{Reference Type}: Review
{Title}: Mediastinal Rosai-Dorfman Disease with KRAS mutation case report and literature review.
{Author}: Zhang W;Fang L;Wang J;Ma X;Hu X;Liu W;
{Journal}: J Cardiothorac Surg
{Volume}: 19
{Issue}: 1
{Year}: 2024 Apr 1
{Factor}: 1.522
{DOI}: 10.1186/s13019-024-02668-0
{Abstract}: <B>BACKGROUND: </B>Rosai-Dorfman Disease (RDD) is a rare self-limiting histiocytosis, more prevalent in children and young adults. It typically manifests as painless bilateral massive cervical lymphadenopathy but may also extend to extra-nodal sites, with intrathoracic RDD noted in 2% of cases. Distinguishing mediastinal RDD from thymoma on imaging poses challenges, underscoring the reliance on pathological features and immunohistochemical staining for diagnosis.<BR><B>METHODS: </B>Patient, male, 33 years old, underwent lung a CT revealing an enlarged round soft tissue shadow in the anterior superior mediastinum, compared to a year ago. Surgical resection removed the entire mass, thymus, and part of the pericardium, confirming RDD on pathology. Genetic testing using second-generation testing technology identified a KRAS gene point mutation.<BR><B>CONCLUSIONS: </B>No established treatment protocol currently exists for this disease. However, as genetic mutation research progresses, a novel therapeutic avenue is emerging: targeted therapy integrated with surgical interventions.