Diagnostic and Therapeutic Problems in Multicentric Renal Angiomyolipoma

Abstract

Multicentric renal angiomyolipoma is a rare form of benign tumor. However, its effective incidence as evaluated in autopsy studies may be as high as 8%. There are 2 main types of renal angiomyolipoma, that is isolated forms and those associated with other diseases, such as phakomatosis, polycystic kidneys and fibromuscular dysplasia. The tumor may also display malignant behavior with local invasiveness and regional lymph node involvement. However, the clinical course is benign and multicentricity is important for prognosis. Histopathological diagnosis often is difficult. Immunohistochemical analysis of surgical specimens using a panel of monoclonal antibodies, including HMB-45 and actin, enabled us to make a definitive diagnosis in 3 cases of multicentric renal angiomyolipoma.

Perivascular Epithelioid Cell Tumor (PEComa) in the Genitourinary Tract

“…Multifocality and regional lymph node involvement can occur and this is considered to represent a multifocal growth pattern rather than metastasis. 17,18 Three cases of sarcoma developing in sporadic angiomyolipoma have been reported, although a similar event has not been described in tuberous sclerosis patients. [19][20][21] Angiomyolipoma frequently shows loss of heterozygosity of variable portions of the TSC2 gene locus in both sporadic and tuberous sclerosis-associated tumors.…”

Management of Renal Angiomyolipoma:Analysis of 15 Cases

“…It occurs more commonly in the isolated form and, rarely, as part of the tuberous sclerosis (TBS) complex. The isolated form is usually small and asymptomatic and is found predominantly in women in the 4th to 7th decades of life; the combined form is encountered in 40–80% of the patients with TBS and appears as multiple bilateral tumors [2]. Though hamartomas in adults usually do not have growth potential, studies have shown that AMLs may grow and compress surrounding tissue [3, 4].…”

Massive bilateral angiomyolipomas resulting in respiratory failure

“…They occur commonly as an isolated form and less often in association with tuberous sclerosis [2]. Patients with tuberous sclerosis are more likely to have bilateral disease, are more symptomatic and require surgery more frequently [3].…”