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

• Ano de defesa: 2019
• Autor(a) principal: Thompson, Ruth Pamela Mauger
• Tipo de documento: Dissertação
• Tipo de acesso: Acesso aberto
• Idioma: por
• Instituição de defesa: Universidade Federal de Santa Maria; Brasil; Medicina Veterinária; UFSM; Programa de Pós-Graduação em Medicina Veterinária; Centro de Ciências Rurais
• Palavras-chave em Português: 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
• Link de acesso: http://repositorio.ufsm.br/handle/1/16520

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.