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In this series, we take a look at what’s happening in kidney cancer each month! From treatments and clinical trials to genetic testing and government regulations, the KCA’s Medical Director, Sallie McAdoo, MS, CGC, will show you what’s new and why it matters.

After a quiet July, kidney cancer is back in the headlines. This month, let’s discuss the approval of two renal cell carcinoma (RCC) drug therapies, a new paper on genetic risk assessment for hereditary RCC, and the KCA’s partnership with the Sickle Cell Disease Association of America.

Keytruda® plus Lenvima® is another first-line RCC treatment option

Earlier in August, the drug therapy combination of Keytruda (pembrolizumab) plus Lenvima (lenvatinib) received FDA approval for people with advanced RCC, giving them another promising first-line treatment option.   

The approval was granted based on the CLEAR /KEYNOTE-581 study, which showed improved outcomes for people with RCC compared to those treated with the widely used Sutent (sunitinib). The study showed participants treated with the Keytruda/Lenvima combination showed a reduction in tumor size (response rate) almost double that of Sutent. Those on the combination treament also lived  approximately 2.5  longer without their disease progressing (progression free survival).

FDA approves combination RCC therapy

First-line treatment for advanced RCC

VHL-associated tumors get first therapy ever with WeliregTM (belzutifan) approval

Also last month, the FDA approved the first and only therapy to treat RCC tumors associated with Von-Hippel Lindau (VHL) Disease. VHL is an inherited condition that causes both cancerous and non-cancerous solid tumors and fluid-filled cysts in various parts of the body. Approximately 10,000 people in the US and 200,000 in the world are estimated to be affected by VHL. The study that led to FDA approval looked at people with known mutations causing VHL that did not have metastases (e.g. spread of a cancer from the kidney to other areas of the body).  Although it can take an average of 8 months to see an effect, the study results showed an overall 40% reduction in tumor size for patients with these specific RCC tumors. Welireg is approved for patients that do not immediately require surgery and was also shown to reduce the size of other tumors associated with VHL (pancreatic neuroendocrine tumors and hemangioblastomas). 

About VHL

FDA approves Welireg

Belzutifan and VHL disease

First consensus statement published on genetic risk assessment for inherited RCC.

In late 2019, a group of 33 researchers, healthcare providers, and advocates with expertise in hereditary RCC came together to sort out their thoughts on how to care for patients with suspicion of an inherited form of kidney cancer. Current guidelines may not be sufficient to account for all the possible scenarios in which inherited genetic risk assessment would be useful. This group answered a series of questions relating to who, when, and how genetic risk assessment should be performed. More than 85% of the specialists had to agree in order to become part of the consensus recommendations. The summary of their results were published in the August 2021 edition of the scientific journal Cancer.

Importantly, the group addressed who should be assessed and with what kinds of tests thoroughly and in strong agreement:

  • Who should undergo genetic risk assessment?
    • People with certain kidney tumors (papillary type 1, chromophobe, renal angiomyolipomas and clear cell RCC) in both kidneys or multiple tumors in one kidney 
    • People with hybrid renal tumors (oncocytomas/chromophobe), SDH-tumors, or FH-deficient tumors
    • People with more than 1 of the following tumors outside the kidney:  pheochromocytoma or paraganglioma, endolymphatic sac tumor, uveal melanoma, and FH-deficient uterine fibroid
    • Certain people with 1st and/or 2nd degree relatives with RCC or features suggestive of an inherited cancer syndrome. 
  • What type of genetic testing should be performed and how?
    • Anyone undergoing testing should have pre-test counseling by someone with expertise in inherited cancer syndromes either in-person or by telemedicine.
    • If there are signs of a specific condition, only that gene should be tested, otherwise a test of multiple genes should be done in those with more general risk factors.
    • Single gene testing by blood is also preferred following the finding of a mutation in the tumor itself in order to check for it being an inherited form or when pretest counseling cannot be done.

There was less clear agreement on other aspects of genetic risk assessment. For example, many weren’t sure whether age was sufficient to recommend genetic testing, though most of the group felt the threshold should be somewhere at or above 46 years of age.  Similarly, there was less agreement on what lab/medical results are needed before genetic testing and if that genetic testing should be done before starting treatment. Because there are still remaining areas of controversy and because it is still unclear how some genetic results may impact treatment and outcomes, more research and discussion will surely continue.

Read the guidelines

KNOW and TELL Initiative with Sickle Cell Disease Association of America (SCDAA)

In June, the KCA partnered with the SCDAA to embark on a year-long educational initiative to raise awareness of sickle cell trait (SCT) and its connection to kidney cancer. The partnership was announced on June 18th; a day that magically fell between World Kidney Cancer Day (June 17th) and World Sickle Cell Disease Awareness Day (June 19th).  Although not everyone with SCT will get cancer, more than 95% of people with renal medullary carcinoma (RMC, a rare and aggressive type of kidney cancer) also have SCT. Since RMC can be confused with other cancer types and be hard to detect early, it is important to tell your doctor when you are experiencing any symptoms of RMC especially when you are a positive carrier for SCT.  Since September is sickle cell awareness month, we wanted to reiterate the importance of knowing your sickle cell status and the signs of RMC and telling your family and healthcare team when you have either. 

Learn about KNOW and TELL

Upcoming 2nd Annual Virtual Patient Symposium – September 17-18

Last, but certainly not least, is a reminder that the KCA’s Virtual Patient Symposium is open for registration. This online event taking place September 17th and 18th is an opportunity for people impacted by kidney cancer to learn from medical professionals, researchers, and fellow patients and caregivers. We’ll be covering topics from treatment and genetics to managing cancer finances and how to navigate a new diagnosis. The symposium is free to anyone touched by kidney cancer.  We hope to see you there!

Register here

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