Posterior mediastinal soft-tissue Rosai–Dorfman disease mimicking a neurogenic tumor

Rosai–Dorfman disease (RDD), a rare non-Langerhans cell histiocytic disorder that primarily involves lymph nodes, rarely manifests as isolated posterior mediastinal extranodal lesions. We report a woman in her 30s with incidentally detected paravertebral masses in the posterior mediastinum on routine screening. Contrast-enhanced CT showed progressive homogeneous enhancement of the lesions; MRI revealed T1 isointensity, mild T2 hyperintensity, and marked fat-suppressed T2 hyperintensity relative to muscle. A neurogenic tumor was initially suspected. Video-assisted thoracoscopic excision established RDD: large pale histiocytes with emperipolesis, S100 and CD68 positive, CD1a negative. The patient had an uncomplicated postoperative course, with no clinical or radiologic recurrence over 2 years of follow-up. This case underscores extranodal soft-tissue RDD as a rare mimic of posterior mediastinal neurogenic tumors, highlighting the need for mandatory radiologic-pathologic correlation to prevent misdiagnosis and guide surgical management and long-term surveillance.

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Publication Details

Journal
Radiology Case Reports
Published
2026-08-21
DOI
https://doi.org/10.1016/j.radcr.2026.07.118
Primary Topic
Histiocytic Disorders and Treatments
Type
article
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article

Posterior mediastinal soft-tissue Rosai–Dorfman disease mimicking a neurogenic tumor

Jingfeng Huang, Zeyu Xiao, Yin He, Xinyu Li
Radiology Case Reports
Histiocytic Disorders and Treatments
article

Posterior mediastinal soft-tissue Rosai–Dorfman disease mimicking a neurogenic tumor

Jingfeng Huang, Zeyu Xiao, Yin He, Xinyu Li
article en

Abstract

Rosai–Dorfman disease (RDD), a rare non-Langerhans cell histiocytic disorder that primarily involves lymph nodes, rarely manifests as isolated posterior mediastinal extranodal lesions. We report a woman in her 30s with incidentally detected paravertebral masses in the posterior mediastinum on routine screening. Contrast-enhanced CT showed progressive homogeneous enhancement of the lesions; MRI revealed T1 isointensity, mild T2 hyperintensity, and marked fat-suppressed T2 hyperintensity relative to muscle. A neurogenic tumor was initially suspected. Video-assisted thoracoscopic excision established RDD: large pale histiocytes with emperipolesis, S100 and CD68 positive, CD1a negative. The patient had an uncomplicated postoperative course, with no clinical or radiologic recurrence over 2 years of follow-up. This case underscores extranodal soft-tissue RDD as a rare mimic of posterior mediastinal neurogenic tumors, highlighting the need for mandatory radiologic-pathologic correlation to prevent misdiagnosis and guide surgical management and long-term surveillance.

Radiology Case ReportsVol. 21(11)
Ningbo University Affiliated Hospital (CN), First Affiliated Hospital of Jinan University (CN), Ningbo First Hospital (CN)
Good health and well-being
Openalex Percentile: Top 10%
Histiocytic Disorders and Treatments
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