Cancer of unknown primary (CUP) is a metastatic cancer where a primary tissue of origin (ToO) evades detection despite extensive clinical investigation. CUP is typically treated with empiric chemotherapy, which provides limited survival benefit for most patients. Recent approaches to the workup and treatment of CUP have involved gene-expression profiling to resolve the likely ToO, enabling site specific therapy, and mutational profiling to identify targeted treatments. Current evidence from our group and others is that both of these methods can have some clinical benefit but only in a minority of cases.
Immunotherapy, which is efficacious across many cancer types, may also be effective in CUP. As part of the Solving Unknown Primary Cancer (SUPER) study we have reviewed the outcomes of 18 CUP patients treated with anti-PD1/PD-L1 immune checkpoint inhibition (ICI) therapy, showing that seven had partial response, five had stable disease and six had progressive disease. As part of SUPER, genomic data is available for 245 patients involving gene-expression profiling by custom Nanostring panel assay and/or cancer panel DNA sequencing. We used the genomic data from 217 of these patients to explore interdependencies between predicted ToO (based gene-expression testing), immune gene-expression patterns, mutation profile and ICI response. Tumour mutation burden was weakly correlated with a gene-expression score for adaptive immune response (AIR) based on averaged z-score values of six genes (CD8A, IFNG, PRF1, PD-L1, GZMA, GZMB) (Spearman rho=0.136) with many outliers exhibiting low TMB but high AIR scores. Amongst 18 patients treated with ICIs genomic data was available and we found that an elevated AIR score was more predictive of ICI response than TMB. All patients with either stable disease or partial response to ICI treatment had positive AIR scores, whereas all patients with progressive disease had negative AIR scores.
Our results demonstrate that ICI therapy may benefit some CUP patients and that an adaptive immune response gene-expression score may be predictive of ICI treatment response.