【摘要翻译】伴有结节性硬化症的血管平滑肌脂肪瘤的治疗

伴有结节性硬化症的血管平滑肌脂肪瘤的治疗
（Management of Renal Angiomyolipomas Associated With Tuberous Sclerosis Complex ）

TORU HARABAYASHI*; NOBUO SHINOHARA; HIDENORI KATANO; KATSUYA NONOMURA; TADASHI SHIMIZU; TOMOHIKO KOYANAGI
J Urol. 2004 Jan;171(1):102-5.
PMID: 14665854
ABSTRACT
Purpose:
Intensive management is generally recommended for angiomyolipomas associated with tuberous sclerosis complex (TSC), which are known to have a more aggressive nature than sporadic tumors. In this study we evaluated the management of these tumors.

Materials and Methods:
The records of 6 men and 6 women with TSC and a mean age of 28.8 years) with angiomyolipomas treated from 1984 to 2000 were retrospectively analyzed. All patients had bilateral multiple tumors (mean size 6.6 cm). There were 10 lesions classified as small (less than 4 cm), 6 medium (4 to 10 cm) and 8 large lesions (10 cm or greater). Computerized tomography was repeated annually for followup observation and semiannually after embolization.

Results:
Excluding from study 3 tumors treated prophylactically intervention was required in 20%, 50% and 100% of small, medium and large tumors, respectively. Among 14 tumors followed by observation, 8 grew and 4 required intervention. Uncontrollable hemorrhage did not develop in any patient. While some tumors showed remarkable growth in decade 3, growth did not exceed 1.5 cm per year. Embolization was performed in 11 tumors and tumors shrank in 10. Although 1 patient experienced renal atrophy, all patients but 1 are alive without renal failure with a mean followup of 60 months.

Conclusions:
Although asymptomatic angiomyolipomas associated with TSC grow gradually, severe hemorrhage is rare and most tumors can be managed conservatively with annual computerized tomography. Embolization is the first choice of intervention but should be reserved until symptoms develop.

中文摘要
目的:
对伴有结节性硬化症 (TSC) 的血管平滑肌脂肪瘤，现在普遍认为应加强治疗。这类疾病比偶发肿瘤具有较强侵袭性的自然特点。在这一项研究中，我们评估了这类肿瘤的治疗措施。

材料和方法:
回顾性分析了1984－2000年间伴结节性硬化症的血管平滑肌瘤患者的临床资料（6男、6女，平均年龄28.8岁）。所有的患者都有双侧多发肿瘤(平均大小6.6cm)。其中10个为小肿瘤(小于4cm)，6个为中等大小的肿瘤(4－10 cm)，8个为大肿瘤(10cm或更大的)。对随访观察的病例每年重复一次CT检查，而行肿瘤栓塞术后的则每半年一次CT检查。

结果:
除研究的3个肿瘤采用预防性干预外，小、中等和大肿瘤采用干预措施的比例分别为20％、50％和100％。在14个被随访观察的肿瘤中,8个继续长大，4个需要干涉治疗。无法控制的出血没有发生在任何患者中。而一些肿瘤在第3个十年中表现为显著的长大，不超过每年1.5cm。11个肿瘤行栓塞术治疗，其中10个肿瘤缩小。经过60个月的随访，除1例外，所有的患者均没有发生肾衰竭而继续生存，尽管1例患者出现了肾萎缩。

结论:
尽管无症状的伴有结节性硬化症的血管平滑肌脂肪瘤逐渐地生长，但严重的出血是很罕见的，绝大多数肿瘤可以采取每年一次CT复查的保守治疗。栓塞术是干预治疗的第一选择，但是应该被保留直到症状出现。