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 A rare parenchymal renal mass that accounts for <10% of
all renal tumors.
 Incidence in general population 0.3% and 0.13% with
autopsy series and ultrasound-screened populations
 consists of thick-walled aneurysmal vessels, smooth
muscle, and varying levels of mature adipose tissue.
 Angiomyolipoma is now considered to be of neural crest
origin, possibly derived from perivascular epithelioid cells
(PEC)
 Its growth may be hormone dependent, as suggested by
its female predominance and rarity before puberty
 Pregnancy appears to increase the risk of hemorrhage
fromAML.
Types
Associated with Tuberous
sclerosis
Approximately 20% of patients with
AMLs have tuberous sclerosis
(TS), an autosomal dominant
disease characterized by epilepsy,
mental retardation, adenoma
sebaceum, retinal phakomas, and
hamartomas of the kidneys, brain,
and other viscera.
Bilateral, multifocal& large
• sporadic
(unilateral, unifocal, small, slow
growth rate and are usually
detected incidentally)
• Extra renal
• retroperitoneal lymph nodes, liver,
and spleen have been noted to
have AMLs identical to the
primary renal tumor. However,
these have been considered to
represent multifocality rather than
metastases
Asymptomatic (accidentally discovered) 50%1
Flank pain, Flank mass, GIT symptoms (compression)2
Hematuria3
Hemorrhage (hypotension)4
1. History taking
2. Clinical examination : associated features of perirenal haemorrage,
flank mass, hematuria
3. Radiological:
 K.U.B: soft-tissue mass with radiolucent areas in 10%.
 USG : well-circumscribed, highly echogenic
 C.T : hipodens lesion, HU <-20
 MRI :T1-weighted image: ↑ signal intensity (due to high fat content)
 Most accurate
 on cross-sectional imaging. The presence of fat (confirmed on non-enhanced
thin-cut CT by a value of −20 Hounsfield Units [HU] or less) within a renal
lesion is considered the diagnostic hallmark.
Solid renal mass : as usual but the challenge is presented with,
 liposarcoma (compressing the renal parenchyma, not
inside the kidney)
 Possibility of fat-containing RCC(calcifications)
 possibility of a fat-poor angiomyolipoma
resembling an RCC(14% of cases)
Nephrectomy
Partial nephrectomy
Small (<4cm)
Observation
Embolization
Angiomyolipoma

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Angiomyolipoma

  • 1.
  • 2.  A rare parenchymal renal mass that accounts for <10% of all renal tumors.  Incidence in general population 0.3% and 0.13% with autopsy series and ultrasound-screened populations  consists of thick-walled aneurysmal vessels, smooth muscle, and varying levels of mature adipose tissue.
  • 3.  Angiomyolipoma is now considered to be of neural crest origin, possibly derived from perivascular epithelioid cells (PEC)  Its growth may be hormone dependent, as suggested by its female predominance and rarity before puberty  Pregnancy appears to increase the risk of hemorrhage fromAML.
  • 4. Types Associated with Tuberous sclerosis Approximately 20% of patients with AMLs have tuberous sclerosis (TS), an autosomal dominant disease characterized by epilepsy, mental retardation, adenoma sebaceum, retinal phakomas, and hamartomas of the kidneys, brain, and other viscera. Bilateral, multifocal& large • sporadic (unilateral, unifocal, small, slow growth rate and are usually detected incidentally) • Extra renal • retroperitoneal lymph nodes, liver, and spleen have been noted to have AMLs identical to the primary renal tumor. However, these have been considered to represent multifocality rather than metastases
  • 5. Asymptomatic (accidentally discovered) 50%1 Flank pain, Flank mass, GIT symptoms (compression)2 Hematuria3 Hemorrhage (hypotension)4
  • 6. 1. History taking 2. Clinical examination : associated features of perirenal haemorrage, flank mass, hematuria 3. Radiological:  K.U.B: soft-tissue mass with radiolucent areas in 10%.  USG : well-circumscribed, highly echogenic  C.T : hipodens lesion, HU <-20  MRI :T1-weighted image: ↑ signal intensity (due to high fat content)
  • 7.  Most accurate  on cross-sectional imaging. The presence of fat (confirmed on non-enhanced thin-cut CT by a value of −20 Hounsfield Units [HU] or less) within a renal lesion is considered the diagnostic hallmark.
  • 8. Solid renal mass : as usual but the challenge is presented with,  liposarcoma (compressing the renal parenchyma, not inside the kidney)  Possibility of fat-containing RCC(calcifications)  possibility of a fat-poor angiomyolipoma resembling an RCC(14% of cases)