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Spinal Rosai–Dorfman disease—a report of 2 cases and review of literature

Spinal Cord Series and Cases volume 9, Article number: 36 (2023) Cite this article

Subjects

Abstract

Introduction

Rosai–Dorfman Disease (RDD) is a rare benign histiocytic disease that infrequently affects the spine. We report two cases of spinal RDD and review the relevant literature. This report addresses the various diagnostic dilemmas related to the evaluation of Spinal RDD and its treatment.

Case presentation

Case 1: A 32-year-old male presented with low back pain and left anterior thigh for last 8 months. On examination, there was sensory diminution on inner aspects of the thigh with an absent left knee jerk. CT/MRI scans revealed an extradural lesion at L2/3 with neural compression. PET scan showed several hypermetabolic lesions in ribs, humerus, femur, and vertebrae. He underwent en bloc excision of the extradural mass with L2-3 pedicle screw-rod fixation and was later managed with chemotherapy. Case 2: A 42-year-old male presented with spastic paraparesis with urinary incontinence for the last 4 weeks. On examination, he had a neurological level of T6. MRI scan revealed a lesion in posterior elements of T6-7 compressing the spinal cord. He underwent T6-7 laminectomy with decompression. In both cases, the diagnosis was confirmed by histopathology and further managed by Hemato-oncologist. They both did well at 1-year follow-up with improvement in neurology.

Discussion

Spinal RDD to date remains a large diagnostic dilemma with no pathognomonic clinical or radiological features; mimicking many osteolytic lesions in the spine. The diagnosis is purely histopathological and immunological. The lesion’s complete surgical excision is the mainstay of treatment with a better prognosis and decreased chances of recurrences.

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Fig. 1: The imaging presentation of a case with spinal RDD.
Fig. 2: Histopathological examination of the dorsal extradural lesion at L2/3.
Fig. 3: MRI imaging of thoracic spine showing a well-defined epidural mass extending from T6–T8 levels causing significant cord compression.
Fig. 4: Histopathological examination of the extradural space-occupying lesion at T6 and T7.
Fig. 5

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Data availability

Correspondence and requests for materials should be addressed to KG.

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Acknowledgements

We thank Dr Bhaskar Mitra and Dr Debasis Banerjee for reviewing the histopathology slides.

Author information

Author notes

  1. These authors contributed equally: Rohan Gala, Kushal Gohil.

Authors and Affiliations

  1. Head of Department of Spine Surgery, Kothari Medical Centre, Kolkata, India

    Saumyajit Basu

  2. Department of Orthopedics, DY Patil University School of Medicine, Hospital and Research Centre, Nerul Navi Mumbai, India

    Rohan Gala

  3. Department of Orthopedics, Grant Government Medical College and Sir JJ Group of Hospitals, Mumbai, India

    Kushal Gohil

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  1. Saumyajit Basu
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  3. Kushal Gohil
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Contributions

We thank SB for allowing us to share two of his cases. SB was responsible for the overall review and finalizing of the manuscript. KG contributed the first case description and the review, editing, and revision of the manuscript. RG contributed the second case description and writing of the discussion.

Corresponding author

Correspondence to Kushal Gohil.

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The authors declare no competing interests.

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The study was approved institutional ethics committee. Both patients gave consent to publishing their data.

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Basu, S., Gala, R. & Gohil, K. Spinal Rosai–Dorfman disease—a report of 2 cases and review of literature. Spinal Cord Ser Cases 9, 36 (2023). https://doi.org/10.1038/s41394-023-00600-7

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