The document discusses the management of renal cell carcinomas (RCC) associated with genetic syndromes. It notes that for von Hippel-Lindau syndrome, smaller tumors less than 3 cm are observed while larger tumors greater than 3 cm are removed. For hereditary papillary RCC, surgery is emphasized to remove larger lesions and observe smaller ones due to the indolent nature of type 1 papillary RCC. For hereditary leiomyomatosis RCC, delayed intervention is not recommended due to the aggressive nature of the tumors, and wide local excision is recommended even for tumors under 3 cm. The document also discusses staging, treatment options including radical nephrectomy and minimally invasive approaches, management

1. Dr Naina Kumar
SR 1 Surgical oncology, SMS Hospital Jaipur
Management of Renal Cell
Carcinomas
31.08.21

4. Genetic syndromes and RCC :
Management

7. HEREDITARY KIDNEY CANCER SYNDROMES, GENETICS, AND
MOLECULAR BIOLOGY Management:
• Management of renal tumors in patients with VHL now includes surveillance of
smaller tumors (<3 cm) and resection of larger ones (>3 cm) by PN with the goal of
preventing metastases and optimizing renal function by “resetting the biologic clock”
through appropriately timed surgeries.
• The goal of complete tumor removal with wide negative surgical margins is less
appropriate for these patients, where management of localized lesions supplants cure.

8. HPRCC:
• As with VHL, management of renal tumors recognizes the need to remove larger
lesions and observe smaller ones.
• Although no size cutoff for intervention has been established, the biology of type 1
papillary RCC appears to be more indolent than ccRCC, suggesting the risk of death
from kidney cancer in these patients is low.
• Unfortunately, renal mass biopsy cannot reliably make a diagnosis of type 1 papillary
RCC, so surgery is sometimes required.
• As such, PN with renal preservation is emphasized despite the often encountered
diffuse micro- and macromultifocality of these lesions.

9. Hereditary Leiomyomatosis Renal Cell Carcinoma:
• Unlike other hereditary forms of RCC, AS with delayed intervention for small tumors
is not recommended due to the aggressive nature of these tumors. Although tumor
enucleation is recommended for most patients with hereditary tumors, renal lesions
associated with this syndrome tend to be infiltrative, thus wide local excision is
recommended at initial diagnosis, even for tumors <3 cm.

11. Staging and Prognosis:

14. TREATMENT OF LOCALIZED
RENAL CELL CARCINOMA

19. Radical Nephrectomy for Renal
Cell Carcinoma

20. • RN” as currently practiced may be better termed “total” nephrectomy, as it often omits
several of the components of the original, “radical” nephrectomy, which always included
extrafascial nephrectomy, adrenalectomy, and extended lymphadenectomy (LND) from
the crus of the diaphragm to the aortic bifurcation.
• Perifascial dissection is still routinely practiced for larger tumors, as ≥25% of these
tumors extend into the perinephric fat.
• Removal of the ipsilateral adrenal gland is no longer recommended, unless there is
suspicion of direct invasion of the gland by tumor or a radiographically or clinically
suspicious adrenal tumor because of the similar propensity of RCC to metastasize to the
ipsilateral or contralateral adrenal gland.

21. • Finally, extended LND has been shown to be of no therapeutic benefit for patients with clinically
localized RCC as the risks of clinically negative nodes being pathologically involved is <5%.46
The role of LND in high-risk (>pT2 N+ M+) renal tumors remains controversial.
• RN is still a preferred option for some patients with localized RCC, such as those with very large
tumors (most clinical T2 tumors) or the relatively limited subgroup of patients with clinical T1
tumors that are not amenable to nephron-sparing approaches.
• According to the 2017 AUA guidelines, physicians should consider RN for patients with a solid
or Bosniak 3/4 complex cystic renal mass where increased oncologic potential is suggested by
tumor size, renal mass biopsy, and/or imaging characteristics and in whom active treatment is
planned.

22. • In this setting, RN is preferred if all of the following criteria are met:
• (1) high tumor complexity and PN would be challenging even in experienced hands;
• (2) no preexisting chronic kidney disease (CKD) or proteinuria; and
• (3) normal contralateral kidney and new baseline estimated glomerular filtration rate (eGFR)
will likely be >45 mL/min/1.73 m2
• The surgical approach for RN depends on the size and location of the tumor as well as the
patient’s habitus and medical/surgical history. For locally advanced disease and/or bulky
lymphadenopathy, an open surgical approach using either an extended subcostal, midline, or
thoracoabdominal incision is generally used.

23. • Current minimally invasive approaches allow all of the essential steps of RN to be
performed, with the associated benefits of shorter convalescence and reduced morbidity.
• Laparoscopic RN is now established as a preferred approach for moderate to large
volume tumors (≤10 to 12 cm), without invasion of adjacent organs, with limited (or no)
venous involvement, and having manageable (or no) lymphadenopathy.
• Robotic RN may further extend the indications for minimally invasive radical
nephrectomy (MIRN), to include some patients with features previously thought to
mandate open RN as vascular control and suturing is facilitated with this approach.

24. Important pointers

27. Metastatic disease stage IV:

29. Opinion from UpToDate:
Is there a role for adjuvant therapy? — For patients with localized clear cell renal
carcinoma treated with nephrectomy, adjuvant pembrolizumab improved disease-free
survival (DFS) and was well tolerated in a phase III trial. These data are promising, and
we await regulatory approval and/or introduction into consensus guidelines prior to
incorporating adjuvant immunotherapy into routine clinical practice.
We also do not suggest the use of adjuvant sunitinib. Although adjuvant sunitinib
improved DFS in select patients with high-risk disease in one clinical trial, it confers no
clear overall survival (OS) benefit and increases toxicity.

30. Other trials evaluating sunitinib and sorafenib (ASSURE and SORCE), pazopanib
(PROTECT), and axitinib (ATLAS) have failed to demonstrate a recurrence-free or OS
benefit in the adjuvant setting.
●
Everolimus – A randomized clinical trial (EVEREST) is ongoing evaluating mechanistic
(mammalian) target of rapamycin (mTOR) inhibitor everolimus as adjuvant therapy
(NCT01120249).

32. Risk stratification :

43. Follow up

45. Thank you!