Tuesday, March 5, 2013

RENAL TUMORS

Classification

Benign
  • Cortical adenoma
  • Angiomyolipoma

Malignant
  • RCC
  • Upper tract TCC
  • Nephroblastoma
  • Squamous CA of renal pelvis

Cortical adenoma
  • Discovered incidentally, mostly found on autopsy
  • Less than 1 cm without malignant features (pea like)
  • Symptoms are unusual


Microscopically uniform basophilic or acidophilic with monotonous nuclear and cellular composition


Renal angiomyolipoma
  • May occur alone or a part of tuberous sclerosis
  • Hamartomas composed of fat, smooth muscle, and blood vessels
  • AML may be found in the eyes, heart, lung and bone




  • Malignant Tumors

Wilms tumor (nephroblastoma)
 Peak age:2-5 years

Risk factors
. WAGR syndrome-Wilms tumor, aniridia, genital anomalies, and mental retardation
. Beckwith-Wiedemann syndrome
.Tumor suppressor genes
 . WT-l (llp13)
 . WT-2 (llp15)

Gross: large solitary tan mass


Micro
1. Metanephric blastema
ii. Epithelial elements (immature glomeruli and tubules)
iii. Stroma


C/F:
Abdominal mass
Pyrexia
Hematuria
Metastasis occurs early, mainly to lungs.

Inv:
USG
Urography or
CT

Treatment: 
Nephrectomy followed by radiation with or without chemotherapy
If B/L tumor: Partial Nephrectomy

Prognosis: excellent; long-term survival rate of 90%

Renal Cell carcinoma

Epidemiology
- Male: Female  2:1
- 40-60 years of age

Risk factors
i. Cigarette smoking
ii. Chronic analgesic use
iii. Asbestos exposure
iv. Chronic renal failure and acquired cystic disease
v. Von Hippel- Lindau disease

Gross
. Large solitary yellow mass found in the poles (most commonly in the upper pole: most common site- PCT)
. Areas of necrosis and hemorrhage are commonly present
. The tumor often invades the renal vein and may extend into the vena cava and heart



Pathology

Clear cell types
  • more than 80%
  • polygonal or round cells with clear cytoplasm

Papillary variant
  • 14% of RCC
  • macroscopic feature-small, confined to cortex and nearly completely encapsulated
  • Micro: papillae covered by small cells with scanty cytoplasm, arranged in a single layer on the papillary basement membrane with low nuclear grade
Sarcomatoid
  • aggressive behavior and poor prognosis
  • microscopic features- spindle cell pattern
Chromophobe variant
  • 4% of RCC
  • microscopic features light and transparent cytoplasm
  • better prognosis
Signs and symptoms

Classic triad (pain, lump and hematuria) is found only in 10% cases
Weight loss, fever, hematuria, night sweats and sudden development of varicocele
Paraneoplastic syndromes from ectopic hormone production
   . Polycythemia(erythropoietin production)
   . Hypertension (renin production)
   . Cushing syndrome (corticosteroid synthesis)
   . Hypercalcemia (PTH-like hormone)
   . Feminization or masculinization (gonadotropin release)
High incidence of metastasis on initial presentation
May cause amyloidosis, a leukemoid reaction, or eosinophilia

Investigations

IVU


CT scan


  • Renal Angiogram
  • CX-Ray
  • Bone scan
Treatment
  • RCC is highly resistant to radio and chemotherapy
  • Nephrectomy with removal of the perinephric fat

3 comments:

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