Renal angiomyolipomas: At least two diseases. A series of patients treated at two European institutions

Delhorme, Jean-Baptiste; Fontana, A.; Lévy, Antonin; Terrier, Philippe; Fiore, Marco; Tzanis, Dimitrios; Callegaro, Dario; Dratwa, C; Gronchi, Alessandro; Bonvalot, S.

doi:10.1016/j.ejso.2016.11.012

Cited by 14 publications

(7 citation statements)

References 25 publications

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“…They reported that none of their patients developed de novo chronic renal insufficiency through a median follow-up of 8 years, based on only serum creatinine levels which is an inaccurate way to determine renal function. 17,18 Berglund et al 11 in their series reported a 16% rate of loss of a renal unit and, 14% of patients had progressed to new-onset CKD at the last follow-up. Avoiding the development of CKD by avoiding unnecessary renal surgery is an important goal in diagnosing and treating AML renal masses.…”

Section: Discussionmentioning

confidence: 96%

“…Nephron sparing-surgery for sporadic AMLs is known to offer preservation of renal function and is associated with acceptable complication and low local recurrence rates. 11 Boorjian et al 16 in his series of 58 patients undergoing nephron-sparing surgery for AML, reported a 12% complication rate, including 5% of patients with sustained urine leaks. They reported that none of their patients developed de novo chronic renal insufficiency through a median follow-up of 8 years, based on only serum creatinine levels which is an inaccurate way to determine renal function.…”

Section: Discussionmentioning

confidence: 99%

“…Angiomyolipoma is an uncommon benign renal tumor that accounts for <5% of enhancing renal masses. 11 The strategy for the treatment of renal AMLs was based mainly on evidence acquired during the 1980's. 12 As major retroperitoneal bleeding was known to be the most severe complication of AML, prophylactic treatment was indicated to avoid this hemorrhagic event.…”

Section: Discussionmentioning

confidence: 99%

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Angiomyolipoma managed by partial nephrectomy: Outcomes and follow-up

Nerli¹,

Sanikop²,

Deole³

et al. 2020

IJPO

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Angiomyolipoma (AML) is a benign neoplasm and consists of thick-walled poorly organized blood vessels, smooth muscle, and varying levels of mature adipose tissue. We evaluated the impact of clinical characteristics, particularly tumor diameter, and surgical treatment in the form of partial nephrectomy on the outcome of sporadic renal AML. Patients undergoing partial nephrectomy during the period Jan 2000 to Dec 2016 with a final diagnosis of angiomyolipoma formed the study group. Patient demographic data was collected, and data from during and after surgery was also reviewed. The median age of the patients was 51 (38-55) years. At presentation, most patients were asymptomatic (6/8, 75%) with the AML presenting as an incidental finding on imaging done for other reasons. There was classical triphasic AML, consisting of elements of adipose tissue, smooth muscle and abnormal blood vessels, in 5/10 tumours (50%). The more aggressive epitheliod variant AML was present in 2/10 (20%). The median follow-up of the patients was 38 (29-64) months. During the follow-up period one (12.5%) patient who had two lesions progressively showed evidence of rising creatinine and decreasing creatinine clearance. AML is a benign renal neoplasm and should be treated initially conservatively. Surgical intervention when required should be nephron sparing so as to reduce the incidence of perioperative complications, loss of renal units, and development of CKD.

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Angiomyolipoma managed by partial nephrectomy: Outcomes and follow-up

Nerli¹,

Sanikop²,

Deole³

et al. 2020

IJPO

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“…Epithelioid AML (eAML) was reported in 7.7% of AML cases subjected to histopathologic diagnosis ( 93 ). eAML has an aggressive behavior, leading to metastasis, renal vein/inferior vena cava invasion, and in one study only 50% survival at 3 years ( 94 ). eAML has fewer or no fat content and is less frequently bilateral compared to classic AML.…”

Section: Renal Malignancymentioning

confidence: 99%

The Risks of Renal Angiomyolipoma: Reviewing the Evidence

Seyam

Alkhudair

Kattan

et al. 2017

jkcvhl

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Renal angiomyolipoma (RAML), though a rare benign tumor, may impose a significant morbidity or even mortality due to its unique characteristics and the complications subsequent to its treatment. The classic tumor variant is composed of smooth muscular, vascular, and fatty components. The most straightforward diagnosis is when the fat component is abundant and gives a characteristic appearance on different imaging studies. In fat-poor lesions, however, the diagnosis is difficult and presumed a renal cell carcinoma. Yet, some variants of RAML, though rare, express an aggressive behavior leading to metastasis and mortality. The challenge lies in the early detection of benign variants and identifying aggressive lesions for proper management. Another challenge is when the vascular tissue component predominates and poses a risk of hemorrhage that may extend to the retroperitoneum in a massive life-threatening condition. The predicament here is to identify the characteristics of tumors at risk of bleeding and provide a prophylactic treatment. According to the clinical presentation, different treatment modalities, prophylactic or therapeutic, are available that span the spectrum of observation, embolization, or surgery. Renal impairment may result from extensive tumor burden or as a complication of the management itself. Improvement of diagnostic techniques, super-selective embolization, nephron-sparing surgery, and late treatment with the mammalian target of rapamycin inhibitors have provided more effective and safe management strategies. In this review, we examine the evidence pertaining to the risks imposed by RAML to the patients and identify merits and hazards associated with different treatment modalities.

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“…Tumors of uncertain prognosis are characterized by a size above 5 cm or pleomorphism of the tumor cells. The presence of an epithelioid component in renal angiomyolipomas is related to more aggressive clinical behavior and poorer survival . PEComas typically arise sporadically, though a small percentage of PEComas arise in patients with tuberous sclerosis, which is an inherited autosomal dominant syndrome.…”

Section: Introductionmentioning

confidence: 99%

Management of the “Other” retroperitoneal sarcomas

Rutkowski

Mullen

2017

Journal of Surgical Oncology

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The focus of this review is on the management of the less common sarcomas occurring in the retroperitoneal space, including solitary fibrous tumor (SFT), malignant peripheral nerve sheath tumor (MPNST), perivascular epithelioid cell tumor (PEComa), and undifferentiated pleomorphic sarcoma (UPS) of the psoas muscle. As for other retroperitoneal sarcomas, surgical resection is the mainstay of curative therapy, and multidisciplinary preoperative assessment, including percutaneous needle biopsy for histologic confirmation, is the basis for personalized management, as the surgical management, and the integration of systemic therapy and radiation therapy is unique to each histologic subtype.

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Renal angiomyolipomas: At least two diseases. A series of patients treated at two European institutions

Cited by 14 publications

References 25 publications

Angiomyolipoma managed by partial nephrectomy: Outcomes and follow-up

Angiomyolipoma managed by partial nephrectomy: Outcomes and follow-up

The Risks of Renal Angiomyolipoma: Reviewing the Evidence

Management of the “Other” retroperitoneal sarcomas

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