Introduction on rare cancers

Introduction to rare cancer management

Rare cancers are rare occurrences of a common disease affecting less than 6 per 100,000 individuals a year (RARECARE) or one in five new patients diagnosed with cancer.

Rare adult cancers (RAC) comprise a large number of different tumour types. These hundreds of different rare cancer types may affect any of the body’s organs, with varying clinical presentations.

Although there are different groups of cancers affecting different organs, all rare cancers share similar challenges in their management which includes:

  1. The need for an expert pathological and molecular diagnosis, with both numerical expertise and molecular biology platforms enabling state-of-the-art case classifications available mostly, if not exclusively, in reference centers.
  2. The need for regularly updated clinical practice guidelines that require multidisciplinary collaboration between pathologists, molecular biologists, dedicated radiologists or nuclear medicine specialists, medical oncologists, radiotherapists, surgeons and organ specialists, psychologists and participation of cancer patient advocacy groups.
  3. The need for improved communication between patients, advocacy groups, and general practitioners to ensure proper recognition of the signs and symptoms of a rare cancer and also ensure timely referral to specialists to prevent delays in diagnosis and access to optimal treatment. A common characteristic of the medical pathway of patients with rare cancers is misdiagnosis or late diagnosis. So greater awareness about rare cancers at primary care level is crucial.
  4. When a confirmed diagnosis is obtained, patients with rare cancers may also face difficulties in referral to expert centers. Reasons for this include: lack of information about the existence of these centers; the long distance travel required to get to them; financial disincentives for the referring physician. It must also be stressed that some countries lack any dedicated expert reference centers. One of the objectives of EURACAN is to provide cross-border services for these countries, leading ultimately to the establishment of reference centers for rare cancers in all EU countries, and/or cross border access to certain rare and expensive technologies (e.g. proton beam therapy and carbon ion therapy), or very complex surgical procedures.
  5. The need for multidisciplinary management of patients with rare cancers, involving experts in all key specialty fields: pathology, molecular biology, imaging, nuclear medicine, surgery, radiotherapy, medical oncology, sub-specialty oncology, nursing, palliative care, psycho-oncology, rehabilitation, etc. It must be stressed that the major survival gains can be expected from an optimal first line treatment, performed according to the guidelines in reference centers.
  6. Limited access for patients with rare cancers to clinical research studies, including those trialling novel agents, is also a constant challenge for all rare cancers. Global coordinated efforts are needed to develop clinical, translational and basic research specifically dedicated to rare cancers.
  7. The need to improve the availability and exchange of scientific and medical information, clinical expertise, and clinical trials, and to refer patients to expert centers which the complexity of rare cancers requires.

It is now clear from the literature that the diagnosis and treatment of patients with rare cancers is best delivered, and yields increased cure rates, in reference centers. The development of networks of reference centers is therefore the next logical step to improve cure rates, decrease morbidity and decrease the cost of rare cancer management.

Translate »