Angiomyolipoma medical therapy
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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]
Overview
Treatment
Angiomyolipomas found incidentally usually require no therapy. Small solitary AMLs (< 20 mm) probably do not require follow-up due to their slow growth. Lesions that present with retroperitoneal haemorrhage often requires emergency embolisation as a life saving measure. Angiomyolipoma do not normally require surgery unless there is life-threatening bleeding.[1] Some centres may perform preventative selective embolisation of the angiomyolipoma if it is more than 4 cm in diameter, due to the risk of haemorrhage.[2]
People with tuberous sclerosis are advised to have yearly renal scans, though it is possible that patients with very stable lesions could be monitored less frequently. The research in this area is lacking. Even if no angiomyolipoma is found, one can develop at any life stage. The angiomyolipoma can grow rapidly.[3]
In tuberous sclerosis, typically many angiomyolipomas affecting each kidney. It is not uncommon for more than one intervention to be required during lifetime. Since kidney function may already be impaired (up to half the kidney may be lost before function loss is detectable), it is vital to preserve as much kidney as possible when removing any lesion. Large angiomyolipomas are treated by embolisation which reduces the risk of haemorrhage and can also shrink the lesion. A side effect of this treatment is postembolisation syndrome: severe pain and fever however this is easily managed and lasts only a few days.[3]
A ruptured aneurysm in an angiomyolipoma leads to blood loss that must be stopped (though embolisation) and compensated for (through intravenous fluid replacement). Therefore, removal of the affected kidney (nephrectomy) is strongly discouraged though may occur if the emergency department is not knowledgeable about tuberous sclerosis.[3]
Embolisation involves inserting a catheter along the blood vessels to the tumour. The blood vessels are then Vascular occlusion, typically by injecting ethanol or inert particles. The procedure can be very painful, so analgesics are used. The destroyed kidney tissue often causes post-embolisation syndrome, which manifests as nausea, vomiting, fever and abdominal pain, and lasts a few days. Embolisation (in general) has an 8% rate of morbidity and a 2.5% rate of mortality, so is not considered lightly.[2]
Patients with kidney loss should be monitored for hypertension (and treated for it if discovered) and avoid nephrotoxic drugs such as certain pain relievers and IV contrast agents. Such patients who are unable to communicate effectively (due to age or intellectual disability) are at risk of dehydration. Where multiple or large angiomyolipomas have caused chronic kidney disease, dialysis is required.[3]
Rapamycin and related drugs have been studied for their effect on angiomyolipomas due to their action on the pathways that are disrupted when TSC1 and TSC2 genes are broken. Small studies showed the lesions shrank but that the drugs were often toxic. The use of these drugs remains at the research stage.[3] Robotic assisted partial nephrectomy has been proposed as a surgical treatment of a ruptured angiomyolipoma combining the advantages both of a kidney preservation procedure and the benefits of a minimal invasive procedure without compromising the safety of the patient.[4]
References
- ↑ Shin, NY; Kim, MJ; Chung, JJ; Chung, YE; Choi, JY; Park, YN (May–Jun 2010). "The differential imaging features of fat-containing tumors in the peritoneal cavity and retroperitoneum: the radiologic-pathologic correlation" (PDF). Korean journal of radiology : official journal of the Korean Radiological Society. 11 (3): 333–45. doi:10.3348/kjr.2010.11.3.333. PMC 2864861. PMID 20461188.
- ↑ 2.0 2.1 Loffroy, R; Rao, P; Kwak, BK; Ota, S; De Lin, M; Liapi, E; Geschwind, JF (May–Jun 2010). "Transcatheter arterial embolization in patients with kidney diseases: an overview of the technical aspects and clinical indications" (PDF). Korean journal of radiology : official journal of the Korean Radiological Society. 11 (3): 257–68. doi:10.3348/kjr.2010.11.3.257. PMC 2864852. PMID 20461179.
- ↑ 3.0 3.1 3.2 3.3 3.4 Bissler JJ, Henske EP. Renal Manifestations of Tuberous Sclerosis Complex. In: Kwiatkowski DJ, Wiittlemore DJ, Thiele EA, editors. Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics. Wiley-VCH Verlag GmbH; 2010. p. 321–325. ISBN 3-527-32201-9.
- ↑ Ploumidis, A; Katafigiotis, I; Thanou, M; Bodozoglou, N; Athanasiou, L; Ploumidis, A (2013). "Spontaneous Retroperitoneal Hemorrhage (Wunderlich Syndrome) due to Large Upper Pole Renal Angiomyolipoma: Does Robotic-Assisted Laparoscopic Partial Nephrectomy Have a Role in Primary Treatment?". Case reports in urology. 2013: 498694. doi:10.1155/2013/498694. PMC 3784227. PMID 24106637.