Tuberous sclerosis complex associated renal clear cell carcinoma ( a case report and literature review)
10.3760/cma.j.issn.1000-6702.2011.08.004
- VernacularTitle:结节性硬化症相关肾透明细胞癌一例报告并文献复习
- Author:
Hongliang SHEN
;
Ye TIAN
;
Gangyue HAO
;
Lindong DU
;
Peiqian YANG
- Publication Type:Journal Article
- Keywords:
Tuberous sclerosis complex;
Kidney neoplasms;
Angiomyolipoma
- From:
Chinese Journal of Urology
2011;32(8):517-520
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the diagnosis and treatment features of tuberous sclerosis complex associated renal cell carcinoma.Methods A 22-year-old boy with a childhood history of epilepsy and mental retardation presented with a complaint of intermittent painless gross hematuria for the past 2 years.After superselective left renal artery embolization was done twice in the past year, painless gross hematuria was still repeated with 6- 10 months intervals.Physical examination showed retarded face, obesity, visible facial angiofibroma and a ditch fibroma.CT scan showed irregular lesions.The largest cross-section 14.2 cm × 9.0 cm in the left kidney was inhomogeneous enhanced from 45 - 54 HU in the plain phase to 60 - 78 HU in the contrast phase.Filling defect in the left renal vein and multiple fat-density lesions (CT value of -25 - -38 HU) with the largest cross-section 7.2 cm× 5.7 cm in the right kidney were also found in contrast CT scan.The PUBMED and CBM database were reviewed.Results Open retroperitoneal radical left nephrectomy was performed.Pathology showed renal clear cell carcinoma and renal vein thrombosis.There was no tumor recurrence or distant metastasis at 4-month follow-up.Conclusions Tuberous sclerosis complex associated renal cell carcinoma is rarely reported.Timely nephron-sparing surgery is necessary when the diagnosis is established, or radical nephrectomy is also necessary if nephron-sparing surgery is impossible.
