Journal of Hereditary Tumors
Online ISSN : 2435-6808
Case report
Surgical treatment of endometrial cancer in a patient with tuberous sclerosis complex and an enlarged polycystic liver and lymphangioleiomyomatosis
Yoshinari MatsumotoReiko TasakaTomoko NakagawaTomohiro YoshidaMasaaki DeguchiKazumasa SaigohAtsuko IkegawaToshiyuki Seto
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JOURNAL OPEN ACCESS

2022 Volume 22 Issue 1 Pages 17-21

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Abstract

 A patient with tuberous sclerosis complex, who had an enlarged polycystic liver and lymphangioleiomyomatosis, required surgical treatment for endometrial cancer. When tuberous sclerosis complex is accompanied by a severely enlarged polycystic liver and renal angiomyolipoma, the possibility of PKD1/TSC2 contiguous gene syndrome must be considered. In this case, to forestall complications, a radical surgery had to be carefully planned in consideration of the liver and kidney conditions, ongoing dialysis, lymphangioleiomyomatosis, and multiple aneurysms, among other conditions. Strategies for the management of endometrial cancer can include surgery, radiation, cytotoxic agents, and hormonal therapy. In this case, we believe that the most appropriate post-surgical approach was the use of molecular biology therapy.

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© 2022 The Japanese Society for Hereditary Tumors
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