Angiomyolipoma natural history, complications and prognosis: Difference between revisions
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{{Angiomyolipoma}} | {{Angiomyolipoma}} | ||
{{CMG}}''' Associate Editor-In-Chief:''' {{CZ}}, {{Faizan}} | {{CMG}}''' Associate Editor-In-Chief:''' {{CZ}}, {{Faizan}},{{Rekha}} | ||
==Overview== | ==Overview== | ||
If left untreated, small angiomyolipomas and those without dilated blood vessels (aneurysms) cause few | If left untreated, small [[Angiomyolipoma|angiomyolipomas]] and those without dilated [[Blood vessel|blood vessels]] ([[Aneurysm|aneurysms]]) cause few [[Symptom|symptoms]]. Small [[Angiomyolipoma|angiomyolipomas]] are associated with the most favorable [[prognosis]]. Common [[complications]] of [[angiomyolipoma]] include [[infection]], [[hemorrhage]], and [[shock]]. | ||
==Natural History== | ==Natural History== | ||
If left untreated, small angiomyolipomas and those without dilated blood vessels ( | * If left untreated, small [[Angiomyolipoma|angiomyolipomas]] and those without dilated [[Blood vessel|blood vessels]] ([[aneurysm]]s) cause few problems. | ||
== | *[[Angiomyolipoma (patient information)|Angiomyolipomas]] grow as rapidly as 4 cm in one year so regular surveillance is necessary depending upon the size of the [[tumour]].<ref name="pmid24325283">{{cite journal| author=Ouzaid I, Autorino R, Fatica R, Herts BR, McLennan G, Remer EM et al.| title=Active surveillance for renal angiomyolipoma: outcomes and factors predictive of delayed intervention. | journal=BJU Int | year= 2014 | volume= 114 | issue= 3 | pages= 412-7 | pmid=24325283 | doi=10.1111/bju.12604 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24325283 }}</ref><ref name="pmid8230504">{{cite journal| author=Steiner MS, Goldman SM, Fishman EK, Marshall FF| title=The natural history of renal angiomyolipoma. | journal=J Urol | year= 1993 | volume= 150 | issue= 6 | pages= 1782-6 | pmid=8230504 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8230504 }}</ref> | ||
**[[Angiomyolipoma]], less than 2cm, surveillance every 3-4 years by renal [[ultrasound]]. | |||
**[[Angiomyolipoma]] 2-4 cm, annual renal [[ultrasound]] recommended. | |||
* | **[[Angiomyolipoma]] more than 4 cm, and of [[Epithelioid]] variant, renal [[ultrasound]] every 6 months initially and annual thereafter once stabilized, in case if it is not resected. | ||
**[[Angiomyolipoma]] with high risk of [[malignant]] transformation, should undergo whole body [[Computed tomography|CT]]/[[Magnetic resonance imaging|MRI]], 6 months post [[resection]] and then yearly for at least 5 years. | |||
* It has been observed in some studies that the female sex [[Hormone|hormones]] has positive impact on the size of the [[angiomyolipoma]]. | |||
* There has been increased growth and [[hemorrhagic]] [[complications]] seen during the [[pregnancy]] as well as after treatment with [[exogenous]] [[hormonal]] therapy.<ref name="pmid17018483">{{cite journal| author=Gould Rothberg BE, Grooms MC, Dharnidharka VR| title=Rapid growth of a kidney angiomyolipoma after initiation of oral contraceptive therapy. | journal=Obstet Gynecol | year= 2006 | volume= 108 | issue= 3 Pt 2 | pages= 734-6 | pmid=17018483 | doi=10.1097/01.AOG.0000188062.13749.ef | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17018483 }}</ref><ref name="pmid4082575">{{cite journal| author=Lewis EL, Palmer JM| title=Renal angiomyolipoma and massive retroperitoneal hemorrhage during pregnancy. | journal=West J Med | year= 1985 | volume= 143 | issue= 5 | pages= 675-6 | pmid=4082575 | doi= | pmc=1306454 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4082575 }}</ref><ref name="pmid21876331">{{cite journal| author=Zapardiel I, Delafuente-Valero J, Bajo-Arenas JM| title=Renal angiomyolipoma during pregnancy: review of the literature. | journal=Gynecol Obstet Invest | year= 2011 | volume= 72 | issue= 4 | pages= 217-9 | pmid=21876331 | doi=10.1159/000329328 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21876331 }}</ref><ref name="pmid16996772">{{cite journal| author=Raft J, Lalot JM, Meistelman C, Longrois D| title=[Renal angiomyolipoma rupture during pregnancy]. | journal=Gynecol Obstet Fertil | year= 2006 | volume= 34 | issue= 10 | pages= 917-9 | pmid=16996772 | doi=10.1016/j.gyobfe.2006.02.021 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16996772 }}</ref><ref name="pmid2184818">{{cite journal| author=Vandenesch F, Surgot M, Bornstein N, Paucod JC, Marmet D, Isoard P et al.| title=Relationship between free amoeba and Legionella: studies in vitro and in vivo. | journal=Zentralbl Bakteriol | year= 1990 | volume= 272 | issue= 3 | pages= 265-75 | pmid=2184818 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2184818 }}</ref> | |||
* Due to increased rate of growth of [[angiomyolipoma]] and increased risk of its [[rupture]], it is advised to use [[estrogen]] carefully in these patients, and frequent imaging surveillance every six months initially and annual thereafter, once established, is recommended. | |||
==Complications== | ==Complications== | ||
Common complications of angiomyolipoma include: | * Common [[complications]] of [[angiomyolipoma]] include:<ref name="pmid20410812">{{cite journal| author=Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI| title=Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. | journal=Am J Surg Pathol | year= 2010 | volume= 34 | issue= 5 | pages= 715-22 | pmid=20410812 | doi=10.1097/PAS.0b013e3181d90370 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20410812 }}</ref> | ||
* [[Infection]]s | **[[Infection]]s | ||
* [[Urinary obstruction]] | **[[Urinary obstruction]] | ||
* [[Intestinal obstruction]] | **End stage [[Renal insufficiency|renal failure]], in case of bilateral [[angiomyolipoma]] | ||
* [[Shock]] | **[[Intestinal obstruction]] | ||
* [[Hemorrhage]] | **[[Shock]] | ||
**[[Hemorrhage]] | |||
**Rarely, it can transform into [[malignant]] [[angiomyolipoma]], especially if related to [[Tuberous sclerosis|Tuberous Sclerosis]] | |||
==Prognosis== | |||
* Small [[Angiomyolipoma|angiomyolipomas]] are associated with the most favorable [[prognosis]]. An [[angiomyolipoma]] larger than 5 cm and those containing an [[aneurysm]] pose a significant risk of [[rupture]], which is a [[medical emergency]] as it is potentially life-threatening. Cumulative risk of [[hemorrhage]] is estimated to be:<ref name="pmid26165440">{{cite journal| author=Eijkemans MJ, van der Wal W, Reijnders LJ, Roes KC, van Waalwijk van Doorn-Khosrovani SB, Pelletier C et al.| title=Long-term Follow-up Assessing Renal Angiomyolipoma Treatment Patterns, Morbidity, and Mortality: An Observational Study in Tuberous Sclerosis Complex Patients in the Netherlands. | journal=Am J Kidney Dis | year= 2015 | volume= 66 | issue= 4 | pages= 638-45 | pmid=26165440 | doi=10.1053/j.ajkd.2015.05.016 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26165440 }}</ref> | |||
**10% among males | |||
**20% among females | |||
==References== | ==References== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2], Faizan Sheraz, M.D. [3],Rekha, M.D.
Overview
If left untreated, small angiomyolipomas and those without dilated blood vessels (aneurysms) cause few symptoms. Small angiomyolipomas are associated with the most favorable prognosis. Common complications of angiomyolipoma include infection, hemorrhage, and shock.
Natural History
- If left untreated, small angiomyolipomas and those without dilated blood vessels (aneurysms) cause few problems.
- Angiomyolipomas grow as rapidly as 4 cm in one year so regular surveillance is necessary depending upon the size of the tumour.[1][2]
- Angiomyolipoma, less than 2cm, surveillance every 3-4 years by renal ultrasound.
- Angiomyolipoma 2-4 cm, annual renal ultrasound recommended.
- Angiomyolipoma more than 4 cm, and of Epithelioid variant, renal ultrasound every 6 months initially and annual thereafter once stabilized, in case if it is not resected.
- Angiomyolipoma with high risk of malignant transformation, should undergo whole body CT/MRI, 6 months post resection and then yearly for at least 5 years.
- It has been observed in some studies that the female sex hormones has positive impact on the size of the angiomyolipoma.
- There has been increased growth and hemorrhagic complications seen during the pregnancy as well as after treatment with exogenous hormonal therapy.[3][4][5][6][7]
- Due to increased rate of growth of angiomyolipoma and increased risk of its rupture, it is advised to use estrogen carefully in these patients, and frequent imaging surveillance every six months initially and annual thereafter, once established, is recommended.
Complications
- Common complications of angiomyolipoma include:[8]
- Infections
- Urinary obstruction
- End stage renal failure, in case of bilateral angiomyolipoma
- Intestinal obstruction
- Shock
- Hemorrhage
- Rarely, it can transform into malignant angiomyolipoma, especially if related to Tuberous Sclerosis
Prognosis
- Small angiomyolipomas are associated with the most favorable prognosis. An angiomyolipoma larger than 5 cm and those containing an aneurysm pose a significant risk of rupture, which is a medical emergency as it is potentially life-threatening. Cumulative risk of hemorrhage is estimated to be:[9]
- 10% among males
- 20% among females
References
- ↑ Ouzaid I, Autorino R, Fatica R, Herts BR, McLennan G, Remer EM; et al. (2014). "Active surveillance for renal angiomyolipoma: outcomes and factors predictive of delayed intervention". BJU Int. 114 (3): 412–7. doi:10.1111/bju.12604. PMID 24325283.
- ↑ Steiner MS, Goldman SM, Fishman EK, Marshall FF (1993). "The natural history of renal angiomyolipoma". J Urol. 150 (6): 1782–6. PMID 8230504.
- ↑ Gould Rothberg BE, Grooms MC, Dharnidharka VR (2006). "Rapid growth of a kidney angiomyolipoma after initiation of oral contraceptive therapy". Obstet Gynecol. 108 (3 Pt 2): 734–6. doi:10.1097/01.AOG.0000188062.13749.ef. PMID 17018483.
- ↑ Lewis EL, Palmer JM (1985). "Renal angiomyolipoma and massive retroperitoneal hemorrhage during pregnancy". West J Med. 143 (5): 675–6. PMC 1306454. PMID 4082575.
- ↑ Zapardiel I, Delafuente-Valero J, Bajo-Arenas JM (2011). "Renal angiomyolipoma during pregnancy: review of the literature". Gynecol Obstet Invest. 72 (4): 217–9. doi:10.1159/000329328. PMID 21876331.
- ↑ Raft J, Lalot JM, Meistelman C, Longrois D (2006). "[Renal angiomyolipoma rupture during pregnancy]". Gynecol Obstet Fertil. 34 (10): 917–9. doi:10.1016/j.gyobfe.2006.02.021. PMID 16996772.
- ↑ Vandenesch F, Surgot M, Bornstein N, Paucod JC, Marmet D, Isoard P; et al. (1990). "Relationship between free amoeba and Legionella: studies in vitro and in vivo". Zentralbl Bakteriol. 272 (3): 265–75. PMID 2184818.
- ↑ Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI (2010). "Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy". Am J Surg Pathol. 34 (5): 715–22. doi:10.1097/PAS.0b013e3181d90370. PMID 20410812.
- ↑ Eijkemans MJ, van der Wal W, Reijnders LJ, Roes KC, van Waalwijk van Doorn-Khosrovani SB, Pelletier C; et al. (2015). "Long-term Follow-up Assessing Renal Angiomyolipoma Treatment Patterns, Morbidity, and Mortality: An Observational Study in Tuberous Sclerosis Complex Patients in the Netherlands". Am J Kidney Dis. 66 (4): 638–45. doi:10.1053/j.ajkd.2015.05.016. PMID 26165440.