Les tumeurs thoraciques rares

Abstract

Rare thoracic tumors are defined as lung, pleural, mediastinal, or cardiac tumors with unusual histology. Overall, these tumors account for less than 1% of all primary thoracic tumors, while they correspond to more than 100 different histological, clinical, radiological, and prognostic entities. Some rare histological subtypes are specific to the lung or the pleura, whereas others, rarely occurring within the thorax, correspond to tumors more frequent in other organs. Some rare thoracic tumors have been individualized recently in histopathological classifications.The most frequent rare pulmonary tumors are, in decreasing order of frequency: carcinoids, sclerosing hemangioma, inflammatory myofibroblastic tumors, Mucosa-Associated Lymphoid Tissue (MALT) lymphoma, and sarcomatoid carcinoma. In the mediastinum, thymic tumors represent a model of implementation for all rare thoracic tumors.These rare tumors are usually characterized, in addition to a low incidence, by the absence of clinical and radiological actualized characterization, by the low number of specialists for each tumor sub-type, and by the absence of specific therapeutic data.Recognition of rare thoracic tumors is based on specific clinical and radiological signs suggesting the diagnosis, on a specific diagnostic and imaging strategy, and on the determination of their primary or secondary nature. The establishment of specific standards of care for rare pulmonary tumors requires collaboration and networks to better characterize their clinical and therapeutic features, and to optimize the management of patients.