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34 Cards in this Set

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Name 3 benign tumors of the kidney.

Renal papillary adenoma


Angiomyolipoma


Oncocytoma


What is the origin of renal papillary adenoma?

Arising from the renal tubular epithelium.

What are the morphologic characteristic of renal papillary adenoma?

Less than 0.5 cm.


Papillomatous structures with numerous complex fronds (ветвь с листьями)


Cell may grow in tubules, glands, cords and sheets of cells.


The cells are polygonal in shape and have regular small central nuclei, scanty cytoplasm and no atypia.

What low grade tumor is similar to renal papillary adenoma by histological criteria? What is the prognostic feature?

Low grade papillary renal cell carcinoma.


Not metastasize<3 cm>metastasize.

What is an angiomyolipoma?

Benign neoplasm consisting of vessels, smooth muscle and fat originating from perivascular epithelioid cells.

What is causing tuberous sclerosis?

Loss of function mutation in the TSC1 or TSC2 tumor suppressor genes.

What is the connection between angiomyolipoma and tuberous sclerosis?

Angiomyolipomas are present in 25 to 50 % of patients with tuberous sclerosis.

What are the characteristic of tuberous sclerosis? 3

It is characterized by lesions of the cerebral cortex, that produce epilepsy and mental retardation.


Variety of ski abnormalities


Unusual benign tumors of other sites, such as heart.

What is the clinical importance of angiomyolipoma?

Their susceptibility to spontaneous hemorrhage.

What people are more prone to renal cell carcinoma?

Male individuals in their 70s.

What is the most significant risk factor for renal cell carcinoma?

Tobacco. Cigarette smokers have double the incidence of renal cell carcinoma.

What are the major types of renal cell carcinoma? 5

1. Clear cell


2. Papillary carcinoma.


3.chromophobe carcinoma


4. Xp11 translocation carcinoma


5. Collecting duct (Bellini duct) carcinoma.

What is the most common type and the most rare type of renal cell carcinoma?

Clear cell 70-80%.


Collecting duct , less than 1%

T\F

Most cases or clear cell type are familial

F, Most sporadic 95%

What gene is the leading cause of clear cell type carcinoma? What happens to him?

VHL (Von Hippel-Lindau).


Loss of short arm on chromosome 3.

What is the pathogenesis that is caused by mutation of VHL gene?

Inactive VHL→ HIF-1 ( hypoxia-inducible factor 1) remain high→ inappropriate expression of a number of genes, like VEGF-promotes angiogenesis, IGF1 ( insulin like growth factor)- stimulates cell growth + HIF collaborates with MYC to reprogram cellular metabolism→ favors growth.

What %of incidents of papillary carcinoma?

10-15

What is the most common cytogenetic abnormalities in papillary carcinoma? 2

Trisomies 7 and 17


Loss of Y in male patients.

What is the genetic feature of papillary carcinoma?

Mutated , activated MET, a proto-oncogene that encodes the tyrosine kinase receptor for hepatocyte growth factor.

What is the histologic pic of chromophobe carcinoma?

Cells with prominent cell membranes and pale eosinophilic cytoplasm, usually with halo around the nucleus.

How the cells looks like

What is the origin of chromophobe carcinoma?

Thought to grow from intercalated cells of collecting ducts.

Which of the more common types of renal cell carcinoma has the best prognosis?

Chromophobe.

What is Xp11 translocation carcinoma? Whom does it affect more often?

It is genetically distinct type of renal cell carcinoma, defined by translocation of the TFE3 gene located on Xp,.


Occurs more often in young patients.

What is the histological pic of collecting ducct carcinoma?

Malignant cells forming glands within a prominent fibrotic stroma, typically in medullary location


.

What is the reason for the yellow color of renal cell carcinoma?

It ks a consequence of the prominent lipid accumulations in tumor cells.

Can there be bizarre nuclei and giant cell in clear cell type?

Yes. But most of tumors are well differentiated.

What is the striking characteristic of renal cell carcinoma?

Its tendency to invade the renal vain. In which it may grow as a solid column of cells that extends up to the inferior vena cava.

What is the characteristic histological pic of papillary cell carcinoma?


Composed of cuboidal or low columnar cells arranged in papillary formations.


Interstitial foam cells are common in papillary cores.

What is the characteristic histological pic of chromophobe carcinoma?

Pale eosinophilic cells, often with a perinuclear halo , arranged in solid sheets with a concentration around of the largest cells around blood vessels.

What are the histological features of collecting duct carcinoma?

Irregular channels lined by highly atypical epithelium with a hobnail pattern.

Why is renal cell carcinoma considered to be one of the great mimics in medicine?

Produces a diversity of symptoms that are not related to the kidney.


Syndromes that are ascribed to abnormal hormone production: hypercalcemia, feminization, Cushing syndrome, Amyloidosis, eosinophilia.

Cushing syndrome

What is a particularly troublesome feature of renal cell carcinoma?

Its tendency to metastasize widely before giving rise to any local symptoms.

What are the most common location of renal cell metastasis?

Lungs>50%→bones 33%→lymph nodes, liver, brain.

Individuals with Lynch syndrome, what renal tumor they most probably have?

Urothelial carcinoma of renal pelvis.