Rare thoracic cancer

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 tumours.

Rare thoracic tumours correspond to more than 100 different histological, clinical, radiological, and prognostic entities.
Some rare thoracic tumours are unique to the thorax, whereas others, rarely occurring within the thorax, correspond to tumours more frequent in other organs: for example, sarcomas, NET, lymphomas may develop primarily in the lung. As for all rare tumours, rare thoracic tumours suffer from a limited number of experts and limited therapeutic options.

Rare thoracic tumours share histological complexity and require expert pathological diagnosis. Treatment strategies for rare pulmonary tumours 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 relies 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.