**10. Conclusion**

tumors associated with HPRC are candidates for radiologic surveillance until one or more tumors reach 3 cm. Ultrasound is not sufficient in surveillance of hereditary kidney cancer syndromes [10, 25, 54, 55]. In the near future, more evidence on effective monitoring in different kidney cancer syndromes will be gathered. If the gene mutation has not been identified but the family has a significantly increased risk of kidney cancer, a regular renal imaging examination in the first-degree relative of the patient with renal cancer may be considered on a case-by-case basis. Other surveillance procedures depend on the risks of the specific symptoms in observed gene mutation. In multisystem syndromes, monitoring practices depend on the

**Table 7.** Follow-up care recommendations in hereditary cancer syndromes in which the kidney cancer risk is high [10, 25, 54].

In Lynch syndrome, which is a high risk for upper tract ureotelic carcinoma, the risk of colorectal cancer can be reduced by removing intestinal adenomas at regular checkups, which results in the same mortality to colorectal cancer in the carriers of Lynch gene mutation as in

The contents of routine gene panels are based on the knowledge on kidney cancer susceptibility genes. If a patient's DNA sequencing has failed to identify the hereditary mutation, RNA sequencing may identify the specific diagnosis [57]. If there are three patients in the family, trio exome analysis may be a pivotal method. Potentially, tumor tissue-only test would be a useful method to find out novel kidney cancer susceptibility genes and mutations in kidney cancer

probability of the kidney cancer, and ultrasound may be used in monitoring.

**von Hippel-Lindau (VHL) Hereditary**

Annually starting at the age

Biannual brain and spine MRI starting at the age of 11 Ophthalmological and audiological exam Plasma-free metanephrines/ urinary metanephrines

of 10

RCC subtype Clear cell carcinoma Papillary type 1 Papillary type 2,

**papillary RCC (HPRC)**

40 years 50–70 years Less than 40 years 50 years

None Dermatological and

Annually starting at the age of 30

Risk for RCC 40% Nearly 100% 20–30% 30% Gene *VHL MET FH FLCN(BHD)*

**Hereditary leiomyomatosis and RCC (HLRCC)**

collecting duct

Annually starting at the age of 5–20 years

gynecological exam

**Birt-Hogg-Dubé (BHD)**

Annually starting at the age of 20 years

Dermatological exam and CT of the chest

Most often chromophobe oncocytoma

the general population.

Typical age of onset for RCC

12 Evolving Trends in Kidney Cancer

Abdominal magnetic imaging

Other surveillance

**9. Future perspectives**

Knowledge on the clinical significance of mutations helps to improve prognosis in families with hereditary cancer. Understanding of hereditary kidney cancer syndromes on their molecular basis improves the utility of specific diagnosis [64]. In the future, increased knowledge of cancer genetics is likely to enable the development of targeted drug therapies. Today, hereditary mutations are detected by DNA testing of white blood cell lymphocytes. Tumor tissueonly tests to identify the hereditary kidney cancer are not yet available.
