Renal Cell Carcinoma

Question 1

What is the prognosis assoc with Rhabdoid and Sarcamotoid type? What is the tx?

Answer 1

The prognosis is poor with these pathologic subtypes. They are responsive to immunotherapy.

Question 2

What are a few manifestations of HLRCC and what is the gene mutation?

Answer 2

uterine and cutaneous leiomyomas, aggressive RCC. Autosomal dominant-FH gene! Remember this can also present in a somatic form.

Question 3

With Von Hippel Lindau Syndrome what is the mode of inheritance and what type of renal cancer are they most likely to develop?

Answer 3

Autosomal dominant-vHL gene 3p25. They are most predisposed to getting clear cell RCC.

Question 4

In Birt Hogg-Dube Syndrome what are the clinical features? What kidney cancer can they get?

Answer 4

Hereditary hair follicle tumors. Think lung cysts and spontaneous pneumothorax. The most common RCC is chromophobe tumors. Can also develop clear cell and oncocytomas.

Question 5

What is the mutation seen in Birt Hogg-Dube Syndrome?

Answer 5

BHD on chromosome 17

Question 6

What is the guideline for unaffected individuals who have a family history of RCC?

Answer 6

If they have two or more first degree relatives with RCC

Question 7

For those individuals who do not have genetic syndrome of RCC what are the guidelines for genetic referral?

Answer 7

diagnosed at age 46 or younger, that has bilateral or multifocal tumors, or 1 or more first or second degree relative with RCC

Question 8

In terms of staging what is the cutoff difference for pT1 vs pT2?

Answer 8

7cm. Anything less than this is pT1.

Question 9

When treating early stage RCC what is preferred partial or radical nephrectomy?

Answer 9

Partial due to loss of kidney function seen with radical nephrectomy

Question 10

What is the recommendation for adjuvant therapy for Stage II RCC (greater than 7cm)?

Answer 10

You give adjuvant Pembrolizumab only for patients with grade 4 pathology +/- sarcomatoid features

Question 11

What are the guidelines for stage III RCC (tumor invades into renal vein or segmental branches or T2,N1)?

Answer 11

For clear cell pathology-Pembrolizumab, they also mention Sunitinib, but it is a category 3 rec! Surveillance is also an option here.
For non clear cell pathology-surveillance.

Question 12

What are the IMDC criteria for stage IV RCC?

Answer 12

Poor performance status, less than 1 year from nephrectomy, hypercalcemia, anemia, neutrophilia, thrombocytosis. 1-2 intermediate. 3 or more-poor risk.

Question 13

When should you consider cytoreductive nephrectomy in Stage IV RCC?

Answer 13

Only for those patients with favorable risk disease and a very low metastatic disease burden! Also if they are symptomatic from the kidney (pain or bleeding) you can consider it then. Also have option to delayed nephrectomy after systemic therapy.

Question 14

What are the first line treatment options for favorable risk Stage IV RCC?

Answer 14

Just remember that you can’t use Ipi/Nivo in these patients. Lenvatinib/Pembro, Cabo/Nivo, Axitinib/Pembro

Question 15

What are the first line options for intermediate/poor risk Stage IV RCC?

Answer 15

All of the options for favorable risk disease in addition to Ipi/Nivo

Question 16

What are the second line treatment options in Stage IV RCC?

Answer 16

Axitinib, Cabozantinib, Lenvatinib/Everolimus (can be used regardless of prior exposure), Tivozanib-if they received TKI/IO. If they had not received IO/TKI combo before you can use those options. Nivo and Cabo can be used individually if no previous exposure to TKI/IO.

Question 17

What are the options for Stage IV non clear RCC?

Answer 17

Preferred-Cabozantinib
Others-Lenvatinib/Everolimus, Nivo, Cabo/Nivo, Pembro, Sunitinib.

Question 18

What is the treatment for collecting duct tumors?

Answer 18

Gemcitabine/Cisplatin-has a low response rate, ORR-26%

Question 19

What is the treatment for renal medullary tumors that we can see in sickle cell trait patients?

Answer 19

Platinum based chemotherapy even in localized disease.

Question 20

Who are candidates for metastatatectomy in Stage IV RCC? Is there a role for systemic therapy after?

Answer 20

Those with 1-3 sites of disease, those who have a disease free interval of a year or more after nephrectomy, and ECOG of 0 or 1. There is no role for systemic therapy after.

Question 20

In those with VHL RCC what oral therapy has been approved for this? Toxicities?

Answer 20

Belzutifan. Anemia, fatigue, headache, nausea.