Disease overview

Renal cystadenocarcinoma and nodular dermatofibrosis is a multi-organ inherited cancer caused by a mutation in the folliculin gene. This mutation creates dense collagen nodules in the skin, as well as tumors in the kidneys and uterus.

Gene name

FLCN

Inheritance

Autosomal dominant

Signs and symptoms

This is a late-onset disease, with firm nodules in the skin typically presenting by 6 years of age. Signs of kidney involvement include excessive thirst and urination. If the cancer spreads to the lungs, respiratory distress, such as difficulty breathing, may occur.

How is it diagnosed

A veterinarian will conduct a thorough exam, including a review of health history and any clinical signs. Genetic testing is necessary to determine if there is an underlying genetic cause, which can assist veterinarians with diagnosis and help breeders identify affected and carrier dogs.

Breeds at higher risk

Citations

Lingaas F, Comstock KE, Kirkness EF, Sørensen A, Aarskaug T, Hitte C, Nickerson ML, Moe L, Schmidt LS, Thomas R, Breen M, Galibert F, Zbar B, Ostrander EA. A mutation in the canine BHD gene is associated with hereditary multifocal renal cystadenocarcinoma and nodular dermatofibrosis in the German Shepherd dog. Hum Mol Genet. 2003 Dec 1;12(23):3043-53. doi: 10.1093/hmg/ddg336. Epub 2003 Oct 7. PMID: 14532326.

Lium B, Moe L. Hereditary multifocal renal cystadenocarcinomas and nodular dermatofibrosis in the German shepherd dog: macroscopic and histopathologic changes. Vet Pathol. 1985 Sep;22(5):447-55. doi: 10.1177/030098588502200503. PMID: 4049673.

Disclaimer

Disease diagnosis and treatment should always be performed by a veterinarian. The following information is for educational purposes only.