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Objective <p>Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV<sup>+</sup> IFDCS) of the liver is a rare stroma-derived neoplasm of lymphoid tissues. This retrospective study aims to delineate the clinicoradiological characteristics of hepatic EBV<sup>+</sup> IFDCS and propose potential diagnostic clues for this tumor.</p> Materials and Methods <p>Clinical and imaging data from patients with histopathologically confirmed hepatic EBV<sup>+</sup> IFDCS at a tertiary cancer center (July 2017 to July 2024) were retrospectively analyzed. Two abdominal radiologists independently re-evaluated B-mode ultrasound (B-mode), contrast-enhanced ultrasound (CEUS), and contrast-enhanced magnetic resonance imaging (CE-MRI) features, with discrepancies resolved by consensus.</p> Results <p>Eleven patients (8 males, 3 females; median age: 38&#xa0;years; range: 21 − 58&#xa0;years) with 13 lesions were included. Most patients lacked hepatitis B infection (9/11, 81.8%), and elevated absolute eosinophil counts were noted in 54.5% (6/11). Lesion diameters ranged from 12 − 154&#xa0;mm. On B-mode, lesions larger than 5&#xa0;cm (4/5, 80.0%) exhibited patchy hyperechoic areas with a Luffa-Reticulum Sign. On CEUS, most lesions displayed rapid hyperenhancement, accompanied by early (&lt; 45&#xa0;s) “washout” (6/9, 66.7%). Perfluorobutane-enhanced CEUS demonstrated Kupffer-phase defects in all cases (5/5, 100.0%). On CE-MRI, most lesions exhibited heterogeneous hyperenhancement in the arterial phase, while the majority showed iso- or hypointensity in the portal venous and delayed phases. On gadoxetate-enhanced CE-MRI, lesions displayed hypointense signals during transitional- and hepatobiliary-phase (8/8, 100.0%). Notably, characteristic features included capsule appearances (6/12, 50.0%) and targetoid features (8/12, 66.7%).</p> Conclusions <p>Hepatic EBV<sup>+</sup> IFDCS predominantly affects young adults with nonspecific symptoms. The coexistence of the Luffa-Reticulum Sign on B-mode, early “washout” on CEUS, capsule appearances as well as targetoid features on CE-MRI, and elevated eosinophil counts should raise suspicion of EBV<sup>+</sup> IFDCS, whereas definitive differentiation from other neoplasms requires histopathological confirmation.</p>

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Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma of the liver: clinical features, imaging findings and potential diagnostic clues

  • 期刊论文

  • 发表日期：

    2025年12月10日

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  • Gui-Ling Huang,
  • Man-Qian Huang,
  • Yu-Ting Zhang,
  • Hui-Ning Huang,
  • Hong-Tao Liu,
  • Xiao-Qing Pei

摘要

Objective

Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+ IFDCS) of the liver is a rare stroma-derived neoplasm of lymphoid tissues. This retrospective study aims to delineate the clinicoradiological characteristics of hepatic EBV+ IFDCS and propose potential diagnostic clues for this tumor.

Materials and Methods

Clinical and imaging data from patients with histopathologically confirmed hepatic EBV+ IFDCS at a tertiary cancer center (July 2017 to July 2024) were retrospectively analyzed. Two abdominal radiologists independently re-evaluated B-mode ultrasound (B-mode), contrast-enhanced ultrasound (CEUS), and contrast-enhanced magnetic resonance imaging (CE-MRI) features, with discrepancies resolved by consensus.

Results

Eleven patients (8 males, 3 females; median age: 38 years; range: 21 − 58 years) with 13 lesions were included. Most patients lacked hepatitis B infection (9/11, 81.8%), and elevated absolute eosinophil counts were noted in 54.5% (6/11). Lesion diameters ranged from 12 − 154 mm. On B-mode, lesions larger than 5 cm (4/5, 80.0%) exhibited patchy hyperechoic areas with a Luffa-Reticulum Sign. On CEUS, most lesions displayed rapid hyperenhancement, accompanied by early (< 45 s) “washout” (6/9, 66.7%). Perfluorobutane-enhanced CEUS demonstrated Kupffer-phase defects in all cases (5/5, 100.0%). On CE-MRI, most lesions exhibited heterogeneous hyperenhancement in the arterial phase, while the majority showed iso- or hypointensity in the portal venous and delayed phases. On gadoxetate-enhanced CE-MRI, lesions displayed hypointense signals during transitional- and hepatobiliary-phase (8/8, 100.0%). Notably, characteristic features included capsule appearances (6/12, 50.0%) and targetoid features (8/12, 66.7%).

Conclusions

Hepatic EBV+ IFDCS predominantly affects young adults with nonspecific symptoms. The coexistence of the Luffa-Reticulum Sign on B-mode, early “washout” on CEUS, capsule appearances as well as targetoid features on CE-MRI, and elevated eosinophil counts should raise suspicion of EBV+ IFDCS, whereas definitive differentiation from other neoplasms requires histopathological confirmation.