Renal Cell Carcinoma Overview

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Questions and Answers

What is the male to female ratio for renal cell carcinoma (RCC)?

The male to female ratio for RCC is approximately 5:2 to 3:1.

What are some of the key predisposing factors for developing renal cell carcinoma?

Key predisposing factors include smoking, obesity, hypertension, and exposure to heavy metals.

Describe von Hippel–Lindau (VHL) syndrome and its association with RCC.

VHL syndrome is a hereditary condition predisposing individuals to multiple neoplasms, including multicentric bilateral renal cell carcinomas.

What genetic mutation is associated with hereditary leiomyomatosis and renal cell cancer syndrome?

<p>This syndrome is associated with a mutation in the Fumarate Hydratase (FH) gene.</p> Signup and view all the answers

What is the significance of the MET proto-oncogene in hereditary papillary RCC?

<p>The MET proto-oncogene is mutated in hereditary papillary RCC, leading to multiple bilateral carcinomas.</p> Signup and view all the answers

What percentage of all cancers does renal cell carcinoma (RCC) represent?

<p>RCC constitutes about 2–3% of all cancers.</p> Signup and view all the answers

Identify one environmental exposure that is a predisposing factor for RCC.

<p>Exposure to asbestos is a predisposing factor for RCC.</p> Signup and view all the answers

Name a hereditary syndrome associated with bilateral renal cysts and RCC.

<p>Von Hippel–Lindau (VHL) syndrome is associated with bilateral renal cysts and RCC.</p> Signup and view all the answers

What is the common genetic alteration linked to hereditary leiomyomatosis and renal cell cancer syndrome?

<p>A mutation in the Fumarate Hydratase (FH) gene is linked to this syndrome.</p> Signup and view all the answers

What kind of carcinoma is primarily associated with the mutation in the MET proto-oncogene?

<p>The mutation in the MET proto-oncogene is primarily associated with hereditary papillary RCC.</p> Signup and view all the answers

Flashcards

RCC (Renal Cell Carcinoma)

A type of kidney cancer, arising from the tubular epithelium, responsible for a majority of primary kidney cancers.

Incidence of RCC

RCC accounts for a significant portion (up to 90%) of primary kidney cancers and a relatively small percentage of overall cancers (2-3%).

RCC Predisposing Factors

Factors increasing the risk of RCC include smoking, obesity, hypertension, unopposed estrogen therapy, exposure to asbestos, cadmium, petroleum products, and heavy metals, and acquired cystic kidney disease in dialysis patients.

Inherited RCC Syndromes

Certain genetic syndromes increase RCC risk, including von Hippel-Lindau (VHL), hereditary leiomyomatosis and renal cell cancer, hereditary papillary RCC, and Birt-Hogg-Dubé (BHD).

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VHL Syndrome

A genetic syndrome, commonly associated with multiple, potentially life-threatening, cancers in various organs (including the kidneys), including haemangioblastomas (blood vessel tumors), renal cysts, and pheochromocytomas (adrenal gland tumors).

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What is RCC?

Renal Cell Carcinoma (RCC) is a type of kidney cancer that originates from the tubular epithelium (lining of the kidney tubes). It accounts for the majority of all primary kidney cancers.

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What is the most common type of kidney cancer?

RCC is the most prevalent type of kidney cancer, accounting for about 90% of all primary malignant tumors affecting the kidneys.

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How is RCC related to adrenal cortex?

RCC is sometimes called 'hypernephroma' because it resembles the clear cells of the adrenal cortex and often exhibits a yellow color upon gross inspection.

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What are some risk factors for RCC?

Factors that increase your risk of developing RCC include smoking, obesity, hypertension, unopposed estrogen therapy, exposure to asbestos, cadmium, heavy metals, and acquired cystic disease in dialysis patients.

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What is VHL syndrome?

VHL syndrome is a genetic disorder that increases the risk of developing RCC. It also predisposes individuals to other tumors like haemangioblastomas (blood vessel tumors) of the cerebellum and retina, renal cysts, and pheochromocytomas (adrenal gland tumors).

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Study Notes

Renal Cell Carcinoma (RCC)

  • RCC accounts for 90% of primary kidney cancers and 2-3% of all cancers.
  • Also known as hypernephroma due to its resemblance to adrenal cortex cells.
  • Originates from renal tubular epithelium (renal adenocarcinomas).

Epidemiology and Predisposing Factors

  • Male to female ratio is 5:2 to 3:1.
  • Predisposing factors:
    • Smoking
    • Obesity
    • Hypertension
    • Unopposed estrogen therapy
    • Exposure to asbestos, cadmium, petroleum products, and heavy metals
    • Acquired cystic kidney disease in dialysis patients

Genetic Predisposition to RCC

  • Most RCC cases are sporadic.
  • 5% are inherited and associated with:
    • von Hippel–Lindau (VHL) syndrome:
      • Multiple neoplasms (especially haemangioblastomas of cerebellum and retina)
      • Multiple bilateral renal cysts
      • Pheochromocytomas
      • Multicentric bilateral RCC
    • Hereditary leiomyomatosis and renal cell cancer syndrome:
      • Autosomal dominant inheritance
      • Mutation in Fumarate Hydratase (FH) gene
      • Uterine and cutaneous leiomyomas
      • Aggressive papillary RCC
    • Hereditary papillary RCC:
      • Autosomal dominant inheritance
      • Multiple cytogenetic abnormalities
      • Mutation in MET proto-oncogene
      • Multiple bilateral papillary RCCs
    • Birt–Hogg–Dube (BHD) syndrome:
      • Autosomal dominant inheritance
      • Mutation in the BHD gene (encoding folliculin)
      • Skin appendageal tumors (hair follicle origin)
      • Pulmonary cysts
      • Renal tumors

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