania, 95123 Catania, Italy. Electronic address: g.magro@unict.it.
[Ti] Título:Diagnostic utility of cyclin D1 in the diagnosis of small round blue cell tumors in children and adolescents.
[So] Source:Hum Pathol;60:58-65, 2017 Feb.
[Is] ISSN:1532-8392
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Small round blue cell tumors (SRBCTs) of children and adolescents are often diagnostically challenging lesions. With the increasing diagnostic approach based on small biopsies, there is the need of specific immunomarkers that can help in the differential diagnosis among the different tumor histotypes to assure the patient a correct diagnosis for proper treatment. Based on our recent studies showing cyclin D1 overexpression in both Ewing sarcoma/primitive peripheral neuroectodermal tumor (EWS/pPNET) and peripheral neuroblastic tumors (neuroblastoma and ganglioneuroblastoma), we immunohistochemically assessed cyclin D1 immunoreactivity in 128 cases of SRBCTs in children and adolescents to establish its potential utility in the differential diagnosis. All cases of EWS/pPNET and the undifferentiated/poorly differentiated neuroblastomatous component of all peripheral neuroblastic tumors exhibited strong and diffuse nuclear staining (>50% of neoplastic cells) for cyclin D1. In contrast, this marker was absent from rhabdomyosarcoma (regardless of subtype) and lymphoblastic lymphoma (either B- or T-cell precursors), whereas it was only focally detected (<5% of neoplastic cells) in some cases of Wilms tumor (blastemal component) and desmoplastic small round cell tumor. Our findings suggest that cyclin D1 can be exploitable as a diagnostic adjunct to conventional markers in confirming the diagnosis of EWS/pPNET or neuroblastoma/ganglioneuroblastoma. Its use in routine practice may also be helpful for those cases of SRBCT with undifferentiated morphology that are difficult to diagnose after application of the conventional markers.
[Mh] Termos MeSH primário: Biomarcadores Tumorais/análise
Neoplasias Ósseas/química
Ciclina D1/análise
Ganglioneuroblastoma/química
Neuroblastoma/química
Tumores Neuroectodérmicos Primitivos Periféricos/química
Sarcoma de Ewing/química
[Mh] Termos MeSH secundário: Adolescente
Biópsia
Neoplasias Ósseas/patologia
Diferenciação Celular
Criança
Pré-Escolar
Tumor Desmoplásico de Pequenas Células Redondas/química
Tumor Desmoplásico de Pequenas Células Redondas/patologia
Diagnóstico Diferencial
Feminino
Ganglioneuroblastoma/patologia
Seres Humanos
Imuno-Histoquímica
Lactente
Neoplasias Renais/química
Neoplasias Renais/patologia
Masculino
Neuroblastoma/patologia
Tumores Neuroectodérmicos Primitivos Periféricos/patologia
Leucemia-Linfoma Linfoblástico de Células Precursoras/metabolismo
Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia
Valor Preditivo dos Testes
Estudos Retrospectivos
Rabdomiossarcoma/química
Rabdomiossarcoma/patologia
Sarcoma de Ewing/patologia
Tumor de Wilms/química
Tumor de Wilms/patologia
Adulto Jovem
[Pt] Tipo de publicação:EVALUATION STUDIES; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers, Tumor); 0 (CCND1 protein, human); 136601-57-5 (Cyclin D1)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:171111
[Lr] Data última revisão:
171111
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161217
[St] Status:MEDLINE


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[PMID]:27905830
[Au] Autor:Fan C; Kong D; Tan C; Yang J
[Ad] Endereço:a Department of The Cardiovascular Surgery , The Second Xiangya Hospital, Central South University , Changsha , China.
[Ti] Título:Isolated cardiac peripheral primitive neuroectodermal tumor: A case report.
[So] Source:Cancer Biol Ther;18(1):4-7, 2017 Jan 02.
[Is] ISSN:1555-8576
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Peripheral primitive neuroectodermal tumor isolated in the heart, presenting as a primary cardiac tumor is considered as extremely rare. METHODS: We present a 53-year-old Chinese female with a cardiac tumor which was discovered by CT. RESULTS: A hypo-intense tumorous mass was shown extending from the left ventricle by Cardiac CT, and fused FDG positron emission tomography demonstrated no other abnormal FDG active lesions in the body. We performed a total resection surgery of the tumor subsequently and the patient recovered well and discharged from hospital 6 d after surgery. CONCLUSION: The pathological diagnosis was primary cardiac peripheral primitive neuroectodermal tumor. No tumor recurrence was shown by echocardiography during the 24 months follow-up visits.
[Mh] Termos MeSH primário: Tumores Neuroectodérmicos Primitivos Periféricos
[Mh] Termos MeSH secundário: Feminino
Seres Humanos
Meia-Idade
Tumores Neuroectodérmicos Primitivos Periféricos/patologia
Tumores Neuroectodérmicos Primitivos Periféricos/terapia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170922
[Lr] Data última revisão:
170922
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161202
[St] Status:MEDLINE
[do] DOI:10.1080/15384047.2016.1264542


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[PMID]:27878756
[Au] Autor:Zhang Y; Cai P; Chen M; Yi X; Li L; Xiao D; Liu W; Li W; Li Y
[Ad] Endereço:Department of Radiology, Xiangya Hospital, Central South University, 87# XiangYa Road, Changsha, 410008, Hunan, People's Republic of China.
[Ti] Título:Imaging findings of adrenal primitive neuroectodermal tumors: a series of seven cases.
[So] Source:Clin Transl Oncol;19(5):641-649, 2017 May.
[Is] ISSN:1699-3055
[Cp] País de publicação:Italy
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: To explore the imaging features of adrenal primitive neuroectodermal tumors (PNETs). MATERIALS AND METHODS: This retrospective study included seven patients with surgically and pathologically confirmed adrenal PNETs. Among them, six underwent computed tomography (CT) scans, and one underwent magnetic resonance imaging. The imaging findings, including size, shape, margin, hemorrhage, calcification, cystic degeneration, regional lymph nodes involvement, tumor thrombus formation and enhancement pattern, were retrospectively analyzed. RESULTS: Among the seven adrenal PNET patients, six were male, and one was female. The median age was 26 years (range 2-56 years). The disease generally presented with either insidious symptoms (n = 4) or non-specific symptoms, including right flank pain (n = 1) and left upper abdominal discomfort (n = 2). On the pre-enhanced CT images, the tumor usually appeared as a well-defined, rounded or oval, heterogeneous mass without calcification. Certain tissue characteristics, such as cystic degeneration (n = 5), capsule (n = 4) and hemorrhage (n = 2), were observed. Regional lymph node involvement was observed in three cases, and renal vein thrombus was observed in one case. All cases showed mild heterogeneous enhancement of the tumor on the enhanced CT images. CONCLUSION: An adrenal PNET commonly presents as a relatively large, well-defined, heterogeneous mass with cystic degeneration, necrosis and a characteristic mild contrast-enhancement pattern on multiphase enhanced images. PNET should be considered when the diagnosis of common tumors is not favored by signs on images. CLINICAL TRIAL REGISTRATION STATEMENT: This study was approved by the medical ethics committee of Xiangya Hospital, Central South University. The approval number is 201512538.
[Mh] Termos MeSH primário: Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem
Neoplasias das Glândulas Suprarrenais/patologia
Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico por imagem
Tumores Neuroectodérmicos Primitivos Periféricos/patologia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Criança
Pré-Escolar
Feminino
Seres Humanos
Processamento de Imagem Assistida por Computador
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Estudos Retrospectivos
Tomografia Computadorizada por Raios X
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1705
[Cu] Atualização por classe:171109
[Lr] Data última revisão:
171109
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161124
[St] Status:MEDLINE
[do] DOI:10.1007/s12094-016-1580-3


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[PMID]:27762097
[Au] Autor:Avanzini S; Faticato MG; Crocoli A; Virgone C; Viglio C; Severi E; Fagnani AM; Cecchetto G; Riccipetitoni G; Noccioli B; Leva E; Sementa AR; Mattioli G; Inserra A
[Ad] Endereço:Department of Pediatric Surgery, IRCCS G. Gaslini, Genoa, Italy.
[Ti] Título:Comparative retrospective study on the modalities of biopsying peripheral neuroblastic tumors: a report from the Italian Pediatric Surgical Oncology Group (GICOP).
[So] Source:Pediatr Blood Cancer;64(5), 2017 May.
[Is] ISSN:1545-5017
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Peripheral neuroblastic tumors are the most common extracranial solid neoplasms in children. Early and adequate tissue sampling may speed up the diagnostic process and ensure a prompt start of optimal treatment whenever needed. Different biopsy techniques have been described. The purpose of this multi-center study is to evaluate the accuracy and safety of the various examined techniques and to determine whether a preferential procedure exists. METHODS: All children who underwent a biopsy, from January 2010 to December 2014, as a result of being diagnosed with a peripheral neuroblastic tumor, were retrospectively reviewed. Data collected included patients' demographics, clinical presentation, intraoperative technical details, postoperative parameters, complications, and histology reports. The Mann-Whitney U and Fisher's exact tests were used for statistical analysis. RESULTS: The cohort included 100 patients, 32 of whom underwent an incisional biopsy (performed through open or minimally invasive access) (Group A), and the remaining 68 underwent multiple needle-core biopsies (either imaging-guided or laparoscopy/thoracoscopy-assisted) (Group B). Comparing the two groups revealed that Group A patients had a higher rate of complications, a greater need for postoperative analgesia, and required red blood cell transfusion more often. Overall adequacy rate was 94%, without significant differences between the two groups (100% vs. 91.2% for Group A and Group B, respectively, P = 0.0933). CONCLUSIONS: Both incision and needle-core biopsying methods provided sub-optimal to optimal sampling adequacy rates in children affected by peripheral neuroblastic tumors. However, the former method was associated with a higher risk of both intraoperative and postoperative complications compared with the latter.
[Mh] Termos MeSH primário: Biópsia por Agulha/métodos
Biópsia Guiada por Imagem/métodos
Laparoscopia/métodos
Procedimentos Cirúrgicos Minimamente Invasivos/métodos
Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico
Toracoscopia/métodos
[Mh] Termos MeSH secundário: Adolescente
Biópsia por Agulha/efeitos adversos
Criança
Pré-Escolar
Feminino
Seres Humanos
Biópsia Guiada por Imagem/efeitos adversos
Lactente
Recém-Nascido
Complicações Intraoperatórias/epidemiologia
Itália/epidemiologia
Masculino
Procedimentos Cirúrgicos Minimamente Invasivos/efeitos adversos
Complicações Pós-Operatórias/epidemiologia
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE; MULTICENTER STUDY
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170626
[Lr] Data última revisão:
170626
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161021
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.26284


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[PMID]:27680421
[Au] Autor:Hou W; Xu L; Zhan H; Wang H; Xu M; Yu Y
[Ad] Endereço:From the Departments of *Radiology and †Pathology, The First Affiliated Hospital of Anhui Medical University, Hefei, China.
[Ti] Título:Computed Tomography and Magnetic Resonance Imaging Characteristics of Peripheral Primitive Neuroectodermal Tumor: A Retrospective Analysis of 16 Cases.
[So] Source:J Comput Assist Tomogr;41(2):224-230, 2017 Mar/Apr.
[Is] ISSN:1532-3145
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: The aim of this study was to analyze the radiological features of peripheral primitive neuroectodermal tumor (pPNET). MATERIALS AND METHODS: The radiological and clinical findings for 16 patients with pPNETs were retrospectively reviewed. The 16 tumors were classified into 4 groups (meninges group, n = 4; spine group, n = 3; bone group, n = 5; soft-tissue group, n = 4), and clinical data, size, and common and unique CT/MRI characteristics were assessed. RESULTS: Peripheral primitive neuroectodermal tumors presented as large solid masses with aggressive extension into the neighboring tissue. Most tumors (11/16) presented with necrosis, and 5 of the 16 cases showed signs of hemorrhage. The "dural tail sign" was observed in the meninges and spine groups. The pPNETs of bone demonstrated bony destruction with spiculated periosteal reaction, and small nourishing vessels were found in tumors in the soft-tissue group. CONCLUSIONS: Peripheral primitive neuroectodermal tumor should be suggested as an important differential diagnosis when the tumor presents as a large, ill-defined solid mass with aggressive extension and significant enhancement.
[Mh] Termos MeSH primário: Imagem por Ressonância Magnética/métodos
Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico por imagem
Tomografia Computadorizada por Raios X/métodos
[Mh] Termos MeSH secundário: Adolescente
Adulto
Criança
Pré-Escolar
Diagnóstico Diferencial
Feminino
Seres Humanos
Lactente
Masculino
Meia-Idade
Estudos Retrospectivos
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170414
[Lr] Data última revisão:
170414
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160930
[St] Status:MEDLINE
[do] DOI:10.1097/RCT.0000000000000496


  9 / 1392 MEDLINE  
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[PMID]:27521765
[Au] Autor:Emerson RE; Kao CS; Eble JN; Grignon DJ; Wang M; Zhang S; Wang X; Fan R; Masterson TA; Roth LM; Cheng L
[Ad] Endereço:Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
[Ti] Título:Evidence of a dual histogenetic pathway of sacrococcygeal teratomas.
[So] Source:Histopathology;70(2):290-300, 2017 Jan.
[Is] ISSN:1365-2559
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:AIMS: Sacrococcygeal teratomas are rare tumours that occur most frequently in neonates, although adult cases also occur. The molecular pathogenesis of these tumours and their long-term prognosis is uncertain. We investigated the i(12p) status of a large number of primary sacrococcygeal teratomas in both children and adults, including cases with malignant germ cell tumour elements. METHODS AND RESULTS: Fifty-four sacrococcygeal teratoma specimens from 52 patients were identified, and available follow-up information was obtained. Fluorescence in-situ hybridization analysis was performed to identify isochromosome 12p [i(12p)] abnormalities on paraffin blocks of the tumours. Among the 48 paediatric patients, there were 44 teratomas and four tumours with combined teratoma and yolk sac tumour (one of whom also had primitive neuroectodermal tumour). The teratomas included 37 mature teratomas and 11 immature teratomas (four grade 1, two grade 2, and five grade 3). The 44 teratomas lacking a yolk sac tumour component were all negative for i(12p). The four tumours with a yolk sac tumour component were all positive for i(12p). The four adult cases all lacked non-teratomatous germ cell tumour components, immature elements, and i(12p). Follow-up information was available for 32 patients. Two patients with teratoma had recurrence, but were alive with no evidence of disease after long-term follow-up. One patient with combined teratoma and yolk sac tumour had recurrence 7 months after resection. The other patients were alive with no evidence of disease at last follow-up. CONCLUSIONS: Our data suggest that paediatric sacrococcygeal teratomas should be considered as two distinct groups with divergent histogenetic pathways. The prognosis of these tumours is excellent, despite rare recurrence.
[Mh] Termos MeSH primário: Cromossomos Humanos Par 12/genética
Teratoma/genética
Teratoma/patologia
[Mh] Termos MeSH secundário: Adulto
Criança
Pré-Escolar
Tumor do Seio Endodérmico/patologia
Feminino
Seres Humanos
Hibridização in Situ Fluorescente
Lactente
Recém-Nascido
Isocromossomos/genética
Masculino
Meia-Idade
Tumores Neuroectodérmicos Primitivos Periféricos/patologia
Região Sacrococcígea
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160814
[St] Status:MEDLINE
[do] DOI:10.1111/his.13062


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[PMID]:27500883
[Au] Autor:Ke Ch; Duan Q; Yang H; Zhu F; Yan M; Xu SP; Zhou S; Wan F; Shu K; Lei T; Xia LM
[Ad] Endereço:Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
[Ti] Título:Meningeal Ewing Sarcoma/Peripheral PNET: Clinicopathological, Immunohistochemical and FISH study of four cases.
[So] Source:Neuropathology;37(1):35-44, 2017 02.
[Is] ISSN:1440-1789
[Cp] País de publicação:Australia
[La] Idioma:eng
[Ab] Resumo:Meningeal Ewing Sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) is a rare diagnostically challenging small round cell tumor in the CNS. This study investigates the clinical pathological features of four cases of this tumor from archives of 6 years in our hospital. Patients were within the median age of 21.5 years and male to female ratio was 1:1. The tumors distributed at the supra-tentorial location, posterior fossa and lumbar vertebral canal, usually presenting as the dura-sited nodule or having close connection with the meninges within the cranium or vertebral canal. Histopathologically, small round undifferentiated tumor cells with hypercellularities, scant cytoplasm and inconspicuous nucleoli were observed, although some components such as atypical larger vesicular nuclei, prominent nucleoli of tumor cells, necrotic foci and mesenchymal collagen proliferation forming the lobular structure, were also appreciated. Immunohistochemally, tumor cells displayed membranous positivity of CD99 (4/4), nuclear positivity of FLI-1 (4/4) and NKX2.2 (4/4), negativity of EMA, GFAP and synaptophysin expression. The histochemical PAS staining showed weak positivity in one case. Fluorescence in situ hybridization (FISH) test using EWSR1 (22q12) dual color break apart rearrangement probe showed positive results in two cases. Results suggest that using a panel of immunohistochemical markers, including NKX2.2, CD99, FLI-1, EMA, GFAP and synaptophysin, combined with the supplementary EWSR1 FISH test, helps to define the diagnosis of meningeal ES/pPNET of CNS.
[Mh] Termos MeSH primário: Neoplasias Meníngeas/diagnóstico
Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico
Sarcoma de Ewing/diagnóstico
[Mh] Termos MeSH secundário: Antígeno 12E7/metabolismo
Adolescente
Adulto
Biomarcadores Tumorais/genética
Biomarcadores Tumorais/metabolismo
Proteínas de Ligação a Calmodulina/genética
Proteínas de Ligação a Calmodulina/metabolismo
Dura-Máter/metabolismo
Dura-Máter/patologia
Feminino
Proteínas de Homeodomínio/metabolismo
Seres Humanos
Imuno-Histoquímica
Hibridização in Situ Fluorescente
Masculino
Neoplasias Meníngeas/genética
Neoplasias Meníngeas/metabolismo
Neoplasias Meníngeas/patologia
Tumores Neuroectodérmicos Primitivos Periféricos/genética
Tumores Neuroectodérmicos Primitivos Periféricos/metabolismo
Tumores Neuroectodérmicos Primitivos Periféricos/patologia
Proteína Proto-Oncogênica c-fli-1/metabolismo
Proteína EWS de Ligação a RNA
Proteínas de Ligação a RNA/genética
Proteínas de Ligação a RNA/metabolismo
Sarcoma de Ewing/genética
Sarcoma de Ewing/metabolismo
Sarcoma de Ewing/patologia
Fatores de Transcrição/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (12E7 Antigen); 0 (Biomarkers, Tumor); 0 (Calmodulin-Binding Proteins); 0 (EWSR1 protein, human); 0 (FLI1 protein, human); 0 (Homeodomain Proteins); 0 (Nkx-2.2 homedomain protein); 0 (Proto-Oncogene Protein c-fli-1); 0 (RNA-Binding Protein EWS); 0 (RNA-Binding Proteins); 0 (Transcription Factors)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160809
[St] Status:MEDLINE
[do] DOI:10.1111/neup.12325



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