Renal Neoplasia Overview

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Questions and Answers

Which statement correctly describes the pathogenesis of renal cell carcinoma?

Renal cell carcinoma primarily arises from mesenchymal tissues.
Loss of the VHL tumor suppressor gene leads to a decrease in IGF-1 levels.
Loss of the VHL tumor suppressor gene results in increased levels of HIF transcription factor. (correct)
Increased levels of VEGF and PDGF cause the suppression of tumor growth.

What is the most common clinical symptom of renal cell carcinoma?

Weight loss
Hematuria (correct)
Palpable mass
Flank pain

Involvement of the left renal vein by carcinoma is most likely to lead to which condition?

Left-sided varicocele (correct)
Right-sided varicocele
Renal vein thrombosis
Hematogenous spread to the liver

Which demographic is most commonly affected by sporadic renal cell carcinoma?

Adult males in their 60s (A) Signup and view all the answers

Which statement is true regarding Wilms tumor?

Associated with genetic syndromes like WAGR and Denys-Drash. (C) Signup and view all the answers

Which histological feature is most characteristic of the common variant of renal cell carcinoma?

Clear cytoplasm in histological examination (D) Signup and view all the answers

Which of the following is a classic symptom associated with Wilms tumor?

Unilateral flank mass (C) Signup and view all the answers

What distinguishes a hereditary Wilms tumor from a sporadic case?

Hereditary cases often present bilaterally. (C) Signup and view all the answers

Flashcards

What is an Angiomyolipoma?

A benign tumor of the kidney composed of blood vessels, smooth muscle, and fat tissue. It is often associated with the genetic disorder tuberous sclerosis.

What is Renal Cell Carcinoma?

A type of kidney cancer that arises from the epithelial cells of kidney tubules. Classic symptoms include blood in the urine (hematuria), a palpable mass in the flank, and flank pain, though all three symptoms occur rarely together.

What is the Pathogenesis of Renal Cell Carcinoma?

Mutations in the VHL gene, a tumor suppressor gene, leading to increased levels of IGF-1, which promotes cell growth, and increased HIF transcription factor, which stimulates the production of VEGF and PDGF, promoting blood vessel formation.

What is a Wilms Tumor?

The most common malignant kidney tumor in children, typically presenting with a large, unilateral flank mass, hematuria, and hypertension due to renin secretion. It often arises from primitive kidney structures, including blastema, glomeruli, and tubules.

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What is WAGR Syndrome?

A rare genetic disorder that is associated with an increased risk of Wilms Tumor, Aniridia (absence of the iris), genital abnormalities, and mental and motor retardation. It results from a deletion of the WT1 tumor suppressor gene located on chromosome 11p13.

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What is Denys-Drash Syndrome?

A syndrome characterized by Wilms Tumor, progressive renal (glomerular) disease, and male pseudohermaphroditism (ambiguous genitalia). It is linked to mutations in the WT1 tumor suppressor gene.

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What is Beckwith-Wiedemann Syndrome?

A rare syndrome presenting with Wilms Tumor, aniridia, hemihypertrophy (overgrowth of one side of the body), and genital abnormalities. It results from a mutation in the WT1 tumor suppressor gene and often involves a deletion on chromosome 11.

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Study Notes

Renal Neoplasia

Angiomyolipoma:
- A hamartoma composed of blood vessels, smooth muscle, and adipose tissue.
- Increased frequency in tuberous sclerosis.
Renal Cell Carcinoma:
- A malignant epithelial tumor originating from kidney tubules.

Additional Renal Tumor Information

Classic triad of renal tumors:
- Hematuria, palpable mass, and flank pain (rarely occur together).
- Fever, weight loss, or paraneoplastic syndromes (EPO, renin, PTHrP, or ACTH) may be present
Left-sided varicocele:
- Possible complication from blockage of the left renal vein by carcinoma.
- Left spermatic vein blockage causes varicocele.
- Right spermatic vein drains into the IVC; thus preventing a right-sided varicocele.
Gross and microscopic appearance:
- Yellow mass microscopically shows clear cytoplasm (clear cell type) in the most common variant.
Pathogenesis (Renal Cell Carcinoma):
- Involves loss of the VHL (3p) tumor suppressor gene.
- Leads to increased IGF-1, promoting growth.
- Increased HIF transcription factor for VEGF and PDGF
Tumor types:
- Sporadic:
  - Common in adult males (average age 60).
  - Typically single tumor in the kidney's upper pole.
  - Cigarette smoking is a risk factor
- Hereditary:
  - Arises in younger adults.
  - Typically bilateral tumors.
  - Von Hippel-Lindau disease: Autosomal dominant linking to VHL gene inactivation and increased risk for hemangioblastoma of the cerebellum and renal cell carcinoma.
Staging:
- T-stage: based on size and involvement of the renal vein
  - Risk of spread to the lungs and bones
- N-stage: spread to retroperitoneal lymph nodes

Wilms Tumor

A malignant kidney tumor, composed of blastema, primitive glomeruli, tubules and stromal cells.
Most common malignant renal tumor in children (average age 3 years).
Presents with a large, unilateral, flank mass, hematuria and hypertension.
90% are sporadic, some are syndromic:
- WAGR syndrome: Wilms tumor, aniridia, genital abnormalities, mental and motor retardation, linked to a deletion of the WT1 tumor suppressor gene (11p13).
- Denys-Drash syndrome: Wilms tumor, progressive renal disease, and male pseudohermaphroditism, related to WT1 mutations.
Beckwith-Wiedemann syndrome:
- Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (including tongue).
- Associated with mutations in the WT2 gene cluster (imprinted genes at 11p15.5), particularly IGF-2.