RENAL PATHOLOGY 4 Flashcards

This deck covers Renal Neoplasms

1
Q

This malignant renal neoplasm is closely resembles a benign neoplasm microscopically. What is the malignant neoplasm?

A

Chromophobe carcinoma - closely resembles oncocytoma. The two are distinguished by the presence of a perinuclear halo in chromophobe carcinoma (see image attached)

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2
Q

Is this a feature of nephroblastoma or neuroblastoma?

elevated urine levels of the metabolites vanillylmandelic acid [VMA] and homovanillic acid [HVA]).

A

Neuroblastoma

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3
Q

most common site for metastasis in RCC

A

Lungs (cannon ball lesions)

The example shown here reveals multiple, bilateral, rounded soft tissue density masses on the chest x-ray.

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4
Q

Cell of origin of this tumor

A

Papillary RCC - cell of origin is distal convoluted tubules

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5
Q

Clinical consequence of invasion of left renal vein by RCC

A

Left renal vein invasion→ impaired drainage of the left spermatic vein→ left sided varicocele

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6
Q

precursor lesions of nephroblastoma

A

nephrogenic rests

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7
Q

Neuroblastoma/ nephroblastoma?

A

Neuroblastoma

The attached image shows blueberry muffin baby with multiple light bluish subcutaneous nodules over trunk and extremities due to cutaneous metastases from neuroblastoma

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8
Q

Clinical significance of renal adenomas

A

Close resemblance to low grade papillary RCC; potentially malignant

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9
Q

Identify this syndrome associated with Wilms tumor:

Wilms tumor, aniridia, genitourinary anomalies, and intellectual disability (retardation)

A

WAGR

Wilms tumor, aniridia, genitourinary anomalies, and intellectual disability (retardation)

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10
Q

Identify the tumor described

This benign renal neoplasm (see attached image) arises form intercalated cells of the collecting duct and

A

Oncocytoma

Gross image shows: tan or mahogany brown, relatively homogeneous, and usually well encapsulated with a central scar

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11
Q

Underlying molecular basis of neuroblastoma

A

amplification of the N-MYC oncogene

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12
Q

Identify this syndrome associated with Wilms tumor:

organomegaly, macroglossia, hemihypertrophy, omphalocele, and adrenal cytomegaly

Loss of maternal imprinting/ uniparental paternal disomy of IGF-2

Localized to WT 2 locus on 11p15.5

A

Beckwith-Wiedemann syndrome (BWS)

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13
Q

most common primary renal tumor of childhood

A

Wilms Tumor (Nephroblastoma)

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14
Q

What is the clinical significance of detecting amplification of the NMYC oncogene in neuroblastoma?

A

The presence of MYCN amplification “bumps” the tumor to the “high”-risk category, irrespective of age, stage, or histology

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15
Q

Most common type of renal cell carcinoma (RCC)

A

Clear cell

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16
Q

See attached microscopic image and identify the tumor

A

Oncocytoma

Observe the large eosinophilic cells having small, round, benign-appearing nuclei that have large nucleoli, intensely pink cytoplasm due to the abundant mitochondria

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17
Q

How does having VHL syndrome lead to tumor and cyst development?

A

Deletion of VHL gene → impaired ubiquitination and elimination of hypoxia-inducible factor 1α → loss of function → tumor and cyst development

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18
Q

What is the reason for the yellow appearance of clear cell carcinoma?

A

prominent lipid accumulations in tumor cells.

19
Q

Most significant risk factor for renal cell carcinoma

A

Smoking

20
Q

Identify the tumor described

5 year old, abdominal mass, hematuria

See attached image

A

Nephroblastoma (Wilms tumor)

21
Q

25-50% cases of angiomyolipomas are associated with this syndrome

A

Tuberous sclerosis - hypomelanotic (ash leaf) macules, angiofibromas, cardiac rhabdomyomas, Shagreen patches.

22
Q

List 2 poor prognostic features for renal cell carcinoma

A

Invasion of the renal vein

Sarcomatoid change (histologic)

23
Q

cell of origin of oncocytoma

A

intercalated cells of collecting ducts

24
Q

most critical determinant of prognosis in Wilms

A

Anaplastic histology

25
Q

Histopathologic features of angiomyolipoma

A

mixtures of well-differentiated adipose tissue, smooth muscle and thick-walled vessels

26
Q

See attached microscopic image.

Identify the tumor and the reason for this appearance

A

Clear cell type of renal cell carcinoma.

Cells appear clear due to prominent lipid accumulations in tumor cells

Description: Individual tumor cells are rounded or polygonal, abundant clear or granular cytoplasm, which contains glycogen and lipids, delicate branching vasculature

27
Q

Cell of origin of clear cell carcinoma

A

proximal tubular epithelium

28
Q

Identify this paraneoplastic syndrome associated with RCC

hepatic abnormalities with no evidence of hepatic metastases

Elevations of AST, ALT, ALP, and PT , gammaglobulin and bilirubin (any 3 for diagnosis

Secretes hepatotoxins, lysosomal enzymes, IL-6

A

Stauffer’s Syndrome

29
Q

Identify this syndrome associated with Wilms tumor:

Wilms tumor, Diffuse mesangial sclerosis (early-onset nephrotic syndrome), Dysgenesis of gonads (male pseudohermaphroditism).

Risk of gonadoblastomas

A

Denys-Drash syndrome

30
Q

4 major paraneoplastic syndromes associated with RCC

A
  1. Hypercalcemia
  2. Hypertension
  3. Polycythemia
  4. Stauffer’s syndrome
31
Q

Why is angimyolipoma clinically significant?

A

susceptibility to spontaneous hemorrhage

32
Q

What are the IHC markers for this tumor presenting as an abdominal/ flank mass in a child?

A

This is a neuroblastoma.

Neuron specific enolase, synaptophysin, chromogranin , neurofilament

33
Q

Syndrome associated with both familial and sporadic forms of renal cell carcinoma

A

§Von Hippel-Lindau (VHL) syndrome

34
Q

Syndromes associated with Wilms tumor

A
  1. WAGR syndrome
  2. Denys-Drash syndrome
  3. Beckwith-Wiedemann syndrome (BWS)
35
Q

Cell of origin of angiomyolipomas

A

perivascular epithelioid cell (PEC)

36
Q

Risk factors for this tumor?

A

This is urothelial carcinoma.

smoking (most common), aromatic amines (aniline dyes), and cyclophosphamide

37
Q

Most cases of Wilms tumor are sporadic or familial?

A

90% are sporadic

38
Q

Neuroblastoma/ nephroblastoma?

A

small, primitive-appearing cells with dark nuclei, scant cytoplasm, and poorly defined cell borders and Homer-Wright pseudorosettes (tumor cells concentrically arranged about a central space filled with neuropil)

This is a neuroblastoma

39
Q

Microscopic features of Wilms tumor

A

Blastemal – sheets of small blue cells

Stromal - spindle cells with smooth muscle /fibroblast differentiation

Epithelial- abortive tubules or glomeruli

40
Q

Constituents of VHL syndrome

A

Remember HIPPEL

Hemangioblastoma

Increased risk of renal cell carcinoma

Pheochromocytoma

Pancreatic lesions (cysts, cystadenomas , and neuroendocrine tumors )

Eye Lesions (retinal angiomas or hemangioblastomas)

41
Q

Cell of origin of neuroblastoma

A

neural crest origin

42
Q

Significance of identifying TP53 mutations in Wilms tumor

A

TP53 mutations are associated with :

an especially poor prognosis

Relative unresponsiveness to cytotoxic chemotherapy.

distinctive anaplastic histologic appearance

43
Q

significance of identifying nephrogenic rests

A

patients are at an increased risk of developing Wilms tumors in the contralateral kidney and require frequent and regular surveillance

44
Q

Most common presenting clinical feature for this tumor

A

Hematuria