Renal angiomyolipoma. A clinicopathologic study of 32 cases

Abstract

T h e data from 32 cases of angiomyolipoma (renal hamartoma) are presented: 23 of the tumors were removed a t surgery and nine a t autopsy. T h e histopathologic findings are reviewed and the spectrum of gross renal involvement is emphasized. O n the basis of the duration of symptoms and findings a n d the subsequent course of patients subjected to surgical resection, the clinical behavior of the tumor is assessed. Of particular interest is the relationship of this tumor to the tuberous sclerosis complex. Data presented indicate that many of the patients have various components of the syndrome, which in some patients have familial manifestations. I t is inferred from this study that the tuberous sclerosis complex may vary in expression from a solitary renal tumor to the fully developed clinical syndrome. HE ANGIOMYOLIPOMA OR RENAL HAMAR-T toma is a n uncommon tumor: only about 150 cases appear in the literature.16 Although the pathologic features have been well described,8.9, 1 3~1 5 the neoplastic features and biologic behavior should be re-evaluated and the clinicopathologic aspects, particularly the relationship to tuberous sclerosis, brought into sharper focus.This report is based on the study of 32 cases of renal angiomyolipoma seen at the Mayo Clinic during a 50-year period; 23 of the tumors were seen at surgical exploration and nine at autopsy. Four of these cases have been previously reported.10, 15 The gross specimen was available and examined in all but three cases. T h e original histopathologic slides and the new slides made from samplings of tissue from several areas of each tumor were examined. Clinical and follow-up information was obtained from the records and from questionnaires sent to the patient or the family. PATHOLOGIC FEATURESThese turn or s were char act e r i s t i c a 11 y located within the parenchyma of the kidney. From the Mayo Clinic and Mayo Foundation: Section of Surgical Pathology (Drs. Farrow and Harrison),

Imaging of fat thrombus in the inferior vena cava originating from an angiomyolipoma

“…It has a tendency to invade parenchyma [1] but extrarenal growth is rare and vascular extension into the IVC has been reported to date in only 7 cases, to our knowledge [2][3][4][5][6][7]. Although venous involvement represents aggressive local invasion, histology showed no evidence of malignant transformation in our patient or any of the previous cases.…”

Giant Hepatic Angiomyolipoma Associated with Disseminated Intravascular Coagulation.

“…With immunohistochemical studies, smooth muscle cells are always positive for smooth muscle myosin, desmin, actin, and vimetin, adipose cytes are commonlypositive for S-100, and the endothelial cells of the blood vessels are positive for factor VHI-related antigen (16). Furthermore recently HMB-45, a monoclonal antibody previously regarded as specific for melanoma, has been recognized to stain the smooth muscle cells in angiomyolipoma (13,14,(21)(22)(23). In our case the tumor contained these four components and HMB-45 -positive smooth muscle.…”

Clinical and Molecular Insights into Tuberous Sclerosis Complex Renal Disease

“…3c, d). Very rarely, renal angiomyolipomas can infiltrate the surrounding tissue [55]. Although very rare for the TSC patient population, epithelioid angiomyolipomas can exhibit an aggressive phenotype [56], recur after resection, and even be fatal [49,57,58].…”