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Liver and intrahepatic bile ducts - tumor

Other malignancies

Follicular dendritic cell tumors


Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● Rare tumor, ~15 cases reported in liver (Hepatobiliary Pancreat Dis Int 2011;10:443), ~ 100 cases reported at all sites, usually lymph nodes, head and neck
● Usually Asian women, mean age 47 years, range 19 to 82 years
● Usually associated with Epstein–Barr virus (EBV), weight loss, fever
● Indolent behavior; may recur without causing death
● Inflammatory pseudotumor-like variant: marked female predominance; selective localization in intra-abdominal sites, especially liver and spleen; frequent systemic symptoms; indolent behavior; prominent lymphoplasmacytic infiltration; consistent association with EBV (Am J Surg Pathol 2001;25:721)

Case reports
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● 51 year and 57 year old women with 30-bp deletion in Exon 3 of LMP1 gene in tumor (Mod Pathol 2001;14:354)
● 82 year old man with EBV- tumor (Arch Pathol Lab Med 2005;129:1480)

Gross description
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● Solitary, fleshy, often > 10 cm (range, 4-20 cm) with focal hemorrhage and necrosis; surrounding liver tissue unremarkable

Gross images
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Well circumscribed tumor with hemorrhage and necrosis

Micro description
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● Well-demarcated from surrounding liver
● Syncytial growth of spindle or ovoid tumor cells in background of lymphocytes and plasma cells
● Tumor cells have vesicular nuclei, distinct nucleoli and variable nuclear atypia with some cells resembling Reed-Sternberg cells
● Focal spindle cell fascicles; occasional vessels with fibrinoid deposits

Micro images
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Various images-liver




Various images-sites other than liver


Inflammatory pseudotumor–like variant (site unspecified) composed of loosely arranged spindle to oval cells (A) with prominent mixed inflammatory infiltrate consisting primarily of lymphocytes and plasma cells (B)

Positive stains
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● CD21 / CD35 (best), CD23, CNA.42, EBV-LMP1 (focal/weak), EBV (in situ hybridization)

Negative stains
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● ALK1, CD30, CD1a

Electron microscopy description
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● Long, thin cytoplasmic processes connected by desmosomes

Molecular images
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EBER in situ hybridization

Differential diagnosis
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Hodgkin lymphoma
Inflammatory myofibroblastic tumor
Sarcomas

End of Liver and intrahepatic bile ducts - tumor > Other malignancies > Follicular dendritic cell tumors


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