Cranio-spinal Rosai Dorfman disease: case series and literature review

Abstract

Rosai-Dorfman disease (RDD) is a rare condition similar to lymphomas, presenting with cervical lymphadenopathy in young adults. Extra-nodal involvement is relatively common but involvement of the central nervous system (CNS) is rare. Cranial RDD presents with symptoms of raised intracranial pressure, focal or generalised seizures, while spinal RDD presents with pain, peripheral neurological deficits and radiculopathy. In contrast to other similar neoplastic or degenerative conditions affecting the CNS, RDD is a benign, non-infective, granulomatous disorder. Radiologically cranio-spinal RDD often mimics commoner dural-based lesions like meningioma, with only subtle radiological differentiating findings on Magnetic Resonance Imaging (MRI). The histopathology of RDD is diagnostic. Surgical excision is preferred modality of treatment. However, adjuvant therapies like steroids and radiation may help controlling residual or recurrent disease. There are multiple sporadic reports and short case publications in the literature, often focusing on a particular aspect of RDD. In this study, authors aim to present five cases of craniospinal RDD, and comprehensive review of literature and highlight neurological complications of systemic RDD.

Keywords: Rosai-Dorfman disease; differential diagnosis of meningioma; emperipolesis; extradural granulomatous lesion; lymphadenopathy.