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Renal II > Renal neoplasms > Flashcards

Renal neoplasms Flashcards

1
Q

Benign kidney tumors

A

Renal papillary adenoma
Angiomyolipoma
Oncocytoma

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2
Q

Malignant kidney tumors

A

Renal cell carcinoma
Transitional cell carcinoma of renal pelvis
Wilms tumor/nephroblastoma
Adrenal neuroblastoma

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3
Q

Benign kidney tumor that grossly appears as small cortical pale yellow-gray nodules (<0.5 cm in diameter) that arises from tubular epithelium

A

Renal papillary adenoma

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4
Q

Microscopy of kidney lesion shows complex, branching papillary process

A

Renal papillary adenoma or renal cell adenocarcinoma –> hard to differentiate

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5
Q

Benign tumor of kidney with blood vessels, smooth muscle, and fat. Present in 25-50% of tuberous sclerosis due to loss of function in tumor suppressor gene, TSC1 and TSC2

A

Angiomyolipoma

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6
Q

Syndrome characterized by hamartomatous lesions in the cerebral cortex causing epilepsy and mental retardation, skin lesions (Shagreen patches and ash leaf patches), and other benign tumors (like rhabdomyoma of the heart).

A

Tuberous sclerosis

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7
Q

Benign renal tumor that is clinically important due to tendency to bleed spontaneously

A

Angiomyolipoma

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8
Q

Gross appearance of renal angiomyolipoma

A

Large, yellowish, and firm tumor

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9
Q

Gross morphology of renal oncocytoma

A

Tan or mahogany brownish colored lesion with central scar

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10
Q

Benign kidney tumor that arises from intercalated cells of collecting ducts. Large pink cells on microscopy. Electron microscopy shows cells rich in mitochondria.

A

Oncocytoma

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11
Q

Description of oncocytes

A

Large cells with are very pink

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12
Q

Exposure that has a strong association with renal cell carcinoma

A

Cigarette smoking

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13
Q

Familial variants (4%) of renal cell carcinoma

A

Von Hippel-Lindau disease
Hereditary/familial clear cell carcinoma
Hereditary papillary carcinoma

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14
Q

Normal function of VHL gene

A

Downregulates hypoxia inducing factor-1 –> decreased production of VEGF, PDGF, and IGF-1

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15
Q

Result of abnormal VHL gene in Von Hippel-Lindau disease

A

Increased cell growth and angiogenesis

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16
Q

Manifestations of Von Hippel-Lindau disease not seen in hereditary/familial clear cell carcinoma

A

Hemangioblastomas of cerebellum, cysts, and multiple RCC, other than renal

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17
Q

Mutation in hereditary papillary carcinoma

A

MET proto-oncogene resulting in multiple bilateral kidney tumors

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18
Q

Inheritance of hereditary papillary carcinoma

A

Autosomal dominant

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19
Q

Gene involved in 98% of all clear cell carcinomas, both familial and sporadic

A

VHL gene on chromosome 3p

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20
Q

What is also regulated by VHL gene, other than HIF-1?

A

Expression of EPO

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21
Q

Classifications of renal cell carcinoma

A

Clear cell carcinoma
Papillary carcinoma
Chromophobe renal cell carcinoma (great prognosis)
Collecting/Bellini duct carcinoma

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22
Q

Renal tumor with a propensity to invade the renal V to spread to IVC and right heart

A

Renal cell carcinoma

23
Q

Spherical, bright yellow or gray-white, kidney lesion with sharply defined margins on upper or lower pole. Accompanied by hemorrhage and necrosis.

A

Renal cell carcinoma

24
Q

Microscopic features of clear cell renal carcinoma

A

Clear cells in nests, cords, and sheets with PAS positive cytoplasm filled with lipid and glycogen and delicate branching vasculature.

25
Q

Microscopic features of renal papillary carcinoma

A

Papillae with foam cells in the fibrovascular cores
Psammoma bodies may also be present

26
Q

Classic triad of clinical features of renal cell carcinoma seen in 10%

A

Costovertebral pain
Palpable mass
Hematuria

27
Q

Paraneoplastic syndromes associated with renal cell carcinoma

A

Polycythemia
Hypercalcemia
HTN
Hepatic dysfunction
Cushing syndrome
Eosinophilia
Leukemoid reaction
Amyloidosis
Feminization or masculinization

28
Q

Associated with increased incidence of transitional cell carcinoma of renal pelvis

A

Balkan nephropathy
Analgesic nephropathy

29
Q

Papillary, nodular, or flat kidney lesion. Microscopy shows delicate papillae lined by malignant urothelial cells (>7 thick). May be low or high grade depending on mitosis, polarity, or pleomorphism.

A

Transitional cell carcinoma of renal pelvis

30
Q

Why does TCC of renal pelvis become symptomatic early?

A

Due to its location

31
Q

Symptoms of TCC of renal pelvis

A

Hematuria
Obstruction causing palpable hydronephrosis and flank pain

32
Q

Concomitant tumor seen with TCC of renal pelvis

A

Bladder tumor

33
Q

Four groups of congenital malformations associated with Wilms tumor

A

WAGR syndrome
Denys-Drash syndrome
Beckwith-Weidemann syndrome
Mutations in gene encoding beta-catenin

34
Q

Syndrome associated with germline deletion of 11p13 (WT1)

A

WAGR syndrome

35
Q

Features of WAGR syndrome

A

Wilms tumor
Aniridia
Genital abnormalities
Mental retardation

36
Q

Mutation associated with Denys-Drash syndrome

A

Germline abnormalities in WT1 affecting DNA-binding properties

37
Q

Features of Denys-Drash syndrome

A

Gonadal dysgenesis
Early onset nephropathy –> diffuse mesangial sclerosis

38
Q

Syndrome associated with Wilms tumor and increased risk for gonadoblastoma

A

Denys-Drash syndrome

39
Q

Protein critical for normal renal and gonadal development that is associated with Wilms tumor when mutated

A

WT1

40
Q

Syndrome associated with non-classical example of tumorigenesis involving WT2 on chromosome 11p15.5

A

Beckwith-Weidemann syndrome

41
Q

Characteristics of Beckwith-Weidemann syndrome

A

Organomegaly
Macroglossia
Risk for other tumors

42
Q

Pathway affected by beta-catenin mutation in Wilms tumor

A

WNT pathway

43
Q

Precursor lesion of Wilms tumor

A

Nephroblastoma

44
Q

Nephrogenic rests that share genetic alterations with Wilms tumor and are considered pre-neoplastic. When present, increase risk of development of Wilms tumor in contralateral kidney

A

Nephroblastoma

45
Q

Large, solitary, well-circumscribed renal mass that is soft and tan-gray, with a foci of hemorrhage and necrosis. May also have cysts.

A

Wilms tumor

46
Q

Classic triphasic microscopic features of Wilms tumor

A

Epithelial - abortive glomeruli and tubules
Blastemal - small blue cells
Stromal - spindle cells and myxoid areas

47
Q

Describe anaplasia, sometimes seen in Wilms tumor

A

Large hyperchromatic pleomorphic nuclei with abnormal mitosis due to p53 mutation

48
Q

Significance of presence of anaplasia in Wilms tumor

A

Resistant to chemotherapy

49
Q

Features associated with poor prognosis in Wilms tumor

A

Anaplasia
Loss of genetic material on chromosomes 11q and 16q
Gain in chromosome 1q

50
Q

Malignancies with increased risk after radiation for Wilms tumor

A

Bone and soft tissue sarcomas
Leukemias and lymphomas
Breast cancer

51
Q

Tumor composed of small, primitive appearing cells that have dark nuclei and scant cytoplasm, with poorly defined cell borders growing in sheets with pleomorphism. Background is often faintly eosinophilic fibrillar material. Arranged in Homer-Wright pseudorosettes

A

Adrenal neuroblastoma

52
Q

Metabolites in the synthesis of epinephrine that are often elevated in urine with neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and pheochromocytoma.

A

VMA and HVA

53
Q

What is a sign of poor prognosis in adrenal neuroblastoma?

A

Absence of VMA production

54
Q
A

Renal II (15 decks)

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