6533b85bfe1ef96bd12baa8f

RESEARCH PRODUCT

A Follicular Dendritic Cell Sarcoma of the Mediastinum With Immature T Cells and Association With Myasthenia Gravis

Alexander MarxChristian-friedrich VahlMarc HartertPhilipp StröbelWilfred NixManfred Dahm

subject

AdultMalePathologymedicine.medical_specialtyT-LymphocytesDendritic Cell Sarcoma FollicularMediastinal NeoplasmsFollicular cellPathology and Forensic MedicineAzathioprineMyasthenia GravisBiomarkers TumormedicineHumansAntigen-presenting cellFollicular dendritic cellsbusiness.industryMediastinumDendritic cellThymectomymedicine.diseaseImmunohistochemistryMyasthenia gravismedicine.anatomical_structureFollicular dendritic cell sarcomaSurgeryLymph NodesSarcomaAnatomybusinessImmunosuppressive Agents

description

Follicular dendritic cell (FDC) sarcoma is a very rare neoplasm showing morphologic and phenotypic features of FDCs. It occurs primarily in lymph nodes but also in extranodal sites. So far, there have been no reports on FDC sarcoma associated with myasthenia gravis. In the following we will present a case of an FDC tumor of the mediastinum associated with paraneoplastic myasthenia gravis in a 39-year-old man. The tumor contained a major proportion of immature T cells, which may be connected to this patient's very unusual clinical presentation with autoimmune phenomena. Extranodal FDC sarcomas still seem hardly noticed, and their clinical and pathologic characteristics remain to be better defined.

https://doi.org/10.1097/pas.0b013e3181d7a2ee