Rare tumors: a blue ocean of investigation.

Shuhang Wang; Peiwen MA; Ning Jiang; Yale Jiang; Yue YU; Yuan Fang; Huilei Miao; Huiyao Huang; Qiyu Tang; Dandan Cui; Hong Fang; Huishan Zhang; Qi Fan; Yuning Wang; Gang Liu; Zicheng YU; Qi Lei; Ning LI

Return

Rare tumors: a blue ocean of investigation.

Author: Shuhang WANG ¹ ; Peiwen MA ¹ ; Ning JIANG ¹ ; Yale JIANG ¹ ; Yue YU ¹ ; Yuan FANG ¹ ; Huilei MIAO ¹ ; Huiyao HUANG ² ; Qiyu TANG ¹ ; Dandan CUI ¹ ; Hong FANG ¹ ; Huishan ZHANG ² ; Qi FAN ¹ ; Yuning WANG ¹ ; Gang LIU ³ ; Zicheng YU ⁴ ; Qi LEI ¹ ; Ning LI ⁵
Author Information

1. Clinical Trial Center, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China.
2. Phase I Clinical Trial Center, Fujian Medical University Cancer Hospital/Fujian Cancer Hospital, Fuzhou, 350014, China.
3. Key Laboratory of Molecular Epigenetics of the Ministry of Education, Northeast Normal University, Changchun, 130024, China.
4. GenePlus-Shenzhen, Shenzhen, 518118, China.
5. Clinical Trial Center, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China. lining@cicams.ac.cn.
Publication Type:Review
Keywords: clinical trials recommendation; diagnosis flowchart; rare tumors; treatment strategy
MeSH: Humans; Neoplasms/pathology*; Biomarkers; Prognosis; Oceans and Seas; China/epidemiology*
From: Frontiers of Medicine 2023;17(2):220-230
CountryChina
Language:English
Abstract: Advances in novel drugs, therapies, and genetic techniques have revolutionized the diagnosis and treatment of cancers, substantially improving cancer patients' prognosis. Although rare tumors account for a non-negligible number, the practice of precision medicine and development of novel therapies are largely hampered by many obstacles. Their low incidence and drastic regional disparities result in the difficulty of informative evidence-based diagnosis and subtyping. Sample exhaustion due to difficulty in diagnosis also leads to a lack of recommended therapeutic strategies in clinical guidelines, insufficient biomarkers for prognosis/efficacy, and inability to identify potential novel therapies in clinical trials. Herein, by reviewing the epidemiological data of Chinese solid tumors and publications defining rare tumors in other areas, we proposed a definition of rare tumor in China, including 515 tumor types with incidences of less than 2.5/100 000 per year. We also summarized the current diagnosis process, treatment recommendations, and global developmental progress of targeted drugs and immunotherapy agents on the status quo. Lastly, we pinpointed the current recommendation chance for patients with rare tumors to be involved in a clinical trial by NCCN. With this informative report, we aimed to raise awareness on the importance of rare tumor investigations and guarantee a bright future for rare tumor patients.