Spinal Rosai–Dorfman disease: case report and literature review

Xu, Haocheng; Zhang, Fan; Lu, Feizhou; Jiang, Jianyuan

doi:10.1007/s00586-017-4975-0

Cited by 19 publications

(12 citation statements)

References 60 publications

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“…The mean age of spinal RDD is 34.7 years (range 2.5–78 years) with a male predominance (M/F = 1.48) with 71.4% of cases of spinal RDD being isolated, 21.4% of cases have associated lymphadenopathy, and 19.7% of patients have intracranial lesions simultaneously. [ 28 ] Before our case (case-no.1), only one case of skull involvement reported by Allegranza et al . in 1991,[ 3 ] making it be an extremely rare occurrence.…”

Section: Discussionmentioning

confidence: 88%

Rosai-Dorfman disease of cranial and spinal origin - A case series

Safi

Murshed

Ali

et al. 2020

Surgical Neurology International

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Background: Rosai-Dorfman disease (RDD) is an idiopathic nonneoplastic lymphadenopathy disorder which is characterized by lymph node enlargement, but it may also presents primarily involving a variety of extranodal sites, including central nerves system and craniospinal axis. This study reports five cases of craniospinal RDD, with review of epidemiology, clinical presentation, imaging, and histopathological features with current management strategies. Case Description: Five cases of RDD are diagnosed at Hamad General Hospital, Qatar, during 2013–2018. Two cases had dural-based cranial lesions with overlying cranial involvement while three cases were having extradural thoracic spine lesions. All cases underwent surgical intervention and confirmed by histopathology. Conclusion: Craniospinal RDD is a rare clinical presentation and poses significant diagnostic challenges preoperatively due to its similarity with other neoplastic or inflammatory diseases. Surgical option to remove compressive neural pathology provides a good clinical outcome with no recurrence in long-term follow-up.

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Section: Discussionmentioning

confidence: 88%

Rosai-Dorfman disease of cranial and spinal origin - A case series

Safi

Murshed

Ali

et al. 2020

Surgical Neurology International

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“…Spinal involvement in RDD is uncommon, manifesting as extramedullary dural-based lesions in most cases (4). Most lesions present as homogeneous, iso to hypointense masses on T2W images with or without perilesional edema, a presentation that closely mimics meningiomas (31,32). Distinction from this more commonly occurring imaging abnormality usually requires histologic confirmation.…”

Section: Discussionmentioning

confidence: 99%

“…Distinction from this more commonly occurring imaging abnormality usually requires histologic confirmation. Rarely, the presence of bone hyperostosis may enable the differentiation (32).…”

Section: Discussionmentioning

confidence: 99%

Multimodality imaging manifestations of Rosai-Dorfman disease

Vaidya

Mahajan

Rane

2020

Acta Radiologica Open

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Background Rosai-Dorfman disease (RDD) is a rare lympho-histiocytic disorder of indeterminate etiology usually presenting with lymph node involvement, and infrequently with extra-nodal manifestations. The diagnosis of this condition is challenging due to the wide spectrum of disease manifestations. Purpose To elucidate the radiologic features of this disease using multimodality imaging in histopathologically proven cases and to identify characteristic features that would enable its differentiation from its mimics. Material and Methods We retrospectively evaluated imaging studies of 19 patients with histopathologically confirmed RDD presenting to our institute between January 2004 and March 2016. Imaging modalities included magnetic resonance imaging (MRI), computed tomography, FDG-positron emission tomography (PET) CT, mammography, and ultrasonography. Results Lymphadenopathy was the most common imaging feature in our study, seen in 11 (57.8%) cases followed by sino-nasal involvement in 7 (36.8%) cases and intracranial masses in 5 (26.3%) cases. Bilateral homogeneously enhancing cervical lymphadenopathy with avidity on FDG-PET scans was the predominant abnormality on imaging. Sino-nasal involvement manifested as homogeneously enhancing soft-tissue masses occupying the paranasal sinuses. Intracranial disease manifested as sellar/suprasellar masses, dural-based lesions along the cerebral hemispheres and choroid plexus enlargement. Unusual disease manifestations included spinal, osseous, and breast lesions. Conclusion Due to the high likelihood of multifocal involvement, the recognition of RDD at one site necessitates screening of other sites for disease. Homogeneously enhancing, FDG-avid lymphadenopathy and sino-nasal masses in association with hypointense extra-nodal lesions on T2-weighted MRI are imaging features which could aid the diagnosis of RDD and facilitate its differentiation from pathologies that present in a similar manner.

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“…A retrospective analysis on clinical records dated from 1969 to 2016 revealed that only 60 cases of isolated spinal RDD were reported during this time period. [ 7 ] Of these 60 cases, a significant majority of them (58 cases) were extramedullary dura-based lesions with intramedullary lesions affecting only 2 patients. [ 7 ] Interestingly, Chhabria et al [ 8 ] has reported RDD presenting as Conus–Cauda syndrome in which the mass lesion extended from the cauda equina to the sacral level.…”

Section: Discussionmentioning

confidence: 99%

“…[ 7 ] Of these 60 cases, a significant majority of them (58 cases) were extramedullary dura-based lesions with intramedullary lesions affecting only 2 patients. [ 7 ] Interestingly, Chhabria et al [ 8 ] has reported RDD presenting as Conus–Cauda syndrome in which the mass lesion extended from the cauda equina to the sacral level. Our case report is of a 28-year-old patient who presented with an isolated epidural mass at the level of the thoracic vertebra, in the absence of intracranial lesions.…”

Section: Discussionmentioning

confidence: 99%

Isolated extradural Rosai–Dorfman disease causing the spinal cord compression

Li¹,

Wang²,

Gao³

et al. 2018

Medicine

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Rationale:Rosai-Dorfman disease (RDD) is a rare benign histiocytic disease that is commonly characterized by massive painless cervical lymphadenopathy and systemic manifestations. Isolated extranodal involvement, especially spinal involvement, is extremely rare.Patient concerns:A 28-year-old man presented with intermittent dorsodynia and bilateral lower-limb weakness and numbness. A magnetic resonance scan (MRI) showed an extradural lesion of the T6-T9 thoracic spine that lead to cord compression.Diagnoses:Histopathological findings showed distinctive emperipolesis and immunohistochemistry results that were positive for cluster of differentiation CD68 and S100. Therefore, we diagnosed the Rosai-Dorfman disease.Interventions:we performed a nearly total surgical resection and a limited T6-T9 laminectomy.Outcomes:Postoperatively, the patient's symptoms were partially relieved and experienced no recurrence during the 6-month follow-up. The preoperative diagnosis of isolated spinal RDD still remains challenging.Lessons:Thus, we should consider RDD in the differential diagnosis of the central nervous system. Besides surgical resection, the treatment also included radiation, chemotherapy or monoclonal antibodies. However, the optimal treatment remains controversial. Therefore, we should exert all our energies on the exploration of etiology and adjuvant therapy for this disease.

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Spinal Rosai–Dorfman disease: case report and literature review

Cited by 19 publications

References 60 publications

Rosai-Dorfman disease of cranial and spinal origin - A case series

Rosai-Dorfman disease of cranial and spinal origin - A case series

Multimodality imaging manifestations of Rosai-Dorfman disease

Isolated extradural Rosai–Dorfman disease causing the spinal cord compression

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