RENAL PATHOLOGY 4 Flashcards
This deck covers Renal Neoplasms
This malignant renal neoplasm is closely resembles a benign neoplasm microscopically. What is the malignant neoplasm?
Chromophobe carcinoma - closely resembles oncocytoma. The two are distinguished by the presence of a perinuclear halo in chromophobe carcinoma (see image attached)
Is this a feature of nephroblastoma or neuroblastoma?
elevated urine levels of the metabolites vanillylmandelic acid [VMA] and homovanillic acid [HVA]).
Neuroblastoma
most common site for metastasis in RCC
Lungs (cannon ball lesions)
The example shown here reveals multiple, bilateral, rounded soft tissue density masses on the chest x-ray.
Cell of origin of this tumor
Papillary RCC - cell of origin is distal convoluted tubules
Clinical consequence of invasion of left renal vein by RCC
Left renal vein invasion→ impaired drainage of the left spermatic vein→ left sided varicocele
precursor lesions of nephroblastoma
nephrogenic rests
Neuroblastoma/ nephroblastoma?
Neuroblastoma
The attached image shows blueberry muffin baby with multiple light bluish subcutaneous nodules over trunk and extremities due to cutaneous metastases from neuroblastoma
Clinical significance of renal adenomas
Close resemblance to low grade papillary RCC; potentially malignant
Identify this syndrome associated with Wilms tumor:
Wilms tumor, aniridia, genitourinary anomalies, and intellectual disability (retardation)
WAGR
Wilms tumor, aniridia, genitourinary anomalies, and intellectual disability (retardation)
Identify the tumor described
This benign renal neoplasm (see attached image) arises form intercalated cells of the collecting duct and
Oncocytoma
Gross image shows: tan or mahogany brown, relatively homogeneous, and usually well encapsulated with a central scar
Underlying molecular basis of neuroblastoma
amplification of the N-MYC oncogene
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
Beckwith-Wiedemann syndrome (BWS)
most common primary renal tumor of childhood
Wilms Tumor (Nephroblastoma)
What is the clinical significance of detecting amplification of the NMYC oncogene in neuroblastoma?
The presence of MYCN amplification “bumps” the tumor to the “high”-risk category, irrespective of age, stage, or histology
Most common type of renal cell carcinoma (RCC)
Clear cell
See attached microscopic image and identify the tumor
Oncocytoma
Observe the large eosinophilic cells having small, round, benign-appearing nuclei that have large nucleoli, intensely pink cytoplasm due to the abundant mitochondria
How does having VHL syndrome lead to tumor and cyst development?
Deletion of VHL gene → impaired ubiquitination and elimination of hypoxia-inducible factor 1α → loss of function → tumor and cyst development
What is the reason for the yellow appearance of clear cell carcinoma?
prominent lipid accumulations in tumor cells.
Most significant risk factor for renal cell carcinoma
Smoking
Identify the tumor described
5 year old, abdominal mass, hematuria
See attached image
Nephroblastoma (Wilms tumor)
25-50% cases of angiomyolipomas are associated with this syndrome
Tuberous sclerosis - hypomelanotic (ash leaf) macules, angiofibromas, cardiac rhabdomyomas, Shagreen patches.
List 2 poor prognostic features for renal cell carcinoma
Invasion of the renal vein
Sarcomatoid change (histologic)
cell of origin of oncocytoma
intercalated cells of collecting ducts
most critical determinant of prognosis in Wilms
Anaplastic histology
Histopathologic features of angiomyolipoma
mixtures of well-differentiated adipose tissue, smooth muscle and thick-walled vessels
See attached microscopic image.
Identify the tumor and the reason for this appearance
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
Cell of origin of clear cell carcinoma
proximal tubular epithelium
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
Stauffer’s Syndrome
Identify this syndrome associated with Wilms tumor:
Wilms tumor, Diffuse mesangial sclerosis (early-onset nephrotic syndrome), Dysgenesis of gonads (male pseudohermaphroditism).
Risk of gonadoblastomas
Denys-Drash syndrome
4 major paraneoplastic syndromes associated with RCC
- Hypercalcemia
- Hypertension
- Polycythemia
- Stauffer’s syndrome
Why is angimyolipoma clinically significant?
susceptibility to spontaneous hemorrhage
What are the IHC markers for this tumor presenting as an abdominal/ flank mass in a child?
This is a neuroblastoma.
Neuron specific enolase, synaptophysin, chromogranin , neurofilament
Syndrome associated with both familial and sporadic forms of renal cell carcinoma
§Von Hippel-Lindau (VHL) syndrome
Syndromes associated with Wilms tumor
- WAGR syndrome
- Denys-Drash syndrome
- Beckwith-Wiedemann syndrome (BWS)
Cell of origin of angiomyolipomas
perivascular epithelioid cell (PEC)
Risk factors for this tumor?
This is urothelial carcinoma.
smoking (most common), aromatic amines (aniline dyes), and cyclophosphamide
Most cases of Wilms tumor are sporadic or familial?
90% are sporadic
Neuroblastoma/ nephroblastoma?
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
Microscopic features of Wilms tumor
Blastemal – sheets of small blue cells
Stromal - spindle cells with smooth muscle /fibroblast differentiation
Epithelial- abortive tubules or glomeruli
Constituents of VHL syndrome
Remember HIPPEL
Hemangioblastoma
Increased risk of renal cell carcinoma
Pheochromocytoma
Pancreatic lesions (cysts, cystadenomas , and neuroendocrine tumors )
Eye Lesions (retinal angiomas or hemangioblastomas)
Cell of origin of neuroblastoma
neural crest origin
Significance of identifying TP53 mutations in Wilms tumor
TP53 mutations are associated with :
an especially poor prognosis
Relative unresponsiveness to cytotoxic chemotherapy.
distinctive anaplastic histologic appearance
significance of identifying nephrogenic rests
patients are at an increased risk of developing Wilms tumors in the contralateral kidney and require frequent and regular surveillance
Most common presenting clinical feature for this tumor
Hematuria
