Angiomyolipoma medical therapy: Difference between revisions
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{{Angiomyolipoma}} | {{Angiomyolipoma}} | ||
{{CMG}} {{AE}}{{Faizan}} | {{CMG}} {{AE}}{{Faizan}},{{Rekha}} | ||
==Overview== | ==Overview== | ||
Treatment options include selective renal artery [[embolization]], [[nephron]] sparing [[surgery]]( [[enucleation]] or partial [[nephrectomy]]), complete and radical [[nephrectomy]], radio-frequency ablation and [[everolimus]]. Treatment and management of the [[angiomyolipoma]] depends largely on | |||
# Size of the [[Tumor|tumors]] | |||
# Signs and [[Symptom|symptoms]] | |||
# Presence or absence of Life threatening [[hemorrhage]] | |||
# Its association with the [[tuberous sclerosis]] and | |||
# The presence of the [[comorbidities]]. | |||
[[Angiomyolipoma|Angiomyolipomas]] found incidentally, asymptomatic, < 4 cm in size, generally requires no intervention beside periodic evaluation. Medium sized lesion(4-8 cm) should be closely followed with the serial imaging studies(every 6 month and then yearly once stable) and elective intervention provided if the change in the size or [[symptoms]] noticed, to increases the chances of [[Kidney|renal]] function salvage. Larger [[lesion]](>8cm), shown to progress to the development of the [[Complications|complication]] so treated electively. | |||
==Medical Therapy== | ==Medical Therapy== | ||
[[Everolimus]], an [[mTOR]] inhibitor, is [[Food and Drug Administration|FDA]] approved for the treatment of [[Angiomyolipoma|angiomyolipomas]]. It is most commonly used in [[Tuberous sclerosis]] associated [[angiomyolipoma]] rather than sporadic cases. It has been shown to reduce the [[tumor]] volume as well as its risk of progression.<ref name="pmid24729041">{{cite journal| author=Kingswood JC, Jozwiak S, Belousova ED, Frost MD, Kuperman RA, Bebin EM et al.| title=The effect of everolimus on renal angiomyolipoma in patients with tuberous sclerosis complex being treated for subependymal giant cell astrocytoma: subgroup results from the randomized, placebo-controlled, Phase 3 trial EXIST-1. | journal=Nephrol Dial Transplant | year= 2014 | volume= 29 | issue= 6 | pages= 1203-10 | pmid=24729041 | doi=10.1093/ndt/gfu013 | pmc=4852219 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24729041 }}</ref><ref name="pmid19493268">{{cite journal| author=Sooriakumaran P, Gibbs P, Coughlin G, Attard V, Elmslie F, Kingswood C et al.| title=Angiomyolipomata: challenges, solutions, and future prospects based on over 100 cases treated. | journal=BJU Int | year= 2010 | volume= 105 | issue= 1 | pages= 101-6 | pmid=19493268 | doi=10.1111/j.1464-410X.2009.08649.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19493268 }}</ref> | |||
Patients with loss of renal function should be monitored for: | |||
=== Indications of Everolimus in Angiomyolipoma: === | |||
* Multiple large [[angiomyolipoma]]<ref name="pmid3520013">{{cite journal| author=Oesterling JE, Fishman EK, Goldman SM, Marshall FF| title=The management of renal angiomyolipoma. | journal=J Urol | year= 1986 | volume= 135 | issue= 6 | pages= 1121-4 | pmid=3520013 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3520013 }}</ref><ref name="pmid9617489">{{cite journal| author=Dickinson M, Ruckle H, Beaghler M, Hadley HR| title=Renal angiomyolipoma: optimal treatment based on size and symptoms. | journal=Clin Nephrol | year= 1998 | volume= 49 | issue= 5 | pages= 281-6 | pmid=9617489 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9617489 }}</ref> | |||
* Bilateral [[angiomyolipoma]] | |||
* Progression of the [[lesion]]( > 5mm/year) | |||
* Hx of prior [[nephrectomy]] or [[embolization]] | |||
Patients with loss of [[renal]] function should be monitored for: | |||
*[[Hypertension]] | *[[Hypertension]] | ||
*Adverse effects of nephrotoxic drugs such as: | *Adverse effects of [[Nephrotoxicity|nephrotoxic]] drugs such as: | ||
**[[NSAIDS]] | **[[NSAIDS]] | ||
**IV contrast agents | **IV contrast agents | ||
Latest revision as of 16:24, 8 November 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2],Rekha, M.D.
Overview
Treatment options include selective renal artery embolization, nephron sparing surgery( enucleation or partial nephrectomy), complete and radical nephrectomy, radio-frequency ablation and everolimus. Treatment and management of the angiomyolipoma depends largely on
- Size of the tumors
- Signs and symptoms
- Presence or absence of Life threatening hemorrhage
- Its association with the tuberous sclerosis and
- The presence of the comorbidities.
Angiomyolipomas found incidentally, asymptomatic, < 4 cm in size, generally requires no intervention beside periodic evaluation. Medium sized lesion(4-8 cm) should be closely followed with the serial imaging studies(every 6 month and then yearly once stable) and elective intervention provided if the change in the size or symptoms noticed, to increases the chances of renal function salvage. Larger lesion(>8cm), shown to progress to the development of the complication so treated electively.
Medical Therapy
Everolimus, an mTOR inhibitor, is FDA approved for the treatment of angiomyolipomas. It is most commonly used in Tuberous sclerosis associated angiomyolipoma rather than sporadic cases. It has been shown to reduce the tumor volume as well as its risk of progression.[1][2]
Indications of Everolimus in Angiomyolipoma:
- Multiple large angiomyolipoma[3][4]
- Bilateral angiomyolipoma
- Progression of the lesion( > 5mm/year)
- Hx of prior nephrectomy or embolization
Patients with loss of renal function should be monitored for:
- Hypertension
- Adverse effects of nephrotoxic drugs such as:
- NSAIDS
- IV contrast agents
References
- ↑ Kingswood JC, Jozwiak S, Belousova ED, Frost MD, Kuperman RA, Bebin EM; et al. (2014). "The effect of everolimus on renal angiomyolipoma in patients with tuberous sclerosis complex being treated for subependymal giant cell astrocytoma: subgroup results from the randomized, placebo-controlled, Phase 3 trial EXIST-1". Nephrol Dial Transplant. 29 (6): 1203–10. doi:10.1093/ndt/gfu013. PMC 4852219. PMID 24729041.
- ↑ Sooriakumaran P, Gibbs P, Coughlin G, Attard V, Elmslie F, Kingswood C; et al. (2010). "Angiomyolipomata: challenges, solutions, and future prospects based on over 100 cases treated". BJU Int. 105 (1): 101–6. doi:10.1111/j.1464-410X.2009.08649.x. PMID 19493268.
- ↑ Oesterling JE, Fishman EK, Goldman SM, Marshall FF (1986). "The management of renal angiomyolipoma". J Urol. 135 (6): 1121–4. PMID 3520013.
- ↑ Dickinson M, Ruckle H, Beaghler M, Hadley HR (1998). "Renal angiomyolipoma: optimal treatment based on size and symptoms". Clin Nephrol. 49 (5): 281–6. PMID 9617489.