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], 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

Pathogenesis

Genetics

Genes involved in the pathogenesis of angiomyolipoma include:[3][4]


Associated Conditions

Conditions associated with angiomyolipoma include:[5][6][7][8]

Gross Pathology

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

Angiomyolipoma Gross Pathology[9]

Microscopic Pathology

On microscopic histopathological analysis the following are characteristic findings of angiomyolipoma:[10]

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:[10]

Immunohistochemistry

Immunohistochemistry markers of angiomyolipoma include:

References

  1. Siroky BJ, Yin H, Dixon BP, Reichert RJ, Hellmann AR, Ramkumar T; et al. (2014). "Evidence for pericyte origin of TSC-associated renal angiomyolipomas and implications for angiotensin receptor inhibition therapy". Am J Physiol Renal Physiol. 307 (5): F560–70. doi:10.1152/ajprenal.00569.2013. PMC 4868369. PMID 24920756.
  2. Lane BR, Aydin H, Danforth TL, Zhou M, Remer EM, Novick AC; et al. (2008). "Clinical correlates of renal angiomyolipoma subtypes in 209 patients: classic, fat poor, tuberous sclerosis associated and epithelioid". J Urol. 180 (3): 836–43. doi:10.1016/j.juro.2008.05.041. PMID 18635231.
  3. Walsh SN, Sangüeza OP (2009). "PEComas: a review with emphasis on cutaneous lesions". Semin Diagn Pathol. 26 (3): 123–30. PMID 20043511.
  4. Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F (2008). "PEComas: the past, the present and the future". Virchows Arch. 452 (2): 119–32. doi:10.1007/s00428-007-0509-1. PMC 2234444. PMID 18080139.
  5. Warncke JC, Brodie KE, Grantham EC, Catarinicchia SP, Tong S, Kondo KL; et al. (2017). "Pediatric Renal Angiomyolipomas in Tuberous Sclerosis Complex". J Urol. 197 (2): 500–506. doi:10.1016/j.juro.2016.09.082. PMID 27678300.
  6. Walsh SN, Sangüeza OP (2009). "PEComas: a review with emphasis on cutaneous lesions". Semin Diagn Pathol. 26 (3): 123–30. PMID 20043511.
  7. Sugimoto M, Takamura S (1997). "[Renal angiomyolipoma and renal cell carcinoma associated with tuberous sclerosis: a case report]". Hinyokika Kiyo. 43 (1): 33–5. PMID 9046419.
  8. Walsh SN, Sangüeza OP (2009). "PEComas: a review with emphasis on cutaneous lesions". Semin Diagn Pathol. 26 (3): 123–30. PMID 20043511.
  9. Image courtesy of Dr Andrew Ryan. Radiopaedia (original file [1]).[http://radiopaedia.org/licence Creative Commons BY-SA-NC
  10. 10.0 10.1 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)
  11. 11.0 11.1 11.2 11.3 http://librepathology.org/wiki/index.php/Angiomyolipoma
  12. 12.0 12.1 Template:Ref GUP
  13. Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F (2008). "PEComas: the past, the present and the future". Virchows Arch. 452 (2): 119–32. doi:10.1007/s00428-007-0509-1. PMC 2234444. PMID 18080139.
  14. Walsh SN, Sangüeza OP (2009). "PEComas: a review with emphasis on cutaneous lesions". Semin Diagn Pathol. 26 (3): 123–30. PMID 20043511.
  15. 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)
  16. 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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