Surgical Management of Angiomyolipoma: Nephron-sparing Surgery for Symptomatic Tumour

Abstract

Angiomyolipoma (AML) is a benign renal tumour that occasionally requires intervention. We evaluated the surgical management of symptomatic angiomyolipoma, with special attention to the feasibility of nephron-sparing surgery. A retrospective study was conducted enlisting 23 patients (including 3 patients with tuberous sclerosis) who had their AML treated surgically from 1991 to 1998. The study included 7 males and 16 females, with a mean age of 49 years (range 24-75). The mean tumour size was 12.3 cm (range 1.5-30.0) including 7 lesions < or = 4 cm and 16 lesions > 4 cm. The presenting features included abdominal/loin pain (9 patients), spontaneous rupture (5 patients), non-specific symptoms (7 patients) and incidental findings (2 patients). AML was diagnosed by computed tomography in 16 patients and malignancy was suspected in 7 patients. The latter group included 5 patients with lesions smaller than 4 cm, one of them having coexisting AML and renal cell carcinoma. All patients underwent elective surgery, with the exception of one, who required a semi-emergency operation. Nephron-sparing surgery using frozen section in 5 patients (all diagnostic) was carried out on 16 patients, including all 5 patients with previous haemorrhage and 2 patients with suspected lesions from preoperative studies. Total nephrectomy was performed in 7 patients, including the other 5 patients with lesions suspected to be malignant. There was no operative mortality. Two complications were encountered in the entire group. We conclude that elective nephron-sparing surgery is feasible, even for massive angiomyolipoma or after previous rupture, especially when the diagnosis is made by preoperative imaging and/or intraoperative frozen section.

Dual-Tracer PET/CT in Renal Angiomyolipoma and Subtypes of Renal Cell Carcinoma

“…However, when the percentage of fat component is low, particularly when the tumor is also small, the radiological diagnosis of renal angiomyolipoma can be difficult and very often indistinguishable from other hypervascular tumors, especially renal cell carcinoma (RCC). 5,6 The fat content can be less than 25% in 50% of angiomyolipoma 7,8 and as much as 30% of angiomyolipoma were initially diagnosed as RCC by CT. 6 Other atypical findings of angiomyolipoma include increased spindle cell content 9 and hemorrhage. On the other hand, it is also well known that RCC may sometimes contain lipid by infiltrating or entrapping the perinephric fat or sometimes by tumor necrosis and fatty degeneration.…”

Pneumothorax in a female with renal angiomyolipoma

“…As reported, two-thirds of the cases occur as a result of sporadic mutations and will have no family history of the disease [11]. In sporadic LAM, somatic mutations have been found in LAM cells in the lungs, lymph nodes, and AMLs, predominantly for TSC2 [12].…”

Bilateral nephron sparing surgeries for multiple renal angiomyolipomas in Bourneville’s disease

“…The efficacy of partial nephrectomy for sporadic angiomyolipomas [6] and its application in solitary kidneys has been established [7]. Good results for unilateral partial nephrectomy have been reported even in angiomyolipomas associated with TSC [8,9].…”