Management of Renal Angiomyolipoma in Complex Clinical Situations

Abstract

Renal angiomyolipoma (AML) is associated with complex clinical situations such as tumour in a solitary kidney, bilateral, large or multicentric tumours or those associated with tuberous sclerosis (TS) or pregnancy. Management in these situations may be challenging. Fifteen patients (20 kidneys) were admitted with symptomatic AML over last 10 years. Eleven patients had one or the other complicating factor. Ten patients had a tumour of >10 cm, 4 had TS, 5 had multiple and bilateral tumours, 1 patient was pregnant and 1 had a solitary functioning kidney. With the newer imaging modalities correct diagnosis was possible in 12 cases and renal cell carcinoma (RCC) was suspected in 3 cases. Selective angioembolization (SAE) was done in 3 patients, which successfully controlled bleeding in all. Nephron-sparing surgery (NSS) was performed in 5 patients. Total nephrectomy was done in 4 cases, in 3 due to suspicion of RCC and in 1 due to extensive involvement of the kidney. Three patients with multiple and bilateral tumours were chosen for conservative treatment and none developed recurrence of bleeding on strict follow-up. In a pregnant patient, bleeding was successfully controlled with angioembolization. However, 1 patient with a solitary functioning kidney with large-sized tumour (20 × 18 cm) underwent NSS. In conclusion, the basis of management of AML is preservation of renal tissue, which can be effectively achieved with SAE or NSS. In a solitary functioning kidney, NSS or SAE is the ideal treatment, if feasible. The patients in the TS group are usually more complicated and require life-long follow-up after initial management with NSS or SAE. Pregnant AML patients can be safely managed with SAE. Conservative treatment without any intervention and regular follow-up may be more helpful in some patients with multiple, bilateral extensive tumours.

Increasing Severity of Haematuria with Successive Pregnancies in a Woman with Renal Angiomyolipoma

“…Consequently, for women who plan to have more children, excisional surgery in between pregnancies would appear to be the treatment of choice. As in most other reports, the risk of spontaneous haemorrhage is more during the last trimester of pregnancy again as demonstrated by our patient [11][12][13][14][15].…”

Renal angiomyolipoma with malignant transformation, simultaneous occurrence with malignity and other complex clinical situations

“…The tumor rupture and acute bleeding are the most serious complications associated with angiomyolipoma, even very small tumors carry these risks [1,[11][12][13]. Secondary bleeding in angiomyolipoma case is called ''Wunderlich syndrome'' [7].…”

“…In most cases, diagnosis can be done by computerized tomography with no need for angiography. In ultrasonography, hyperechogenic areas are seen where the tumor is present [1,11]. However, diagnosis is difficult in small tumors, cases with low fat content, and in the presence of hemorrhage and necrosis [11,14].…”

“…Angiomyolipoma is a benign hamartomatous kidney tumor, which characteristically exhibits mature or immature fat tissue, thick-walled blood vessels, and smooth muscle fibers [1]. The histologic characteristics of renal angiomyolipoma were initially described by Fischer in 1951 and the term ''angiomyolipoma'' is first used by Morgan and his colleagues [2][3][4][5][6][7].…”

Didelės abipusės inkstų angiomiolipomos sergant tuberozine skleroze: atvejo pristatymas

“…Pirmenybė skiriama nefronus tausojančioms operacijoms bei selektyviai arterijų embolizacijai [5,6,7]. Svarbus argumentas gydomosioms intervencijoms yra pasitaikantys atvejai, kai ilgai ir aktyviai stebint angiomiolipomą vystosi inksto ląstelių vėžys.…”