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 definition of a rare cancer. Hence, the analysis by localization 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.
1
2
Gatta G, et al, Europ J Cancer 2011; 47 : 2493-2511
Greenlee RT, et al, Public Health Rep 2010 ; 125 : 28-43
French Cancer plan 2009-2013 : measures 20 and 23
www.e-cancer.fr
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 complex therapies are often limited to a few institutions ;
•
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.
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Description of organisational
framework
It was built through 4 competitive calls for proposals
since 2009. Twenty-three national clinical expert centres 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 clinician. Expert centres have collaborative links with local
cancer teams.
Double reading and access to molecular tumor profiling 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 direction 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, multidisciplinary 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 international level;
•
to participate in the drafting or updating of national recommendations for good clinical practice,
taking into account as appropriate European or international 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 relatives, 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 conference 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 supported 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).
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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 sarcomas
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 neuroendocrine 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 Bordeaux
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
LOC (lymphome oculaire 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
Primitive lymphomas of the
central nervous system
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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
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Available on www.e-cancer.fr