Angiomyolipoma natural history, complications and prognosis: Difference between revisions

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Revision as of 07:59, 15 August 2018

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]

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.

Angiomyolipoma, less than 2cm, surveillance every 3-4 years by renal ultrasound.
Angiomyolipoma 2-4cm, annual renal ultrasound recommended.
Angiomyolipoma more than 4cm, and of Epitheloid variant, renal ultrasound every 6 months initially and annual thereafter once stabilized, in case if 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.^[1]^[2]^[3]^[4]^[5].Due to increased rate of growth of Angiomyolipoma and increased risk of its rupture, it is advised to use estrogen cautionally in these patients, and frequent imaging surveillance every six months initially and annual thereafter, once stablised,is recommended.

Complications

Common complications of angiomyolipoma include:

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^[6]

Retroperitoneal hemorrhage from renal angiomyolipoma.^[7]

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:

10% among males
20% among females

References

↑ 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.
↑ Image courtesy of Dr Matt A. Morgan and Dr Behrang Amini et alRadiopaedia(original file [1]). Creative Commons BY-SA-NC

Template:WikiDoc Sources

[pmid17018483-1] 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.

[pmid4082575-2] Lewis EL, Palmer JM (1985). "Renal angiomyolipoma and massive retroperitoneal hemorrhage during pregnancy". West J Med. 143 (5): 675–6. PMC 1306454. PMID 4082575.

[pmid21876331-3] 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.

[pmid16996772-4] 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.

[pmid2184818-5] 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.

[pmid20410812-6] 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.

[radio-7] Image courtesy of Dr Matt A. Morgan and Dr Behrang Amini et alRadiopaedia(original file [1]). Creative Commons BY-SA-NC

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 ==Natural History==
-If left untreated, small angiomyolipomas and those without dilated blood vessels ([[aneurysm]]s) 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.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.<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 cautionally in these patients, and frequent imaging surveillance every six months initially and annual thereafter, once stablised,is recommended.
+If left untreated, small angiomyolipomas and those without dilated blood vessels ([[aneurysm]]s) 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.
+* Angiomyolipoma, less than 2cm, surveillance every 3-4 years by renal ultrasound.
+* Angiomyolipoma 2-4cm, annual renal ultrasound recommended.
+* Angiomyolipoma more than 4cm, and of Epitheloid variant, renal ultrasound every 6 months initially and annual thereafter once stabilized, in case if 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.<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 cautionally in these patients, and frequent imaging surveillance every six months initially and annual thereafter, once stablised,is recommended.
 ==Complications==