Rare adult solid cancers
Rare cancers represent 24% of all human cancers but cause 30% of all cancer deaths. Each year in Europe, about 500,000 people will be diagnosed with a rare cancer.
Rare cancers represent an extremely fragmented group of diseases. There are probably over 300 different rare cancer entities, as defined by an incidence under 6/100,000/year.
Within these rare cancer groups there is a further fragmentation, with many different histological or molecular subtypes.
For example, for sarcomas or brain tumours, which represent 1–2% of all cancers, over 150 different subtypes are identified.
Patients with rare cancers do not have access to the same levels of scientific evidence to build clinical practice guidelines compared to patients with frequent cancers, this results from the limited number of clinical trials and frequent lack of centralised care in the past.
Specialised care with experienced teams is often difficult to find for these patients.
Patients with rare cancers therefore frequently face delays in access to diagnosis and care and difficulties in accessing expert reference centres. They have fewer options for clinical trials and frequently receive inadequate treatment.
All these factors contribute to a worse prognosis of rare cancer patients which is consistently reported in the literature.
Click on the colored spots to find out about rare adult solid cancers specific to that area of the human body.
Find out more about rare adult solid cancers
Rare cancer of the connective tissues (sarcoma)
Sarcoma can arise anywhere in the human body, limbs and limb-girdles being the most common primary sites, but all other sites can potentially be affected, including viscera.
Rare cancer of the female genital organs and placenta
These tumours can be divided into three groups: gestational trophoblastic disease (GTD), rare ovarian tumours and all other rare gynaecological cancers arising in the uterus, cervix, vagina and vulva.
Rare cancer of the male genital organs and of the urinary tract
Testicular cancers are rare but they are the most common solid malignancy in men aged between 20 and 40.
Rare cancer of the neuroendocrine system
Neuroendocrine tumours can arise from any organ of the body but most commonly the small bowel, pancreas and lung.
Rare cancer of digestive tract
Rare digestive tract cancers gather a heterogeneous group of malignancies which include primary peritoneal malignancies, biliary tract cancer and anal cancers.
Rare endocrine cancer
Rare thyroid cancers are mainly classified as differentiated thyroid cancer (DTC), anaplastic thyroid cancer (ATC) and medullary thyroid cancer (MTC).
Rare head and neck cancer
Rare head and neck cancers include tumors originating from four different subsites: nasopharyngeal cancer, nasal and paranasal sinus cancers, salivary gland cancers and middle ear epithelial cancers.
Rare thoracic cancer
Rare thoracic tumors correspond to more than 100 different histological, clinical, radiological, and prognostic entities.
Rare skin cancer and uveal melanoma
Rare skin cancers consist of skin sarcomas, Kaposi sarcoma, Merkel cell carcinomas and adnexal tumours.
Rare cancer of the brain and of the spinal cord
Primary brain tumours are often diffuse glial tumours including astrocytomas and oligodendrogliomas from grades II to IV. Other types of rare brain tumours can be merged into a number of clinically relevant groups.