Tumeurs thoraciques rares

Abstract

Rare pulmonary tumors are defined as lung tumors with unusual histology. Overall, these tumors account for less than 1 % of all lung primary tumors, while they correspond to more than 100 different histological, clinical, radiological, and prognostic entities. Rare pulmonary tumors can develop from orthotopic, heterotopic, or haematopoietic tissues. Some rare histological subtypes are specific to the lung, whereas others, rarely occurring within the lung, correspond to tumors more frequent in other organs. Some rare pulmonary tumors have been individualized recently in histopathological classifications.These rare tumors are usually characterized, in addition to a low incidence, by the absence of clinical and radiological updated characterization, by the low number of specialists for each tumor subtype, and by the absence of specific therapeutic data. For patients, rare pulmonary tumors may then hamper equal access to the care.Orphan thoracic oncology looks at the study and the management of rare thoracic tumors, arising in the lung, the pleura, or the mediastinum. 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.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. Molecular profiling is of importance both for diagnosis and targeted treatment. The establishment of specific standards of care for rare thoracic tumors requires collaboration to better characterize their clinical and therapeutic features.