Nerve.  2023 Oct;9(2):222-229. 10.21129/nerve.2023.00479.

Diagnostic Challenges of Rosai-Dorfman Disease Presenting as Pachymeningitis and Meningiomatosis: A Report of Two Unusual Cases Involving the Spinal Cord

• Park JH
• Lee CK
• Kim SH
• Chang JH
• Shin DA
• Yi S
• Ha Y
• Kim KN

• KMID: 2547523
• DOI: http://doi.org/10.21129/nerve.2023.00479

Abstract

Rosai-Dorfman disease (RDD) is an exceptionally rare medical condition, with spinal cord involvement even more infrequent. It is frequently misidentified as another malignancy or infectious disease. In this report, we present two cases of RDD with spinal cord involvement. Each patient presented with spinal intradural extramedullary masses within the thoracic and cervical levels, respectively. The differential diagnoses remained uncertain, with the potential for pachymeningitis and meningiomatosis due to the shared nature of nonspecific imaging findings and clinical symptoms in both conditions. Given the presence of neurologic symptoms and spinal cord compression, the individual lesions were surgically excised. Subsequent pathological analysis definitively confirmed the diagnosis of RDD. As an adjunctive therapeutic measure, both patients were orally administered prednisolone. Both patients exhibited favorable neurologic improvement. These cases highlight the diagnostic challenges of RDD when solely relying on imaging studies. Surgical intervention, in conjunction with a meticulous pathologic examination, is imperative for a precise diagnosis and guides appropriate treatment strategies. The multifaceted spatial manifestation of this disease further underscores the potential for multifocal occurrences within the central nervous system, providing an avenue for improved comprehension of the underlying mechanisms contributing to its dissemination.

Keyword

Rosai-Dorfman disease; Spinal cord neoplasms; Meningioma; Meningitis