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[PMID]:27538186
[Au] Autor:Hammer J; Léonard D; Chateau F; Abbes Orabi N; Ciccarelli O; Bachmann R; Remue C; Lengelé B; Kartheuser A
[Ad] Endereço:a Colorectal Surgery Unit, Department of Abdominal Surgery and Transplantation , Cliniques universitaires Saint-Luc , Brussels , Belgium.
[Ti] Título:Reconstruction of an abdominal wall defect with biologic mesh after resection of a desmoid tumor in a patient with a Gardner's syndrome.
[So] Source:Acta Chir Belg;117(1):55-60, 2017 Feb.
[Is] ISSN:0001-5458
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Desmoid tumors are rare proliferative and invasive benign lesions. They can be sporadic, but in most instances, desmoid tumors develop in the context of Gardner's syndrome with principal localization in the abdominal cavity and abdominal wall. CASE-REPORT: We report the case of a 24-year-old female presenting Gardner's syndrome with a symptomatic abdominal wall desmoid tumor. Lack of response to medical treatment led to surgical management consisting in a complete resection and parietal reconstruction with a biologic mesh. Postoperative course was uneventful and there was no evidence of recurrence at 12 months of follow-up. DISCUSSION: Conventional treatment of abdominal wall desmoid tumors consists in a wide and radical resection. However, complete resection is not always feasible because of difficulty to differentiate the desmoid tumor from adjacent tissues. The surgical approach may require different techniques to repair the parietal defect including prosthetic material such as synthetic or biologic meshes. Biological mesh is an ideal alternative to synthetic graft, mainly in case of infection. CONCLUSION: We have encountered a case of a symptomatic growing desmoid tumor of the abdominal wall in a young patient with Gardner's syndrome, successfully treated by complete resection and reconstruction with a biologic mesh to correct the parietal defect.
[Mh] Termos MeSH primário: Parede Abdominal/cirurgia
Fibromatose Abdominal/cirurgia
Síndrome de Gardner/complicações
Telas Cirúrgicas
[Mh] Termos MeSH secundário: Feminino
Fibromatose Abdominal/complicações
Fibromatose Abdominal/patologia
Síndrome de Gardner/cirurgia
Seres Humanos
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170320
[Lr] Data última revisão:
170320
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160819
[St] Status:MEDLINE
[do] DOI:10.1080/00015458.2016.1212499


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[PMID]:27391496
[Au] Autor:Oliveira MR; Rodrigues WC; Gabrielli MF; Gabrielli MA; Onofre MA; Filho VA
[Ad] Endereço:Department of Diagnosis and Surgery, Araraquara Dental School, São Paulo State University (UNESP), Araraquara, SP, Brazil.
[Ti] Título:Gardner Syndrome With Unusual Maxillofacial Manifestation.
[So] Source:J Craniofac Surg;27(5):1253-5, 2016 Jul.
[Is] ISSN:1536-3732
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Gardner syndrome is a rare autosomal-dominant condition characterized by the presence of intestinal polyposis, multiple osteomas, and tumors of the hard and soft tissues. This paper describes a patient of Gardner syndrome with unusual maxillofacial manifestation with presence of fibromyxomatous injury in jaw, coronoid hyperplasia, and multiple osteomas diffusely distributed in the craniomaxillofacial skeleton. Imaging examinations have identified craniofacial manifestations and the patient was referred to the gastroenterologist who confirmed the diagnosis of Gardner syndrome. The early diagnosis of this syndrome is important since intestinal polyps have high potential for malignant transformation. It is therefore essential that dentists are familiar with the maxillofacial features of this condition, since they precede the intestinal polyposis and encourage early diagnosis. In addition to classic maxillofacial signs of this syndrome, one must consider that, although it is rare, other injuries may be present such as those described in this clinical patient.
[Mh] Termos MeSH primário: Diagnóstico Precoce
Fibroma/diagnóstico
Síndrome de Gardner/diagnóstico
Neoplasias Mandibulares/diagnóstico
[Mh] Termos MeSH secundário: Biópsia
Criança
Fibroma/cirurgia
Seres Humanos
Masculino
Neoplasias Mandibulares/cirurgia
Osteotomia/métodos
Radiografia Panorâmica
Tomografia Computadorizada por Raios X
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:D
[Da] Data de entrada para processamento:160709
[St] Status:MEDLINE
[do] DOI:10.1097/SCS.0000000000002741


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[PMID]:27377916
[Au] Autor:Li W; Zhou Y; Li Q; Tong H; Lu W
[Ad] Endereço:Department of Oncology, Zhongshan Hospital, Fudan University, Shanghai, China.
[Ti] Título:Intestinal perforation during chemotherapeutic treatment of intra-abdominal desmoid tumor in patients with Gardner's syndrome: report of two cases.
[So] Source:World J Surg Oncol;14(1):178, 2016 Jul 04.
[Is] ISSN:1477-7819
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: A minority of intra-abdominal desmoid tumors is associated with Gardner's syndrome in which desmoid tumors become an important cause of morbidity and mortality if they are surgically unresectable. CASE PRESENTATION: Here, we report two cases of intestinal perforation during chemotherapy in patients with Gardner's syndrome-associated intra-abdominal desmoids. One female and one male patients who developed inoperable desmoids were given the chemotherapeutic regimen of doxorubicin plus dacarbazine, followed by meloxicam. Significant tumor regression was observed clinically. However, intestinal perforation happened in both patients. They were subjected to emergency surgery, follow-up management, and survived up to now. CONCLUSIONS: The doxorubicin plus dacarbazine chemotherapy is an effective treatment for intra-abdominal demoid tumors in patients with Gardner's syndrome. On the other hand, given severe adverse events might occur, clinicians should pay more attention that tumor quick regression may cause intestinal perforation in which urgent surgical intervention is necessary.
[Mh] Termos MeSH primário: Neoplasias Abdominais/tratamento farmacológico
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos
Fibromatose Agressiva/tratamento farmacológico
Síndrome de Gardner/complicações
Perfuração Intestinal/induzido quimicamente
[Mh] Termos MeSH secundário: Neoplasias Abdominais/etiologia
Adolescente
Adulto
Antineoplásicos/efeitos adversos
Antineoplásicos/uso terapêutico
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
Dacarbazina/efeitos adversos
Dacarbazina/uso terapêutico
Doxorrubicina/efeitos adversos
Doxorrubicina/uso terapêutico
Feminino
Fibromatose Agressiva/etiologia
Seres Humanos
Perfuração Intestinal/diagnóstico
Masculino
Tiazinas/efeitos adversos
Tiazinas/uso terapêutico
Tiazóis/efeitos adversos
Tiazóis/uso terapêutico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antineoplastic Agents); 0 (Thiazines); 0 (Thiazoles); 7GR28W0FJI (Dacarbazine); 80168379AG (Doxorubicin); VG2QF83CGL (meloxicam)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170317
[Lr] Data última revisão:
170317
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160706
[St] Status:MEDLINE
[do] DOI:10.1186/s12957-016-0935-0


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[PMID]:27291484
[Au] Autor:Guignard N; Cartier C; Crampette L; Akkari M
[Ad] Endereço:Service d'ORL et chirurgie cervicofaciale, CHU de Montpellier, université de Montpellier, hôpital Gui-de-Chauliac, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 5, France.
[Ti] Título:Gardner's syndrome presenting with a fibromatous tumour of the parotid.
[So] Source:Eur Ann Otorhinolaryngol Head Neck Dis;133(5):357-359, 2016 Nov.
[Is] ISSN:1879-730X
[Cp] País de publicação:France
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Gardner's syndrome is the association of familial adenomatous polyposis (FAP) with an anaphase promoting complex (APC) gene mutation and several extradigestive manifestations: osteomas, epidermal cysts and desmoid tumours. Only 2 cases of FAP associated with parotid tumour have been reported in the literature: one carcinoma and one pleomorphic adenoma. CASE REPORT: We report the case of a 23-year-old man with Gardner's syndrome presenting with a fibromatous tumour of the left parotid gland. The other clinical manifestations were an osteoma of the skull base and a mesentery desmoid tumour. Left total parotidectomy was performed, followed by gastroscopy and colonoscopy, demonstrating numerous colonic adenomas. Genetic analysis revealed an APC gene mutation, confirming the diagnosis. Total prophylactic colectomy was performed. CONCLUSIONS: This is the first reported case of Gardner's syndrome associated with a fibromatous tumour of the parotid. Early management of FAP is essential to prevent colorectal cancer that occurs in 100% of untreated cases. The rarity of this syndrome and the parotid site can lead to delayed diagnosis.
[Mh] Termos MeSH primário: Fibroma/patologia
Síndrome de Gardner/diagnóstico
Neoplasias Parotídeas/patologia
[Mh] Termos MeSH secundário: Síndrome de Gardner/genética
Seres Humanos
Masculino
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170807
[Lr] Data última revisão:
170807
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160614
[St] Status:MEDLINE


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[PMID]:27178686
[Au] Autor:Schadt CR
[Ad] Endereço:University of Louisville Division of Dermatology, Louisville, KY. Electronic address: courtney.schadt@louisville.edu.
[Ti] Título:The cutaneous manifestations of gastrointestinal malignancy.
[So] Source:Semin Oncol;43(3):341-6, 2016 Jun.
[Is] ISSN:1532-8708
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The skin is often the herald of an underlying systemic illness, and gastrointestinal malignancies can present in numerous ways in the skin. Paraneoplastic phenomenon, such as acanthosis nigricans and tripe palm, may be the first indicator of a gastrointestinal malignancy. In addition, gastrointestinal cancers can metastasize to the skin, as described in the well-known Sister Mary Joseph's nodule. Inflammatory systemic conditions such as dermatomyositis and multicentric reticulohistiocytosis can be associated with underlying malignancy. Finally, in numerous genetic syndromes with underlying malignancies, such as Muir-Torre, recognition of the skin signs leads to early diagnosis and screening.
[Mh] Termos MeSH primário: Neoplasias Gastrointestinais/patologia
Síndromes Paraneoplásicas/etiologia
Neoplasias Cutâneas/patologia
[Mh] Termos MeSH secundário: Carcinoma Basocelular/etiologia
Carcinoma Basocelular/patologia
Dermatomiosite/etiologia
Dermatomiosite/patologia
Neoplasias Esofágicas/etiologia
Neoplasias Esofágicas/patologia
Síndrome de Gardner/etiologia
Síndrome de Gardner/patologia
Seres Humanos
Hipertricose/etiologia
Hipotricose/etiologia
Hipotricose/patologia
Ceratodermia Palmar e Plantar/etiologia
Ceratodermia Palmar e Plantar/patologia
Síndrome de Muir-Torre/etiologia
Síndrome de Muir-Torre/patologia
Síndromes Paraneoplásicas/patologia
Neoplasias Cutâneas/etiologia
Neoplasias Cutâneas/secundário
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170515
[Lr] Data última revisão:
170515
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160515
[St] Status:MEDLINE


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[PMID]:26995577
[Au] Autor:Pena AH; Chaudhry A; Seidman RJ; Peyster R; Bangiyev L
[Ad] Endereço:SUNY at Stony Brook, Stony Brook, NY. Electronic address: andreshernanp@gmail.com.
[Ti] Título:Ectopic craniopharyngioma of the fourth ventricle in a patient with Gardner syndrome.
[So] Source:Clin Imaging;40(2):232-6, 2016 Mar-Apr.
[Is] ISSN:1873-4499
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Ectopic craniopharyngioma is uncommon and a craniopharyngioma confined purely within the fourth ventricle is extremely rare. We report a craniopharyngioma of the fourth ventricle in a 20-year-old man with Gardner syndrome. Imaging characteristics of craniopharyngiomas and fourth ventricle lesions are discussed with a review of the literature regarding the pathogenesis of craniopharyngiomas and the possible association with Gardner syndrome.
[Mh] Termos MeSH primário: Craniofaringioma/diagnóstico
Quarto Ventrículo/patologia
Síndrome de Gardner/complicações
Neoplasias Hipofisárias/diagnóstico
[Mh] Termos MeSH secundário: Craniofaringioma/complicações
Diagnóstico Diferencial
Seres Humanos
Imagem por Ressonância Magnética
Masculino
Neoplasias Hipofisárias/complicações
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1611
[Cu] Atualização por classe:161230
[Lr] Data última revisão:
161230
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160321
[St] Status:MEDLINE


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[PMID]:26850131
[Au] Autor:Ponti G; Tomasi A; Manfredini M; Pellacani G
[Ad] Endereço:Department of Surgical, Medical, Dental and Morphological Sciences with Interest transplant, Oncological and Regenerative Medicine, University of Modena and Reggio Emilia, Italy.
[Ti] Título:Oral mucosal stigmata in hereditary-cancer syndromes: From germline mutations to distinctive clinical phenotypes and tailored therapies.
[So] Source:Gene;582(1):23-32, 2016 May 10.
[Is] ISSN:1879-0038
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:Numerous familial tumor syndromes are associated with distinctive oral mucosal findings, which may make possible an early diagnosis as an efficacious marker for the risk of developing visceral malignancies. In detail, Familial Adenomatous Polyposis (FAP), Gardner syndrome, Peutz-Jeghers syndrome, Cowden Syndrome, Gorlin Syndrome, Lynch/Muir-Torre Syndrome and Multiple Endocrine Neoplasia show specific lesions of the oral mucosa and other distinct clinical and molecular features. The common genetic background of the above mentioned syndromes involve germline mutations in tumor suppressor genes, such as APC, PTEN, PTCH1, STK11, RET, clearly implied in both ectodermal and mesodermal differentiation, being the oral mucosal and dental stigmata frequently associated in the specific clinical phenotypes. The oral and maxillofacial manifestations of these syndromes may become visible several years before the intestinal lesions, constituting a clinical marker that is predictive for the development of intestinal polyps and/or other visceral malignancies. A multidisciplinary approach is therefore necessary for both clinical diagnosis and management of the gene-carriers probands and their family members who have to be referred for genetic testing or have to be investigated for the presence of visceral cancers.
[Mh] Termos MeSH primário: Polipose Adenomatosa do Colo/genética
Genes Supressores de Tumor
Terapia de Alvo Molecular
Mucosa Bucal/patologia
[Mh] Termos MeSH secundário: Polipose Adenomatosa do Colo/complicações
Polipose Adenomatosa do Colo/patologia
Polipose Adenomatosa do Colo/terapia
Síndrome de Gardner/complicações
Síndrome de Gardner/genética
Síndrome de Gardner/patologia
Síndrome de Gardner/terapia
Mutação em Linhagem Germinativa/genética
Heterozigoto
Seres Humanos
Mucosa Bucal/metabolismo
Síndrome de Muir-Torre/complicações
Síndrome de Muir-Torre/genética
Síndrome de Muir-Torre/patologia
Síndrome de Muir-Torre/terapia
Neoplasia Endócrina Múltipla/complicações
Neoplasia Endócrina Múltipla/genética
Neoplasia Endócrina Múltipla/patologia
Neoplasia Endócrina Múltipla/terapia
Síndrome de Peutz-Jeghers/complicações
Síndrome de Peutz-Jeghers/genética
Síndrome de Peutz-Jeghers/patologia
Síndrome de Peutz-Jeghers/terapia
Fenótipo
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1608
[Cu] Atualização por classe:160304
[Lr] Data última revisão:
160304
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160207
[St] Status:MEDLINE


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[PMID]:26840078
[Au] Autor:Dahl NA; Sheil A; Knapke S; Geller JI
[Ad] Endereço:*Department of Pediatrics Divisions of †Pathology and Laboratory Medicine ‡Human Genetics §Cancer and Blood Disease Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH.
[Ti] Título:Gardner Fibroma: Clinical and Histopathologic Implications of Germline APC Mutation Association.
[So] Source:J Pediatr Hematol Oncol;38(5):e154-7, 2016 Jul.
[Is] ISSN:1536-3678
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The proportion and clinical characteristics of Gardner fibromas (GAFs) that are sporadic versus familial adenomatous polyposis (FAP)-associated have not been clearly established. We report on 7 patients diagnosed with GAF who underwent APC sequencing and duplication/deletion testing. Three (43%) were found to have underlying APC germline perturbations consistent with FAP; these patients had multifocal (1) or large; unresectable (2) GAFs. The 4 patients with negative APC testing each had a single resectable GAF. ß-catenin reactivity was noted in all FAP-associated GAFs and in 1/4 APC wild-type cases. FAP-associated GAFs may be less common than sporadic GAFs and can demonstrate clinically distinct features.
[Mh] Termos MeSH primário: Fibroma
Síndrome de Gardner
Genes APC
Mutação em Linhagem Germinativa
[Mh] Termos MeSH secundário: Polipose Adenomatosa do Colo/patologia
Pré-Escolar
Feminino
Seres Humanos
Lactente
Masculino
Estudos Retrospectivos
Análise de Sequência de DNA
beta Catenina/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (CTNNB1 protein, human); 0 (beta Catenin)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170907
[Lr] Data última revisão:
170907
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160204
[St] Status:MEDLINE
[do] DOI:10.1097/MPH.0000000000000493


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[PMID]:26049684
[Au] Autor:Artiles Armas M; Marchena Gómez J; Kanouzi Masuh G; Ramírez Felipe JA
[Ad] Endereço:Servicio de Cirugía General y Digestiva, Hospital Universitario de Gran Canaria Dr. Negrín, Las Palmas de Gran Canaria, España. Electronic address: martarmcgd@gmail.com.
[Ti] Título:Total gastroduodenectomy with pancreatic preservation for the treatment of Gardner's syndrome with gastroduodenal polyposis and malignant transformation.
[So] Source:Cir Esp;94(3):196-8, 2016 Mar.
[Is] ISSN:1578-147X
[Cp] País de publicação:Spain
[La] Idioma:eng; spa
[Mh] Termos MeSH primário: Síndrome de Gardner
[Mh] Termos MeSH secundário: Neoplasias Colorretais
Gastrectomia
Genes APC
Seres Humanos
Pólipos Intestinais/cirurgia
Intestinos
Mutação
Estômago
Odontopatias
[Pt] Tipo de publicação:JOURNAL ARTICLE
[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:150608
[St] Status:MEDLINE


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[PMID]:26793832
[Au] Autor:Vargo R; Bilodeau EA; Potluri A
[Ti] Título:Supernumerary Teeth: Case Comparison and Review of the Literature.
[So] Source:J Mich Dent Assoc;97(12):30-4, 2015 Dec.
[Is] ISSN:0026-2102
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Displasia Cleidocraniana/diagnóstico
Síndrome de Gardner/diagnóstico
Dente Supranumerário/diagnóstico
[Mh] Termos MeSH secundário: Adolescente
Feminino
Seres Humanos
Neoplasias Mandibulares/diagnóstico
Odontoma/diagnóstico
Osteoma/diagnóstico
Dente Impactado/diagnóstico
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; COMPARATIVE STUDY; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1602
[Cu] Atualização por classe:160122
[Lr] Data última revisão:
160122
[Sb] Subgrupo de revista:D
[Da] Data de entrada para processamento:160123
[St] Status:MEDLINE



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