Le comité des malades - Institut National Du Cancer

1
Rare cancers of adult:
a specific organisational framework in
France
The definition of rare cancers
There is no international consensus for the definition
of a rare cancer. Whereas a prevalence of under
50/100,000 persons per year delineates a rare disease,
it is a low incidence that serves as a basis for the defi-
nition of a rare cancer. Hence, the analysis by localiza-
tion and by histological subtype leads to a proposed
cut-off of 6/100,000 in Europe1, and 15/100,000 in the
USA2 for the definition of a rare cancer.
The specific organizational framework for rare cancers
in adults promoted by the French national cancer
institute (INCa) and the Ministry of Health targets
both:
• cancers with incidence under 6/100,000 persons
per year;
• and cancers requiring a highly specialized care,
owing to their localization or their occurrence in a
very specific context or their complexity.
Histological or molecular rare subtypes of frequent
cancers are excluded from this specific organizational
framework.
These cancers present specific problems:
• primarily the difficulty of diagnosis. Indeed, the
rarity of cases may lead to diagnosis uncertainty
and several weeks delay, or even misdiagnosis,
which may in turn lead to inadequate treatment;
• but also a problem of care management. Few
clinical practice guidelines are available for these
pathologies; expertise and access to highly com-
plex therapies are often limited to a few institu-
tions ;
• thirdly, an insufficient access to clinical trials, due
to the rarity of these cancers ;
• finally, and above all, the rarity of these tumors,
subsequent delayed diagnosis and/or treatment
breads feelings of isolation for the patients and
their relatives.
1 Gatta G, et al, Europ J Cancer 2011; 47 : 2493-2511
2 Greenlee RT, et al, Public Health Rep 2010 ; 125 : 28-43
www.e-cancer.fr
French Cancer plan 2009-2013 : measures 20 and 23

2
Description of organisational
framework
It was built through 4 competitive calls for proposals
since 2009. Twenty-three national clinical expert cen-
tres and 4 national pathological networks have been
identified.
For a given group of rare cancers, the management of
care relies on regional or interregional expert centres
that cover the whole national territory, including the
French overseas Departments and Territories and are
coordinated at the national level by a national expert
centre under the responsibility of a coordinating clini-
cian. Expert centres have collaborative links with local
cancer teams.
Double reading and access to molecular tumor profil-
ing if needed, is organized either in the context of one
of the national pathological expert networks, or via
groups of expert pathologists working under the direc-
tion of an expert pathologist designated at a national
level. They assume responsibility for the organization
and quality of this double reading for all patient cases
presented at a regional or national referral RCP
(réunion de concertation pluridisciplinaire, multidisci-
plinary meeting). The double reading makes use of
telepathology networks to shorten delays.
Referral RCPs are organized (by web conferences most
of the time) at a regional and/or a national level by the
expert centres. The patient cases are discussed by
experts in the presence of the patient’s oncologist, at
diagnosis stage, during treatment, during follow-up, or
when the disease recurs.
Expert centres are requested:
• to contribute to research promotion on these rare
cancers through multicentre research studies –
basic, translational and clinical- at national or in-
ternational level;
• to participate in the drafting or updating of na-
tional recommendations for good clinical practice,
taking into account as appropriate European or in-
ternational recommendations;
• to contribute to the epidemiological surveillance
and observation of these cancers by establishing a
database and collect all cases;
• to train health care providers;
• to provide information to patients and their rela-
tives, through relationships with national patients
associations, and communication to the general
public on this rare cancer;
• to participate in the follow-up of this specific
organizational framework implemented by INCa;
• to liaise with the expert pathologist and integrate
double reading into this framework.
Accordingly, each and every patient affected by one of
these pathologies should benefit from best care in the
institution of his/her choice, with a definite diagnosis
through the double reading process of his/her tumoral
tissue samples, a multidisciplinary consensus confer-
ence on his/her case, the choice of an appropriate
treatment – very often innovative in the context of a
clinical trial- and the support of a patient association.
The rare cancers organizational framework is support-
ed through an annual grant of the LFSS (Loi de
Financement de la Sécurité Sociale, social security
funding Act) under MIGAC (mission of general interest
and contract support).

3
National clinical expert centres for rare adult cancers
RARE CANCERS NETWORK NAME NATIONAL COORDINATOR
(AND CO-COORDINATORS)
NATIONAL EXPERT CENTRE
(ONE OR MORE SITES
Soft tissue and visceral sar-
comas NETSARC
Pr Jean-Yves Blay
Dr Binh Bui
Dr Axel Le Cesne
Centre Léon Bérard
Institut Bergonié
Institut Gustave Roussy
Bone sarcomas RESOS Pr François Gouin CHU de Nantes
Rare malignant neuroendo-
crine tumours RENATEN Pr Patricia Niccoli Hôpital de la Timone, AP-HM
Rare primary tumours of the
nervous system TUCERA Pr Hugues Loiseau Hôpital Pellegrin, CHU de Bor- deaux
High-grade oligodendroglial
tumours POLA Pr Jean-Yves Delattre
Pr Dominique Figarella-Branger
Hôpital Pitié Salpêtrière, AP-HP
Hôpital de la Timone, AP-HM
Rare cutaneous carcinomas CARADERM Pr Laurent Mortier CHRU de Lille
Rare malignant ENT tumours REFCOR Dr François Janot
Pr Baujat
Institut Gustave Roussy
Hôpital Tenon, AP-HP
Thymoma and thymic
carcinoma RYTHMIC Dr Benjamin Besse Pr Nicolas Girard
Institut Gustave Roussy,
Hôpital Louis Pradel, HCL
Malignant pleural
mesothelioma MESOCLIN
Pr Arnaud Scherpereel
Pr Françoise Le Pimpec-Barthes
Pr Jacques Margery
CHRU Lille
Hôpital Européen
Georges Pompidou, AP-HP
Institut Gustave Roussy
Rare renal carcinomas CARARE Dr Bernard Escudier Institut Gustave Roussy
Rare ovarian carcinomas
Observatoire des
tumeurs malignes rares
gynécologiques
Dr Isabelle Ray-Coquard
Pr Eric Pujade-Lorraine
Dr Patricia Pautier
Centre Léon Bérard
Hôtel-Dieu, AP-HP
Institut Gustave Roussy
Rare peritoneal tumours RENAPE Pr François Gilly Centre hospitalier Lyon Sud, HCL
Adrenal cancers COMETE
Pr Xavier Bertagna
Pr Pierre-François Plouin
Dr Eric Baudin
Hôpital Cochin, AP-HP
Hôpital européen
Georges Pompidou, AP-HP
Institut Gustave Roussy
Cutaneous lymphomas
Réseau d’experts du
groupe français d’étude
des lymphomes cutanés
(GFELC)
Pr Martine Bagot Hôpital Saint-Louis, AP-HP
Primitive lymphomas of the
central nervous system
LOC (lymphome ocu-
laire et cérébral)
Pr Khê Hoang-Xuan
Dr Carole Soussain
Hôpital Pitié Salpêtrière, AP-HP
Centre Hospitalier Spécialisé
Institut Curie, site de Saint-Cloud
Lymphomas associated with
coeliac disease CELAC
Pr Christophe Cellier
Pr Olivier Hermine
Pr Elizabeth Macintyre
Hôpital européen
Georges Pompidou, AP-HP
Hôpital Necker-Enfants Malades,
AP-HP
Uveal melanoma MELACHONAT Dr Laurence Desjardins Institut Curie
Refractory thyroid cancers TUTHYREF Pr Martin Schlumberger
Pr Françoise Borson-Chazot
Institut Gustave Roussy
Hospices civils de Lyon
AIDS and non-AIDS related
malignancies CANCERVIH
Pr Jean-Philippe Spano
Dr Isabelle Poizot-Martin
Pr François Boue
Hôpital Pitié Salpêtrière, AP-HP
Hôpital Sainte-Marguerite, AP-HM
Hôpital Antoine Béclère, AP-HP

4
Cancers occurring during
pregnancy CALG
Pr Emile Daraï
Dr Olivier Mir
Pr Philippe Morice
Hôpital Tenon, AP-HP
Hôpital Cochin, AP-HP
Institut Gustave Roussy
Gestational trophoblastic
tumors MTG Pr Daniel Raudrant Centre hospitalier Lyon Sud, HCL
von Hippel-Lindau disease
and inherited predisposition
to renal cancer
PREDIR Pr Stéphane Richard Hôpital Bicêtre*, AP-HP
Virally induced cances in
transplant recipients K-VIROGREF
Pr Véronique Leblond
Dr Corinne Bezu
Pr Camille Francès
Groupe hospitalier
Pitié Salpêtrière, AP-HP
Hôpital Tenon
AP-HM : assistance publique-hôpitaux de Marseille ; AP-HP : assistance publique-hôpitaux de Paris ; HCL : Hospices civils de Lyon
*: national expert centre with multiple sites including the following institutions: AP-HP (hospitals Necker, hôpital européen
Georges Pompidou, Beaujon, Lariboisière, Cochin), and Institut Gustave Roussy.
National pathological networks for rare adult cancers and lymphomas
RARE CANCERS NETWORK NAME NATIONAL COORDINATOR
(AND CO-COORDINATORS)
NATIONAL EXPERT CENTRE
(ONE OR MORE SITES
Soft tissue and visceral
sarcomas CRePS-TMV
Pr Jean-Michel Coindre
Dr Philippe Terrier
Dr Dominique Ranchère-Vince
Institut Bergonié
Institut Gustave Roussy
Centre Léon Bérard
Malignant pleural
mesotheliomas and rare
peritoneal tumours
MESOPATH-IM@EC Pr Françoise Galateau-Sallé CHU de Caen
Rare malignant
neuroendocrine tumours TENpath Pr Jean-Yves Scoazec Hôpital Édouard Herriot, HCL
Lymphomas LYMPHOPATH Pr Georges Delsol
Pr Philippe Gaulard
CHU Toulouse
Hôpital Henri Mondor, AP-HP
Institut National du Cancer
52, avenue André Morizet
92100 Boulogne-Billancourt
France
Tel. [phone]
Fax [phone]
Available on www.e-cancer.fr