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=12 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=

Pancreatic neuroendocrine neoplasms (PNEN) are tumors that originate from neuroendocrine cells. Only about 1% patients are related to mutation of tuberous sclerosis complex gene. Here, we reported a rare case with involvement of multiple organs and space-occupying lesions. Initially, the patient was thought to have metastasis of a pancreatic 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 presented with a heterozygous variant. Tuberous sclerosis often presents with widespread tumors, but it is less common to present with pancreatic neuroendocrine tumors and liver perivascular tumors as highlighted in the case. So we analyzed the relationship between TSC gene mutations and related tumors. And we also reviewed the current molecular mechanisms and treatments for tuberous sclerosis complex.