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Angiomyolipoma

MedGen UID:
64622
•Concept ID:
C0206633
•
Neoplastic Process
Synonym: Angiomyolipomas
SNOMED CT: Angiomyolipoma (19929002); Angiomyolipoma (1293131004)
 
Monarch Initiative: MONDO:0002603

Definition

A neoplasm with perivascular epithelioid cell differentiation often associated with tuberous sclerosis. It is characterized by a mixture of epithelioid cells, smooth muscle, vessels, and mature adipose tissue. The kidney is the most common site of involvement. Other sites of involvement include the liver, lung, lymph nodes, and retroperitoneum. The vast majority of cases follow a benign clinical course. However, cases of metastatic angiomyolipomas with sarcomatoid features have been described. [from NCI]

Professional guidelines

PubMed

Recommendations for the diagnosis and management of LAM: Looking towards the future.
Gupta N, McCarthy C
Respir Med Res 2023 Jun;83:101016. Epub 2023 Apr 21 doi: 10.1016/j.resmer.2023.101016. PMID: 37087907
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197
The management of renal angiomyolipoma.
Oesterling JE, Fishman EK, Goldman SM, Marshall FF
J Urol 1986 Jun;135(6):1121-4. doi: 10.1016/s0022-5347(17)46013-7. PMID: 3520013

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Soft Tissue Perivascular Epithelioid Cell Tumors.
Hammer PM, Tan SY
Surg Pathol Clin 2024 Mar;17(1):105-118. Epub 2023 Aug 1 doi: 10.1016/j.path.2023.06.002. PMID: 38278600
Epithelioid angiomyolipoma: a pathological entity discovered in Verona with the endorsement of Doctor Rosai.
Caliò A, Brunelli M, Marletta S, Zamboni G, Bonetti F, Pea M, Martignoni G
Pathologica 2021 Oct;113(5):307-315. doi: 10.32074/1591-951X-335. PMID: 34837089Free PMC Article
Renal manifestation of tuberous sclerosis complex.
Bissler JJ, Christopher Kingswood J
Am J Med Genet C Semin Med Genet 2018 Sep;178(3):338-347. Epub 2018 Oct 11 doi: 10.1002/ajmg.c.31654. PMID: 30307110
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197
Imaging of angiomyolipomas.
Lemaitre L, Claudon M, Dubrulle F, Mazeman E
Semin Ultrasound CT MR 1997 Apr;18(2):100-14. doi: 10.1016/s0887-2171(97)90054-8. PMID: 9163829

Diagnosis

Epithelioid angiomyolipoma: a pathological entity discovered in Verona with the endorsement of Doctor Rosai.
Caliò A, Brunelli M, Marletta S, Zamboni G, Bonetti F, Pea M, Martignoni G
Pathologica 2021 Oct;113(5):307-315. doi: 10.32074/1591-951X-335. PMID: 34837089Free PMC Article
Notch sign in renal angiomyolipoma.
Milosevic S, Djuric-Stefanovic A
Abdom Radiol (NY) 2020 Apr;45(4):1216-1217. doi: 10.1007/s00261-020-02418-5. PMID: 31970438
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197
Anterior mediastinal angiomyolipoma.
Candaș F, Berber U, Yildizhan A, Yiyit N, Görür R, Ișitmangil T
Ann Thorac Surg 2013 Apr;95(4):1431-2. doi: 10.1016/j.athoracsur.2012.07.066. PMID: 23522204
Renal angiomyolipoma.
Lienert AR, Nicol D
BJU Int 2012 Dec;110 Suppl 4:25-7. doi: 10.1111/j.1464-410X.2012.11618.x. PMID: 23194120

Therapy

mTOR inhibitor therapy as a disease modifying therapy for tuberous sclerosis complex.
Franz DN, Krueger DA
Am J Med Genet C Semin Med Genet 2018 Sep;178(3):365-373. Epub 2018 Oct 11 doi: 10.1002/ajmg.c.31655. PMID: 30307123
Lymphangioleiomyomatosis.
Johnson SR, Taveira-DaSilva AM, Moss J
Clin Chest Med 2016 Sep;37(3):389-403. doi: 10.1016/j.ccm.2016.04.002. PMID: 27514586
Tuberous sclerosis complex.
Henske EP, Jóźwiak S, Kingswood JC, Sampson JR, Thiele EA
Nat Rev Dis Primers 2016 May 26;2:16035. doi: 10.1038/nrdp.2016.35. PMID: 27226234
Renal embolization.
Grenier N, Petitpierre F, Le Bras Y, Lasserre AS, Cornelis F
Nephrol Ther 2016 Apr;12 Suppl 1:S139-43. Epub 2016 Mar 8 doi: 10.1016/j.nephro.2016.01.009. PMID: 26968474
Tuberous sclerosis complex: the past and the future.
De Waele L, Lagae L, Mekahli D
Pediatr Nephrol 2015 Oct;30(10):1771-80. Epub 2014 Dec 23 doi: 10.1007/s00467-014-3027-9. PMID: 25533384

Prognosis

Angiomyolipoma Mimic: Myelolipoma and Adrenal-Renal Fusion.
Schwenke M, Greene J, Braverman S, Singh M
J Vasc Interv Radiol 2020 Jun;31(6):977. doi: 10.1016/j.jvir.2019.12.004. PMID: 32444111
Tuberous sclerosis complex: the past and the future.
De Waele L, Lagae L, Mekahli D
Pediatr Nephrol 2015 Oct;30(10):1771-80. Epub 2014 Dec 23 doi: 10.1007/s00467-014-3027-9. PMID: 25533384
Focal liver lesions: MR imaging-pathologic correlation.
Bartolozzi C, Cioni D, Donati F, Lencioni R
Eur Radiol 2001;11(8):1374-88. doi: 10.1007/s003300100845. PMID: 11519546
Lymphangioleiomyomatosis.
Kelly J, Moss J
Am J Med Sci 2001 Jan;321(1):17-25. doi: 10.1097/00000441-200101000-00004. PMID: 11202475
Retroperitoneal extrarenal angiomyolipoma.
Law SY, Fok M, Shek WH, Ma LT, Wong J
Aust N Z J Surg 1994 Jun;64(6):449-51. doi: 10.1111/j.1445-2197.1994.tb02252.x. PMID: 8010912

Clinical prediction guides

Radiologist's Disease: Imaging for Renal Cancer.
Chung A, Raman SS
Urol Clin North Am 2023 May;50(2):161-180. doi: 10.1016/j.ucl.2023.01.006. PMID: 36948664
GPNMB expression identifies TSC1/2/mTOR-associated and MiT family translocation-driven renal neoplasms.
Salles DC, Asrani K, Woo J, Vidotto T, Liu HB, Vidal I, Matoso A, Netto GJ, Argani P, Lotan TL
J Pathol 2022 Jun;257(2):158-171. Epub 2022 Mar 29 doi: 10.1002/path.5875. PMID: 35072947Free PMC Article
Lymphangioleiomyomatosis.
Doubková M, Štefániková M, Čan V, Merta Z, Svoboda M
Klin Onkol 2019 Fall;32(5):367-374. doi: 10.14735/amko2019367. PMID: 31610670
Immunohistochemical approach for the diagnosis of a liver mass on small biopsy specimens.
Choi WT, Ramachandran R, Kakar S
Hum Pathol 2017 May;63:1-13. Epub 2017 Jan 11 doi: 10.1016/j.humpath.2016.12.025. PMID: 28087475
Nasal angiomyolipoma.
Erkiliç S, Koçer NE, Mumbuç S, Kanlikama M
Acta Otolaryngol 2005 Apr;125(4):446-8. doi: 10.1080/00016480510029419. PMID: 15823821

Recent systematic reviews

Clinical features and outcomes of male patients with lymphangioleiomyomatosis: A review.
Zhang H, Hu Z, Wang S, Wu K, Yang Q, Song X
Medicine (Baltimore) 2022 Dec 30;101(52):e32492. doi: 10.1097/MD.0000000000032492. PMID: 36596036Free PMC Article
The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis.
Wang Q, Luo M, Xiang B, Chen S, Ji Y
Respir Res 2020 Feb 14;21(1):55. doi: 10.1186/s12931-020-1316-3. PMID: 32059669Free PMC Article
The efficacy and adverse events of mTOR inhibitors in lymphangioleiomyomatosis: systematic review and meta-analysis.
Gao N, Zhang T, Ji J, Xu KF, Tian X
Orphanet J Rare Dis 2018 Aug 14;13(1):134. doi: 10.1186/s13023-018-0874-7. PMID: 30107845Free 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
Transarterial Embolization of Angiomyolipoma: A Systematic Review.
Murray TE, Doyle F, Lee M
J Urol 2015 Sep;194(3):635-9. Epub 2015 Apr 25 doi: 10.1016/j.juro.2015.04.081. PMID: 25916674

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Reviews

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