AUTHOR=Zhang Xinhe , Zhong Xinping , Lin Xuyong , Li Xuedan , Tian Haoyu , Chang Bing , Wang Ying , Tong Jing , Wang Ningning , Li Dan , Jin Xiuli , Huang Die , Wang Yanmeng , Cui Huipeng , Guan Lin , Li Yiling TITLE=Tuberous Sclerosis Complex With Multiple Organ Tumors: Case Report and Literature Review JOURNAL=Frontiers in Oncology VOLUME=Volume 12 - 2022 YEAR=2022 URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2022.916016 DOI=10.3389/fonc.2022.916016 ISSN=2234-943X ABSTRACT=We reported a rare case with involvement of multiple organs and space-occupying lesions. Initially, the patient was thought to be metastasis of pancreatic endocrine tumor. However, the patient was diagnosed as pancreatic neuroendocrine tumors, liver perivascular epithelioid tumors, splenic hamartoma, and renal angiomyolipoma by pathological examination after surgery. We performed genetic mutation detection to identify that tuberous sclerosis complex 2 gene (TSC2) presented with a heterozygous variant. Finally, the patient was diagnosed with possible tuberous sclerosis complex with multiple organ involvement. In addition, we reviewed the current molecular mechanisms and treatments for tuberous sclerosis complex. We also analyzed the relationship between TSC gene mutations and related tumors.