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[PMID]:29480840
[Au] Autor:De Nola R; Di Naro E; Schonauer LM; Lucarelli G; Battaglia M; Fiore MG; Mastrolia SA; Loverro G
[Ad] Endereço:Department of Biomedical Sciences and Human Oncology, Gynaecologic and Obstetrics Clinic.
[Ti] Título:Clinical management of a unique case of PNET of the uterus during pregnancy, and review of the literature.
[So] Source:Medicine (Baltimore);97(2):e9505, 2018 Jan.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: PNETs (primitive neuroectodermal tumors) are a family of highly malignant neoplasms characterized by small round cells of neuroepithelial origin. They usually involve bone and soft tissues, and have a higher incidence in childhood. PATIENT CONCERNS: In this case report, we describe the obstetric and oncological outcome of a huge mass diagnosed as a leiomyoma in a 39-year-old pregnant woman who complained of low back pain, dysuria, and urinary frequency at 22 weeks of gestation. DIAGNOSES: During the 25th week of pregnancy, the patient was referred to our hospital at night with severe anemia and suspected hemoperitoneum. She underwent an emergency caesarean section, delivering a female fetus weighing 400 g, with an Apgar score of 7 at 1 minute and 9 at 5 minutes. INTERVENTION: During surgery, we found a huge uterine sarcoma-like metastatic tumor, invading the pelvic peritoneum and parametria bilaterally; the adnexae seemed disease-free. We performed a type B radical hysterectomy, bilateral salpingo-oophorectomy, pelvic peritonectomy, omentectomy, appendectomy, and excision of a bulky lymph node. Seven days after delivery, staging computed tomography (CT) scan demonstrated a large lombo-aortic lymph node compressing the left renal vein and we completed debulking with a second surgery, including diaphragmatic peritonectomy and excision of a huge lymph node by lombo-aortic lymphadenectomy, requiring partial reconstruction of an infiltrated renal vein. OUTCOME: Ten days after the second surgery, echo-color Doppler showed a regular microcirculation in the left kidney. The patient was discharged after 10 days, and the baby after 1 month, both in good health.Histological examination revealed a uterine body cPNET (central primitive neuroectodermal tumor) orienting the clinical management toward chemotherapy with cisplatin and etoposide. LESSONS: PNETs are aggressive neoplasms, usually diagnosed at an advanced stage. Due to their low incidence, universally accepted guidelines are still unavailable. Radical surgery leaving no macroscopic residual disease is mandatory in advanced stages. A good fertility-sparing procedure can be performed only in young women at early stages of disease, when the wish for childbearing is not yet fulfilled.
[Mh] Termos MeSH primário: Tumores Neuroectodérmicos Primitivos/cirurgia
Complicações Neoplásicas na Gravidez/cirurgia
Neoplasias Uterinas/cirurgia
[Mh] Termos MeSH secundário: Adulto
Cesárea
Serviços Médicos de Emergência
Feminino
Seres Humanos
Recém-Nascido
Tumores Neuroectodérmicos Primitivos/diagnóstico por imagem
Tumores Neuroectodérmicos Primitivos/tratamento farmacológico
Tumores Neuroectodérmicos Primitivos/patologia
Gravidez
Complicações Neoplásicas na Gravidez/diagnóstico por imagem
Complicações Neoplásicas na Gravidez/tratamento farmacológico
Complicações Neoplásicas na Gravidez/patologia
Neoplasias Uterinas/diagnóstico por imagem
Neoplasias Uterinas/tratamento farmacológico
Neoplasias Uterinas/patologia
Útero/diagnóstico por imagem
Útero/patologia
Útero/cirurgia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180302
[Lr] Data última revisão:
180302
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180227
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009505


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[PMID]:29443778
[Au] Autor:He X; Chen Z; Dong Y; Tong D
[Ad] Endereço:Department of Radiology.
[Ti] Título:A primitive neuroectodermal tumor in an adult: Case report of a unique location and MRI characteristics.
[So] Source:Medicine (Baltimore);97(7):e9933, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Central nervous system primitive neuroectodermal tumors (CNS PNETs) mostly occur in children and present as cerebellar medulloblastoma. A few cases of PNETs occur in the cerebral hemisphere. The presence of a PNET in ventricles is extremely rare. The prognosis of CNS PNET is extremely poor, and the 5-year survival rate does not exceed 35%. In the present study, we describe the first case of a PNET in the ventricles with good prognosis. PATIENT CONCERNS: The case of a 36-year-old man is reported, who presented with a progressively worsening headache for 2 months. DIAGNOSES: Magnetic resonance imaging (MRI) revealed multiple tubercula on the walls of the lateral and third ventricles. Histopathologic analysis revealed a hypercellular tumor with small round cells containing hyperchromatic nuclei and a high nucleus:cytoplasm ratio. The analysis was consistent with PNET. INTERVENTIONS: Radiation therapy covering the entire craniospinal axis was administered, with Temozolomide for synchronous auxiliary treatment. OUTCOMES: The patient was follow-up for a year and showed no signs of recurrence. LESSONS: We present the first CNS PNET located in the ventricles with good prognosis. In this case, radiotherapy with Temozolomide auxiliary treatment presented good efficacy and safety to treat PNET. Additional studies on biomarkers may be useful in predicting personalized therapeutic response.
[Mh] Termos MeSH primário: Neoplasias Encefálicas/diagnóstico por imagem
Ventrículos Cerebrais/diagnóstico por imagem
Imagem por Ressonância Magnética
Tumores Neuroectodérmicos Primitivos/diagnóstico por imagem
[Mh] Termos MeSH secundário: Adulto
Antineoplásicos Alquilantes/uso terapêutico
Neoplasias Encefálicas/tratamento farmacológico
Neoplasias Encefálicas/radioterapia
Quimioterapia Adjuvante
Dacarbazina/análogos & derivados
Dacarbazina/uso terapêutico
Seres Humanos
Masculino
Tumores Neuroectodérmicos Primitivos/tratamento farmacológico
Tumores Neuroectodérmicos Primitivos/radioterapia
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antineoplastic Agents, Alkylating); 7GR28W0FJI (Dacarbazine); YF1K15M17Y (temozolomide)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180220
[Lr] Data última revisão:
180220
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180215
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009933


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[PMID]:27770281
[Au] Autor:Indini A; Schiavello E; Biassoni V; Bergamaschi L; Magni MC; Puma N; Chiaravalli S; Pallotti F; Seregni E; Diletto B; Pecori E; Gandola L; Poggi G; Massimino M
[Ad] Endereço:Pediatric Oncology Unit, Fondazione IRCCS, Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, Italy. alice.indini@istitutotumori.mi.it.
[Ti] Título:Long-term safety of growth hormone replacement therapy after childhood medulloblastoma and PNET: it is time to set aside old concerns.
[So] Source:J Neurooncol;131(2):349-357, 2017 01.
[Is] ISSN:1573-7373
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:To assess the long-term safety of administering growth hormone (GH) in patients with GH deficiency due to treatment for childhood medulloblastoma and primitive neuroectodermal tumor (PNET). Data were retrospectively retrieved on children receiving GH supplementation, assessing their disease-free and overall survival outcomes and risk of secondary malignancies using Kaplan-Meier and Cox models. Overall 65 children were consecutively collected from May 1981 to April 2013. All patients had undergone craniospinal irradiation (total dose 18-39 Gy), and subsequently received GH for a median (interquartile range, IQR) of 81 (50.6-114.9) months. At a median (IQR) of 122.4 months (74.4-149.5) after the end of their adjuvant cancer treatment, two patients (3 %) experienced recurrent disease and 8 (12.3 %) developed secondary malignancies, all but one of them (an osteosarcoma) related to radiation exposure and occurring within the radiation fields. There was no apparent correlation between the administration of GH replacement therapy (or its duration) and primary tumor relapse or the onset of secondary malignancies [HR: 1.01 (95 % CI: 0.98, 1.03) for every additional 12 months of GH supplementation; p = 0.36). At univariate analysis, the large cell or anaplastic medulloblastoma subtype, metastases and myeloablative chemotherapy correlated with a higher risk of secondary malignancies (p < 0.1), but multivariate analysis failed to identify any factors independently associated with this risk. Our data supports once more the safety of long-term GH replacement therapy in children treated for medulloblastoma/PNET, previously reported in larger data sets. The neurooncology community now need to warrant large-scale meta-analyses or international prospective trials in order to consolidate our knowledge of factors other than GH, such as genetic predisposition, high-grade/metastatic disease, high-dose chemotherapy and era of treatment, in promoting the occurrence of secondary malignancies.
[Mh] Termos MeSH primário: Neoplasias Encefálicas/tratamento farmacológico
Hormônio do Crescimento/efeitos adversos
Terapia de Reposição Hormonal/efeitos adversos
Meduloblastoma/tratamento farmacológico
Tumores Neuroectodérmicos Primitivos/tratamento farmacológico
[Mh] Termos MeSH secundário: Criança
Feminino
Hormônio do Crescimento/uso terapêutico
Seres Humanos
Estimativa de Kaplan-Meier
Masculino
Estudos Retrospectivos
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
9002-72-6 (Growth Hormone)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:180119
[Lr] Data última revisão:
180119
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161023
[St] Status:MEDLINE
[do] DOI:10.1007/s11060-016-2306-7


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[PMID]:29245277
[Au] Autor:Wang G; Guo F
[Ad] Endereço:aDepartment of NeurosurgerybDepartment of Neurosurgery, Key Laboratory of Neurosurgical Diseases, The First Affiliated Hospital of Zhengzhou University, ZhengZhou, HeNan Province, PR China.
[Ti] Título:Primary intramedullary primitive neuroectodermal tumor: A case report and review of the literature.
[So] Source:Medicine (Baltimore);96(49):e9001, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Primary spinal primitive neuroectodermal tumors (PNETs) are highly malignant tumors, which are extremely rare entities and primary intramedullary PNETs are extremely rare. Till now, only 24 cases of primary intramedullary PNET have been reported. PATIENT CONCERNS: A 26-year-old male presented with progressive low back and lower limb pain for 1 month. DIAGNOSES: Based on MRI and histopathological findings, he was diagnosed with primary intramedullary PNET. INTERVENTIONS: The patient was treated two times with microsurgical resections. OUTCOMES: Follow-up visit at 14 months after the first surgery showed that the patient is neurologically intact and free of disease. LESSONS: PNETs should be considered in the differential diagnosis of an intramedullary spinal cord tumor manifesting as progressive neurological deterioration.
[Mh] Termos MeSH primário: Tumores Neuroectodérmicos Primitivos/diagnóstico
Neoplasias da Medula Espinal/diagnóstico
[Mh] Termos MeSH secundário: Adulto
Seres Humanos
Vértebras Lombares/patologia
Imagem por Ressonância Magnética
Masculino
Tumores Neuroectodérmicos Primitivos/diagnóstico por imagem
Tumores Neuroectodérmicos Primitivos/cirurgia
Neoplasias da Medula Espinal/diagnóstico por imagem
Neoplasias da Medula Espinal/cirurgia
Vértebras Torácicas/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180105
[Lr] Data última revisão:
180105
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171217
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009001


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[PMID]:28509337
[Au] Autor:Szychot E; Seunarine K; Mankad K; Thust S; Clark C; Gaze MN; Michalski A
[Ad] Endereço:Department of Clinical Studies, The Institute of Cancer Research, London, United Kingdom.
[Ti] Título:Impact of induction chemotherapy, hyperfractionated accelerated radiotherapy and high-dose thiotepa on brain volume loss and functional status of children with primitive neuroectodermal tumour.
[So] Source:Pediatr Blood Cancer;64(11), 2017 Nov.
[Is] ISSN:1545-5017
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The introduction of aggressive chemo-radiotherapy regimens has improved overall survival in children with primitive neuroectodermal tumours (PNET). However, these combinations may result in neurotoxicity. Previously reported magnetic resonance imaging abnormalities in children receiving intensive sequential chemotherapy, hyperfractionated accelerated radiotherapy (HART) and high-dose thiotepa prompted us to investigate the degree of brain volume loss and patients' functional status after therapy. METHODS: We retrospectively reviewed clinico-radiological data of children with PNET treated in this way at our centre. RESULTS: We studied 14 children treated between December 2009 and April 2013. Data were not complete for one child. Performance status was severely restricted in four children, and mildly to moderately impaired in 7 of the 13 children. Eleven of 13 children showed mild-to-severe generalised neuroparenchymal atrophy, in 7 of whom neuroparenchymal volume loss was moderate to severe. Of these seven, six had received high-dose thiotepa. There was no correlation between brain volume loss and Lansky performance status. However, unexpected neurotoxicities, such as symptoms of transverse myelitis, were observed. CONCLUSION: Measurement of brain volume loss in patients treated with HART and high-dose thiotepa may not be sufficient to predict function. However, correlation of brain volume loss due to late neurotoxicity with performance decline may be more obvious over longer period of follow-up. The combination of HART and myeloablative courses of thiotepa is associated with severe neurotoxicity and subsequent decline in performance status in a significant proportion of patients.
[Mh] Termos MeSH primário: Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
Neoplasias Encefálicas/patologia
Quimiorradioterapia
Quimioterapia de Indução
Tumores Neuroectodérmicos Primitivos/patologia
[Mh] Termos MeSH secundário: Adolescente
Neoplasias Encefálicas/terapia
Carboplatina/administração & dosagem
Criança
Pré-Escolar
Ciclofosfamida/administração & dosagem
Fracionamento de Dose
Etoposídeo/administração & dosagem
Feminino
Seguimentos
Seres Humanos
Masculino
Estadiamento de Neoplasias
Tumores Neuroectodérmicos Primitivos/terapia
Prognóstico
Estudos Retrospectivos
Taxa de Sobrevida
Tiotepa/administração & dosagem
Carga Tumoral
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
6PLQ3CP4P3 (Etoposide); 8N3DW7272P (Cyclophosphamide); 905Z5W3GKH (Thiotepa); BG3F62OND5 (Carboplatin)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171031
[Lr] Data última revisão:
171031
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170517
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.26619


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[PMID]:28438654
[Au] Autor:Nguyen HS; Doan N; Gelsomino M; Shabani S
[Ad] Endereço:Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, Wisconsin. Electronic address: hsnguyen@mcw.edu.
[Ti] Título:Dysembryoplastic Neuroectodermal Tumor: An Analysis from the Surveillance, Epidemiology, and End Results Program, 2004-2013.
[So] Source:World Neurosurg;103:380-385, 2017 Jul.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Dysembryoplastic neuroectodermal tumor (DNT) is a rare neoplasm. Though the pathology is commonly considered benign, there have been various reports documenting rapid growth, recurrence/progression, sudden death, and malignant transformation. Most studies have addressed outcomes regarding seizure control, but limited data exist regarding incidence and survival. Consequently, we explore the Surveillance, Epidemiology, and End Results (SEER) database to explore the epidemiology of DNT. METHODS: From the SEER-18 registry database, information from all patients diagnosed with intracranial DNT between 2004 and 2013 was extracted, including age, sex, race, marital status, tumor location, tumor size, receipt of surgery, extent of primary surgery, receipt of radiation, and follow-up data. Age-adjusted incidence rates and overall survival (OS) were calculated. A Cox proportional hazards model was used to assess relationships between various demographic/treatment variables and OS. RESULTS: A total of 381 cases were identified in the SEER-18 database. The incidence of DNT within the large subset of the United States population represented by SEER was 0.033 per 100,000 person-years (95% confidence interval [CI], 0.030-0.037). The median duration of follow-up was 50 months. The median OS was not attained. The 3-, 5-, and 9-year OS was 99.363% (95% CI, 97.428%-99.844%), 97.993% (95% CI, 95.168%-99.174%), and 96.296% (95% CI, 91.834%-98.341%), respectively. Seven of the 381 patients passed at their last follow up. Of all demographic/treatment factors, only receipt of radiation demonstrated a significant relationship with OS (hazard ratio, 0.051; 95% CI, 0.01-0.267; P < 0.01). CONCLUSIONS: Although the prognosis for DNT is generally favorable, the pathology can lead to poor outcomes in rare cases. Common demographic factors, treatment with surgery, and the extent of surgical resection did not show significant associations with OS. In contrast, treatment with radiation was associated with poorer OS.
[Mh] Termos MeSH primário: Neoplasias Encefálicas/mortalidade
Tumores Neuroectodérmicos Primitivos/mortalidade
[Mh] Termos MeSH secundário: Adolescente
Adulto
Distribuição por Idade
Idoso
Criança
Pré-Escolar
Morte Súbita/epidemiologia
Feminino
Seres Humanos
Incidência
Lactente
Masculino
Meia-Idade
Prognóstico
Programa de SEER
Estados Unidos/epidemiologia
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170426
[St] Status:MEDLINE


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[PMID]:28408259
[Au] Autor:Fu W; Ju Y; Zhang S; You C
[Ad] Endereço:Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.
[Ti] Título:Pediatric Basal Ganglia Region Tumors: Clinical and Radiologic Features Correlated with Histopathologic Findings.
[So] Source:World Neurosurg;103:504-516, 2017 Jul.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: To summarize the clinical and radiologic features of pediatric basal ganglia region tumors (PBGRT) in correlation with their histopathologic findings to reduce inappropriate surgery and identify tumors that can benefit from maximal safe resection. METHODS: The records of 35 children with PBGRT treated in our hospital from December 2011 to December 2015 were analyzed retrospectively. The clinical and radiologic features of these tumors were summarized and correlated with their histopathologic diagnosis. RESULTS: Our series included 15 astrocytomas and 11 germ cell tumors (GCTs). Basal ganglia astrocytomas were characterized by various clinical presentations and an ill-circumscribed mass with the involvement of surrounding structures on neuroimaging and mostly occurred in the first decade of life (n = 10; 66.7%). Basal ganglia GCT mostly occurred in the second decade of life (n = 8; 72.7%) with hemiparesis as the most common symptom (n = 9; 81.8%). The tumors were located predominantly in the caput of caudate nucleus (n = 8; 72.7%) with hemiatrophy as the typical sign (n = 8; 72.7%). Occasionally, other tumors also could occur in this region, including primitive neuroectodermal tumor (n = 1), atypical teratoid/rhabdoid tumor (n = 1), anaplastic ependymoma (n = 1), lymphoma (n = 1), extraventricular neurocytoma (n = 1), gangliogliomas (n = 2), oligodendroglioma (n = 1), and dysembryoplastic neuroepithelial tumor (n = 1). CONCLUSIONS: Astrocytoma and GCT are the most common PBGRTs. Low-grade astrocytomas could benefit from maximal surgical resection, whereas GCTs merit neoadjuvant chemoradiation therapy followed by second-look surgery. We advocate routine testing of tumor markers and analysis of their clinical and radiologic features to optimize the therapeutic strategy.
[Mh] Termos MeSH primário: Astrocitoma/terapia
Doenças dos Gânglios da Base/terapia
Biomarcadores Tumorais/metabolismo
Neoplasias Encefálicas/terapia
Quimiorradioterapia
Terapia Neoadjuvante
Neoplasias Embrionárias de Células Germinativas/terapia
Procedimentos Neurocirúrgicos
[Mh] Termos MeSH secundário: Adolescente
Astrocitoma/complicações
Astrocitoma/diagnóstico por imagem
Astrocitoma/metabolismo
Doenças dos Gânglios da Base/complicações
Doenças dos Gânglios da Base/diagnóstico por imagem
Doenças dos Gânglios da Base/metabolismo
Neoplasias Encefálicas/complicações
Neoplasias Encefálicas/diagnóstico por imagem
Neoplasias Encefálicas/metabolismo
Núcleo Caudado/diagnóstico por imagem
Núcleo Caudado/cirurgia
Criança
Pré-Escolar
Tontura/etiologia
Ependimoma/complicações
Ependimoma/diagnóstico por imagem
Ependimoma/metabolismo
Ependimoma/terapia
Feminino
Ganglioglioma/complicações
Ganglioglioma/diagnóstico por imagem
Ganglioglioma/metabolismo
Ganglioglioma/terapia
Cefaleia/etiologia
Seres Humanos
Lactente
Linfoma/complicações
Linfoma/diagnóstico por imagem
Linfoma/metabolismo
Linfoma/terapia
Masculino
Técnicas de Diagnóstico Molecular
Neoplasias Embrionárias de Células Germinativas/complicações
Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem
Neoplasias Embrionárias de Células Germinativas/metabolismo
Neoplasias Neuroepiteliomatosas/complicações
Neoplasias Neuroepiteliomatosas/diagnóstico por imagem
Neoplasias Neuroepiteliomatosas/terapia
Neurocitoma/complicações
Neurocitoma/diagnóstico por imagem
Neurocitoma/metabolismo
Neurocitoma/terapia
Tumores Neuroectodérmicos Primitivos/complicações
Tumores Neuroectodérmicos Primitivos/diagnóstico por imagem
Tumores Neuroectodérmicos Primitivos/metabolismo
Tumores Neuroectodérmicos Primitivos/terapia
Oligodendroglioma/complicações
Oligodendroglioma/diagnóstico por imagem
Oligodendroglioma/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers, Tumor)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170415
[St] Status:MEDLINE


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[PMID]:28376845
[Au] Autor:Guo H; Chen S; Liu S; Wang K; Liu E; Li F; Hou Y
[Ad] Endereço:Department of Urology, First Hospital of Jilin University, Changchun, Jilin, 130021, China.
[Ti] Título:Rare adrenal gland incidentaloma: an unusual Ewing's sarcoma family of tumor presentation and literature review.
[So] Source:BMC Urol;17(1):24, 2017 Apr 04.
[Is] ISSN:1471-2490
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Members of the Ewing's sarcoma family of tumor (ESFT) are malignant neoplasms and rarely observed in the adrenal gland. CASE PRESENTATION: We report an extremely exceptional case of ESFT rising from the adrenal gland in a 57-year-old Chinese man. The patient was hospitalized with abdominal swelling for 2 months. Computed tomography (CT) scan revealed a nearly-circular mass measuring about 8.1 × 10.6 cm in the right adrenal region. The patient underwent right adrenal resection. Histopathologic examination found the tumor was composed of small round blue cells forming typical Homer-Wright rosettes in focal area. The immunohistochemical analysis confirmed the case to be ESFT, which was positive for membranous CD99 and nuclear FLI-1. The patient was scheduled for four courses of large doses of chemotherapy and died for cancer metastasis one year later after surgery. CONCLUSIONS: Histopathological evidence of Homer-Wright rosettes and immunohistochemical markers positivity, such as CD99 and FLI-1, are valuable factors for ESFT diagnosis, although cytogenetic analysis is considered as the gold standard. Complete surgery is the treatment of choice for ESFT and adjuvant radiotherapy and combination chemotherapy can significantly improve the survival rate of postoperative patients.
[Mh] Termos MeSH primário: Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem
Sarcoma de Ewing/diagnóstico por imagem
[Mh] Termos MeSH secundário: Adolescente
Neoplasias das Glândulas Suprarrenais/patologia
Adulto
Criança
Pré-Escolar
Feminino
Seres Humanos
Achados Incidentais
Masculino
Meia-Idade
Tumores Neuroectodérmicos Primitivos/patologia
Sarcoma de Ewing/patologia
Tomografia Computadorizada por Raios X
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170821
[Lr] Data última revisão:
170821
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170406
[St] Status:MEDLINE
[do] DOI:10.1186/s12894-017-0217-3


  9 / 1567 MEDLINE  
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[PMID]:28319177
[Au] Autor:Yi X; Liu W; Zhang Y; Xiao D; Yin H; Long X; Li L; Zai H; Chen M; Li W; Sun L
[Ad] Endereço:Department of Radiology, Xiangya Hospital, Central South University; Changsha, P.R. China.
[Ti] Título:Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases.
[So] Source:PLoS One;12(3):e0173536, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVES: To characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions. METHODS: Eighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed. RESULTS: Of eighteen PNET patients, fifteen were male and three were female, with a median age of 36 years (range, 2-65 years). The onset of symptoms was most often nonspecific and insidious. The mean tumor diameter was 7.2 cm (range, 3.0-12.1 cm), with necrosis in fifteen cases, cystic changes in eight, partition structure in five, calcification in five, hemorrhage in two, and mural nodules in one. Contrast enhanced computed tomography showed multiple tiny feeding arteries within the masses in six cases, resulting in a crab-like appearance, and mild ring enhancement pattern in five cases. Eleven cases showed surrounding invasion and metastasis. Of the eighteen PNET cases, nine cases showed smooth, well-defined margins, and nine cases had irregular, ill-defined margins. A median survival was 10.0±1.6 months. However, chemotherapy had efficacy on patients even those with advanced disease. CONCLUSIONS: Primary intra-abdominal and retroperitoneal PNETs are rare, and imaging features documented here may help the diagnosis of this severe disease. Notably, two signs present in retroperitoneal PNET tumors, including a mild ring enhancement pattern and a crab-like appearance of the tiny feeding arteries, may have the potential to help us improve the ability to make a relatively reliable diagnosis.
[Mh] Termos MeSH primário: Neoplasias Abdominais/diagnóstico por imagem
Imagem por Ressonância Magnética
Tumores Neuroectodérmicos Primitivos/diagnóstico por imagem
Neoplasias Retroperitoneais/diagnóstico por imagem
Tomografia Computadorizada por Raios X
[Mh] Termos MeSH secundário: Neoplasias Abdominais/irrigação sanguínea
Neoplasias Abdominais/patologia
Neoplasias Abdominais/terapia
Adolescente
Adulto
Idoso
Criança
Pré-Escolar
Meios de Contraste
Feminino
Seguimentos
Seres Humanos
Masculino
Meia-Idade
Tumores Neuroectodérmicos Primitivos/irrigação sanguínea
Tumores Neuroectodérmicos Primitivos/patologia
Tumores Neuroectodérmicos Primitivos/terapia
Prognóstico
Neoplasias Retroperitoneais/irrigação sanguínea
Neoplasias Retroperitoneais/patologia
Neoplasias Retroperitoneais/terapia
Análise de Sobrevida
Carga Tumoral
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Contrast Media)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170823
[Lr] Data última revisão:
170823
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170321
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0173536


  10 / 1567 MEDLINE  
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[PMID]:28249000
[Au] Autor:Malchenko S; Sredni ST; Bi Y; Margaryan NV; Boyineni J; Mohanam I; Tomita T; Davuluri RV; Soares MB
[Ad] Endereço:Department of Cancer Biology & Pharmacology, University of Illinois College of Medicine, Peoria, Illinois, United States of America.
[Ti] Título:Stabilization of HIF-1α and HIF-2α, up-regulation of MYCC and accumulation of stabilized p53 constitute hallmarks of CNS-PNET animal model.
[So] Source:PLoS One;12(3):e0173106, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Recently, we described a new animal model of CNS primitive neuroectodermal tumors (CNS-PNET), which was generated by orthotopic transplantation of human Radial Glial (RG) cells into NOD-SCID mice's brain sub-ventricular zone. In the current study we conducted comprehensive RNA-Seq analyses to gain insights on the mechanisms underlying tumorigenesis in this mouse model of CNS-PNET. Here we show that the RNA-Seq profiles derived from these tumors cluster with those reported for patients' PNETs. Moreover, we found that (i) stabilization of HIF-1α and HIF-2α, which are involved in mediation of the hypoxic responses in the majority of cell types, (ii) up-regulation of MYCC, a key onco-protein whose dysregulation occurs in ~70% of human tumors, and (iii) accumulation of stabilized p53, which is commonly altered in human cancers, constitute hallmarks of our tumor model, and might represent the basis for CNS-PNET tumorigenesis in this model. We discuss the possibility that these three events might be interconnected. These results indicate that our model may prove invaluable to uncover the molecular events leading to MYCC and TP53 alterations, which would be of broader interest considering their relevance to many human malignancies. Lastly, this mouse model might prove useful for drug screening targeting MYCC and related members of its protein interaction network.
[Mh] Termos MeSH primário: Fatores de Transcrição Hélice-Alça-Hélice Básicos/metabolismo
Neoplasias Encefálicas/metabolismo
Subunidade alfa do Fator 1 Induzível por Hipóxia/metabolismo
Tumores Neuroectodérmicos Primitivos/metabolismo
Proteínas Proto-Oncogênicas c-myc/metabolismo
Proteína Supressora de Tumor p53/metabolismo
[Mh] Termos MeSH secundário: Animais
Fatores de Transcrição Hélice-Alça-Hélice Básicos/genética
Neoplasias Encefálicas/genética
Células Cultivadas
Seres Humanos
Subunidade alfa do Fator 1 Induzível por Hipóxia/genética
Camundongos
Camundongos Endogâmicos NOD
Camundongos SCID
Tumores Neuroectodérmicos Primitivos/genética
Proteínas Proto-Oncogênicas c-myc/genética
Proteína Supressora de Tumor p53/genética
Regulação para Cima
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Basic Helix-Loop-Helix Transcription Factors); 0 (Hif1a protein, mouse); 0 (Hypoxia-Inducible Factor 1, alpha Subunit); 0 (Proto-Oncogene Proteins c-myc); 0 (Tumor Suppressor Protein p53); 0 (endothelial PAS domain-containing protein 1)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170302
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0173106



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