Cancer is categorized as “rare” according to its very low incidence and its very specific location. There are hundreds like the corticosurrenaloma from which actress Emilie Dequenne suffered.
Actress Emilie Dequenne died on March 16, 2025 of the consequences, of a corticosurrenaloma (cancer of the adrenal glands), an extremely rare cancer which affects about 1 to 2 people out of a million per year in the world.
What is the definition of rare cancer?
We consider a cancer is rare when its incidence is less than 6 cases per year per 100,000 inhabitantsindicates the National Cancer Institute. Cancer is also considered rare if it is in a specific and unusual place (Thymus, uvés or digestive tract for example, or if it occurs in a specific framework (during pregnancy for example) or if it is particularly complex, little known and requiring highly specialized care. “The term ‘rare cancer’ is unhappybecause if they are brought together, they concern 22% of all cancer patients (either 70,000 cases each year in France)“, Note Dr. Charles Honoré, visceral surgeon and responsible for the” OS – soft parts “committee to Gustave Roussy. Not all rare cancers are aggressive Or unfavorable prognosis (for example, survival observed after 5 years for children aged 0 to 14 who have a retinoblastoma is 96 %). They are not necessarily hereditary either. However, genetic anomalies predispose to certain rare cancers: this is the case with Von Hippel-Lindau’s disease or certain rare neuroendocrine cancers.
The list of rare cancers
The list of rare cancers has been established According to the classification published in 2011 by the Rarecare working groupin charge of monitoring rare cancers in Europe. Here are several examples:
► Pediatric cancers: retinoblastoma, neuroblastoma, medulloblastoma
► Hematological cancers: Classic Hodgkin lymphoma, chronic myeloid leukemia, mastocytosis, Fanconi anemia …
► Environment -related cancers
► Rare thoracic cancers: Thymus tumors, trachea tumors and lungs …
► Rare cancers of the skin or eyes: Gorlin syndrome, Cowden syndrome …
► Rare cancers of the digestive sphere: Anal canal page, intrahepatic cholangiocarcinoma, family adenomatous polyposis …
► Rare gynecological cancers: Breast Paget disease, breast cancer in humans, li-fraumeni syndrome …
► Rare Cancers ENT: Salivary head and neck tumor, type 2 neurofibromatosis …
► Sarcomas: Ewing Sunlaughter of soft fabrics, Kaposi sarcoma …
► Neuroendocrine tumors: Endocrine neoplasia, family leiomyomatosis …
► Rare brain tumors and central nervous system: Carcinoma of choroids, ependymal tumors …
► Rare urological cancers: Adenocarcinoma of the urethra, Birt-Hogg-Dubé syndrome …
More difficult to treat cancer
Because of their rarity or their specificity, these cancers are often the subject ofA delay in diagnosis And difficulty in setting up a treatment suitable for each patient, which often requires inclusion in a clinical trial. So these are cancers more difficult to treat. This is why in 2009, the National Cancer Institute decided to Better organize the care offer For rare cancers, in order to improve the management, diagnostic and therapeutic, patients. For this, he has labeled networks bringing together various care establishments coordinated by Reference centers : Refps for rare ENT cancers, rhythmic for thymus cancers, carare for rare rein tumors, netsarc for sarcomes …
- Specific regional cancer system in Île-de-France
- The answers to your questions about rare cancers – Inca
