MSCT Features and Clinicopathological Analysis of Mediastinal Follicular Dendritic Cell Sarcoma with Paraneoplastic Pemphigus and Paraneoplastic Myasthenia Gravis
Objective To investigate the MSCT manifestations,clinical and pathological features of mediastinal follicu-lar dendritic cell sarcoma with paraneoplastic pemphigus and paraneoplastic myasthenia gravis,in order to improve the clini-cal understanding of this rare disease,avoid misdiagnosis and missed diagnosis,assist clinical improvement of related diag-nosis and treatment,and reduce the mortality.Methods All imaging,pathological and clinical data of 2 patients with me-diastinal FDCS accompanied by PNP and/or MG diagnosed pathologically by our hospital from January 2012 to December 2022 were retrospectively analyzed.Results In this group,2 cases were male patients with concurrent PNP,and the main clinical symptoms were oral ulcers,red and dark red rashes scattered throughout the body,some of which were fused into tablets,and one patient was accompanied by MG and severe dyspnea.Plain CT scan showed that the lesions in both cases occurred at the subgrade level of the right tracheal carina in the middle and posterior mediastinum,with single solid mass accompanied by multiple cystic necrosis.Spot-like or branching calcification could be seen in both cases,and the right infe-rior lobe bronchi were pushed to different degrees,enhancing unevenly and strengthening.Meanwhile,the lesions in the pa-tients with MG were gradually enlarged and the cystic necrosis area was gradually increased within 3 months.Conclusion FDCS accompanied by PNP and/or MG is rare and easy to be misdiagnosed and missed in early stage,but its clinical manifestations have certain specificity,and some characteristic imaging findings of MSCT are conducive to the early diagno-sis of this disease.It is necessary to fully understand the internal relationship between PNP,MG and FDCS,so as to achieve timely and accurate diagnosis to assist clinical treatment as early as possible.
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