Angiomyolipoma

A benign tumor containing vascular, adipose, and muscle elements. It occurs most often in the kidney with smooth muscle elements (angiolipoleiomyoma) in association with tuberous sclerosis. (Dorland, 27th ed)

Also Known As:

Networked: 1197 relevant articles (56 outcomes, 110 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Tuberous Sclerosis (Bourneville's Disease)
2.	Neoplasms (Cancer)
3.	Astrocytoma (Pilocytic Astrocytoma)
4.	Angiofibroma
5.	Lymphangioleiomyomatosis (Lymphangiomyomatosis)

Experts

1.	Moss, Joel: 21 articles (01/2019 - 12/2004)
2.	Henske, Elizabeth P: 20 articles (10/2021 - 03/2002)
3.	Kwiatkowski, David J: 19 articles (01/2022 - 10/2002)
4.	Bissler, John J: 16 articles (01/2019 - 11/2002)
5.	Curatolo, Paolo: 12 articles (01/2020 - 06/2008)
6.	Franz, David N: 11 articles (01/2022 - 01/2008)
7.	Sauter, Matthias: 11 articles (10/2021 - 12/2012)
8.	Belousova, Elena: 11 articles (01/2020 - 03/2013)
9.	Zonnenberg, Bernard A: 11 articles (12/2019 - 03/2013)
10.	Franz, David Neal: 10 articles (01/2020 - 01/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Angiomyolipoma:

1.	EverolimusFDA Link 09/01/2020 - "Everolimus was effective in causing volumetric reduction of angiomyolipomas by 25% or greater in most patients but was associated with a high rate of treatment discontinuation." 09/01/2020 - "The primary outcomes were the efficacy of everolimus in the volumetric reduction of angiomyolipomas by 25% or more, and the safety and tolerability of everolimus. " 05/01/2018 - "While previous reports have largely assessed the effect of everolimus for angiomyolipomas of < 10 cm in the longest diameter, our findings suggest that this drug might also be effective for huge lesions of > 20 cm in diameter. " 06/01/2018 - "The aim of this study was to evaluate the influence of components of angiomyolipoma (AML) on the efficacy of everolimus. " 09/01/2020 - "We sought to determine the safety and efficacy of everolimus in the volumetric reduction of sporadic angiomyolipomas. "
2.	trans-sodium crocetinate (crocetin)IBA 01/01/2019 - "A total of 1497 TSC patients met the study criteria (896 renal angiomyolipoma only, 411 SEGA only and 190 both). " 12/01/2018 - "This study's objective is to describe the age-stratified morbidity, treatment patterns, and health-related quality of life of TSC patients with renal angiomyolipomas in the United States. " 12/01/2018 - "Our current study aims to further explore whether there is such a correlation in patients with TSC-associated renal angiomyolipoma (TSC-RAML). " 12/01/2017 - "A multidisciplinary expert panel developed a series of recommendations based on current evidence and professional experience for imaging studies in adults and children with TSC-associated renal angiomyolipoma. " 07/01/1998 - "This study suggests that hormonal factors play a role in the pathogenesis of TSC-associated angiomyolipomas."
3.	MTOR InhibitorsIBA 06/01/2012 - "The role of mTOR inhibitors in the management of angiomyolipomas needs to be defined. " 11/01/2013 - "Targeted therapies, such as mTOR inhibitors, which have demonstrated efficacy in maintaining and reducing renal angiomyolipoma size, are of great benefit to patients. " 01/01/2016 - "We specifically reviewed case series of partial nephrectomy, selective arterial embolization and ablative therapies as well as trials of mTOR inhibitors for angiomyolipoma from 1999 to 2014. " 10/01/2018 - "There are several cases of malignant angiomyolipoma which exhibit transient responses to mTOR inhibitors, forming the basis of current practice guidelines in malignant PEComa. " 01/01/2016 - "We also review recent advances in the medical treatment of patients with tuberous sclerosis complex associated angiomyolipomas with mTOR inhibitors. "
4.	Sirolimus (Rapamycin)FDA Link 01/01/2021 - "Our study showed that sirolimus reduces the size of angiomyolipomas by decreasing primarily their highly-vascularized and fat-poor compartments. " 11/01/2014 - "Studies that reported an angiomyolipoma response or adverse events after the treatment of sirolimus were included in the analysis. " 09/01/2011 - "In two studies, angiomyolipoma size decreased by 26-50% after 12 months of sirolimus treatment. " 01/01/2021 - "Volume reduction following treatment with sirolimus was higher in fat-poor than fat-rich angiomyolipomas. " 01/01/2021 - "Thirty angiomyolipomas from 14 patients treated with sirolimus were retrospectively evaluated. "
5.	lipoarabinomannan (LAM)IBA 05/23/2000 - "In this study, we report the identification of somatic TSC2 mutations in five of seven angiomyolipomas from sporadic LAM patients. " 01/01/2019 - "Immunohistochemistry showed strong nuclear expression in both LAM and angiomyolipoma tumours. " 01/01/2018 - "We present a young woman with LAM with an aggressive renal angiomyolipoma confirmed at autopsy." 03/01/2017 - "The first had about 50% regression of the pelvic LAM and renal angiomyolipoma (AML). " 01/01/2016 - "LAM and angiomyolipoma are significantly associated, and may coexist in the lungs in rare cases. "
6.	TOR Serine-Threonine KinasesIBA 09/01/2013 - "Although the mammalian target of rapamycin inhibitor seems to be effective for treating epithelioid angiomyolipoma, its long-term effects remain unknown. " 09/01/2013 - "Role of mammalian target of rapamycin inhibitor in the treatment of metastatic epithelioid angiomyolipoma: a case report." 09/01/2013 - "Editorial comment to role of mammalian target of rapamycin inhibitor in the treatment of metastatic epithelioid angiomyolipoma: a case report." 04/19/2003 - "Mutation in TSC2 and activation of mammalian target of rapamycin signalling pathway in renal angiomyolipoma." 05/01/2017 - "Fluorine-18-FDG PET/CT in a patient with angiomyolipoma: Response to mammalian target of rapamycin inhibitor therapy."
7.	LipidsIBA 01/01/2023 - "To evaluate the prevalence of angular interface and the "drooping" sign in exophytic renal angiomyolipomas (AMLs) and the diagnostic performance in differentiating exophytic lipid-poor AMLs from other solid renal masses. " 07/01/2022 - "• It is important to differentiate renal epithelioid lipid-poor angiomyolipoma (E-lpAML) from other lpAML because of differences in clinical management. " 07/01/2022 - "To identify specific imaging and clinicopathological features of a rare potentially malignant epithelioid variant of renal lipid-poor angiomyolipoma (E-lpAML). " 07/01/2022 - "CT imaging findings of renal epithelioid lipid-poor angiomyolipoma." 01/01/2021 - "Lipid-poor angiomyolipomas (lpAMLs) constitute up to 5% of renal angiomyolipomas and are challenging to differentiate from malignant renal lesions on imaging alone. "
8.	Mechanistic Target of Rapamycin Complex 1IBA 01/01/2018 - "mTORC1 Inhibition Is an Effective Treatment for Sporadic Renal Angiomyolipoma." 10/01/2023 - "Although TSC1 or TSC2 inactivating mutations that lead to mTORC1 hyperactivation have been reported in hepatic angiomyolipomas (hAML), the role of other somatic genetic events that may contribute to hAML development is unknown. " 11/01/2018 - "In clinical trials, allosteric inhibitors of mTORC1 decrease angiomyolipoma size, but the tumours regrow after treatment cessation. " 06/15/2011 - "This study investigated the efficacy and safety of the mTORC1 inhibitor sirolimus for treatment of renal angiomyolipomas in patients with these disorders. " 11/01/2018 - "This Review describes important advances in the TSC field and highlights several remaining critical knowledge gaps: the factors that promote aggressive behaviour by a subset of TSC-associated RCCs; the molecular mechanisms underlying early-onset cystogenesis in TSC2-PKD1 contiguous gene deletion syndrome; the effect of early, long-term mTORC1 inhibition on the development of TSC renal disease; and the identification of the cell or cells of origin of angiomyolipomas."
9.	Ethanol (Ethyl Alcohol)IBA 09/01/2021 - "We sought to determine the efficacy and safety of selective arterial embolization for renal angiomyolipoma (AML) using ethanol alone or ethanol with additional embolic materials and to analyze the factors influencing safety and efficacy. " 09/01/2021 - "Radiologic and clinical results of transarterial ethanol embolization for renal angiomyolipoma." 01/01/2020 - "Selective Arterial Embolizations of Renal Angiomyolipomas Using 96% Ethanol: A Case Series of 5 Patients." 10/01/2014 - "Transcatheter arterial embolization (TAE) with absolute ethanol is widely accepted as a therapeutic procedure for renal angiomyolipoma (AML). " 10/01/2014 - "Evaluation of split renal function before and after renal arterial embolization for angiomyolipoma using absolute ethanol."
10.	Ethiodized Oil (Ethiodol)FDA Link 08/01/2014 - "Embolization with a mixture of lipiodol and PVA particles is an effective and safe treatment for symptomatic renal angiomyolipoma. " 08/01/2014 - "Seven patients underwent eight episodes of embolization using a mixture of lipiodol and PVA particles to treat symptomatic renal angiomyolipoma. " 08/01/2014 - "We performed a retrospective review of the seven patients who underwent embolization with a mixture of lipiodol and PVA particles to treat symptomatic renal angiomyolipoma in our center between February 2011 and December 2013. " 08/01/2014 - "Embolization of symptomatic renal angiomyolipoma with a mixture of lipiodol and PVA, a mid-term result." 09/08/2021 - "Selective Arterial Embolization of Renal Angiomyolipomas with a N-Butyl Cyanoacrylate-Lipiodol Mixture: Efficacy, Safety, Short- and Mid-Term Outcomes."

Therapies and Procedures

1.	Therapeutics 12/01/2012 - "In the absence of a curative treatment, recent publications show promising results in molecular therapy to prevent functional decline and to control the size of the angiomyolipomas. " 05/01/2019 - "Angiomyolipoma of the Kidneys: Current Perspectives and Challenges in Diagnostic Imaging and Image-Guided Therapy." 02/01/2018 - "Giant Renal Angiomyolipoma Following Ovarian Stimulation Therapy." 01/01/2018 - "If an angiomyolipoma diagnosis cannot be established with imaging, further diagnostics are appropriate or if malignant progression is suspected, then multidisciplinary discussion for TNM-staging based uro-oncologic therapy is usual. " 01/01/2016 - "Based on recent evidence, new guidelines should be considered that support the earlier initiation of mTOR inhibitor therapy for the management of renal angiomyolipomas to prevent future serious complications, rather than try to rescue patients after the complications have occurred."
2.	Nephrectomy 03/01/2018 - "To present the long-term result and efficacy of robotic partial nephrectomy (RPN) for renal angiomyolipomas (AMLs) with perioperative outcome and renal function preservation. " 03/01/2016 - "To present the experience and efficacy of Robotic Partial Nephrectomy (RPN) for the management of renal angiomyolipomas (AMLs) with regard to renal function preservation and perioperative outcomes. " 01/01/2023 - "Comparisons of the Safety and Effectiveness of Robot-Assisted vs Laparoscopic Partial Nephrectomy for Central Renal Angiomyolipomas: A Propensity Score-Matched Analysis Study." 09/01/2021 - "Immediate nephrectomy was performed and histopathological and further immunohistochemical study, revealed the epithelioid variant of angiomyolipoma." 09/01/2015 - "The study included 406 patients who had 47 angiomyolipomas without visible fat and 359 RCCs smaller than 4 cm, all of which were diagnosed on the basis of findings from nephrectomy or percutaneous biopsy performed at our institution between 2000 and 2011. "
3.	Radiofrequency Ablation 02/01/2012 - "To show that radiofrequency ablation (RFA) is safe and effective treatment for renal angiomyolipoma (AML). " 12/01/2019 - "The present study analyzed the safety and efficacy of superselective arterial embolization (SAE) combined with radiofrequency ablation (RFA) to treat renal angiomyolipoma (RAML). " 12/01/2019 - "A new management strategy for renal angiomyolipomas: superselective arterial embolization in combination with radiofrequency ablation." 02/01/2016 - "[A clinical observation of cool-tip radiofrequency ablation assisted enucleation for giant renal angiomyolipoma]." 01/01/2016 - "The combination of superselective transarterial embolization and radiofrequency ablation seems to be a feasible, safe, and efficient treatment of large renal angiomyolipomas. "
4.	Therapeutic Embolization 07/01/1989 - "Moreover, therapeutic embolization for angiomyolipoma was concluded to be very useful." 07/01/1989 - "[Therapeutic embolization of renal angiomyolipoma: a case report]." 11/01/1987 - "This case and others encountered in our review of the literature underscore the usefulness of therapeutic embolization for renal angiomyolipoma." 11/01/1987 - "Therapeutic embolization for renal angiomyolipoma: case report and review of the literature." 06/01/1982 - "[Therapeutic embolization of renal angiomyolipoma--a case report]."
5.	Aftercare (After-Treatment) 12/01/2012 - "Recently, regression of angiomyolipoma size has also been achieved with mTOR inhibitor therapy, but recurrent lesion growth after treatment cessation has to be expected. " 06/01/2011 - "Development of hepatic angiomyolipoma accompanied with focal nodular hyperplasia long after treatment of pelvic rhabdomyosarcoma." 11/01/2018 - "In clinical trials, allosteric inhibitors of mTORC1 decrease angiomyolipoma size, but the tumours regrow after treatment cessation. " 09/01/2011 - "The protective effect of sirolimus was lost after treatment discontinuation, with a parallel lung function decline in both groups, similar to the increase in angiomyolipoma size. " 12/01/2012 - "Reduction in size of renal angiomyolipoma after treatment with everolimus in lung transplantation due to lymphangioleiomyomatosis."