Kidney cancer: diagnosis and management

  • NICE guideline
  • NG256
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Managing renal cell carcinoma in people with a heritable renal cell carcinoma predisposition syndrome

People have the right to be involved in discussions and make informed decisions about their care, as described in NICE's information on making decisions about your care.

Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations, and has information about prescribing medicines (including off-label use), professional guidelines, standards and laws (including on consent and mental capacity), and safeguarding.

Healthcare professionals should follow our general guidelines for people delivering care:

1.17 Genetic assessment to diagnose heritable renal cell carcinoma predisposition syndromes

See also NICE's visual summary on diagnosis and management of renal cell carcinoma (RCC) in people with a heritable RCC predisposition syndrome.

1.17.1

Assess people with RCC to determine whether they meet any of the following criteria associated with having a heritable RCC predisposition syndrome:

  • aged 46 or younger (or an age eligible for inherited renal cancer genetic testing based on the rare and inherited disease eligibility criteria in the National Genomic Test Directory [NGTD], if different)

  • multiple renal tumours

  • family history of renal cancer (first- or second-degree relative)

  • signs or symptoms associated with a hereditable RCC predisposition syndrome (for example, cerebellar or spinal haemangioblastoma, or spontaneous pneumothorax)

  • tumour type commonly associated with heritable RCC predisposition syndromes, for example, fumarate hydratase (FH)-deficient RCC or succinate dehydrogenase (SDH)-deficient RCC.

1.17.2

If a person with RCC meets any of the criteria in recommendation 1.17.1, healthcare professionals who:

  • can directly request genetic testing for heritable RCC predisposition syndromes, according to local policies and procedures, should:

    • check the rare and inherited disease eligibility criteria in the NGTD to determine who should be tested for conditions that are associated with an inherited predisposition for RCC and which gene panel or panels they should be tested for, and

    • request any relevant tests following local policies and procedures, and after discussing with the person.

  • cannot directly request genetic testing for a heritable RCC predisposition syndrome should:

    • consult local policies and procedures and

    • refer the person to a relevant healthcare professional who can do a detailed assessment and request testing, if needed.

1.17.3

Before offering testing to a person with RCC for a heritable RCC predisposition syndrome, discuss the following with them:

  • the expected waiting time at their centre to have the genetic test and get their results

  • most people will have a negative result unless there are additional indicators of a heritable RCC predisposition syndrome

  • a positive result can:

    • influence treatment options and follow-up for RCC

    • indicate a risk of developing non-RCC conditions related to the syndrome

    • have implications for family members

  • access to specialist genetic services for ongoing support and advice will be available if the result is positive

  • it may be possible to have the test in the future if they decline it and then change their mind.

1.17.5

When a person with RCC is diagnosed with a heritable RCC predisposition syndrome, discuss with them:

  • what the results could mean for them and their family members

  • that there are other non-RCC conditions related to the syndrome that they have an increased chance of developing, and how and where these would be managed

  • how to access genetic counselling and support services for their condition.

For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on genetic assessment to diagnose heritable RCC predisposition syndromes.

Full details of the evidence and the committee's discussion are in evidence review G: genetic assessment and management of renal cell carcinoma (RCC) associated with heritable RCC syndromes and evidence review D: information needs.

1.18 Managing localised and locally advanced RCC in people with a heritable RCC predisposition syndrome

Localised RCC in people with a heritable RCC predisposition syndrome

Active surveillance for suspected or confirmed localised RCC in people with a heritable RCC predisposition syndrome
1.18.1

For people with a renal lesion and a heritable RCC predisposition syndrome associated with more aggressive RCC, such as hereditary leiomyomatosis and RCC (HLRCC) syndrome which is associated with FH-deficient RCC, consider either:

1.18.2

For people with a renal lesion and a heritable RCC predisposition syndrome that is not associated with more aggressive RCC, consider active surveillance (using MRI or ultrasound) for any renal lesions that are less than 3 cm in diameter.

1.18.3

Consider coordinating imaging:

  • within the uro-oncology multidisciplinary team, when the person has multiple renal lesions that are at different stages of management (including active surveillance) or is having follow-up after treatment and

  • with other specialities involved in managing the heritable RCC predisposition syndrome, such as clinical genetics, endocrinology for von Hippel–Lindau (VHL) syndrome and respiratory for Birt–Hogg–Dubé (BHD) syndrome.

1.18.4

For people with a renal lesion and a heritable RCC predisposition syndrome that is not associated with more aggressive RCC who are undergoing active surveillance, consider using the following imaging schedule, making changes if more frequent imaging is needed based on the renal lesion's and person's clinical characteristics, and the person's preferences:

  • year 1: imaging between 3 and 6 months, and again at 12 months, after the start of active surveillance

  • year 2 onward: imaging at least annually or as decided by the uro‑oncology multidisciplinary team.

1.18.5

For people with a renal lesion and a heritable RCC predisposition syndrome associated with more aggressive RCC who are undergoing active surveillance, agree a more frequent imaging schedule than in recommendation 1.18.4, based on the person's clinical needs and preferences.

1.18.6

Offer all people with a heritable RCC predisposition syndrome who are undergoing active surveillance the opportunity to discuss and agree a personalised care plan with their healthcare professional, which is given to them and their GP, and documented in their health record. The plan should include:

  • the person's active surveillance imaging schedule and, when possible, their coordinated imaging schedule

  • clarity that active surveillance is managed in secondary care

  • a named healthcare professional and examples of symptoms that the person should contact them (such as if they have blood in their urine or persistent abdominal pain).

1.18.7

For people with a renal lesion and a heritable RCC predisposition syndrome that is not associated with more aggressive RCC, discuss moving from active surveillance to treatment if any of the following apply:

  • the renal lesion is 3 cm in diameter or larger on the most recent scan

  • the renal lesion's growth rate suggests that it might be 3 cm in diameter or larger before the next scan

  • the person wants to move to treatment for 1 or more renal lesions, and this is still suitable for them, taking into account competing clinical priorities.

For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on active surveillance for suspected or confirmed localised RCC in people with a heritable RCC predisposition syndrome.

Full details of the evidence and the committee's discussion are in evidence review G: genetic assessment and management of renal cell carcinoma (RCC) associated with heritable RCC syndromes.

Surgery for suspected or confirmed localised RCC in people with a heritable RCC predisposition syndrome

See NICE's guideline on perioperative care in adults for considerations around perioperative care and enhanced recovery programmes.

1.18.8

Consider partial nephrectomy when this can completely remove the renal lesion or lesions based on location and size, and the person's clinical characteristics.

1.18.9

Consider other nephron-sparing treatments, such as thermal ablation or stereotactic ablative radiotherapy (SABR), if:

  • these can completely destroy the renal lesion and

  • partial nephrectomy is not possible or is likely to be very challenging because of previous partial nephrectomy.

1.18.10

Consider total nephrectomy or surgically removing more tissue around the lesion during partial nephrectomy for people with syndromes associated with more aggressive RCC.

For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on surgery for suspected or confirmed localised RCC in people with a heritable RCC predisposition syndrome.

Full details of the evidence and the committee's discussion are in evidence review G: genetic assessment and management of renal cell carcinoma (RCC) associated with heritable RCC syndromes.

Systemic anticancer therapy for localised RCC in people with a heritable RCC predisposition syndrome

See also recommendation 1.1.8 in the section on information for healthcare professionals to support people with suspected or confirmed RCC.

1.18.11

Belzutifan is recommended with managed access through the Cancer Drugs Fund as an option for treating von Hippel–Lindau (VHL) syndrome in adults who need treatment for VHL associated RCCs, central nervous system hemangioblastomas or pancreatic neuroendocrine tumours, when localised procedures are unsuitable or undesirable. For full details, see NICE's technology appraisal guidance on belzutifan (TA1011, 2024).

Locally advanced RCC in people with a heritable RCC predisposition syndrome

See the section on surgery for suspected or confirmed locally advanced RCC.

Follow-up of localised or locally advanced RCC after treatment in people with a heritable RCC predisposition syndrome

1.18.12

Offer follow-up to people who have completed treatment for localised or locally advanced RCC.

1.18.13

Offer all people with a heritable RCC predisposition syndrome who are undergoing follow-up the opportunity to discuss and agree a personalised care plan with their healthcare professional, which is given to them and their GP, and documented in their health record. The plan should include

  • the person's risk of recurrence, follow-up imaging schedule and, when possible, their coordinated imaging schedule

  • that follow-up is managed in secondary care

  • a named healthcare professional

  • examples of symptoms that could indicate recurrence or metastases that the person should contact their healthcare professional about (such as if they have blood in their urine or persistent abdominal pain).

    For an example follow-up letter, see section 7.2 in the GIRFT guide Urology: Towards better care for patients with kidney cancer. See also section 3.9 on post-surgery follow-up in the GIRFT guide, particularly the information on patient support during follow-up, principles for good communication with patients and information to communicate to patients.

1.18.16

After 5 years, discuss with the clinical team that oversees standard surveillance for heritable RCC predisposition syndromes when to stop follow-up and return solely to standard surveillance.

For a short explanation of why the committee made these recommendations and how they might affect practice, see the rationale and impact section on follow-up of localised or locally advanced RCC after treatment in people with a heritable RCC predisposition syndrome.

Full details of the evidence and the committee's discussion are in evidence review G: genetic assessment and management of renal cell carcinoma (RCC) in people with heritable RCC syndromes.

1.19 Managing advanced RCC in people with a heritable RCC predisposition syndrome

See the section on managing advanced RCC.