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Erschienen in: Virchows Archiv 2/2006

01.08.2006 | Original Article

Extranodal follicular dendritic cell sarcoma: clinical, pathologic, and histogenetic characteristics of an underrecognized disease entity

verfasst von: Jinru Shia, Wen Chen, Laura H. Tang, Diane L. Carlson, Jin Qin, Jose G. Guillem, Jennifer Nobrega, W. Douglas Wong, David S. Klimstra

Erschienen in: Virchows Archiv | Ausgabe 2/2006

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Abstract

It has been more than 10 years since follicular dendritic cell (FDC) sarcoma was first reported to occur in extranodal sites, yet extranodal FDC sarcoma still appears underrecognized, and its clinical and pathological characteristics remain to be defined. This study analyzed the clinical and pathological findings of three such cases that the authors encountered recently and 43 previously reported cases identified in the literature. Assessment of all 46 cases showed a slight female predominance (1.2:1) with a median age of 41.5 years. One-third of the cases were misdiagnosed at initial evaluation mainly because the possibility of FDC sarcoma was not considered. When considered, this disease had distinct pathological characteristics that allowed an accurate diagnosis. Staining for FDC markers, CD21, CD35, and clusterin was particularly helpful. The pathogenesis of the disease appeared heterogeneous, and associated factors included Epstein–Barr virus infection (in hepatic cases) and inflammatory pseudotumor-like conditions. Treatment modality varied widely although surgical resection was often included. With a median follow-up of 18 months, 43% of the cases recurred and 7% died of disease. The 5-year recurrence-free survival was 27.4%. From data available at the current time, we were not able to identify prognostically significant pathologic factors.
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Metadaten
Titel
Extranodal follicular dendritic cell sarcoma: clinical, pathologic, and histogenetic characteristics of an underrecognized disease entity
verfasst von
Jinru Shia
Wen Chen
Laura H. Tang
Diane L. Carlson
Jin Qin
Jose G. Guillem
Jennifer Nobrega
W. Douglas Wong
David S. Klimstra
Publikationsdatum
01.08.2006
Verlag
Springer-Verlag
Erschienen in
Virchows Archiv / Ausgabe 2/2006
Print ISSN: 0945-6317
Elektronische ISSN: 1432-2307
DOI
https://doi.org/10.1007/s00428-006-0231-4

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