Several types of common hereditary kidney cancer

Kidney cancer has two forms of onset: hereditary and sporadic. Hereditary kidney cancer accounts for about 4% of all kidney cancers. Common hereditary kidney cancers include the following:

Von Hippel-Lindaudiase disease: It is an autosomal dominant genetic disease and a familial tumor syndrome involving multiple systemic lesions. The clinical features are hemangioblastomas of the cerebellum, retina, and spinal cord, renal cell carcinoma, pheochromocytoma, and renal cysts. The incidence of renal cancer in VHL disease is as high as 28% to 45%, and all pathological types are clear cell carcinoma. Clinically, VHL renal cancer is mostly bilateral multiple renal cancer, which progresses slowly and metastasizes late. It is generally believed that when the tumor is small, it can be observed clinically. When the tumor diameter is greater than 3 cm, tumor resection can be performed and the residual kidney should be preserved as much as possible.

Hereditary papillary renal carcinoma (HPRC): HPRC is an autosomal dominant genetic disease, and the pathological type of the tumor is all type I papillary renal carcinoma. The clinical characteristics of HPRC are similar to those of VHL disease renal carcinoma, mostly bilateral multiple renal carcinomas, with a later age of onset and slower progression. Tumor resection is generally recommended when the tumor is large.

Hereditary leiomyoma renal cancer (HLRCC): HLRCC is an autosomal dominant genetic disease, manifested as renal cancer with multiple skin leiomyoma, multifocal uterine leiomyoma or uterine leiomyosarcoma, among which the pathological type of renal cancer is mostly type II papillary renal cancer. The clinical characteristics of HLRCC are significantly different from other types of hereditary renal cancer. HLRCC is mostly a single tumor occurring on one side, with an early onset age, strong invasiveness, and easy early metastasis. Therefore, early diagnosis and surgical treatment are very important.

BHD syndrome: It is an autosomal dominant genetic syndrome involving multiple systems of the body. Typical manifestations are multiple fibrofollicular tumors on the head, neck and upper limbs, and may be accompanied by lung cysts and colon polyps. Most patients have benign skin lesions such as fibrofollicular proliferation. Multiple small round papules on the face, neck and upper trunk are the characteristic lesions of this syndrome.