Cancer of unknown primary—state of the art

Thorsten Fuereder1
1Department of Medicine I and CCC, Division of Oncology, Medical University of Vienna, Vienna, Austria

Tóm tắt

SummaryThe Cancer of Unknown Primary (CUP) syndrome does not represent a single clinical entity but comprises a diverse group of malignant tumors. CUP is defined as a histologically confirmed metastatic malignant disease for which the primary tumor cannot be identified after completing initial diagnostics. Although its incidence has decreased, managing CUP patients remains a significant clinical challenge, particularly for those in the poor prognosis group, where the overall survival is merely 3 months. In this context, accurate diagnostics and prompt treatment initiation are crucial. While platinum-based chemotherapy still plays a central role in CUP therapy, the future holds promise with the advent of next-generation targeted therapies and immunotherapies guided by the results of advanced sequencing tools. These advancements offer hope for improving the bleak outcomes faced by these patients.

Từ khóa


Tài liệu tham khảo

Krämer A, Bochtler T, Pauli C, et al. Cancer of unknown primary: ESMO clinical practice guideline for diagnosis, treatment and follow-up. Ann Oncol. 2023;34(3):228–46. https://doi.org/10.1016/j.annonc.2022.11.013.

Kolling S, Ventre F, Geuna E, et al. “Metastatic cancer of unknown primary” or “primary metastatic cancer”? Front Oncol. 2019;9:1546. https://doi.org/10.3389/fonc.2019.01546.

Rassy E, Pavlidis N. The currently declining incidence of cancer of unknown primary. Cancer Epidemiol. 2019;61:139–41. https://doi.org/10.1016/j.canep.2019.06.006.

Rassy E, Pavlidis N. Progress in refining the clinical management of cancer of unknown primary in the molecular era. Nat Rev Clin Oncol. 2020;17(9):541–54. https://doi.org/10.1038/s41571-020-0359-1.

NCCN clinical practice guidelines in oncology: occult primary.. https://www.nccn.org/professionals/physician_gls/pdf/occult.pdf..

CUP Syndrom – Krebserkrankungen mit unbekanntem Primärtumor.. https://www.onkopedia.com/de/onkopedia/guidelines/cup-syndrom-krebserkrankungen-mit-unbekanntem-primaertumor/@@guideline/html/index.html..

Kwee TC, Kwee RM. Combined FDG-PET/CT for the detection of unknown primary tumors: systematic review and meta-analysis. Eur Radiol. 2009;19(3):731–44. https://doi.org/10.1007/s00330-008-1194-4.

Pentheroudakis G, Golfinopoulos V, Pavlidis N. Switching benchmarks in cancer of unknown primary: from autopsy to microarray. Eur J Cancer. 2007;43(14):2026–36. https://doi.org/10.1016/j.ejca.2007.06.023.

Karim Fizazi A. A phase 3 trial of empiric chemotherapy with cisplatin and gemcitabine or systemic treatment tailored by molecular gene expression analysis in patients with carcinomas of an unknown primary site. ESMO https://doi.org/10.1093/annonc/mdz394.

Lee MS, Sanoff HK. Cancer of unknown primary. BMJ. 2020;371:m4050. https://doi.org/10.1136/bmj.m4050.

Gatalica Z, Xiu J, Swensen J, Vranic S. Comprehensive analysis of cancers of unknown primary for the biomarkers of response to immune checkpoint blockade therapy. Eur J Cancer. 2018;94:179–86. https://doi.org/10.1016/j.ejca.2018.02.021.

Mateo J, Chakravarty D, Dienstmann R, et al. A framework to rank genomic alterations as targets for cancer precision medicine: the ESMO scale for clinical actionability of molecular targets (ESCAT). Ann Oncol. 2018;29(9):1895–902. https://doi.org/10.1093/annonc/mdy263.

Golfinopoulos V, Pentheroudakis G, Salanti G, Nearchou AD, Ioannidis JP, Pavlidis N. Comparative survival with diverse chemotherapy regimens for cancer of unknown primary site: multiple-treatments meta-analysis. Cancer Treat Rev. 2009;35(7):570–3. https://doi.org/10.1016/j.ctrv.2009.05.005.

Tanizaki J, Yonemori K, Akiyoshi K, et al. Open-label phase II study of the efficacy of nivolumab for cancer of unknown primary. Ann Oncol. 2022;33(2):216–26. https://doi.org/10.1016/j.annonc.2021.11.009.