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34 Cards in this Set
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- 3rd side (hint)
Name 3 benign tumors of the kidney. |
Renal papillary adenoma Angiomyolipoma Oncocytoma |
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What is the origin of renal papillary adenoma? |
Arising from the renal tubular epithelium. |
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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. |
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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. |
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What is an angiomyolipoma? |
Benign neoplasm consisting of vessels, smooth muscle and fat originating from perivascular epithelioid cells. |
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What is causing tuberous sclerosis? |
Loss of function mutation in the TSC1 or TSC2 tumor suppressor genes. |
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What is the connection between angiomyolipoma and tuberous sclerosis? |
Angiomyolipomas are present in 25 to 50 % of patients with tuberous sclerosis. |
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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. |
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What is the clinical importance of angiomyolipoma? |
Their susceptibility to spontaneous hemorrhage. |
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What people are more prone to renal cell carcinoma? |
Male individuals in their 70s. |
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What is the most significant risk factor for renal cell carcinoma? |
Tobacco. Cigarette smokers have double the incidence of renal cell carcinoma. |
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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. |
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What is the most common type and the most rare type of renal cell carcinoma? |
Clear cell 70-80%. Collecting duct , less than 1% |
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T\F |
Most cases or clear cell type are familial |
F, Most sporadic 95% |
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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. |
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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. |
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What %of incidents of papillary carcinoma? |
10-15 |
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What is the most common cytogenetic abnormalities in papillary carcinoma? 2 |
Trisomies 7 and 17 Loss of Y in male patients. |
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What is the genetic feature of papillary carcinoma? |
Mutated , activated MET, a proto-oncogene that encodes the tyrosine kinase receptor for hepatocyte growth factor. |
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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 |
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What is the origin of chromophobe carcinoma? |
Thought to grow from intercalated cells of collecting ducts. |
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Which of the more common types of renal cell carcinoma has the best prognosis? |
Chromophobe. |
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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. |
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What is the histological pic of collecting ducct carcinoma? |
Malignant cells forming glands within a prominent fibrotic stroma, typically in medullary location . |
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What is the reason for the yellow color of renal cell carcinoma? |
It ks a consequence of the prominent lipid accumulations in tumor cells. |
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Can there be bizarre nuclei and giant cell in clear cell type? |
Yes. But most of tumors are well differentiated. |
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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. |
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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. |
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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. |
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What are the histological features of collecting duct carcinoma? |
Irregular channels lined by highly atypical epithelium with a hobnail pattern. |
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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 |
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What is a particularly troublesome feature of renal cell carcinoma? |
Its tendency to metastasize widely before giving rise to any local symptoms. |
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What are the most common location of renal cell metastasis? |
Lungs>50%→bones 33%→lymph nodes, liver, brain. |
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Individuals with Lynch syndrome, what renal tumor they most probably have? |
Urothelial carcinoma of renal pelvis. |
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