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Renal Cell Carcinoma: (RCC)

   -   Adenocarcinoma of the kidney (most often used term)
   -   It is also known as hyperne...
Paraneoplastic Syndromes:
   - erythrocytosis
   - hypercalcemia
   - hypertension
   - nonmetastatic hepatic dysfunction
...
-   Radical nephrectomy is the standard:
   -   Involves the removal of the kidney and its enveloping fascia (Gerota’s), i...
-   exogenous estrogen administration to the mother during pregnancy associated with increased
       risk
   -   acquired...
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[ ] Renal Cell Carcinoma.

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[ ] Renal Cell Carcinoma.

  1. 1. Renal Cell Carcinoma: (RCC) - Adenocarcinoma of the kidney (most often used term) - It is also known as hypernephroma and clear cell carcinoma of the kidney - In 2000 an estimated 36,000 new cases were diagnosed with 12,000 resulting in death - RCC comprises about 3% of adult tumors - RCC makes up about 85% of all primary malignant renal tumors Who gets RCC? - most commonly occurs in the 5th and 6th decades of life - male:female ratio- 2:1 - the incidence is equivalent between whites and blacks - Hispanics have rates more than 1/3 higher than white Americans Etiology of RCC: - the cause of RCC is unknown - cigarette smoking is only risk factor consistent linked to RCC - roles of cellular oncogenes and tumor suppressor genes is still up for discussion - in patients with acquired cystic disease of the kidneys in uremic states, 4-9% develop RCC Pathology of RCC: - RCC originates from the proximal renal tubular epithelium - RCC originates in the cortex - Average diameter is 7-8 cm - There is equal frequency of RCC in either kidney - It is randomly distributed in the upper and lower poles - Types of RCC and prevalence- - clear cell type- 25% - granular cell type- 25% - mixed cell types- 50% - sarcomatoid variety- 2% Pathogenesis of RCC: - most common site of metastasis is the lung - liver, bone, ipsilateral adjacent lymph nodes, adrenal gland, and the opposite kidney are the sites of disease spread - few patients today have evidence of metastases at presentation Clinical findings of RCC: - classic triad of gross hematuria, flank pain, and a palpable mass occurs in 10% of patients - 60% of patients present with gross or microscopic hematuria - 20% may present with pain or an abdominal mass - dyspnea, cough, and pain secondary to metastatic disease are not common - 30% detected incidentally
  2. 2. Paraneoplastic Syndromes: - erythrocytosis - hypercalcemia - hypertension - nonmetastatic hepatic dysfunction RCC may produce other biologically active products that result in syndromes- - ACTH→ Cushings syndrome - enteroglucagon→ protein enteropathy - prolactic→ galactorrhea - insulin→ hypoglycemia - gonadotropins→ gynecomastia and decreased libido Work up for RCC: - UA, C&S, CBC, SMA-20 - IVP- when patient presents with hematuria - Renal US- 98% accurate in defining simple cysts - CT abdomen- the mass becomes enhanced with the use of IV contrast - MRI- if suspicion of renal vein or venal caval tumor - CXR- PA or lateral - Fine needle aspiration (FNA)- - has a limited role - used to differentiate primary RCC from metastases - used to diagnose masses in patients with solitary kidneys - used when a histologic diagnosis is needed prior to nonsurgical rx - used to distinguish complicated cyst vs RCC Tumor Staging: Size- T1: tumor 2.5 cm or less T2: tumor more than 2.5 cm T3: tumor extends into major veins, adrenal, and perinephric tissues - T3a: invades adrenal but not beyoned Gerota’s fascia - T3b: extends into renal vein or vein cava T4: tumor invades beyond Gerota’s fascia Regional lymph nodes (N)- N1: metastases in single lymph node 2 cm or less N2: metastases in single or multiple lymph nodes- none > 5 cm N3: metastases in lymph node > 5 cm Distant metastases (M)- M0: No distant metastases M1: Distant metastases Treatment- Localized disease:
  3. 3. - Radical nephrectomy is the standard: - Involves the removal of the kidney and its enveloping fascia (Gerota’s), including the ipsilateral adrenal and proximal ureter Alternative treatments: - Nephron sparing surgery- partial nephrectomy - Laprascopic nephrectomy Treatment for metastatic disease: - radical nephrectomy for palliation - spontaneous regression of RCC mets after nephrectomy reported in less than 1% - solitary metastases may be candidates for combined nephrectomy and resection of metastases - hormonal therapy- often gives poor response - chemotherapy- often gives poor response - immunotherapy- interferons, IL-2, monoclonal antibiotics Follow up care: - H&P - CXR - CBC - Serum chemistries - US, CT and bone scans when clinically indicated Prognosis: 5 year survival rates: T1- 90% T2- 70% T3a- 70% T3b- 30% T4- <20% Tumors of the Testis: - the lifetime probability of deverloping testicular cancer is 0.2% for a white male in the USA - overall 5-year survival rates: 95% Etiology of testicular cancer: - cause is unknown - the strongest association has been in cryptorchid testis - 7-10% of testis tumors in patients with a history of cryptorchidism - risk of malignancy - intra-abdominal testes: 1 in 20 - inguinal testes: 1 in 80
  4. 4. - exogenous estrogen administration to the mother during pregnancy associated with increased risk - acquired factors - trauma - infection Classification: - Semimoma- 35% - Nonaseminomatous germ cell tumors (NSGCT) - embryonal (20%)

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