Notes
Urinary Tract Tumors
Sections
Here we'll learn about urinary tract tumors.
Renal cell carcinoma
Accounts for approximately 90% of all primary renal neoplasms.
Tumors arise from tubule epithelial cells in the renal cortex; they tend to occur at the renal poles, and can range from 1 cm to several times the size of the kidney.
Signs, Symptoms, Complications:
Renal cell carcinoma is often asymptomatic until it reaches an advanced stage, at which point patients may present with hematuria, abdominal masses and pain, weight loss, and anemia.
Another sign to look for is a non-reducing right-sided varicocele, which is an enlargement of the veins that drain the testis. Varicoceles are caused by right renal vein occlusion, which drains the right testicular venous system.
Paraneoplastic disease, including hypertension, hypercalcemia, and polycythemia, develops in a minority of patients.
We need to look for metastases, particularly in the lung, liver, brain, and bone.
Be aware that renal cell carcinoma can trigger thrombus formation in the renal vein or inferior vena cava and disseminate hematogenously.
Risk factors for renal cell carcinoma include:
- Acquired polycystic kidneys (i.e., in dialysis patients), cigarette smoking, hypertension, diabetes, chronic analgesic use, and exposure to various chemicals, particularly trichloroethylene.
- Genes associated with renal cell carcinoma include VHL and PBRM-1; both are tumor suppressor genes located on chromosome 3.
- Black men in their 60's and 70's are at higher risk of renal cell carcinoma.
Patients with the familial syndrome Von Hippel Lindau have an increased risk of renal cell carcinoma
- Von Hippel Lindau is an autosomal dominant disorder characterized by the growth of cysts and tumors in the kidneys, CNS, retina, reproductive organs, pancreas, and adrenal glands.
Clear-cell renal carcinoma:
The most common subtype of renal cell carcinoma is clear-cell renal cell carcinoma, followed by the papillary and chromophobe subtypes.
Clear-cell renal cell carcinoma is characterized by strands of fibrosis weaving among collections of polygonal clear cells filled with lipids and carbohydrates, which give the tumor a yellowish color.
Diagnosis: We can diagnose renal cell carcinoma with contrast CT or MIR;
Treatments: include partial or radical nephrectomy with possible chemotherapy.
Research currently focuses on immune-checkpoint therapies, because renal cell cancer cells induce apoptosis of cytotoxic lymphocytes.
Bladder carcinomas
Bladder cancer is the most common neoplasm of the upper urinary tract and the 7th most common cancer worldwide.
It is most often diagnosed in males over 60 years old.
Signs, Symptoms, & Complications:
Hematuria, dysuria, and burning while urinating.
We also need to watch for urinary tract obstructions that can lead to hydronephrosis and infection.
Unfortunately, bladder cancer commonly reoccurs.
We need to watch for metastasis, particularly to the lymph nodes, lungs, liver, and bone.
Diagnosis:
We can use cystoscopy, imaging, and biopsy to diagnose bladder cancer.
Treatments
Depend on how invasive the cancer is: If there is no muscularis invasion, we can use endoscopic resection intravesical therapy. If the muscularis has been invaded, we need to perform cystectomy, possibly with chemotherapy.
Urothelial cell carcinoma is the most common form of bladder cancer (formerly called transitional cell carcinoma), which accounts for approximately 90% of all bladder cancers.
This form of cancer can affect any of the structures lined by urothelial cells: the urethra, bladder, ureters, and renal pelvis.
The mucosal layer lines these structures and comprises transitional cells and lamina propria; carcinoma originates in the transitional cells.
Lesions can be papillary or flat, and start out in the mucosa, but can invade deeper tissues.
Key risk factors include smoking, exposure to paint, petroleum, and certain dyes; urothelial cell carcinoma is also associated with Lynch syndrome (aka hereditary nonpolyposis colorectal cancer).
Squamous cell bladder carcinoma is less common than urothelial cell cancer; in these patients, chronic bladder irritation or infection triggers transitional cell transformation to squamous cells.
A key risk factor is infection with schistosomiasis.
Renal oncocytomas
Common, benign neoplasms that are most often found in patients over 50 years old.
We often see these tumors in Birt-Hogg-Dubé syndrome (BHD is a genetic condition that primarily effects the skin, but also predisposes to benign tumors in the lungs and kidneys).
On gross inspection, we'll see a mahogany-brown mass with a central stellate star;
Histologically, these tumors are characterized by nests of cells with prominent nucleoli and eosinophilic cytoplasm.
Patients have excellent prognosis, but we can remove the tumors to prevent metastasis to the liver and bone.
Papillary adenomas are another common benign neoplasm; they are often seen in older patients with end-stage renal disease.
Nephroblastomas, aka, Wilms tumors
The most common renal cancer in children ages 2-4; they are the most common pediatric cancer overall.
Signs and Symptoms:
A large, palpable flank mass, hematuria, and hypertension.
Prognosis depends in part on the histopathology:
- "Favorable" histology is characterized by embryonic glomerular structures with blastema, epithelial tubules, and mesenchyme (stroma).
- "Unfavorable" histology is characterized by more anaplasia; we'll see hyperchromatic, enlarged pleomorphic nuclei with abnormal mitotic figures.
Nephroblastomas are associated with several childhood syndromes caused by mutations on chromosome 11, including:
- WAGR complex (Wilms tumor, Aniridia, Genitourinary malformation, Range of developmental delays; WT1 deletion)
- Denys-Drash syndrome (Wilms tumor, diffuse mesangial sclerosis, male pseudohermaphroditism.; WTI mutation)
- Beckwith-Wiedemann syndrome (Wilms tumor, macroglossia, organomegaly, hemihyperplasia, omphalocele; alterations in genes that control body growth on chromosome 11).
With favorable histology, prognosis is good; treatment includes surgery and radiation therapy.
Board Review
Renal Cell Carcinoma
Getting ready for boards? Review these concise, bulleted high yield reviews for your exam.
USMLE & COMLEX-USA
Nurse Practitioner (NP)
Physician Assistant (PA)
Internal Medicine (ABIM)
References
- Angori, Silvia, João Lobo, and Holger Moch. "Papillary Renal Cell Carcinoma: Current and Controversial Issues." Current Opinion in Urology 32, no. 4 (July 2022): 344–51. https://doi.org/10.1097/MOU.0000000000001000.
- Coorens, Tim HH, Taryn D Treger, Reem Al-Saadi, Luiza Moore, Maxine GB Tran, Thomas J Mitchell, Suzanne Tugnait, et al. "Embryonal Precursors of Wilms Tumor." Science (New York, N.Y.) 366, no. 6470 (December 6, 2019): 1247–51. https://doi.org/10.1126/science.aax1323.
- Golla, Vishnukamal, Andrew T. Lenis, Izak Faiena, and Karim Chamie. "Intravesical Therapy for Non-Muscle Invasive Bladder Cancer—Current and Future Options in the Age of Bacillus Calmette-Guerin Shortage." Reviews in Urology 21, no. 4 (2019): 145–53.
- Gray, Richard E, and Gabriel T Harris. "Renal Cell Carcinoma: Diagnosis and Management." RENAL CELL CARCINOMA 99, no. 3 (2019): 6.
- Jonasch, Eric, Cheryl Lyn Walker, and W. Kimryn Rathmell. "Clear Cell Renal Cell Carcinoma Ontogeny and Mechanisms of Lethality." Nature Reviews. Nephrology 17, no. 4 (April 2021): 245–61. https://doi.org/10.1038/s41581-020-00359-2.
- Kaseb, Hatem, and Narothama R. Aeddula. "Bladder Cancer." In StatPearls. Treasure Island (FL): StatPearls Publishing, 2022. http://www.ncbi.nlm.nih.gov/books/NBK536923/.
- Leslie, Stephen W., Hussain Sajjad, and Patrick B. Murphy. "Wilms Tumor." In StatPearls. Treasure Island (FL): StatPearls Publishing, 2022. http://www.ncbi.nlm.nih.gov/books/NBK442004/.
- Linehan, W. Marston, and Christopher J. Ricketts. "The Cancer Genome Atlas of Renal Cell Carcinoma: Findings and Clinical Implications." Nature Reviews Urology 16, no. 9 (September 2019): 539–52. https://doi.org/10.1038/s41585-019-0211-5.
- Marcela, Díaz-Montero C., Brian I. Rini, and James H. Finke. "The Immunology of Renal Cell Carcinoma." Nature Reviews. Nephrology 16, no. 12 (December 2020): 721–35. https://doi.org/10.1038/s41581-020-0316-3.
- Mendhiratta, Neil, Peter Muraki, Anthony E. Sisk, and Brian Shuch. "Papillary Renal Cell Carcinoma: Review." Urologic Oncology: Seminars and Original Investigations 39, no. 6 (June 2021): 327–37. https://doi.org/10.1016/j.urolonc.2021.04.013.
- Merck Manuals Professional Edition. "Renal Cell Carcinoma - Genitourinary Disorders." Accessed October 3, 2022. https://www.merckmanuals.com/professional/genitourinary-disorders/genitourinary-cancers/renal-cell-carcinoma.
- "Renal Neoplasia - ClinicalKey." Accessed October 4, 2022. https://www-clinicalkey-com.proxy.ulib.uits.iu.edu/#!/content/book/3-s2.0-B9780323721639000198.
- "Risk Factors for Kidney Cancer." Accessed October 3, 2022. https://www.cancer.org/cancer/kidney-cancer/causes-risks-prevention/risk-factors.html.
- Servaes, Sabah E., Fredric A. Hoffer, Ethan A. Smith, and Geetika Khanna. "Imaging of Wilms Tumor: An Update." Pediatric Radiology 49, no. 11 (October 2019): 1441–52. https://doi.org/10.1007/s00247-019-04423-3.
- "What Are Wilms Tumors?" Accessed October 3, 2022. https://www.cancer.org/cancer/wilms-tumor/about/what-is-wilms-tumor.html.
- Williamson, Sean R. "Clear Cell Papillary Renal Cell Carcinoma: An Update after 15 Years." Pathology 53, no. 1 (January 2021): 109–19. https://doi.org/10.1016/j.pathol.2020.10.002.
- Yamada, Yasutaka, Sho Sugawara, Takayuki Arai, Satoko Kojima, Mayuko Kato, Atsushi Okato, Kazuto Yamazaki, Yukio Naya, Tomohiko Ichikawa, and Naohiko Seki. "Molecular Pathogenesis of Renal Cell Carcinoma: Impact of the Anti-Tumor MiR-29 Family on Gene Regulation." International Journal of Urology 25, no. 11 (2018): 953–65. https://doi.org/10.1111/iju.13783.
- Zhang, Shichao, Erdong Zhang, Jinhua Long, Zuquan Hu, Jian Peng, Lina Liu, Fuzhou Tang, Long Li, Yan Ouyang, and Zhu Zeng. "Immune Infiltration in Renal Cell Carcinoma." Cancer Science 110, no. 5 (2019): 1564–72. https://doi.org/10.1111/cas.13996.