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level: Ch1: Kidney Pathologies

Questions and Answers List

level questions: Ch1: Kidney Pathologies

QuestionAnswer
How can renal masses be classified?Malignant (carcinoma, lymphoma, leiomyosarcoma, liposarcoma, rhabdomyosarcoma, metastasis, invasion, wilm's tumor) Benign (simple cyst, angiomyolipoma, oncocytoma) Inflammatory (abscess, pyelonephritis, Xanthogranulomatous pyelonephritis, TB, infected cyst, rheumatic granuloma.)
How is dx of renal mass?History : flank pain , hematuria , HTA.. P.E : abdominal mass , Lab studies : anemia , hypercalcemia …. Radio: US (differentiate cyst and mass), CT (very important, delineates nature of renal mass), IVP, MRI, renal arteriography (rare usage). Renal Mass biopsy CT/MRI guided (indication [small mass to r/o malignancy, sus of renal abscess or RCC differentiation from metastatic malignant disease/renal lymphoma)
How do we differentiate between benign and renal cyst?The differentiation between a benign   renal cyst and a cystic RCC remains one of the more common and difficult problems in   renal imaging. Bosniak has developed a useful classification scheme that divides renal cystic lesions into four categories that are distinct from one another in terms of the likelihood of malignancy This radiological differentiation is based on the CTscan findings or MRI (regularity of the wall of the cyst presence or absence of calcifications, septations, enhancement more than 20 housefield unit after IVcontrast)
How is bosniak classification of renal masses?.
What are bengin renal tumors?1-Benign   Renal Cyst :most common benign renal mass -Exist in 50% of normal population above age 50 2-Renal Cortical Adenoma: The diagnosis of renal adenoma remains controversial, with many believing that all solid renal epithelial-derived masses are potentially malignant and should be treated as such. 3-Oncocytoma :represents 3% to 7% of all solid   renal masses. A central scar is commonly found, but prominent necrosis or hypervascularity is lacking. In 3 to 30% , oncocytoma coexist with RCC 4-angiomylolipoma: varying amounts of mature adipose tissue, smooth muscle, and thick-walled vessels Approximately 20% of AMLs are found in patients with tuberous sclerosis syndrome (TS) (autodominant - retard epilepsy, adenoma - CT most reliable dX)
How is tx of benign renal masses?1.Less than 4cm : a/asymptomatic observation b/ symptomatic embolization 2.More than 4 cm :nephrectomy 3.Embolization in case of solitary kidney,bilateral disease , renal insufficiency and a disease affecting the renal function
What are other renal tumors?Other Benign Tumors fibromas, lipomas, lymphangiomas, and hemangiomas Leiomyoma a slow-growing, benign neoplasm, can arise from the capsule or peripelvic tissues and, less often, from the renal vein Multiloculated Cystic Nephroma
How is incidence of RCC (renal cell carcinoma)?Incidence RCC, which accounts for 3% of all adult malignancies, is the most lethal of the urologic cancers RCC in childhood is uncommon, representing only 2.3% to 6.6% of all renal tumors in children Etiology (Established: Tobacco, Obesity and HTA, putative: lead and other chemicals)
What is familial renal cell carcinoma?1/von Hippel-Lindau +++: RCC develops in about 50% of patients with vonHippel-Lindau disease and is distinctive for its early age at onset (often in the third, fourth, or fifth decade of life) and for its bilateral and multifocal involvement due to a mutation in VHL tumor suppressor gene 2/Hereditary papillary RCC 3/Familial Leiomyomatosis and RCC
How is clinical presentation of renal masses?Many renal masses remain asymptomatic and nonpalpable until they are advanced -50% incidental findings on imaging - The classic triad of flank pain, gross hematuria, and palpable abdominal mass is now rarely found -Paraneoplastic syndromes are found in 20% of patients with RCC
How is clinical presentation of RCC?Incidental 50% Local Tumor Growth (Hematuria, Flank pain, Abdominal mass, Perirenal hematoma) Metastases (Persistent cough, Bone pain, Cervical lymphadenopathy, Constitutional symptoms, Weight loss/fever/malaise) Obstruction of the Inferior Vena Cava (Bilateral lower extremity edema, Nonreducing or right-sided varicocele) Paraneoplastic Syndromes (Hypercalcemia, Hypertension, Polycythemia, Stauffer’s syndrome( abnormal liver function tests))
How is staging of RCC?.
How is radio staging of RCC?The radiographic staging of RCC can be accomplished with a high-quality abdominal CT scan and a routine chest radiograph in most cases. Metastatic evaluation in all cases should include a routine chest radiograph, careful and systematic review of the abdominal and pelvic CT, and liver function tests.
What are prognostic factors of RCC?Pathologic stage has proven to be the single most important prognostic factor for RCC Lymph node involvement has long been recognized as a dire prognostic sign, as it is associated with 5- and 10-year survival rates of 5% to 30% and 0% to 5%, respectively Tumor size :less than 4 cm better prognosis Nuclear grade and histologic subtype
How is tx of localized RCC?Radical Nephrectomy (GOLD STD, laparoscopic/robotic assisted most accepted) Partial Nephrectomy (indication : pt w/bilateral RCC, RCC involving functioning kidney alone, pt affected kidney w/other kidney risk for failure, tumor <4cm most commonly) Surveillance (elderly not fit for surgery/<3cm, AS not advisable if healthy individual) Thermal ablation (cryotherapy or radiofrequency but the local recurrence rates may be somewhat higher than that reported for traditional surgical approaches)
How is tx of locally advanced RCC?1. Inferior Vena Caval Involvement: Involvement of the inferior vena cava with RCC occurs in 4% to 10% of patients 2. For patients with nonmetastatic RCC involving the vena cava, 5-year survival rates of 47% to 69% have been reported after complete surgical therapy 3. Radical nephrecotmy is the gold standard + targeted therapy ( ?)
How is tx of metastatic RCC?Nephrectomy Approximately one third of patients with RCC exhibit metastatic disease at the time of initial presentation Chemotherapy : resistance Radiation Therapy: resistance Immunobiologic Therapy: A review of clinical results in 1714 patients treated with IL-2 monotherapy indicated an overall objective response rate of 15.4% Now , the new era of immunotherapy check point inhibitors of PD -1( present on T cells ) and PD-L1 ( present on the tumor cells) are introduced as second line therapy after the TKI target therapy Targeted therapy Tyrosine kinase inhibitors (TKI): anti angiogenesis most recent ++
How is multimodality therapy of RCC?include (1) initial adjuvant nephrectomy followed by immunotherapy or targeted therapy , (2) initial immunotherapy followed by adjuvant nephrectomy for responders, and (3) nephrectomy and immunotherapyand/or targeted therapy followed by resection of residual or recurrent metastatic lesions.
How is tx of sarcomas of the kidney?- Sarcomas represent 1% to 2% of all malignant renal tumors in adults -Specific findings suggestive of sarcoma rather than RCC include apparent origin from the capsule or perisinuous region, growth to large size in the absence of lymphadenopathy, presence of fat or bone suggestive of liposarcoma or osteosarcoma, and hypovascular pattern on angiography, although one notable exception is the hemangiopericytoma, which is highly vascular -Leiomyosarcoma is the most common histologic subtype of renal sarcoma, accounting for 50% to 60% of such tumors
How is tx of renal lymphoma and leukemia?- Renal involvement with hematologic malignancies, which include the various lymphomas and leukemias, is common—found at autopsy in approximately 34% of patients dying of progressive lymphoma or leukemia. -Renal lymphoma can present as multiple distinct renal masses; as a solitary renal mass, which can be difficult to differentiate from RCC; as diffuse renal infiltration; or as direct invasion of the kidney from enlarged retroperitoneal nodes
How is tx of metastatic tumors?Metastatic tumors are the most common malignancies in the kidney . Autopsy studies have shown that 12% of patients dying of cancer have renal metastases The most common sources of renal metastases include lung(most common), breast, and gastrointestinal cancers; malignant melanoma; and hematologic malignancies