Kidney Cancer

Siddhi Andika
Urologist
Head of Dept of Surgery – Pasar Minggu District Hospital Jakarta
What is a Kidney?
• Kidney is the primary organs for
maintaining fluid and electrolyte balance,
and play a large role in maintaining acid-
base balance.
• They produce renin, which plays a vital
role in controlling blood pressure,and
erythropoietin, which affects red blood
cell production.
• They affect calcium metabolism, in
particular calcium absorption, by
converting a precursor of vitamin D to
the most active form, 1,25-
dihydroxyvitamin D.
• Typically each kidney weighs 150 g in the male and 135 g in the female.
The kidneys generally measure 10 to 12 cm vertically, 5 to 7 cm
transversely, and 3 cm in the anteroposterior dimension
• Because of compression by the liver, the right kidney tends to be
somewhat shorter and wider.
RENAL VASCULATURE
• The renal pedicle classically consists of a
single artery and a single vein that enter
the kidney via the renal hilum
• The artery is branch from the aorta and
inferior vena cava
• Renal arteries, right renal artery and left
renal artery move posteriorly as they
enter the kidney. Also, both arteries have
branches to the respective adrenal gland,
renal pelvis, and ureter.
 Renal Cell Carcinoma

Papillary type 1 Papillary type 2 Chromophobe Oncocytoma

Type Clear cell 5% 10% 5% 5%

Incidence (%) 75% c-Met FH BHD BHD

Associated
mutations
VHL

BHD=Birt-Hogg-Dubé; FH=fumarate hydratase; VHL=von Hippel-Lindau.

Modified from Linehan WM et al. J Urol. 2003;170:2163-2172.
Epidemiology
• RCC accounts for 2% to 3% of all adult malignant ,
85% of all primary malignant renal tumors, is the
most lethal of the urologic cancers
• Approximately 54,000 new diagnoses of RCC are
made each year in the United States, and 13,000
patients die of disease
• RCC occurs most commonly in 5th~6th decade, male-
female ratio 1.6:1
Etiology
Etiology

• 2-4% of RCC associated with inherited disorder

* Von Hippel-Lindau disease
- familial cancer syndrome of retinal angiomas, CNS
hemangioblastomas, pheochromocytomas and clear cell RCC.
* Hereditary papillary renal cancer
- Multiple, bilateral papillary renal tumors , C-met oncogene on
ch 7
* Birt-Hogg-Duke syndrome
- Fibrofolliculomas, lung cysts, and RCC, Mutation in BHD gene ch
17p
Clinical Presentation
• Because of the sequestered location of the kidney within the
retroperitoneum, many renal masses remain asymptomatic and
nonpalpable until they are advanced
• With the more pervasive use of noninvasive imaging for the
evaluation of a variety of nonspecific symptom complexes, more than
50% of RCCs are now detected incidentally
• The classic triad of flank pain, gross hematuria, and palpable
abdominal mass is now rarely found
Imaging
• Ultrasonography
• Intravenous Urography (IVU)
• CT with contrast scanning: most sensitive, mass+renal hilum,
perinephric space and vena cava, adrenals, regional LN and adjacent
organs
• Renal Angiography
• MRI: to evaluate collecting system and IVC involvement
Diagnosis

• No screening for the general population

• No bio-marker available
• Radiographic evaluation
 IVU of right RCC

CT Scan of Left RCC

 Right Cystic RCC

RCC invading renal vein

 Neovascularity in Renal
CT scan with 3D reconstruction Angiography
associated with RCC
Tissue Diagnosis
Tumor Staging (Robson System)
Tumor Staging
(International TNM
Staging System)
 Treatment
A. Localized disease:
• Surgical removal---only potentially curative therapy

• Radical Nephrectomy (en bloc removal of the kidney and

Gerota’s fascia including ipsilateral adrenal, proximal ureter,
regional lymphadenectomy
 Robotic Radical Nephrectomy
Laparoscopic Radical Nephrectomy
Treatment
Localized disease:
• Partial Nephrectomy(nephron-sparing surgery, NSS )
--polar tumor
--tumor size<4cm
--bilateral RCC
--solitary kidney
Treatment
B. Metastatic disease:
• nephrectomy--- reducing tumor burden
• radiation--- radioresistant tumor, metastases 2/3 effective
• chemotherapy--- <10% effective
• immunotherapy--- IL-2/interferon-alpha, 30% response rate
• molecular therapy---eg. sorafenib
Prognosis

• Stage 5-year survival rate

• I 88~100%
• II 60%
• III 15~20%
• IV 0~20%
Pediatric Renal Tumors
• Wilms tumor, RCC, RTK, Clear cell sarcoma of
the kidney
• Wilms tumor is most common- accounts for 87% of
pediatric renal masses.
• Occurs 1:10,000 persons
• Peak incidence 3-4 yrs. 80% present< 5 yrs.
• Bilateral tumors in 4-13% of children
Wilms tumor (nephroblastoma)
• Embryonal tumor develops from
remnants of immature kidney.
Athough an excellent outcome is
now expected for most children,
there are subgroups of high-risk
patients for whom more effective
treatment is needed
• Prognosis influenced by stage at diagnosis
• Overall survival 64% for all stages
• Treatment
• Radical nephrectomy and regional lymphadenectomy
• Resistant to chemotherapy
• Interferon/Interleukin post-op for higher grade tumors ?
• Can have late recurrences

Lowe, L., Isuani, B. et al. Pediatric Renal Masses: Wilms Tumor and Beyond. Radiographis. 2000;20: 1585-1603.
Rhabdoid tumor of the kidneys
• Rhabdoid tumor of the kidney (RTK) is the most aggressive and lethal childhood
renal tumor, which accounts for 2% of renal tumors registered to the NWTSG.
• Clinical manifestations
• Hematuria
• Fever
• Anemia
• Symptoms of metastatic disease-most pt. present with advanced disease
• Associated with synchronus/ metachronus primary intracranial masses
• Hypercalcemia

Amar, Aneesa M.; Thomlinson, Gail, et al. Clinical Presentation of Rhabdoid Tumors of the Kidney. Journal of Pediatric Hematology. Vol. 23(2),
February 2001, pp 105-108.
• Treatment
• Worst prognosis of all renal tumors
• 18 month survival-20%
• Resection, chemotherapy, radiation therapy(? Benefit)
• Improved survival with VDCy alternating with ICE-Yamamoto, Suzuki, et al
• High dose chemo/autologous stem cell rescue under study
• May need to discuss palliative therapy/hospice care early
Clear Cell Sarcoma
• Uncommon renal neoplasm of childhood-20 new cases/yr. U.S.
• Aggressive behavior-higher rate of relapse/mortality than WT
• Long term survival-60-70%
• Survival assoc. with stage at diagnosis
• Usually presents with abd. Mass
• Treatment-nephrectomy/chemotherapy
• Addition of Doxorubicin to VCR/Dactinomycin improved survival
• Bone metastasis most common
• Can recur long after therapy

Argani, Perlman et al. Clear Cell Sarcoma of the Kidney: A Review of 351 cases from the NWTSG Pathology Center. The American Journal of Surgical Pathology. Vol.
24(1), January 2000.
Long term effects
• 4-5% develop kidney failure
• Higher incidence of renal dysfunction
• Reproductive dysfunction
• Pulmonary fibrosis if lung radiation used
• Congestive heart failure-Green et al (2001) cumulative incidence of CHF 4.4% 20 yrs after initial tx with
Doxorubicin
• Scoliosis, asymmetry
• Muscle atrophy
• 2nd primary tumors
• Long-term follow-up guidelines: www.childrensoncologygroup.org

Nelson, MB and Meeske, K. Recognizing Health Risks in Childhood Cancer Survivors. Journal of the American Academy of Nurse Practitioners. 2005.