J Pathol Transl Med.  2025 Mar;59(2):133-138. 10.4132/jptm.2024.09.27.

Uncommon granulomatous manifestation in Epstein-Barr virus–positive follicular dendritic cell sarcoma: a case report

• Shen HGD
• Zhang Y
• Leow WQ

• KMID: 2566163
• DOI: http://doi.org/10.4132/jptm.2024.09.27

Abstract

Hepatic Epstein-Barr virus–positive inflammatory follicular dendritic cell sarcoma (EBV+ IFDCS) represents a rare form of liver malignancy. The absence of distinct clinical and radiological characteristics, compounded by its rare occurrence, contributes to a challenging diagnosis. Here, we report a case of a 54-year-old Chinese female with a background of chronic hepatitis B virus treated with entecavir and complicated by advanced fibrosis presenting with a liver mass found on her annual surveillance ultrasound. Hepatectomy was performed under clinical suspicion of hepatocellular carcinoma. Immunomorphologic characteristics of the tumor were consistent with EBV+ IFDCS with distinct non-caseating granulomatous inflammation. Our case illustrates the importance of considering EBV+ IFDCS in the differential diagnosis of hepatic inflammatory lesions. Awareness of this entity and its characteristic features is essential for accurately diagnosing and managing this rare neoplasm.

Keyword

Dendritic cell sarcoma, follicular; Epstein-Barr virus infections; Hepatitis B, chronic; Granuloma; Case report

Figure

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  Fig. 1. Radiological and histological findings of hepatic tumor. (A) Computed tomography scan revealing a 1.3 cm lesion (arrow) in hepatic segment IVb. (B) Hematoxylin and eosin–stained image showing a well-demarcated tumor from the surrounding hepatic tissue. (C) The tumor contains a mixture of atypical spindle cells within a background of small lymphocytes and plasma cells. (D) Abundant prominent noncaseating epithelioid granulomas within the tumor. (E) Multinucleated histiocytes are more readily observable at higher magnification. (F) Periodic acid–Schiff staining did not detect fungal organisms. (G) Ziehl-Neelsen staining was also negative for acid-fast bacilli, such as Mycobacterium tuberculosis.

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  Fig. 2. Immunohistochemical profile of hepatic tumor. Immunohistochemistry shows that the neoplastic cells were strongly positive for CD23 (A) and CD35 (B) markers, consistent with a follicular dendritic cell phenotype. (C) In situ hybridization for Epstein-Barr virus–encoded RNA highlights the neoplastic cell nuclei. (D) Anaplastic lymphoma kinase immunostaining is negative.

Reference

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