Follicular dendritic cell sarcoma (FDCS) is an intermediate-grade malignancy of follicular dendritic cells, which are derived from mesenchymal stem cells. Nodal FDCS is well-recognized. However, when it occurs at an extranodal site, it may not be recognized and is often misdiagnosed. These tumors exhibit a variable spindle to epithelioid cell morphology with a lymphocytic infiltrate and a distinct immunophenotype. The World Health Organization has classified this entity under tumors of hematopoietic and lymphoid tissue, that is, histiocytic and dendritic cell neoplasms. However, its occurrence at extranodal sites and its behavior and management more closely resemble that of a soft tissue tumor. Increased awareness about the existence of FDCS at extranodal sites may aid in the reduction of diagnostic errors. We wish to draw attention to this entity by reporting our experience of 54 cases of extranodal FDCS encountered over a period of 14 years and present a review of the literature of this underrecognized entity. We also describe the ontogeny and molecular pathogenesis of this uncommon lesion.
Follicular dendritic cell (FDC)-sarcoma is a rare neoplasm with morphologic and phenotypic features of FDCs. It shows an extremely heterogeneous morphology, therefore, its diagnosis relys on the phenotype of tumor cells. Aim of the present study was the identification of new specific markers for FDC-sarcoma by whole transcriptome sequencing (WTS). Candidate markers were selected based on gene expression level and biological function. Immunohistochemistry was performed on reactive tonsils, on 22 cases of FDC-sarcomas and 214 control cases including 114 carcinomas, 87 soft tissue tumors, 5 melanomas, 5 thymomas and 3 interdigitating dendritic cell sarcomas. FDC secreted protein (FDCSP) and Serglycin (SRGN) proved to be specific markers of FDC and related tumor. They showed better specificity and sensitivity values than some well known markers used in FDC sarcoma diagnosis (specificity: 98.6%, and 100%, respectively; sensitivity: 72.73% and 68.18%, respectively). In our cohorts CXCL13, CD21, CD35, FDCSP and SRGN were the best markers for FDC-sarcoma diagnosis and could discriminate 21/22 FDC sarcomas from other mesenchymal tumors by linear discriminant analysis. In summary, by WTS we identified two novel FDC markers and by the analysis of a wide cohort of cases and controls we propose an efficient marker panel for the diagnosis of this rare and enigmatic tumor.
Prosthetic joint infection with Mycobacterium tuberculosis usually involves the hips or knees and can result from either local reactivation, or less often from hematogenous spread. Predisposing conditions include rheumatoid arthritis, chronic steroid use and pulmonary diseases. The most common symptom at presentation is pain, and the most common physical finding is joint swelling and/or a draining sinus tract. The sedimentation rate is helpful when elevated but is nonspecific, and initial skin testing is only helpful when positive. The diagnosis depends on culture and histologic examination of tissue. Removal of the joint combined with oral antituberculous treatment is necessary when the infection is discovered greater than six weeks post joint replacement. Early diagnosis leads to decreased morbidity. Tuberculous infection of prosthetic joints is a rare disease and its diagnosis depends on a high degree of clinical suspicion.
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