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Renal angiomyolipoma in Japanese tuberous sclerosis patients |
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| Authors: | Kitano Yoshihiro Honna Toshiro Nihei Kenji Miyauchi Jun Matsuoka Kentarou Kuroda Tatsuo Tanaka Kiyoshi Morikawa Nobuyuki Fuchimoto Yasushi |
| Institution: | a Department of Surgery, National Center for hild Health and Development, Tokyo, Japan b Department of Neurology, National Center for Child Health and Development, Tokyo, Japan c Department of Pathology, National Center for Child Health and Development, Tokyo, Japan |
| Abstract: | PurposeRenal angiomyolipoma (AML) is a benign neoplasm that may grow massive in tuberous sclerosis (TS) patients. The aim of this study was to document the characteristics of renal AML in Japanese TS patients.MethodsMedical records of 29 TS patients followed up at the authors’ center were reviewed for the presence, size, symptom, and treatment of renal AML.ResultsTwenty-four patients screened for renal AML were subdivided into 4 groups: group 0 (n = 8), no mass; group 1 (n = 5), AML less than 1 cm in diameter; group 2 (n = 4), AML 1 to 4 cm in diameter; group 3 (n = 7), AML greater than 4 cm in diameter. When present, AML always affected both kidneys and were multiple. All patients in groups 1 and 2 were symptom free, and the tumors seemed stable in size. All tumors in group 3 grew progressively causing various symptoms. Total or partial nephrectomy or transarterial embolization was performed in 5 patients with limited success.ConclusionsAML in TS patients can be stable or aggressive. Pediatric surgeons aware of this problem should be involved in a follow-up program. |
| Keywords: | Angiomyolipoma tuberous sclerosis selective arterial embolization |
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