Renal Tumor/Cancer Flashcards
Wilms tumor (Nephroblastoma)
general
Second most common abdominal tumor in children, with a majority of these being sporadic occurrence
Some Wilms tumors occur in the setting of associated malformations or syndromes
Occurs most commonly between years 2 and 5, mean age of diagnosis is 4 years
Median age at diagnosis is related both to gender and laterality:
Bilateral tumors present at younger age
Males diagnosed earlier than females
460 new cases of Wilms tumor occur annually in the United States
Represents 5-6% of cancers in children under the age of 15
WILMS Tumor-
RF
May be associated with other GU abnormalities
Wilms tumor, Aniridia, Genitourinary malformations, and Mental retardation associated with chromosome 11 abnormalities
Beckwith-Wiedemann syndrome:
Wilms tumor
Adrenal cytomegaly
Hemihypertrophy
Wilms tumor (Nephroblastoma)-
S/Sx
Most children present with increasing size of abdomen or asymptomatic abdominal mass
Usually incidentally discovered by parent or health care provider
Mass is smooth and firm, well demarcated and rarely crosses the midline
It can extend inferiorly into the pelvis
25% of patients are hypertensive at presentation
Gross hematuria is an uncommon presentation, but microscopic hematuria occurs in 25% of patients
Wilms tumor (Nephroblastoma)
labs
CBC usually normal but can be anemic due to hemorrhage into the tumor.
Blood urea nitrogen (BUN) and serum creatinine are usually normal.
Urinalysis may have some blood or leukocytes.
Wilms tumor (Nephroblastoma)
Imaging/Staging
- Assess both kidneys with Ultrasound or CT scan of abdomen
Will establish presence of intrarenal mass
Look for the contralateral kidney and evaluate function
Rule out synchronous Wilms tumor - Evaluate inferior vena cava with Ultrasonography with Doppler flow
Look for presence and extent of tumor propagation - Image the liver
Look for metastatic disease - Order Chest CT
Look for pulmonary metastases
10% of patients will have metastatic disease at time of diagnosis
Of these, 80% will have pulmonary disease and 15% have liver metastases
Bone and brain metastases are extremely uncommon with Wilms tumor, so bone scans and brain imaging are not routinely ordered
Clinical stage is ultimately decided at surgery and confirmed by pathologist
Wilms tumor (Nephroblastoma)
Surgical Tx
Surgical
In the United States, surgical exploration of the abdomen via an anterior surgical approach to allow for inspection and palpation of the contralateral kidney first
Liver and lymph nodes are inspected
Suspicious areas are biopsied or excised
En bloc resection of the tumor is performed
Every attempt is made to avoid tumor spillage at surgery as this may increase staging and treatment
Therapy is tailored to tumor stage
Wilms tumor (Nephroblastoma)
Prognosis/staging
Along with staging the tumor, the histologic type matters for therapy and prognosis
Favorable histology refers to the classic triphasic Wilms tumor and its variants
VS
Unfavorable histology refers to diffuse anaplasia (extreme nuclear atypia) and is present in 5% of Wilms tumors
They are given a stage I-V and labeled either favorable histology (FH) or unfavorable histology (UH) for treatment purposes after excision and pathologic examination
Wilms tumor (nephroblastoma)
Tx and prognosis
Total nephrectomy followed by chemotherapy is 80-90% cure rate
Partial nephrectomy if bilateral involvement
Post-surgery radiation therapy if extends beyond the renal capsule, pulmonary metastasis, or a large tumor.
Improvement in the treatment of Wilms tumor has resulted in a cure rate of 80-90%
They changed the treatment regimen to now having a more intense therapy during the initial treatment phase while shortening the treatment duration from 60 weeks to 24 weeks
Renal Cell Carcinoma
general
Tumor of the proximal convoluted renal tubule cells
95% of primary tumors originating in the kidney (clear cell most common histological pattern)
More common in men versus women
Usually diagnosed 50-70 years of age
Usually asymptomatic until advanced stage
Most common sites of metastasis:
Lymph
Lungs- Cannon ball metastases
Liver
Bone
Brain
Renal Cell Carcinoma
RF
Risk factors:
Tobacco abuse
Obesity
Hypertension
Men
Cadmium or industrial exposure
Increased risk population:
Family History
End Stage Renal Disease on HD > 3 years
Prior Kidney Radiation
Renal cell carcinoma
Clin man
Presentation:
Triad: FLANK PAIN, PALPABLE ABDOMINAL or RENAL MASS, and HEMATURIA
Abdominal pain
Abdominal mass
Malaise
Weight loss
Hypertension
Hematuria (40% of cases)
Left-sided varicocele- if the tumor blocks left testicular vein drainage
renal cell carcinoma
PE and Labs/imaging
PE: Abdominal Mass
Labs looking for: Anemia, Hypercalcemia, Hematuria
Imaging: May be seen as an incidental finding; test of choice is Abdominal CT, renal US or MRI can be used
Biopsy usually done at time of nephrectomy
Renal Cell carcinoma
Treatment
Nephrectomy: Partial nephrectomy versus radical nephrectomy if no metastatic disease
Stage I-III: radical nephrectomy with immune mediated therapy like interleukin-2 and monoclonal antibody molecular targeted treatment
Usually resistance to chemo and radiation therapy
If patient has localized Renal Cell Carcinoma (RCC) treated with nephrectomy, adjuvant immunotherapy with 1 year of adjuvant Pembrolizumab improved disease-free survival
Partial nephrectomy patients with bilateral involvement of solitary kidney or an option in early disease
Surgery is curative!
Surveillance for 5 years post-cure
Bladder Cancer
general
Bladder cancer is the second most common urologic cancer.
More common in men than women (3.1:1) and mean age of diagnosis is 73 years.
Risk factors include cigarette smoking and exposure to industrial dyes or solvents.
In the United States, almost all primary bladder cancers (98%) are epithelial malignancies.
Urothelial cell carcinomas (90%), also known as transitional cell carcinoma (TCC)
Squamous Cell Cancers (7%)
Associated with schistosomiasis, vesical calculi (bladder stones), or prolonged catheter use
Adenocarcinomas (2%)
bladder cancer
Sx
Symptoms:
Hematuria is the presenting symptom in 85-90% of patients with bladder cancer.
Gross
Microscopic
Chronic
Intermittent
Patients may have irritative voiding symptoms depending on where the tumor is located within the bladder.
Urinary frequency
Urinary urgency
Most patients are asymptomatic.
Due to its superficial nature
bladder cancer
PE
On Physical Exam:
Abdominal masses may be present on bimanual examination with large-volume or deeply infiltrating cancers.
Hepatomegaly, palpable lymphadenopathy, lymphedema of the lower extremities, or metastases to pelvic lymph nodes may be found on physical exam from locally advanced cancers.
bladder cancer
Labs
Urinalysis revels microscopic or gross hematuria in most cases.
Pyuria and azotemia may be present.
Anemia can occur due to chronic blood loss or bone marrow metastases.
Urine cytology will be positive and can be useful to initially diagnosis patient or if bladder cancer reoccurs.
Urine cytology is sensitive in detecting cancers of higher grade and stage (80-90%) but less so in detecting superficial or well-differentiated lesions (50%).
bladder cancer
imaging
Bladder cancers can be noted on ultrasound, CT, or MRI as masses.
Presence of cancer is confirmed by cystoscopy and biopsy, with imaging used to evaluate the upper tract and to stage more advanced lesions.
bladder cancer
Cystoscopy and Biopsy
Cystoscopy is performed in the OR with local anesthesia or in the office with lidocaine injected into the urethra to look for a bladder tumor.
If a bladder tumor is noted, then patient is scheduled back in the OR with either general or regional anesthesia for transurethral resection of the bladder tumor (TURBT).
Bladder Cancer-Pathology and Staging
Grading is based on:
Size
Pleomorphism
Miotic rate
Hyperchromatism
Staging is based on:
Extent (depth) of bladder wall penetration
Presence of regional or distant metastases
T-Primary Tumor
N- Regional Lymph Nodes
M-Distant Metastasis
bladder cancer
tx
Treatment Options:
Surgical Treatment
Intravesical Therapy
Chemotherapy
Radiotherapy
Immunotherapy
Transurethral resection is the initial form of treatment for all bladder tumors since it is diagnostic, allows proper staging, and controls superficial cancers.
But if the tumor is not just superficial and invading the muscle, it will require more aggressive treatment.
Consider a partial cystectomy for patients that have a muscle invasive cancer, solitary lesion at the bladder dome, or cancer in a bladder diverticulum.
Radical cystectomy requires a urinary diversion in all patients.
Most patients will have a conduit of the ileum or colon performed, called a urostomy.
Bladder Cancer
Urostomy
Urostomy overview
Section of the small intestine ileal conduit
Stoma the hole in the abdominal wall to allow urine to leave the body
Bladder cancer
Intravesical Therapy
Intravesical therapy is using chemotherapy or immunotherapy that is delivered directly into the bladder by a urethral catheter to help reduce reoccurrence after a complete transurethral resection.
Agents are given weekly for 6-12 weeks.
Recommend to patients they keep the liquid in their bladder for 2 hours if possible.
Can be given in the urologist’s office.
Most effective agent to reduce disease progression is bacillus Calmette-Guerin (BCG).
Side effects include irritative voiding symptoms and hemorrhagic cystitis.
bladder cancer
chemo
15% of patients with newly diagnosed bladder cancer will have metastases.
Metastases will develop up to 40% of patients within 2 years of having their bladder removed.
Cisplatin-based combination therapy is the preferred approach for them, so offering adjuvant chemotherapy is helpful for those with high risk for reoccurrence.
Neoadjuvant chemotherapy appears to benefit all patients with muscle-invasive cancer prior to having a cystectomy performed.
Chemotherapy is considered for those with bulky lesions or regional metastases.
Bladder Cancer
Radiation
External Beam Radiation
Delivered in fractions over a 6–8-week period.
10-15% of patients will develop bladder, bowel, or rectal complications.
30-70% of patients will have reoccurrence with radiotherapy alone.
Systemic chemotherapy is offered in conjunction with radiation to some patients.
Bladder Cancer
Immunotherapy
Immunotherapy consists of drugs called Anti-PDL-1 inhibitors.
These checkpoint inhibitors are approved as second line therapy for metastatic urothelial cancer by the FDA after the patient has received platinum-based chemotherapy.
Immune checkpoints are molecules on certain immune cells that need to be activated or inactivated to start an immune response.
Checkpoint inhibitors don’t work directly on the tumor but allows the immune response that has only started, to progress to full force.
PD-1 is a checkpoint protein on T cells. Normally it is an “off switch” that helps keep the T cells from attacking the other cells in the body.
bladder cancer
Examples of PD-L1 inhibitors include:
Durvalumab (Imfinzi)
Avelumab (Bavencio)
Atezolizaumab (Tecentriq)
Examples of PD-1 inhibitors include:
Nivolumab (Opdivo)
Pembrolizumab (Keytruda)
Response rates range from 17%-25% in locally advanced and metastatic urothelial bladder cancer.
Bladder Cancer
Treatment, who gets it?
So who gets what?
Complete transurethral resection + a single dose of intravesical chemotherapy immediately after resection:
Best for patients with superficial non-muscle invasive cancers (Tis, Ta, T1)
Additional intravesical chemotherapy for the following subset patients:
Carcinoma in situ (Tis), those undergoing resection of large, high-grade, recurrent Ta lesions or T1 cancers
bladder cancer
So who gets what tx?
Neoadjuvant chemotherapy followed by a radical cystectomy
Patients with muscle invasive (T2+) but still localized are at risk for both nodal metastases and progression, require this more aggressive treatment.
Particularly important for higher-stage or bulky tumors to improve their resect- ability.
This is gold standard for these patients as it offers a better survival rate than just cystectomy alone.
bladder cancer
Prognosis
The frequency and progression are correlated with grade.
Progression is more common with poorly differentiated lesions and carcinoma in situ that is associated with papillary bladder cancers.
At initial presentation, 50-80% of bladder cancers are superficial that include stage Ta, Tis, T1.
When properly treated, survival rate is excellent at 81%.
5-year survival of patients with T2 and T3 diseases are 50-75% after radical cystectomy.
Long term survival is rare for patients with metastatic disease at time of presentation.
Summary of Diagnosis of Bladder Cancer
Gross or microscopic hematuria.
Irritative voiding symptoms.
Positive urinary cytology in most patients.
Filling defect within bladder noted on imaging.
Bladder Cancer-When to Refer?
Refer all patients to a urologist with persistent hematuria for evaluation of upper urinary track imaging and need for cystoscopy.
Refer when histologic diagnosis and staging require endoscopic resection of cancer.
Metastatic urothelial cancer should be referred to a medical oncologist.
