Renal Neoplasia - Pathoma Flashcards Preview

CRRAB II Week 4 - WLB > Renal Neoplasia - Pathoma > Flashcards

Flashcards in Renal Neoplasia - Pathoma Deck (18):

What is an Angiomyolipoma?

  • Hamartoma composed of blood vessels, smooth muscle, and adipose tissue
    • Angio = blood vessel
    • myo = muscle
    • lipo = fat (adipose tissue)


In what cases is there an increased frequency of Angiomyolipomas?

Tuberous sclerosis


What type of Renal neoplasia is a malignant epithelial tumor that arises from the kidney tubules?

Renal Cell Carcinoma


What is the classic presentation of Renal Cell Carcinoma?

  • Triad:
    • Hematuria
    • Palpable mass
    • Flank pain
  • Also common:
    • fever
    • weight loss
    • paraneoplastic syndrome (EPO, renin, parathyroid horomone related peptide, ACTH)


How does a varicocele form in the case of Renal Cell Carcinoma?

  • Left-side only
    • carcinoma obstructs gonadal vein draining into left renal vein
    • blood backs up => varicocele
  • Not on right side
    • gonadal veing drains directly into inferior vena cava


Which type of renal neoplasm reveals a yellow mass on gross exam and clear cytoplasm microscopically?

Renal Cell Carcinoma

(called clear cell type)


The pathogenesis of Renal Cell Carcinoma involves the loss of what gene?

VHL (3p) = tumor suppressor gene


What happens when you lose the expression of VHL in Renal Cell Carcinoma?

  • Increased IGF-3 (insulin-like growth factor)
    • promotes growth
  • Increased HIF transcription factor
    • increases VEGF and PDGF (platelet-derived growth factor)


What are the two pathways in the development of Renal Cell Carcinoma?

  • Sporadic Pathway
    • single tumor in upper pole of kidney
    • usually in smoker
  • Hereditary
    • tumors that arise in younger patients
    • multiple tumors and bilateral


***Both involve loss of VHL expression!


What is one autosomal dominant hereditary disorder that is associated with the inactivation of the VHL gene?

Von-Hippel Lindau Disease


Patients with Von Hippel-Lindau are at increased risk to develop what?

  • Hemangioblastoma of the cerebellum
  • Renal Cell Carcinoma


What is staging of Renal Cell Carcinoma based on?

  • T = based on tumor size and involvement of renal vein
    • increases the risk of hematogenous spread to the lungs and bones
  • N = spread to retroperitoneal lymph nodes


What do you call a malignant tumor of the kidney comprised of blastema, primitive glomeruli and tubules, and stromal cells?

Wilms Tumor


What is the most common malignant renal tumor in children?

Wilms Tumor

(average age = 3 yoa)


What is the classic presentation of Wilms Tumor?

  • Large, unilateral flank mass
  • Hematuria
  • HTN
    • due to renin secretion


What mutation is Wilms Tumor associated with?

WT1 mutations

(especially syndromic cases)


What is the WAGR Syndrome?

  • W = Wilm's tumor
  • A = Aniridia
  • G = Genital abnormalities
  • R = Mental and motor Retardation


What WT1 mutation associated syndrome consists of Wilms Tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (e.g. tongue)?

Beckwith-Wiedmann Syndrome