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[PMID]:29400034
[Au] Autor:Kharoubi S
[Ti] Título:[Benign tumors of the nasal cavity, new classification and review of the literature. Report of 54 cases].
[So] Source:Rev Laryngol Otol Rhinol (Bord);136(4):131-42, 2015.
[Is] ISSN:0035-1334
[Cp] País de publicação:France
[La] Idioma:fre
[Ab] Resumo:Benign tumors of the nasal cavity is an uncommon disease and very diverse about histological variety. Theses tumors are seen at any age without specificity in semiological aspect. The diagnostic approach is based on nasal endoscopy, imaging (CT-MRI) and biopsy. In some anatomo clinical varieties the result of biopsy is so difficult and we must analyze all the tumor specimen after surgery with modern biological procedure (immunohistochemistry, molecular biology). The treatment is surgery especially with endonasal endoscopic procedure. Our series included 54 benign endonasal tumors between January 1998 and December 2008. The mean age of our population is 28 years with a female sex ratio of 1.16. Endoscopic examina­tion and a CT scan of facial bones were systematic. The tumors of nasal septum were predominant. The histological variety are dominated by vascular tumors (hemangioma) in 36 cases (66.6%) and papilloma (papilloma and inverted papilloma) in 15 cases (27.7%), two cases of fibroma (37%) and one case of leiomyoma. The treatment was surgical with an endonasal approach in 37 cases (68.5%), endonasal and endoscopic in 11 cases (20.3%) and external 6 cases (11.1%). A review of the literature on tumors of the nasal cavity is made during this study.
[Mh] Termos MeSH primário: Cavidade Nasal/patologia
Neoplasias Nasais/patologia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Criança
Endoscopia
Feminino
Fibroma/patologia
Fibroma/cirurgia
Hemangioma/patologia
Hemangioma/cirurgia
Seres Humanos
Leiomioma/patologia
Leiomioma/cirurgia
Masculino
Meia-Idade
Cavidade Nasal/cirurgia
Neoplasias Nasais/cirurgia
Papiloma/patologia
Papiloma/cirurgia
Estudos Retrospectivos
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180308
[Lr] Data última revisão:
180308
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180206
[St] Status:MEDLINE


  2 / 9266 MEDLINE  
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[PMID]:29429162
[Au] Autor:Gong QX; Li H; Zhang ZH; Fan QH
[Ad] Endereço:Department of Pathology, the First Affiliated Hospital to Nanjing Medical University, Nanjing 210029, China.
[Ti] Título:[Pulmonary microcystic fibromyxoma: report of a case with review of literature].
[So] Source:Zhonghua Bing Li Xue Za Zhi;47(2):110-113, 2018 Feb 08.
[Is] ISSN:0529-5807
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:To study the clinicopathologic features, diagnosis and differential diagnosis of pulmonary microcystic fibromyxoma. In March 2014, at the First Affiliated Hospital to Nanjing Medical University a 58-year-old female patient of pulmonary microcystic fibromyxoma was collected. The clinicopathologic, immunohistochemical and genetic profile of a case of pulmonary microcystic fibromyxoma were studied, and the relevant literature reviewed. The patient was a 58-year-old female who presented with cough and sputum for 1 month. CT scan disclosed a 15 mm nodule in her right middle lobe of lung. The patient underwent a wedge resection with negative margin. Grossly, a well-demarcated peripheral lung nodule was detected, measuring 1.5 cm×1.5 cm×1.0 cm, with myxoid tan-white cut surface containing microcysts. Microscopically, the tumor was composed of bland spindled to stellate-shaped cells widely spaced within prominent fibromyxoid stroma with prominent cystic change. No mitosis or necrosis was present. There were inconspicuous slim curvilinear capillaries and occasional collection of stromal lymphocytes and plasma cells. Immunohistochemically, the tumor cells were positive for vimentin, but negative for CD34, SMA, desmin, S-100 protein, ALK, CKpan, EMA, calretinin and TTF1. Fluorescence in situ hybridization did not show chromosomal translocation involving EWSR1, DDIT3 or FUS genes. The patient was recurrence or metastasis free after follow-up for 38 months. Pulmonary microcystic fibromyxoma is a rare benign lesion that should be differentiated from other lung tumors with myxoid characteristics.
[Mh] Termos MeSH primário: Fibroma/química
Fibroma/patologia
Neoplasias Pulmonares/química
Neoplasias Pulmonares/patologia
[Mh] Termos MeSH secundário: Proteínas de Ligação a Calmodulina/análise
Diagnóstico Diferencial
Feminino
Seres Humanos
Hibridização in Situ Fluorescente
Meia-Idade
Proteína EWS de Ligação a RNA/análise
Proteínas S100/análise
Tomografia Computadorizada por Raios X
Vimentina/análise
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Calmodulin-Binding Proteins); 0 (EWSR1 protein, human); 0 (RNA-Binding Protein EWS); 0 (S100 Proteins); 0 (Vimentin)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180306
[Lr] Data última revisão:
180306
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180213
[St] Status:MEDLINE
[do] DOI:10.3760/cma.j.issn.0529-5807.2018.02.006


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[PMID]:29384895
[Au] Autor:Yang MX; Zhao ZH; Yang JF; Chen B; Shen XZ; Wei JG; Wang BY
[Ad] Endereço:Department of Radiology.
[Ti] Título:Imaging findings of gastric plexiform fibromyxoma with a cystic change: A case report and review of literature.
[So] Source:Medicine (Baltimore);96(52):e8967, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Plexiform fibromyxoma (PF) is an extremely rare mesenchymal tumor of the stomach, and its radiological findings have not been well described. Here, we analyzed the imaging features of a case of PF. To our knowledge, this is a rare reported case with a remarkable cystic change in the imaging literature. PATIENT CONCERNS: A previously healthy 50-year-old woman presented with a 1-day history of abdominal pain. Then, she underwent computed tomography (CT) and magnetic resonance imaging (MRI). A cystic-solid well-circumscribed extraluminal mass was located in the posterior wall of the gastric upper body. The solid portion appeared as heterogeneous attenuation/intensity with progressive enhancement while the cystic region had no enhancement. DIAGNOSES: The potential for malignancy could not be excluded. INTERVENTIONS: Laparoscopic partial gastric resection was performed. OUTCOMES: Based on pathological findings, a diagnosis of PF was made. The patient was alive without any recurrence or metastasis of the tumor after 2 years of follow-up. LESSONS: As far as we know, a gastric PF with a remarkable cystic change has never been reported. Additionally, the tumor exhibited a progressive enhancement pattern which is a characteristic radiographic feature in our case. Our report may help increase the awareness of this rare but important new disease entity.
[Mh] Termos MeSH primário: Fibroma/diagnóstico por imagem
Neoplasias Gástricas/diagnóstico por imagem
[Mh] Termos MeSH secundário: Feminino
Fibroma/patologia
Fibroma/cirurgia
Gastroscopia
Seres Humanos
Imagem por Ressonância Magnética
Meia-Idade
Neoplasias Gástricas/patologia
Neoplasias Gástricas/cirurgia
Tomografia Computadorizada por Raios X
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180209
[Lr] Data última revisão:
180209
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180201
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008967


  4 / 9266 MEDLINE  
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[PMID]:29245307
[Au] Autor:Shen S; Chen M; Jug R; Yu CQ; Zhang WL; Yang LH; Wang L; Yu JH; Lin XY; Xu HT; Ma S
[Ad] Endereço:aDepartment of Neurology, Shengjing Hospital of China Medical University, Shenyang, Liao Ning, ChinabDepartment of Pathology, Duke University Medical Center, Durham, NCcDepartment of Pathology, First Affiliated Hospital of China Medical University and College of Basic Medical Sciences, Shenyang, Liao Ning, China.
[Ti] Título:Radiological presentation of chondromyxoid fibroma in the sellar region: A CARE-compliant article and literature review.
[So] Source:Medicine (Baltimore);96(49):e9049, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Chondromyxoid fibroma (CMF) is a rare benign bone neoplasm which often occurs in the lower extremities. Little is known about the radiological and histological presentation of CMF in the sellar region. PATIENT CONCERNS: A 16-year-old Asian male presented to the hospital 12 months ago with bilateral diplopia involving right visual fields, intermittent headaches, and dizziness. INTERVENTIONS: After the patient underwent enough examinations, the lesion was surgically removed by curettage. DIAGNOSIS: Postoperatively, the lesion was pathologically confirmed to be CMF. OUTCOMES: There was no recurrence at the 12-month follow-up. LESSONS: To the best of our knowledge, this is the second reported case of CMF in the sellar region which was clinically suspected to be a pituitary macroadenoma, craniopharyngioma, or schwannoma due to its location and radiographic features. We reviewed the morbidity, symptoms, radiographic features, pathological findings, and differential diagnosis of CMF. Because of its rarity, attention should be paid to avoid misdiagnosis of this lesion.
[Mh] Termos MeSH primário: Fibroma/diagnóstico por imagem
Radiografia/métodos
Sela Túrcica/diagnóstico por imagem
Neoplasias Cranianas/diagnóstico por imagem
[Mh] Termos MeSH secundário: Adolescente
Seres Humanos
Masculino
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171226
[Lr] Data última revisão:
171226
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171217
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009049


  5 / 9266 MEDLINE  
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[PMID]:28457065
[Au] Autor:Duek I; Bishara T; Gil Z; Cohen JT
[Ad] Endereço:Head and Neck Center, Department of Otolaryngology Head and Neck Surgery.
[Ti] Título:Transoral Robotic Appraoch for Resection of a Giant Hypopharyngeal Fibroma.
[So] Source:Isr Med Assoc J;19(2):121-122, 2017 Feb.
[Is] ISSN:1565-1088
[Cp] País de publicação:Israel
[La] Idioma:eng
[Mh] Termos MeSH primário: Fibroma
Neoplasias Hipofaríngeas
Cirurgia Endoscópica por Orifício Natural/métodos
Procedimentos Cirúrgicos Robóticos/métodos
[Mh] Termos MeSH secundário: Fibroma/patologia
Fibroma/fisiopatologia
Fibroma/cirurgia
Seres Humanos
Neoplasias Hipofaríngeas/patologia
Neoplasias Hipofaríngeas/fisiopatologia
Neoplasias Hipofaríngeas/cirurgia
Imagem por Ressonância Magnética/métodos
Masculino
Meia-Idade
Tomografia Computadorizada por Raios X/métodos
Resultado do Tratamento
Carga Tumoral
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171130
[Lr] Data última revisão:
171130
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170501
[St] Status:MEDLINE


  6 / 9266 MEDLINE  
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[PMID]:28821190
[Au] Autor:Kuba MG; Lester SC; Giess CS; Bertagnolli MM; Wieczorek TJ; Brock JE
[Ad] Endereço:Department of Pathology.
[Ti] Título:Fibromatosis of the Breast: Diagnostic Accuracy of Core Needle Biopsy.
[So] Source:Am J Clin Pathol;148(3):243-250, 2017 Sep 01.
[Is] ISSN:1943-7722
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Objectives: Fibromatosis of the breast is an uncommon neoplasm with potential for local recurrence. Treatment has traditionally been surgical excision with current trends toward conservative management. Given the option of observation after diagnosis by core needle biopsy (CNB), we sought to evaluate the accuracy of CNB for diagnosing fibromatosis. Methods: We identified a total of 31 cases in which fibromatosis had been diagnosed or included in the differential diagnosis on a CNB, an excision, or both. Morphology and immunohistochemical results were reviewed. Results: Aberrant nuclear immunoreactivity for ß-catenin and absent staining for CD34 were the most useful studies to diagnose fibromatosis, and one or both were performed in 21 (68%) cases. High molecular weight cytokeratins and p63 were helpful to exclude spindle cell carcinoma. Of 26 cases confirmed as fibromatosis on excision, 22 (85%) were diagnosed as fibromatosis or fibromatosis was favored in the differential diagnosis on CNB. More frequent use of immunohistochemistry would likely have resulted in a greater number of definitive diagnoses. Fibromatosis was rarely mistaken for other nonmalignant stromal lesions, with no cases misdiagnosed as carcinoma. Conclusions: CNB can be an accurate method of diagnosing fibromatosis, allowing observation for a select group of patients.
[Mh] Termos MeSH primário: Neoplasias da Mama/diagnóstico
Fibroma/diagnóstico
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Biópsia com Agulha de Grande Calibre
Neoplasias da Mama/metabolismo
Neoplasias da Mama/patologia
Diagnóstico Diferencial
Feminino
Fibroma/metabolismo
Fibroma/patologia
Seres Humanos
Imuno-Histoquímica
Queratinas/metabolismo
Meia-Idade
Sensibilidade e Especificidade
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
68238-35-7 (Keratins)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170906
[Lr] Data última revisão:
170906
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170820
[St] Status:MEDLINE
[do] DOI:10.1093/ajcp/aqx065


  7 / 9266 MEDLINE  
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[PMID]:28774260
[Au] Autor:Guarnieri V; Seaberg RM; Kelly C; Jean Davidson M; Raphael S; Shuen AY; Baorda F; Palumbo O; Scillitani A; Hendy GN; Cole DEC
[Ad] Endereço:Medical Genetics, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy. v.guarnieri@operapadrepio.it.
[Ti] Título:Large intragenic deletion of CDC73 (exons 4-10) in a three-generation hyperparathyroidism-jaw tumor (HPT-JT) syndrome family.
[So] Source:BMC Med Genet;18(1):83, 2017 Aug 03.
[Is] ISSN:1471-2350
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Inactivating mutations of CDC73 cause Hyperparathyroidism-Jaw Tumour syndrome (HPT-JT), Familial Isolated Hyperparathyroidism (FIHP) and sporadic parathyroid carcinoma. We conducted CDC73 mutation analysis in an HPT-JT family and confirm carrier status of the proband's daughter. METHODS: The proband had primary hyperparathyroidism (parathyroid carcinoma) and uterine leiomyomata. Her father and daughter had hyperparathyroidism (parathyroid adenoma) but no other manifestations of HPT-JT. CDC73 mutation analysis (sequencing of all 17 exons) and whole-genome copy number variation (CNV) analysis was done on leukocyte DNA of the three affecteds as well as the proband's unaffected sister. RESULTS: A novel deletion of exons 4 to 10 of CDC73 was detected by CNV analysis in the three affecteds. A novel insertion in the 5'UTR (c.-4_-11insG) that co-segregated with the deletion was identified. By in vitro assay the 5'UTR insertion was shown to significantly impair the expression of the parafibromin protein. Screening for the mutated CDC73 confirmed carrier status in the proband's daughter and the biochemistry and ultrasonography led to pre-emptive surgery and resolution of the hyperparathyroidism. CONCLUSIONS: A novel gross deletion mutation in CDC73 was identified in a three-generation HPT-JT family emphasizing the importance of including screening for large deletions in the molecular diagnostic protocol.
[Mh] Termos MeSH primário: Adenoma/genética
Fibroma/genética
Hiperparatireoidismo/genética
Neoplasias Maxilomandibulares/genética
Deleção de Sequência
Proteínas Supressoras de Tumor/genética
[Mh] Termos MeSH secundário: Regiões 5' não Traduzidas
Adenoma/patologia
Adolescente
Adulto
Alelos
Animais
Sequência de Bases
Criança
DNA/química
DNA/isolamento & purificação
DNA/metabolismo
Variações do Número de Cópias de DNA
Éxons
Feminino
Fibroma/patologia
Testes Genéticos
Células HEK293
Seres Humanos
Hiperparatireoidismo/patologia
Neoplasias Maxilomandibulares/patologia
Leucócitos/metabolismo
Masculino
Meia-Idade
Linhagem
Alinhamento de Sequência
Proteínas Supressoras de Tumor/metabolismo
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (5' Untranslated Regions); 0 (CDC73 protein, human); 0 (Tumor Suppressor Proteins); 9007-49-2 (DNA)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170917
[Lr] Data última revisão:
170917
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170805
[St] Status:MEDLINE
[do] DOI:10.1186/s12881-017-0445-0


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[PMID]:28751104
[Au] Autor:Krings G; McIntire P; Shin SJ
[Ad] Endereço:Department of Pathology, University of California San Francisco (UCSF), San Francisco, CA, USA. Electronic address: gregor.krings@ucsf.edu.
[Ti] Título:Myofibroblastic, fibroblastic and myoid lesions of the breast.
[So] Source:Semin Diagn Pathol;34(5):427-437, 2017 Sep.
[Is] ISSN:0740-2570
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Myofibroblastic, fibroblastic and/or myoid lesions are rare in the breast but comprise the majority of mammary mesenchymal spindle cell lesions. Whereas most have similar features to their counterparts at extramammary sites, pseudoangiomatous stromal hyperplasia is considered a breast-specific myofibroblastic proliferation on the same spectrum as myofibroblastoma. Other lesions with myofibroblastic/fibroblastic differentiation include fibromatosis and nodular fasciitis, as well as more aggressive tumors such as the rarely reported myofibrosarcoma, inflammatory myofibroblastic tumor and fibrosarcoma. Lesions with myoid differentiation include benign leiomyoma, myoid hamartoma and leiomyomatous myofibroblastoma, but primary leiomyosarcoma and rhabdomyosarcoma may also rarely arise in the breast. Furthermore, fibroepithelial lesions and metaplastic carcinomas can demonstrate myoid metaplasia. Diagnosis can be challenging, particularly on core biopsy, but benign lesions with or without recurrence potential must be distinguished from more aggressive tumors, especially metaplastic carcinoma and phyllodes tumors. This article will review lesions with myofibroblastic, fibroblastic and myoid differentiation in the breast, with special emphasis on differential diagnosis.
[Mh] Termos MeSH primário: Neoplasias da Mama/patologia
Fibroblastos/patologia
Fibroma/patologia
Miofibroblastos/patologia
Neoplasias de Tecido Muscular/patologia
[Mh] Termos MeSH secundário: Biomarcadores Tumorais/análise
Biópsia
Neoplasias da Mama/química
Diferenciação Celular
Diagnóstico Diferencial
Feminino
Fibroblastos/química
Fibroma/química
Seres Humanos
Imuno-Histoquímica
Miofibroblastos/química
Neoplasias de Tecido Muscular/química
Valor Preditivo dos Testes
Prognóstico
Sarcoma/química
Sarcoma/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Biomarkers, Tumor)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171016
[Lr] Data última revisão:
171016
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170729
[St] Status:MEDLINE


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[PMID]:28606161
[Au] Autor:Mei F; Zhou B; Cui Y
[Ad] Endereço:Department of Cardiothoracic Surgery, Zhejiang Provincial People's Hospital, No. 158 Shangtang Road, Hangzhou, Zhejiang, 310014, People's Republic of China.
[Ti] Título:Right ventricular outflow tract obstruction caused by right ventricular fibroma in a 5-month-old infant: a case report.
[So] Source:J Cardiothorac Surg;12(1):49, 2017 Jun 12.
[Is] ISSN:1749-8090
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Cardiac fibroma is rarely encountered in children, and even more rare in neonates. We herein report a case of a 5-month-old female with severe right ventricular outflow tract obstruction caused by a large right ventricle fibroma that was successfully surgically resected. CASE PRESENTATION: This report describes the case of a 5-month-old female infant with a large mass measuring 26 × 22 mm in the right ventricle cured successfully with surgery. Physical examination revealed a harsh S1 sound and a grade IV systolic murmur on the left sternal border. Surgical resection was indicated due to severe right ventricular outflow tract obstruction and further follow-up evaluation was uneventful. CONCLUSION: The surgical procedure to excise such a large cardiac fibroma in a 5-month-old infant is feasible and safe.
[Mh] Termos MeSH primário: Procedimentos Cirúrgicos Cardíacos/métodos
Fibroma/complicações
Neoplasias Cardíacas/complicações
Obstrução do Fluxo Ventricular Externo/etiologia
[Mh] Termos MeSH secundário: Ecocardiografia Transesofagiana
Feminino
Fibroma/diagnóstico
Fibroma/cirurgia
Neoplasias Cardíacas/diagnóstico
Neoplasias Cardíacas/cirurgia
Ventrículos do Coração
Seres Humanos
Lactente
Imagem Cinética por Ressonância Magnética
Obstrução do Fluxo Ventricular Externo/diagnóstico
Obstrução do Fluxo Ventricular Externo/cirurgia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171102
[Lr] Data última revisão:
171102
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170614
[St] Status:MEDLINE
[do] DOI:10.1186/s13019-017-0612-6


  10 / 9266 MEDLINE  
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[PMID]:28578783
[Au] Autor:Skubitz KM
[Ad] Endereço:Department of Medicine, University of Minnesota Medical School, Minneapolis. Electronic address: skubi001@umn.edu.
[Ti] Título:Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor.
[So] Source:Mayo Clin Proc;92(6):947-964, 2017 Jun.
[Is] ISSN:1942-5546
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Aggressive fibromatosis, also known as desmoid-type fibromatosis (DTF) or desmoid tumor, is an uncommon locally invasive tumor. Because of its low incidence and variable behavior, DTF is often first seen by physicians who are not familiar with it, and recent advances in understanding this disease have led to changes in treatment approaches. The Wnt (ß-catenin) pathway appears to play a key role in DTF pathogenesis, and recent studies of DTF biology suggest a possible model of DTF pathogenesis. Histologically, DTF shows a poorly circumscribed proliferation of myofibroblast-like cells with variable collagen deposition, similar to the proliferative phase of wound healing, and DTF has been associated with trauma and pregnancy. Desmoid-type fibromatosis may be a useful model of the tumor stroma in carcinomas as well as other fibrosing diseases such as progressive pulmonary fibrosis. The clinical course of DTF can vary greatly among patients, complicating the determination of the optimal treatment approach. Treatment options include surgery, nonsteroidal anti-inflammatory drugs with or without hormonal manipulation, chemotherapy, radiation therapy, and other forms of local therapy. Many treatments have been used, but these are not without toxicities. Because of the variable nature of the disease and the potential morbidity of treatment, some cases of DTF may do better without treatment; simple observation is often the best initial treatment. This review used a PubMed search from January 1, 1980, through October 31, 2016, using the terms fibromatosis and desmoid and discusses DTF disease characteristics, pathophysiology, and treatment options as well as examines several cases illustrating key points in the biology and treatment of this heterogeneous disease.
[Mh] Termos MeSH primário: Fibroma/fisiopatologia
Mutação
beta Catenina/genética
[Mh] Termos MeSH secundário: Fibroma/genética
Fibroma/patologia
Fibroma/cirurgia
Seres Humanos
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (beta Catenin)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170724
[Lr] Data última revisão:
170724
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170606
[St] Status:MEDLINE



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BIREME/OPAS/OMS - Centro Latino-Americano e do Caribe de Informação em Ciências da Saúde