4.2.1 Treatment regimens for patients in the specific–CUP subset

Patients in the specific-CUP subset who have a good prognosis should be treated the same as patients with equivalent known primary tumours with metastatic disease, as shown in Table 1.

Table 1: Treatment of patients in the specific CUP subset (adapted from Fizazi et al. 2015*)

Equivalent known primary tumour

Recommended treatment

Poorly differentiated neuroendocrine carcinoma of unknown primary

Treat as poorly differentiated neuroendocrine carcinomas with a known primary

Well-differentiated neuroendocrine tumour of unknown primary

Treat as well-differentiated neuroendocrine tumour of a known primary site

Peritoneal adenocarcinomatosis of a serous papillary histological type in females

Treat as ovarian cancer

Isolated axillary nodal adenocarcinoma metastases in females

Treat as breast cancer

Squamous cell carcinoma involving

non-supraclavicular cervical lymph nodes

Treat as head and neck squamous cell cancer

CUP with an intestinal phenotype and IHC (CK20+/CDX2+/CK7−) or molecular profile

Treat as metastatic colorectal cancer

Single metastatic deposit from unknown primary

Treat as single metastases by resection or high-dose (ablative) radiotherapy depending on the location

Osteoblastic bone metastases or IHC/serum PSA expression in men

Treat as prostate cancer

Patients with extragonadal germ cell syndrome

Treat as poor-prognosis germ cell tumour (Greco 2013).

Isolated inguinal adenopathy (squamous carcinoma)

Local dissection with or without local radiotherapy (Pavlidis et al. 2015)

Other tumour specific optimal care pathways can be found here.

* Fizazi K, et al. Cancers of unknown primary site: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up, Annals of Oncology 2015; 26 (suppl_5): v133–v138 doi:10.1093/annonc/mdv305. Adapted and reproduced with permission of Oxford University Press on behalf of ESMO. Oxford University Press and ESMO are not responsible or in any way liable for the accuracy of the adaptation, for any errors, omissions or inaccuracies, or for any consequences arising therefore. Cancer Institute NSW is solely responsible for the adapted material in this work. Please visit the ESMO Cancers of Unknown Primary Site website.