Maladie de Castleman médiastinale : mise au point

Castleman disease is a rare lymph nodes disease whose name covers different clinical presentations. The most frequent histology is the hyaline vascular localized form. In this case, Castleman disease occurs in young adults, and is localized to the mediastinum in one third of the cases. The disease is often asymptomatic, but paraneoplasic pemphigus has been described. The management of this form of Castleman disease is based on complete surgical resection. Perioperative immunomodulating treatments may be discussed in case of paraneoplasic pemphigus, mostly when affecting the bronchial tree.