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35 Cards in this Set
- Front
- Back
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Associated with deletions
of chromosome 3p14 to 3p26 |
Sporadic clear cell RCC
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Clinical triad
– Hematuria, flank pain, mass |
Sporadic clear cell RCC
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VHL gene is located on
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chromosome 3p25
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Often related to Von‐Hippel‐
Lindau (VHL) disease |
Familial clear cell RCC
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Knots of capillaries in the retina and other organs
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Angiomatosis seen in VHL
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Features of VHL disease
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Clear cell RCC (multiple and bilateral)
Angiomatosis Phaeochromocytoma pancreatic cysts Hemangioblastomas in cerebellum, retina |
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Tendency to invade renal
vein • Golden yellow becaue of high lipid content in cells • Tumors may also invade renal capsule |
RCC the clear kind
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Majority of RCC are grade
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2 or 3
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describe clear RCCs under microscopy
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• Nests and sheets of clear
epithelial cells • Distinct cell membrane • Contain abundant lipid |
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pT4:Tumor invades beyond
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beyond Gerota’s fascia (
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in renal carcinoma what is T1
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pT1: < 7 cm, limited to the kidney
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in renal carcinoma what is T2
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pT2: >7 cm, limited to the kidney
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Clear Cell RCC
Key Points to Remember |
• Arises in cortex (renal tubules)
• Most common kidney cancer • Composed of lipid rich clear cells • Sporadic and familial – Specific chromosomal or gene abnormalities are seen in each – Loss of chromosome 3p • Where the VHL gene resides – Associated disease in familial type is VHL disease • Different nuclear grades (1 ‐ 4) • Higher the grade the more aggressive the tumor • Prognosis related to grade and stage |
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•Fibrovascular cores may
contain foamy macrophages •Psammoma bodies are common |
Papillary Renal Cell Carcinoma
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Tumor must be > 0.5cm
in order to be called |
papillary RCC
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•Papillae are lined by
eosinophilic cuboidal cells |
Papillary Renal
Cell Carcinoma • Second most common RCC •10‐15% of RCC •Tumor must be > 0.5cm in order to be called papillary RCC •Papillae are lined by eosinophilic cuboidal cells •Fibrovascular cores may contain foamy macrophages •Psammoma bodies are common |
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More likely to be cystic
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Sporadic form of Papillary RCC
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– Trisomy of chromosomes
7, 17, 20 – Loss of Y chromosome |
Sporadic form of Papillary RCC
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– Autosomal dominant
– Involves the c‐MET oncogene – MET gene is on chromosome 7q31 – Trisomy of chr. 7 is seen in the majority of cases – Bilateral, multiple tumors |
Familial/hereditary form of Papillary RCC
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– Histologically identical to
papillary RCC but are < 0.5 cm in size • Share similar cytogenetic abnormalities with papillary RCC |
• PAPILLARY ADENOMAS
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Arises from intercalated cells and histologically similar to an oncocytoma
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Chromophobe Carcinoma
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Chromophobe Carcinoma is
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tumor of the renal medulla and arises from intercalated cells
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well circumscribed solitary tumor arising form the renal medulla intercalated cells
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Chromophobe Carcinoma
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what gene is involved in chromophobe carcinoma of the intercalated cells
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• Gene involved is on
chromosome 17 |
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• Familial
– Associated with Birt‐Hogg‐ Dube (BHD) syndrome (similar to papillary RCC) • Gene involved is on chromosome 17 |
familial chromophobe
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• BHD syndrome is
characterized by: |
– Multiple chromophobe
RCCs – Oncocytomas – Papillary RCC – Lung cysts |
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– Ages 27 ‐ 86
– M=F – Mortality < 10% – Loss of chromosomes: • Chromosome ‐1, ‐2, ‐6, ‐10 |
Chromophobe RCC sporadic
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Cells are large with pale eosinophilic cytoplasm, well defined cell borders and perinuclear halos describesresemble vegetable cell walls
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Chromophobe RCC
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histo of chromophobe RCC resemble vegetable cell walls
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Cells are large with pale eosinophilic cytoplasm, well defined cell borders and perinuclear halos describes
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Specific EM findings
– Microvesicles |
Chromophobe
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• Arises in medulla (intercalated collecting duct epithelium)
• Third most common kidney cancer • Composed of clear and pink cells – Perinuclear halos – Well defined cell borders (“Vegetable cells”) |
chromophobe
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• Good prognosis
• Most are nuclear grade 1 or 2 • Only rarely nuclear grade 4 • Less likely to metastasize than clear cell RCC • Surgery usually curative |
Chromophobe
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• “CDB” arises in renal
pyramid and drains into the renal pelvis and is associated with what |
Collecting Duct Carcinoma
with infiltrative glands |
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• Younger patients
• Mean age 34 |
Collecting Duct Carcinoma
with infiltrative glands • “CDB” arises in renal pyramid and drains into the renal pelvis and is associated with what |
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• Pleomorphic, high grade
tumor • Infiltrative growth pattern • 33% have metastasis at diagnosis • POOR PROGNOSIS • 66% die within 2 yrs |
Collecting Duct
Carcinoma with infiltrative glands |
