Renal angiomyolipoma in Japanese tuberous sclerosis patients.
OBJECTIVE
Renal 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.


METHODS
Medical records of 29 TS patients followed up at the authors' center were reviewed for the presence, size, symptom, and treatment of renal AML.


RESULTS
Twenty-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.


CONCLUSIONS
AML in TS patients can be stable or aggressive. Pediatric surgeons aware of this problem should be involved in a follow-up program.
Kitano
Yoshihiro
Y
Department of Surgery, National Center for hild Health and Development, Tokyo, Japan.
Honna
Toshiro
T
Nihei
Kenji
K
Miyauchi
Jun
J
Matsuoka
Kentarou
K
Kuroda
Tatsuo
T
Tanaka
Kiyoshi
K
Morikawa
Nobuyuki
N
Fuchimoto
Yasushi
Y
D000293:Adolescent; D000328:Adult; D018207:Angiomyolipoma / Q000150:complications*; D002648:Child; D005260:Female; D006801:Humans; D007564:Japan; D007680:Kidney Neoplasms / Q000150:complications*; D008297:Male; D012189:Retrospective Studies; D014402:Tuberous Sclerosis / Q000150:complications*