Rare cancer of the thorax

Domain 8


Rare thoracic cancers share the same difficulties of other rare cancers for diagnosis and treatment. They have in addition the characteristic of being treated in medical and research units where tobacco related lung carcinoma are largely overrepresented, and gathered recently a large part of the attention of research groups and of the public, because of major therapeutic advances, which are not necessarily shared by the rare thoracic tumors.

Rare thoracic tumors correspond to more than 100 different histological, clinical, radiological, and prognostic entities.

Some rare thoracic tumors are unique to the thorax, whereas others, rarely occurring within the thorax, correspond to tumors more frequent in other organs: for example, sarcomas, NET, lymphomas may develop primarily in the lung. As for all rare tumors, rare thoracic tumors suffer from a limited number of experts and limited therapeutic options.


Treatment strategies

Rare thoracic tumors share histological complexity and require expert pathological diagnosis. Treatment strategies for rare pulmonary tumors usually rely on surgery, but some such as lymphoma require to apply strategies developed for more frequent disease location. Mesothelioma are diagnosed with extensive disease in a majority of patients, and treatment rely on chemotherapy, possibly with immunotherapy in refractory disease. Thymic malignancies are usually eligible for surgical resection, but may be associated with autoimmune disorders that complexifies the management of patients. Due to the rarity and the complexity of those diseases, each case should be evaluated in an expert multidisciplinary team, consisting of surgeons, thoracic oncologists, radiologist, radiation oncologist, and pathologists.


Since 2017, the establishment of the European Reference Network EURACAN provided an infrastructure of healthcare providers with high level of multidisciplinary expertise for the diagnosis, and the management and follow-up of patients with rare cancers, including rare thoracic tumors; the objective is to increase the quality of care, while building collaboration to develop clinical and translational research projects.

Meanwhile, a dramatic improvement in our knowledge has occurred in the last few years, through the development of databases, translational research programs, and clinical trials. While access to innovative strategies represents a major challenge, as the rarity of the tumor precludes specific approval of drugs to be obtained, patient-centered initiatives, such as the establishment of dedicated networks, are warranted.

European Patient Advocacy Group (E-PAG) have the important role of representing the wider patient community in the development of ERNs and ensuring that the patient voice can be heard throughout the ERN development process. International societies provide infrastructure for global collaboration, and regional organization is also warranted to support equal access of patients to optimized management.

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