Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão

Detalhes bibliográficos
Ano de defesa: 2019
Autor(a) principal: Thompson, Ruth Pamela Mauger lattes
Orientador(a): Kommers, Glaucia Denise lattes
Banca de defesa: Fernandes, Cristina Gevehr lattes, Basso, Paula Cristina lattes
Tipo de documento: Dissertação
Tipo de acesso: Acesso aberto
Idioma: por
Instituição de defesa: Universidade Federal de Santa Maria
Centro de Ciências Rurais
Programa de Pós-Graduação: Programa de Pós-Graduação em Medicina Veterinária
Departamento: Medicina Veterinária
País: Brasil
Palavras-chave em Português:
Palavras-chave em Inglês:
Área do conhecimento CNPq:
Link de acesso: http://repositorio.ufsm.br/handle/1/16520
Resumo: Renal cystadenocarcinoma -nodular dermatofibrosis (CR-DN syndrome) is rare and mainly affects dogs of the German Shepherd breed. This disease is of autosomal dominant inheritance, caused by a mutation of the folliculin gene (FLCN), and located on chromosome 5 in dogs. The syndrome is characterized by bilateral cystadenomas and cistadenocarcinomas, multiple nodules in the skin and subcutaneous tissue distributed throughout the body and uterine leiomyomas. This study describes 11 cases of CR-DN syndrome in German Shepherd dogs, diagnosed between January 1994 and January 2018 at the Laboratory of Veterinary Pathology of the Federal University of Santa Maria (LPV-UFSM). The affected dogs were eight males and three females, establishing a ratio of 2.67: 1. The age ranged from six to 12 years, with the mean age being 8.7 years. The main clinical signs reported were, in descending order of frequency, multiple skin nodules (nodular dermatofibrosis), dyspnea, anorexia, weight loss, recurrent hematuria, vomiting and polydipsia. This study allowed establishing that the clinical recognition of the syndrome is not always easy, but its peculiar anatomical-pathological characteristics allow a safe diagnosis. Histologically, it was possible to detect all phases (cysts, papillary intratubular hyperplasia, cystadenomas and / or cystadenocarcinomas) of a possible pathological continuum of the renal lesions. Uterine leiomyomas were observed in only one case. Through histochemical techniques, it was possible to establish that type I collagen is present in both cutaneous and renal lesions and to consider its possible involvement in the pathogenesis of renal cystadenocarcinomas. The IHC technique showed partially satisfactory results in the immunostaining of cysts epithelial cells and renal neoplasms for pancytoceratin.
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spelling 2019-05-10T14:27:16Z2019-05-10T14:27:16Z2019-02-27http://repositorio.ufsm.br/handle/1/16520Renal cystadenocarcinoma -nodular dermatofibrosis (CR-DN syndrome) is rare and mainly affects dogs of the German Shepherd breed. This disease is of autosomal dominant inheritance, caused by a mutation of the folliculin gene (FLCN), and located on chromosome 5 in dogs. The syndrome is characterized by bilateral cystadenomas and cistadenocarcinomas, multiple nodules in the skin and subcutaneous tissue distributed throughout the body and uterine leiomyomas. This study describes 11 cases of CR-DN syndrome in German Shepherd dogs, diagnosed between January 1994 and January 2018 at the Laboratory of Veterinary Pathology of the Federal University of Santa Maria (LPV-UFSM). The affected dogs were eight males and three females, establishing a ratio of 2.67: 1. The age ranged from six to 12 years, with the mean age being 8.7 years. The main clinical signs reported were, in descending order of frequency, multiple skin nodules (nodular dermatofibrosis), dyspnea, anorexia, weight loss, recurrent hematuria, vomiting and polydipsia. This study allowed establishing that the clinical recognition of the syndrome is not always easy, but its peculiar anatomical-pathological characteristics allow a safe diagnosis. Histologically, it was possible to detect all phases (cysts, papillary intratubular hyperplasia, cystadenomas and / or cystadenocarcinomas) of a possible pathological continuum of the renal lesions. Uterine leiomyomas were observed in only one case. Through histochemical techniques, it was possible to establish that type I collagen is present in both cutaneous and renal lesions and to consider its possible involvement in the pathogenesis of renal cystadenocarcinomas. The IHC technique showed partially satisfactory results in the immunostaining of cysts epithelial cells and renal neoplasms for pancytoceratin.A síndrome cistadenocarcinoma renal-dermatofibrose nodular (CR-DN) é rara e afeta principalmente cães da raça Pastor Alemão. Esta doença é de herança autossômica dominante, causada por mutação do gene foliculina (FLCN), localizado no cromossomo 5 em cães. A síndrome caracteriza-se por apresentar cistadenomas e cistadenocarcinomas renais bilaterais, múltiplos nódulos na pele e tecido subcutâneo distribuídos em todo o corpo e leiomiomas uterinos. Neste estudo são descritos 11 casos da síndrome CR-DN em cães Pastor Alemão, diagnosticados entre janeiro de 1994 e janeiro de 2018 no Laboratório de Patologia Veterinária da Universidade Federal de Santa Maria (LPV-UFSM). Os cães afetados foram oito machos e três fêmeas, estabelecendo-se uma relação de 2,67:1. A idade variou de seis a 12 anos, sendo a média de idade de 8,7 anos. Os principais sinais clínicos relatados foram, em ordem decrescente de frequência, múltiplos nódulos cutâneos (dermatofibrose nodular), dispneia, anorexia, emagrecimento, hematúria recorrente, vômito e polidipsia. Este estudo permitiu estabelecer que o reconhecimento clínico da síndrome nem sempre é fácil, porém suas características anátomo-patológicas peculiares permitem um diagnóstico com segurança. Histologicamente, foi possível detectar todas as fases (cistos, hiperplasia intratubular papilífera, cistadenomas e/ou cistadenocarcinomas) de um possível continuum patológico das lesões renais. Leiomiomas uterinos foram observados somente em um caso. Através das técnicas histoquímicas foi possível estabelecer que o colágeno tipo I está presente em ambas as lesões, cutâneas e renais, e cogitar seu possível envolvimento na patogênese dos cistadenocarcinomas renais. A técnica de IHQ mostrou resultados parcialmente satisfatórios na imunomarcação das células epiteliais dos cistos e dos neoplasmas renais para pancitoceratina.Conselho Nacional de Pesquisa e Desenvolvimento Científico e Tecnológico - CNPqporUniversidade Federal de Santa MariaCentro de Ciências RuraisPrograma de Pós-Graduação em Medicina VeterináriaUFSMBrasilMedicina VeterináriaAttribution-NonCommercial-NoDerivatives 4.0 Internationalhttp://creativecommons.org/licenses/by-nc-nd/4.0/info:eu-repo/semantics/openAccessCistadenomaCistadenocarcinomaDermatofibrose nodularSíndrome hereditáriaDoenças de cãesRenal cystadenomaRenal cystadenocarcinomaNodular dermatofibrosisHereditary syndromeDiseases of dogsCNPQ::CIENCIAS AGRARIAS::MEDICINA VETERINARIACaracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemãoClinical-epidemiological and pathological characterization of the renal cystadenocarcinoma-nodular dermatofibrosis syndrome in german shepherd dogsinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/masterThesisKommers, Glaucia Denisehttp://lattes.cnpq.br/5818649889964582Fernandes, Cristina Gevehrhttp://lattes.cnpq.br/4498627593892325Basso, Paula Cristinahttp://lattes.cnpq.br/2412593761057734http://lattes.cnpq.br/1198017788313667Thompson, Ruth Pamela Mauger500500000007600432feb45-3af7-4769-84f8-44f81f48e8a71cfbe73d-761f-4970-989b-3b9485976b91aca2cb18-2a7d-4a40-9c51-32e9e00d9099914df3ad-ba8b-4c9a-8be4-0a86ab71bb38reponame:Manancial - Repositório Digital da UFSMinstname:Universidade Federal de Santa Maria (UFSM)instacron:UFSMORIGINALDIS_PPGMV_2019_THOMPSON_RUTH.pdfDIS_PPGMV_2019_THOMPSON_RUTH.pdfDissertação de Mestradoapplication/pdf1821362http://repositorio.ufsm.br/bitstream/1/16520/1/DIS_PPGMV_2019_THOMPSON_RUTH.pdf4b2fc14fcc4f8c75b85c6736374c9435MD51CC-LICENSElicense_rdflicense_rdfapplication/rdf+xml; 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dc.title.por.fl_str_mv Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão
dc.title.alternative.eng.fl_str_mv Clinical-epidemiological and pathological characterization of the renal cystadenocarcinoma-nodular dermatofibrosis syndrome in german shepherd dogs
title Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão
spellingShingle Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão
Thompson, Ruth Pamela Mauger
Cistadenoma
Cistadenocarcinoma
Dermatofibrose nodular
Síndrome hereditária
Doenças de cães
Renal cystadenoma
Renal cystadenocarcinoma
Nodular dermatofibrosis
Hereditary syndrome
Diseases of dogs
CNPQ::CIENCIAS AGRARIAS::MEDICINA VETERINARIA
title_short Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão
title_full Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão
title_fullStr Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão
title_full_unstemmed Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão
title_sort Caracterização clínico-epidemiológica e patológica da síndrome cistadenocarcinoma renal-dermatofibrose nodular em cães pastor alemão
author Thompson, Ruth Pamela Mauger
author_facet Thompson, Ruth Pamela Mauger
author_role author
dc.contributor.advisor1.fl_str_mv Kommers, Glaucia Denise
dc.contributor.advisor1Lattes.fl_str_mv http://lattes.cnpq.br/5818649889964582
dc.contributor.referee1.fl_str_mv Fernandes, Cristina Gevehr
dc.contributor.referee1Lattes.fl_str_mv http://lattes.cnpq.br/4498627593892325
dc.contributor.referee2.fl_str_mv Basso, Paula Cristina
dc.contributor.referee2Lattes.fl_str_mv http://lattes.cnpq.br/2412593761057734
dc.contributor.authorLattes.fl_str_mv http://lattes.cnpq.br/1198017788313667
dc.contributor.author.fl_str_mv Thompson, Ruth Pamela Mauger
contributor_str_mv Kommers, Glaucia Denise
Fernandes, Cristina Gevehr
Basso, Paula Cristina
dc.subject.por.fl_str_mv Cistadenoma
Cistadenocarcinoma
Dermatofibrose nodular
Síndrome hereditária
Doenças de cães
topic Cistadenoma
Cistadenocarcinoma
Dermatofibrose nodular
Síndrome hereditária
Doenças de cães
Renal cystadenoma
Renal cystadenocarcinoma
Nodular dermatofibrosis
Hereditary syndrome
Diseases of dogs
CNPQ::CIENCIAS AGRARIAS::MEDICINA VETERINARIA
dc.subject.eng.fl_str_mv Renal cystadenoma
Renal cystadenocarcinoma
Nodular dermatofibrosis
Hereditary syndrome
Diseases of dogs
dc.subject.cnpq.fl_str_mv CNPQ::CIENCIAS AGRARIAS::MEDICINA VETERINARIA
description Renal cystadenocarcinoma -nodular dermatofibrosis (CR-DN syndrome) is rare and mainly affects dogs of the German Shepherd breed. This disease is of autosomal dominant inheritance, caused by a mutation of the folliculin gene (FLCN), and located on chromosome 5 in dogs. The syndrome is characterized by bilateral cystadenomas and cistadenocarcinomas, multiple nodules in the skin and subcutaneous tissue distributed throughout the body and uterine leiomyomas. This study describes 11 cases of CR-DN syndrome in German Shepherd dogs, diagnosed between January 1994 and January 2018 at the Laboratory of Veterinary Pathology of the Federal University of Santa Maria (LPV-UFSM). The affected dogs were eight males and three females, establishing a ratio of 2.67: 1. The age ranged from six to 12 years, with the mean age being 8.7 years. The main clinical signs reported were, in descending order of frequency, multiple skin nodules (nodular dermatofibrosis), dyspnea, anorexia, weight loss, recurrent hematuria, vomiting and polydipsia. This study allowed establishing that the clinical recognition of the syndrome is not always easy, but its peculiar anatomical-pathological characteristics allow a safe diagnosis. Histologically, it was possible to detect all phases (cysts, papillary intratubular hyperplasia, cystadenomas and / or cystadenocarcinomas) of a possible pathological continuum of the renal lesions. Uterine leiomyomas were observed in only one case. Through histochemical techniques, it was possible to establish that type I collagen is present in both cutaneous and renal lesions and to consider its possible involvement in the pathogenesis of renal cystadenocarcinomas. The IHC technique showed partially satisfactory results in the immunostaining of cysts epithelial cells and renal neoplasms for pancytoceratin.
publishDate 2019
dc.date.accessioned.fl_str_mv 2019-05-10T14:27:16Z
dc.date.available.fl_str_mv 2019-05-10T14:27:16Z
dc.date.issued.fl_str_mv 2019-02-27
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dc.publisher.none.fl_str_mv Universidade Federal de Santa Maria
Centro de Ciências Rurais
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dc.publisher.initials.fl_str_mv UFSM
dc.publisher.country.fl_str_mv Brasil
dc.publisher.department.fl_str_mv Medicina Veterinária
publisher.none.fl_str_mv Universidade Federal de Santa Maria
Centro de Ciências Rurais
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