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Epithelioid type angiomyolipoma

MedGen UID:
1386340
•Concept ID:
C4518194
•
Neoplastic Process
Synonyms: Epithelioid angiomyolipoma; Epithelioid Angiomyolipoma; Epithelioid Type Angiomyolipoma; epithelioid type angiomyolipoma
SNOMED CT: Epithelioid angiomyolipoma (733836008); Epithelioid angiomyolipoma (1293130003)
 
Monarch Initiative: MONDO:0002606

Definition

An angiomyolipoma composed exclusively or predominantly of epithelioid cells. It is often associated with cytologic atypia and may recur or metastasize. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid type angiomyolipoma

Professional guidelines

PubMed

Renal angiomyolipoma with tuberous sclerosis complex: How it differs from sporadic angiomyolipoma in both management and care.
Hatano T, Egawa S
Asian J Surg 2020 Oct;43(10):967-972. Epub 2020 Jan 17 doi: 10.1016/j.asjsur.2019.12.008. PMID: 31959574
Management of hepatic angiomyolipoma: A systematic review.
Klompenhouwer AJ, Verver D, Janki S, Bramer WM, Doukas M, Dwarkasing RS, de Man RA, IJzermans JNM
Liver Int 2017 Sep;37(9):1272-1280. Epub 2017 Mar 6 doi: 10.1111/liv.13381. PMID: 28177188
Clear cell renal cell carcinoma with a syncytial-type multinucleated giant tumor cell component: implications for differential diagnosis.
Williamson SR, Kum JB, Goheen MP, Cheng L, Grignon DJ, Idrees MT
Hum Pathol 2014 Apr;45(4):735-44. Epub 2013 Nov 15 doi: 10.1016/j.humpath.2013.10.033. PMID: 24499686

Recent clinical studies

Etiology

Does epithelioid angiomyolipoma have poorer prognosis, compared with classic angiomyolipoma?
Lee W, Choi SY, Lee C, Yoo S, You D, Jeong IG, Song C, Kim KS, Hong B, Hong JH, Ahn H, Kim CS
Investig Clin Urol 2018 Nov;59(6):357-362. Epub 2018 Oct 4 doi: 10.4111/icu.2018.59.6.357. PMID: 30402567Free PMC Article
Management of hepatic angiomyolipoma: A systematic review.
Klompenhouwer AJ, Verver D, Janki S, Bramer WM, Doukas M, Dwarkasing RS, de Man RA, IJzermans JNM
Liver Int 2017 Sep;37(9):1272-1280. Epub 2017 Mar 6 doi: 10.1111/liv.13381. PMID: 28177188
Hereditary kidney cancer syndromes.
Haas NB, Nathanson KL
Adv Chronic Kidney Dis 2014 Jan;21(1):81-90. doi: 10.1053/j.ackd.2013.10.001. PMID: 24359990Free PMC Article
PEComas: the past, the present and the future.
Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F
Virchows Arch 2008 Feb;452(2):119-32. Epub 2007 Dec 14 doi: 10.1007/s00428-007-0509-1. PMID: 18080139Free PMC Article
The evolving concept of renal neoplasia: impact of emerging molecular and electron microscopic studies.
Picken MM
Ultrastruct Pathol 2005 May-Aug;29(3-4):277-82. doi: 10.1080/01913120590951266. PMID: 16036881

Diagnosis

Diagnostic approach to eosinophilic renal neoplasms.
Kryvenko ON, Jorda M, Argani P, Epstein JI
Arch Pathol Lab Med 2014 Nov;138(11):1531-41. doi: 10.5858/arpa.2013-0653-RA. PMID: 25357116Free PMC Article
Renal cell carcinoma arising in a long pre-existing angiomyolipoma.
Inomoto C, Umemura S, Sasaki Y, Yasuda M, Terachi T, Osamura RY
Pathol Int 2007 Mar;57(3):162-6. doi: 10.1111/j.1440-1827.2006.02075.x. PMID: 17295650
Controversies related to epithelioid variant of renal angiomyolipoma: a review of the literature.
Serrano Frago P, Del Agua Arias Camisón C, Gil Sanz MJ, Allué López M, Gonzalvo Ibarra A, Plaza Mas L, Rioja Sanz LA
Urology 2006 Apr;67(4):846.e3-5. Epub 2006 Apr 5 doi: 10.1016/j.urology.2005.10.030. PMID: 16600344
The evolving concept of renal neoplasia: impact of emerging molecular and electron microscopic studies.
Picken MM
Ultrastruct Pathol 2005 May-Aug;29(3-4):277-82. doi: 10.1080/01913120590951266. PMID: 16036881
Trabecular angiomyolipoma mimicking hepatic cell carcinoma.
Székely E, Schaff Z, Madaras L, Kupcsulik P, Zsirka A
Pathol Oncol Res 2000;6(3):224-6. doi: 10.1007/BF03032377. PMID: 11033464

Therapy

Renal angiomyolipoma with tuberous sclerosis complex: How it differs from sporadic angiomyolipoma in both management and care.
Hatano T, Egawa S
Asian J Surg 2020 Oct;43(10):967-972. Epub 2020 Jan 17 doi: 10.1016/j.asjsur.2019.12.008. PMID: 31959574
Tibial perivascular epithelioid cell tumour (PEComa). A case report and literature review.
Técualt-Gómez R, Atencio-Chan A, Amaya-Zepeda RA, Cario-Méndez AG, González-Valladares R, Rodríguez-Franco JH
Rev Esp Cir Ortop Traumatol (Engl Ed) 2019 May-Jun;63(3):239-245. Epub 2019 Mar 21 doi: 10.1016/j.recot.2018.10.003. PMID: 30905544
Clinical presentations and molecular studies of invasive renal epithelioid angiomyolipoma.
Chuang CK, Lin HCA, Tasi HY, Lee KH, Kao Y, Chuang FL, Chang YH, Lin PH, Liu CY, Pang ST
Int Urol Nephrol 2017 Sep;49(9):1527-1536. Epub 2017 May 25 doi: 10.1007/s11255-017-1629-4. PMID: 28547571
Management of hepatic angiomyolipoma: A systematic review.
Klompenhouwer AJ, Verver D, Janki S, Bramer WM, Doukas M, Dwarkasing RS, de Man RA, IJzermans JNM
Liver Int 2017 Sep;37(9):1272-1280. Epub 2017 Mar 6 doi: 10.1111/liv.13381. PMID: 28177188
Evidence for pericyte origin of TSC-associated renal angiomyolipomas and implications for angiotensin receptor inhibition therapy.
Siroky BJ, Yin H, Dixon BP, Reichert RJ, Hellmann AR, Ramkumar T, Tsuchihashi Z, Bunni M, Dillon J, Bell PD, Sampson JR, Bissler JJ
Am J Physiol Renal Physiol 2014 Sep 1;307(5):F560-70. Epub 2014 Jun 11 doi: 10.1152/ajprenal.00569.2013. PMID: 24920756Free PMC Article

Prognosis

Does epithelioid angiomyolipoma have poorer prognosis, compared with classic angiomyolipoma?
Lee W, Choi SY, Lee C, Yoo S, You D, Jeong IG, Song C, Kim KS, Hong B, Hong JH, Ahn H, Kim CS
Investig Clin Urol 2018 Nov;59(6):357-362. Epub 2018 Oct 4 doi: 10.4111/icu.2018.59.6.357. PMID: 30402567Free PMC Article
Clinical presentations and molecular studies of invasive renal epithelioid angiomyolipoma.
Chuang CK, Lin HCA, Tasi HY, Lee KH, Kao Y, Chuang FL, Chang YH, Lin PH, Liu CY, Pang ST
Int Urol Nephrol 2017 Sep;49(9):1527-1536. Epub 2017 May 25 doi: 10.1007/s11255-017-1629-4. PMID: 28547571
Clinicopathologic features of renal epithelioid angiomyolipoma: report of one case and review of literatures.
Tan G, Liu L, Qiu M, Chen L, Cao J, Liu J
Int J Clin Exp Pathol 2015;8(1):1077-80. Epub 2015 Jan 1 PMID: 25755823Free PMC Article
Controversies related to epithelioid variant of renal angiomyolipoma: a review of the literature.
Serrano Frago P, Del Agua Arias Camisón C, Gil Sanz MJ, Allué López M, Gonzalvo Ibarra A, Plaza Mas L, Rioja Sanz LA
Urology 2006 Apr;67(4):846.e3-5. Epub 2006 Apr 5 doi: 10.1016/j.urology.2005.10.030. PMID: 16600344
PEComa: what do we know so far?
Hornick JL, Fletcher CD
Histopathology 2006 Jan;48(1):75-82. doi: 10.1111/j.1365-2559.2005.02316.x. PMID: 16359539

Clinical prediction guides

Renal angiomyolipoma with tuberous sclerosis complex: How it differs from sporadic angiomyolipoma in both management and care.
Hatano T, Egawa S
Asian J Surg 2020 Oct;43(10):967-972. Epub 2020 Jan 17 doi: 10.1016/j.asjsur.2019.12.008. PMID: 31959574
Does epithelioid angiomyolipoma have poorer prognosis, compared with classic angiomyolipoma?
Lee W, Choi SY, Lee C, Yoo S, You D, Jeong IG, Song C, Kim KS, Hong B, Hong JH, Ahn H, Kim CS
Investig Clin Urol 2018 Nov;59(6):357-362. Epub 2018 Oct 4 doi: 10.4111/icu.2018.59.6.357. PMID: 30402567Free PMC Article
Alterations of the mTOR pathway in hepatic angiomyolipoma with emphasis on the epithelioid variant and loss of heterogeneity of TSC1/TSC2.
Huang SC, Chuang HC, Chen TD, Chi CL, Ng KF, Yeh TS, Chen TC
Histopathology 2015 Apr;66(5):695-705. Epub 2015 Jan 20 doi: 10.1111/his.12551. PMID: 25234729
Renal epithelioid angiomyolipoma with malignant features: Histological evaluation and novel immunohistochemical findings.
Konosu-Fukaya S, Nakamura Y, Fujishima F, Kasajima A, McNamara KM, Takahashi Y, Joh K, Saito H, Ioritani N, Ikeda Y, Arai Y, Watanabe M, Sasano H
Pathol Int 2014 Mar;64(3):133-41. doi: 10.1111/pin.12142. PMID: 24698423
PEComa: what do we know so far?
Hornick JL, Fletcher CD
Histopathology 2006 Jan;48(1):75-82. doi: 10.1111/j.1365-2559.2005.02316.x. PMID: 16359539

Recent systematic reviews

Management of hepatic angiomyolipoma: A systematic review.
Klompenhouwer AJ, Verver D, Janki S, Bramer WM, Doukas M, Dwarkasing RS, de Man RA, IJzermans JNM
Liver Int 2017 Sep;37(9):1272-1280. Epub 2017 Mar 6 doi: 10.1111/liv.13381. PMID: 28177188

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