Angiomyolipoma pathophysiology: Difference between revisions

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==Microscopic Pathology==
==Microscopic Pathology==
Microscopic features of angiomyolipoma:<ref name=pmid15584043>{{Cite journal  | last1 = Crapanzano | first1 = JP. | title = Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases. | journal = Diagn Cytopathol | volume = 32 | issue = 1 | pages = 53-7 | month = Jan | year = 2005 | doi = 10.1002/dc.20179 | PMID = 15584043 }}</ref>
Microscopic features of angiomyolipoma:<ref name="pmid15584043">{{Cite journal  | last1 = Crapanzano | first1 = JP. | title = Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases. | journal = Diagn Cytopathol | volume = 32 | issue = 1 | pages = 53-7 | month = Jan | year = 2005 | doi = 10.1002/dc.20179 | PMID = 15584043 }}</ref>
*[[Smooth muscle]]s
*[[Smooth muscle]]s
*[[Adipose tissue]]
*[[Adipose tissue]]
Line 46: Line 46:


===Cytologic===
===Cytologic===
Cytologic features of angiomyolipoma include:<ref name=pmid15584043>{{Cite journal  | last1 = Crapanzano | first1 = JP. | title = Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases. | journal = Diagn Cytopathol | volume = 32 | issue = 1 | pages = 53-7 | month = Jan | year = 2005 | doi = 10.1002/dc.20179 | PMID = 15584043 }}</ref>
Cytologic features of angiomyolipoma include:<ref name="pmid15584043">{{Cite journal  | last1 = Crapanzano | first1 = JP. | title = Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases. | journal = Diagn Cytopathol | volume = 32 | issue = 1 | pages = 53-7 | month = Jan | year = 2005 | doi = 10.1002/dc.20179 | PMID = 15584043 }}</ref>
*[[Nuclei]] - round/ovoid
*[[Nuclei]] - round/ovoid
*[[Chromatin]] - bland
*[[Chromatin]] - bland
Line 52: Line 52:
===Immunohistochemistry===
===Immunohistochemistry===
Immunohistochemistry markers of angiomyolipoma include:
Immunohistochemistry markers of angiomyolipoma include:
*Melanocytic markers positive<ref name=Ref_GUP324>{{Ref GUP|324}}</ref>
*Melanocytic markers positive<ref name="Ref_GUP324">{{Ref GUP|324}}</ref>
**HMB-45 positive in all cases (15/15)<ref name=pmid23932749>{{Cite journal  | last1 = Esheba | first1 = Gel S. | last2 = Esheba | first2 = Nel S. | title = Angiomyolipoma of the kidney: clinicopathological and immunohistochemical study. | journal = J Egypt Natl Canc Inst | volume = 25 | issue = 3 | pages = 125-34 | month = Sep | year = 2013 | doi = 10.1016/j.jnci.2013.05.002 | PMID = 23932749 }}
**HMB-45 positive in all cases (15/15)<ref name="pmid23932749">{{Cite journal  | last1 = Esheba | first1 = Gel S. | last2 = Esheba | first2 = Nel S. | title = Angiomyolipoma of the kidney: clinicopathological and immunohistochemical study. | journal = J Egypt Natl Canc Inst | volume = 25 | issue = 3 | pages = 125-34 | month = Sep | year = 2013 | doi = 10.1016/j.jnci.2013.05.002 | PMID = 23932749 }}
</ref>
</ref>
**Melan A positive in ~87% of cases (13/15)
**Melan A positive in ~87% of cases (13/15)
*Epithelial markers negative<ref name=Ref_GUP324>{{Ref GUP|324}}</ref>
*Epithelial markers negative<ref name="Ref_GUP324">{{Ref GUP|324}}</ref>
**EMA
**EMA
**AE1/AE3
**AE1/AE3
*SMA positive
*SMA positive
*CD117 positive/negative
*CD117 positive/negative
*Ki-67:<ref name=pmid18839327>{{Cite journal  | last1 = Ooi | first1 = SM. | last2 = Vivian | first2 = JB. | last3 = Cohen | first3 = RJ. | title = The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 559-65 | month =  | year = 2009 | doi = 10.1007/s11255-008-9473-1 | PMID = 18839327 }}</ref>
*Ki-67:<ref name="pmid18839327">{{Cite journal  | last1 = Ooi | first1 = SM. | last2 = Vivian | first2 = JB. | last3 = Cohen | first3 = RJ. | title = The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 559-65 | month =  | year = 2009 | doi = 10.1007/s11255-008-9473-1 | PMID = 18839327 }}</ref>
**Epithelioid variant of [[angiomyolipoma]] positive
**Epithelioid variant of [[angiomyolipoma]] positive
**Conventional angiomyolipoma negative
**Conventional angiomyolipoma negative

Revision as of 16:04, 26 November 2018

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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], Rekha, M.D.

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]

  • Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
    Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
  • Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
    Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
  • Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
    Histopathologic slide of renal angiomyolipoma. Nephrectomy specimen. H & E stain.[3]
  • Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. HMB-45 immunostain.[3]
    Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. HMB-45 immunostain.[3]

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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