Which of the following is rarely associated with renal cell carcinoma?
Ans. c. Cushing's syndrome Cushing syndrome is one of the paraneoplastic manifestations of RCC, though its rare. Renal Cell Carcinoma (Gravitz tumor, Hypernephroma. Internist's tumor, Radiologist's tumor)Q * MC malignant tumor of adult kidney and most lethal0 of all malignancies * More common in males, in 6th and 7th decade * Majority are sporadic * Hereditary variants are VHL syndrome. Hereditary clear cell carcinoma and Hereditary papillary carcinoma0 * The tumor usually involve upper poley Risk Factors: * Most significant risk factors are smoking and tobacco chewing0 * Other risk factors are obesity', hypertension, exposure to Asbestos, petroleum products and cadmium, chronic renal failure (especially due to analgesic nephropathy)0 Spread: * The characteristic feature of RCC is a tendency to invade renal vein. Further extension produces a continuous cord of tumor in IVC and even in the right side of the heart * MC route is hematogenous0 * MC sites of distant metastasis are lungs (cannon ball deposits and pulsating secondaries)> bone> liver> brain. * Lymphatic spread occurs when tumor extends beyond renal capsule. Notable features of RCC 1. Encapsulated in spite of being malignant (pseudocapsule) 2. Spontaneous regression 3. Refractoriness to cytotoxic agents 4. Response to biological response modifiers (1L-2 and f FN-alpha) 5. Prolonged period of stable disease Clinical Features * Classical triad of gross hematuria, abdominal mass and pain is seen in 10% cases0 (Too late triad) * MC and consistent presentation is hematuria0. * Other symptoms are fever, weight loss, malaise, acute and non-reducing varicocele, lower limb edema due to IVC obstruction. RCC: Paraneoplastic Syndromes (20%) 1. Raised ESR: MC paraneoplastic manifestation0 2. Hypercalcemia: - Due to the production of PTH-rp - Only paraneoplastic syndrome in which medical therapies are proven useful. 3. Hypertension (Renin production from tumor) 4. Polycythemia (Erythropoietin production from the tumor) 5. Stauffer's syndrome: - Non-metastatic hepatic dysfunction due to raised IL-6deg leading to increased ALP, PT and bilirubin - Hepatic function normalizes after nephrectomy0 6. Others are: Cushing syndrome, hypoglycemia, anemia, gynecomastia, amenorrhea Renal Cell Carcinoma (Gravitz tumor, Hypernephroma, Internist's tumor, Radiologist's tumor) Diagnosis: * Diagnostic IOC: CT (95% accurate)0 * MRI is the most accurate non-invasive investigation for detecting tumor thrombus in renal vein or IVC. Distinguishes tumor thrombus from bland thrombus * Inferior venocavogram0 is most sensitive and specific but invasive means to detect involvement of IVC. * Renal arteriography is done before renal-sparing surgery (partial nephrectomy), but 3-D helical CT is also sufficient. * Specific plain X-ray finding is central calcification. FNAC is not routinely done in RCC, indications are: 1. Suspected secondaries 2. Suspected lymphoma 3. Clinical suspicion of renal abscess 4. To prove pathological diagnosis in disseminated or unresectable disease
Polycythemia
Amyloidosis
Cushing's syndrome
Hypertension