Chu-Shore Catherine J, Thiele Elizabeth A
Harvard Medical School, Massachusetts General Hospital, 175 Cambridge Street, Suite 340, Boston, MA 02114, USA.
Brain Dev. 2010 Apr;32(4):318-22. doi: 10.1016/j.braindev.2009.04.009. Epub 2009 May 13.
New evidence is emerging that the availability of nutrients plays a key role in regulating the mammalian target of rapamycin complex-1 (mTORC1) signaling pathway in human cancers. Tuberous sclerosis complex (TSC) is a genetic disorder which results in the growth of hamartomatous lesions in multiple organs due to insufficient suppression of the mTORC1 pathway. A minority of patients with TSC who develop epilepsy which is intractable to standard anticonvulsant medical and/or surgical treatments are treated with the ketogenic diet. To provide insight into the effects of nutrient manipulation on tumor growth in this condition, we describe our experience in a unique group of patients with known tuberous sclerosis complex who are on the ketogenic diet for seizure control.
A retrospective chart review was performed of patients with TSC treated with the ketogenic diet between January 2002 and May 2007 at Massachusetts General Hospital.
Five patients with definite TSC underwent serial imaging for tumor growth while on the ketogenic diet or had unchanged imaging prior to the onset of the diet and after termination. Three out of five patients, all children, had progression of a known tumor or tumors or the development of a new tumor while on the ketogenic diet.
In this limited case series of five TSC patients, the ketogenic diet did not induce tumor regression or suppress the growth of TSC-related tumors.
新证据表明，营养物质的可利用性在调节人类癌症中雷帕霉素复合物1（mTORC1）信号通路中起着关键作用。结节性硬化症（TSC）是一种遗传性疾病，由于mTORC1通路抑制不足，导致多个器官出现错构瘤性病变生长。少数患有TSC且癫痫对标准抗惊厥药物和/或手术治疗无效的患者采用生酮饮食治疗。为了深入了解在这种情况下营养调控对肿瘤生长的影响，我们描述了一组独特的已知结节性硬化症患者采用生酮饮食控制癫痫发作的经验。
对2002年1月至2007年5月在马萨诸塞州总医院接受生酮饮食治疗的TSC患者进行回顾性病历审查。
5例确诊为TSC的患者在生酮饮食期间接受了肿瘤生长的系列影像学检查，或者在饮食开始前和结束后影像学检查无变化。5例患者中有3例，均为儿童，在生酮饮食期间已知肿瘤有进展或出现新肿瘤。
在这个由5例TSC患者组成的有限病例系列中，生酮饮食并未诱导肿瘤消退或抑制TSC相关肿瘤的生长。
