Editorial: A structured approach to uncommon cancers: what should a clinician do?

Abstract

In this issue of Annals of Oncology, Pecuchet et al. [1] have produced an elegant, original paper describing an innovative approach to the management of metastatic collecting duct carcinoma, using bevacizumab, gemcitabine and a platinum complex. They have shown surprising anticancer activity, which appears to have been sustained. They have addressed the usual concerns about case selection bias, positive response bias and pathology review of an uncommon tumor, and thus, this regimen certainly will bear confirmatory testing in other structured trials, especially as this triplet really does seem to give a different result from the more conventional gemcitabine–cisplatin combination or the established MVAC regimen (notwithstanding the absence of level 1 data at this time).

The editorial review of this manuscript raised an important generic question, viz. what should a clinician do when approaching a patient with a truly uncommon or rare tumor? Using a cut-off figure of 15 new cases/100 000 of population per year as a definition for ‘rare cancers’, Greenlee et al. [2] have suggested that these tumors cumulatively account for around 25% of incident cases in the United States. This figure seems inflated to me, because of their cut-off value, especially as I do not view testicular cancer as a rare tumor (yet it has an incidence of 6.8/100 000 males per year). Nonetheless, Greenlee et al. make an important point—rare tumors cumulatively do constitute a significant proportion of the total cancers presenting, yet we have remarkably little information available to guide management, when compared with the common tumors that arise in breast, lung, prostate, colon, pancreas and bladder. A similar situation appears to apply to oncology practice in Europe, in a report using a cut-off of less than six new cases/100 000 of population per year, with the accompanying suggestion …...

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