Angiomyolipoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [3], Faizan Sheraz, M.D. [4]

Overview

On gross pathology, well circumscribed and uniform yellow mass is characteristic finding of angiomyolipoma. The components of an angiomyolipoma include vascular cells, immature smooth muscle cells and fat cells. Angiomyolipoma is caused by a defect in TSC1 gene. Diseases associated with angiomyolipoma include von Hippel-Lindau syndrome and tuberous sclerosis. Cytologic features of angiomyolipoma include round nuclei and bland chromatin. Immunohistochemistry markers of angiomyolipoma include positive melanocytic markers.

Pathophysiology

Three components of an angiomyolipoma include:

Angiomyolipoma is derived from a common progenitor cell that suffered the common second hit mutation. Angiomyolipomas are members of the perivascular epithelioid cells tumor group (PEComas) and are composed of variable amounts of these three components.

Genetics

Angiomyolipoma is caused by a defect in:

Associated Conditions

Diseases associated with angiomyolipoma include:

Gross Pathology

On gross pathology, well circumscribed and uniform yellow mass are characteristic findings of angiomyolipoma.

Angiomyolipoma Gross Pathology[1]

Microscopic Pathology

Microscopic features of angiomyolipoma:[2]

Variants

  • Epithelioid angiomyolipoma
    • There is a special variant called an epithelioid angiomyolipoma, composed of epithelial looking cells, often with nuclear atypia

Cytologic

Cytologic features of angiomyolipoma include:[2]

Immunohistochemistry

Immunohistochemistry markers of angiomyolipoma include:

  • Melanocytic markers positive[4]
    • HMB-45 positive in all cases (15/15)[5]
    • Melan A positive in ~87% of cases (13/15)
  • Epithelial markers negative[4]
    • EMA
    • AE1/AE3
  • SMA positive
  • CD117 positive/negative
  • Ki-67:[6]
    • Epithelioid variant of angiomyolipoma positive
    • Conventional angiomyolipoma negative

References

  1. Image courtesy of Dr Andrew Ryan. Radiopaedia (original file [1]).[http://radiopaedia.org/licence Creative Commons BY-SA-NC
  2. 2.0 2.1 Crapanzano, JP. (2005). "Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases". Diagn Cytopathol. 32 (1): 53–7. doi:10.1002/dc.20179. PMID 15584043. Unknown parameter |month= ignored (help)
  3. 3.0 3.1 3.2 3.3 http://librepathology.org/wiki/index.php/Angiomyolipoma
  4. 4.0 4.1 Template:Ref GUP
  5. Esheba, Gel S.; Esheba, Nel S. (2013). "Angiomyolipoma of the kidney: clinicopathological and immunohistochemical study". J Egypt Natl Canc Inst. 25 (3): 125–34. doi:10.1016/j.jnci.2013.05.002. PMID 23932749. Unknown parameter |month= ignored (help)
  6. Ooi, SM.; Vivian, JB.; Cohen, RJ. (2009). "The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma". Int Urol Nephrol. 41 (3): 559–65. doi:10.1007/s11255-008-9473-1. PMID 18839327.

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