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Mit family translocation renal cell carcinoma

Definition Subtype of renal cell carcinoma harboring gene fusions involving two members of MiT family transcription factors, TFE3 (with Xp11 translocation), and TFEB (with t(6;11) translocation) Clinical features Epidemiology The majority of cases are reported in children and young adults. 40% of pediatric RCCs are Xp11 translocation RCCs The incidence is rare in adults, but it is rising because of increased recognition. 2%–4% of adult RCCs…

Renal medullary carcinoma

Definition Highly aggressive type of renal cell carcinoma centered in the medulla, associated with the sickle cell trait and hemoglobinopathies Clinical features Epidemiology Affects young adults in their second to third decades of life, with male-to-female ratio of 2:1 Most cases are reported in black individuals; some in Hispanic or Brazilian patients. Morphologically identical tumors in patients without the sickle cell trait or hemoglobinopathies are designated…

Collecting duct carcinoma

Definition A highly aggressive type of renal carcinoma originating from collecting duct epithelium; also known as carcinoma of the collecting ducts of Bellini Clinical features Epidemiology Less than 1% of renal cancers Occurs mostly in adults; rare cases reported in children Age range, 13–83 years (mean, 55 years) Males more commonly affected You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy…

Fumarate hydratase-deficient renal cell carcinoma, including hereditary leiomyomatosis and renal cell carcinoma syndrome

Definition A variant of renal cell carcinoma defined by loss of protein expression of fumarate hydratase (FH) on immunohistochemistry; the majority of tumors occur in the setting of hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC) with germline mutation in FH gene Clinical features Epidemiology Rare Mean age, 44 years (range, 21–65) Male to female ratio is 1.9:1 You’re Reading a Preview Become a Clinical Tree…

Chromophobe renal cell carcinoma

Definition A renal cell carcinoma (RCC) subtype comprising large pale and/or eosinophilic cells with prominent cell borders, irregular nuclei, and perineuclear halos Clinical features Epidemiology Five percent of RCCs Mean age, 50–60 years (range, 30–90 years) Male-to-female ratio, 1.5:1 You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

Papillary renal cell carcinoma

Definition A histological subtype of renal cell carcinoma (RCC) with predominant tubulopapillary architecture and characteristic genetic changes; 1.5 cm is the size threshold for papillary RCC; smaller low-grade lesions without fibromuscular pseudocapsule are classified as papillary adenoma. Clinical features Epidemiology Second most common RCC subtype Ten to 15% of all RCCs Age distribution similar to clear cell RCC Male predominance (male-to-female ratio, 2:1 to 4:1) You’re…

Multilocular cystic renal neoplasm of low malignant potential

Definition A tumor composed of multiple cysts lined by low-grade clear cells and no solid or expansile nodules Clinical features Epidemiology Less than 5% of clear cell tumors Age and gender distribution similar to clear cell renal cell carcinomas Usually organ-confined You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in…

Clear cell renal cell carcinoma

Definition The most common histological subtype of renal cell carcinoma (RCC), composed of cells with clear or eosinophilic cytoplasm and a prominent but delicate vascular network Clinical features Epidemiology Two percent of all malignancies and approximately 70% of RCCs Primarily in adults, 6th–7th decades Male predominance (male-to-female ratio, 2:1 to 3:1) You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited…

Renal oncocytoma

Definition Benign renal epithelial neoplasm composed of large round-to-polygonal cells with abundant mitochondria-rich eosinophilic cytoplasm Clinical features Epidemiology 4%–8% of adult renal epithelial neoplasms in surgical pathology Wide range of age distribution with a peak incidence in the seventh decade of life Male-to-female ratio of 2:1 Rare cases are associated with Birt-Hogg-Dube syndrome You’re Reading a Preview Become a Clinical Tree membership for Full access and…

Papillary adenoma

Definition Epithelial tumor with tubulopapillary architecture, low nuclear grade, and a diameter of 15 mm or less Clinical features Epidemiology Increased incidence with age No gender predilection You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

Infectious interstitial nephritis

Definition Chronic infection of the renal parenchyma by a specific infectious agent Clinical features Epidemiology Uncommon, but more frequent in immunocompromised patients and transplant recipients. Common bacteria and fungi are the most frequent causative agents during the early post-transplantation period. Viruses and tuberculosis are more common in the late period. Up to 20% of extrapulmonary tuberculosis cases involve the genitourinary system. You’re Reading a Preview Become…

Acute interstitial nephritis

Definition A clinicopathological entity characterized by inflammation in the renal interstitium with various etiologies, often secondary to an allergic reaction to a drug Clinical features You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

Acute kidney injury (acute tubular necrosis)

Definition A clinicopathological entity with pathological changes in the renal tubules when ischemic or toxic injuries affect the kidney Clinical features Epidemiology Acute kidney injury (AKI) is the most common cause of acute renal failure. The precipitating events include ischemic episodes and endogenous or exogenous nephrotoxins. Ischemia-induced AKI represents approximately 60% of the cases. You’re Reading a Preview Become a Clinical Tree membership for Full access…

Malignant nephrosclerosis

Definition A clinicopathological syndrome characterized by a thrombotic microangiopathy induced by severe hypertension (systolic pressure greater than 220 mm Hg or diastolic pressure greater than 120 mm Hg) Clinical features Epidemiology The average age at diagnosis is 40 years, and men are affected more often than women. Cigarette smokers, African Americans, and patients with underlying chronic renal disease are at higher risk. Fifty percent have a…

Benign nephrosclerosis

Definition Pathological changes in the kidney induced by chronic mild to moderate hypertension Clinical features Epidemiology Hypertension affects 20%–40% of the population in Western society. Vascular diseases of the kidneys can be both a cause and a consequence of hypertension. Hypertensive nephrosclerosis is fourfold more common in African Americans than in Caucasians. Diabetes mellitus increases the incidence and severity of the lesions seen in benign nephrosclerosis.…

Renal papillary necrosis

Definition Coagulative necrosis involving the papillae of the renal medulla, classically associated with a number of underlying conditions, all of which predispose the patient to renal medullary ischemia Clinical features You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

Xanthogranulomatous pyelonephritis

Definition A destructive granulomatous inflammatory process of renal parenchyma as the result of long-term urinary tract obstruction and infection Clinical features Epidemiology Seventy percent of patients are female. Ages range widely, from newborn to elderly. Urine cultures most frequently show Escherichia coli and Proteus mirabilis. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are…

Chronic obstructive pyelonephritis

Definition Pathologic changes secondary to chronic obstruction of the urinary outflow Clinical features Epidemiology Obstruction is more common in males during childhood (ureteropelvic obstruction, posterior urethral valves, and other congenital anomalies), in females during adulthood (gynecologic diseases), and in males at an older age (prostatic diseases). Increased pressure, reflux of urine with leakage into the interstitium, and ischemia of the inner medulla are key factors promoting…

Chronic nonobstructive pyelonephritis

Definition Pathological changes observed in the kidney as the result of vesicoureteral reflux (VUR), intrarenal reflux, and repeated UTIs; also termed reflux nephropathy Clinical features Epidemiology VUR occurs in approximately 1% of children and is found in 30%–50% of children investigated for UTIs. Siblings of children affected with VUR are at higher risk. Of the patients receiving dialysis, 11%–20% have a diagnosis of chronic pyelonephritis not…

Acute pyelonephritis

Definition Acute suppurative inflammation of the kidney caused by bacterial infection, due most commonly to ascent from a lower urinary tract infection and less commonly to hematogenous spread in septic patients Clinical features Epidemiology Common, with 15–17 cases annually per 10,000 population in women and 3–4 cases per 10,000 in men Incidence varying with age A trimodal distribution in females, with an elevated incidence in girls…