EURURO Vol. 72 No. 6

985 1030

prognosis, and treatment response PAPMET
(NCT02761057) is one such trial. The availability of rapid
and inexpensive mutation testing in the near future will
simplify the process of molecular classification and allow
targeted therapy to be just that—targeted. This shift in
classification of tumours may, in turn, lead to the
development of more successful combinations of targeted
therapy, for example, would pRCC that has both MET and FH
mutations respond best to a combination of MET kinase
inhibitor (eg, cabozantinib) and vascular endothelial growth
factor receptor blocker (eg, bevacizumab)?
Second, the authors allude to a lack of inclusion of RKCs
in RCC trials and very few trials look at RKCs alone. In an
effort to increase interest in this area, they suggest that RKCs
be classified as an orphan disease. Although when bundled
together, they give an incidence of >5 per 10 000 (the
threshold for an orphan disease), when subtypes are
considered individually they give an incidence low enough
to be an orphan disease. This will encourage pharmaceutical
companies to develop drugs in this niche area by taking
advantage of orphan drugs legislations [7_TD$DIFF]such [8_TD$DIFF]as tax
incentives (Europe 1999; Orphan Drugs Act 1983; Rare
Diseases Act 2002).
Finally, the relatively small numbers of RKCs scattered
across the world necessitates international multicentre
collaboration to allow progress in this disease . The
authors suggest the use of international registries such as
the International mRCC Database Consortium or the USA-
based National Clinical Trials Network biorepository to
centralise tissue specimens for research. In an effort to
support this, they have created a portal
). This portal does not appear to be
at full functionality as yet, but it has a valuable clinical trial
search feature indexing over 300 clinical trials for RKCs. The
use of technology to make it easier for clinicians and
patients to access specialist[3_TD$DIFF] advice[2_TD$DIFF] and trials; enter data
about treatment outcomes; and encourage patients to
participate in trials will be essential for research and
development into RKCs. Likewise, links to international
registries through the RKC website will help. The authors
recommend that we should treat RKCs in specialist centres
and within clinical trials wherever possible. RKCs currently
treated outside of clinical trials represent missed opportu-
nities for [6_TD$DIFF]development in this area.
Whilst in several aspects this paper revisits many of the
conclusions and recommendations of the EAU RCC Guide-
line Panel review and other similar reviews on the
subject , the authors’ intention to inspire action in the
field of RKCs is highly laudable and deserves a reaction from
clinicians, academics, and pharmaceutical companies.
Conflicts of interest: The author has nothing to disclose.
References
[1]
.
[2]
.
[3]
[4]
.
[5]
.
[6]
[7]
.
[8]
[9]
.
[10]
.
E U R O P E A N U R O L O GY 7 2 ( 2 0 17 ) 9 8 4 – 9 8 5 985