Rosai-Dorfman Disease with Massive Intracranial Involvement: Asymmetric Response to Conservative Therapy

Walker, Robert N.; Nickles, Thomas P.; Lountzis, Nektarios; Jacobs, Darren L.; Nawaz, Nava K.

doi:10.1111/j.1552-6569.2009.00404.x

Cited by 8 publications

(5 citation statements)

References 6 publications

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“…12,13 Owing to the location of the lesions complete excision of both lesions was not possible in our case. 12,13 Owing to the location of the lesions complete excision of both lesions was not possible in our case.…”

Section: Discussionmentioning

confidence: 87%

Intracranial Rosai-Dorfman

Said

Abi-Fadel²,

Talwar

et al. 2011

The Neurologist

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Rosai-Dorfman disease has been described as massive painless cervical lymphadenopathy with a benign clinical course and unclear etiology. Isolated extranodal involvement has been rarely described. We present a rare case of isolated intracranial Rosai-Dorfman disease that was diagnosed and treated at our institution. Our diagnostic and clinical challenges are presented with a review of the literature.

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Section: Discussionmentioning

confidence: 87%

Intracranial Rosai-Dorfman

Said

Abi-Fadel²,

Talwar

et al. 2011

The Neurologist

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“…Extranodal involvement is observed in approximately 40% of patients diagnosed with RDD, but CNS involvement is very uncommon, with 210 cases reported in the English medical literature, including our 6 patients (Table 2 ). 1 , 3 – 46 , 48 – 77 , 79 – 82 , 84 , 85 , 87 , 89 – 91 , 94 – 113 , 115 – 127 In 174 of the reported cases, RDD was isolated to the CNS, while in 36 patients it was part of a systemic disseminated disease (see Table 2 ). CNS involvement has been described in both adults and children, with a mean age at presentation of 39 years and a male prevalence (male: female ratio: 1.8: 1.0).…”

Section: Resultsmentioning

confidence: 99%

Rosai-Dorfman Disease of the Central Nervous System

et al. 2014

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Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is an uncommon benign idiopathic lymphoproliferative disorder. The histologic hallmark of RDD is the finding of emperipolesis displayed by lesional histiocytes. While RDD most commonly affects lymph nodes, extranodal involvement of multiple organs has been reported, including the central nervous system (CNS). However, CNS involvement in RDD is rare and is not well characterized. As a result, therapeutic approaches to CNS involvement in RDD are not well established. Herein we report 6 cases of RDD with isolated CNS involvement and review the literature on RDD with CNS involvement. One of the presented cases exhibited intramedullary involvement of the spinal cord—a very rare form of RDD with CNS involvement.

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“…Of interest, there was a case report on a 54 year-old female patient with both intracranial and extracranial sites of RDD involvement that was initially treated with dexamethasone, followed by oral prednisone. Radiologic follow up demonstrated that dermatologic, mediastinal, and retroperitoneal soft tissue masses responded favorably, but intracranial lesions progressed over the course of 9 months [9].…”

Section: Discussionmentioning

confidence: 98%

“…Most commonly, RDD intracranial manifestations are extra-axial and dura-based masses that mimic meningiomas on imaging [3,4,6,7]. Distinguishing RDD from meningomas on imaging is subtle; RDD has low signal intensity on T2-weighted images hypothesized to be due to free radicals released by inflammatory macrophages [9].…”

Section: Discussionmentioning

confidence: 99%

Extensive Multifocal Rosai‐Dorfman Disease Involving the Central Nervous System and Paranasal Sinuses

Kim

Friedel²,

Eloy³

et al. 2011

The Laryngoscope

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ConclusionsExtensive multifocal RDD involving the brain, spine, and paranasal sinuses is exceedingly rare and often mimic meningiomas on imaging. Surgical management can be challenging. We recommend a conservative approach with close observation and reserve surgical treatment for enlarging symptomatic lesions. AbstractIntroduction: Rosai-Dorfman disease (RDD) is a rare benign lymphocytic disorder characterized by idiopathic histiocytic proliferation affecting lymph nodes. RDD in the central nervous system (CNS) without nodal disease is exceedingly uncommon. We describe a rare case of extensive multifocal RDD involving the CNS, skull base, and paranasal sinuses. We review the literature and propose a surgical strategy to manage extensive multifocal disease. Methods: This 39 year-old-male with known RDD and multiple previous surgeries for intracranial and spinal RDD lesions presented with worsening spasticity, gait dysfunction, left facial pain and numbness, and mild dysphagia. Neuroimaging demonstrated multifocal enhancing dural-based lesions in the brain including right frontal, right temporal, right cerebellopontine angle, left clinoidal, left petroclival and bilateral Meckel's cave lesions. There was progressive growth of the left petroclival lesion causing symptomatic brainstem compression. The patient also had obstructive sleep apnea due to RDD occupying the entire nasal and paranasal sinuses. Results: The patient underwent a left-sided retrolabyrinthine transpetrosal-transtentorial approach to remove the left petroclival tumor and decompress the brainstem. The mass was carefully dissected off of cranial nerves IV through XI with excellent decompression of the brainstem. Endoscopic resection of the paranasal sinus mass was performed at a later date. Histologically, both masses were consistent with RDD. Conclusions: Extensive multifocal RDD involving the brain, spine, and paranasal sinuses is exceedingly rare and often mimic meningiomas on imaging. Surgical management can be challenging. We recommend a conservative approach with close observation and reserve surgical treatment for enlarging symptomatic lesions.

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Rosai-Dorfman Disease with Massive Intracranial Involvement: Asymmetric Response to Conservative Therapy

Cited by 8 publications

References 6 publications

Intracranial Rosai-Dorfman

Intracranial Rosai-Dorfman

Rosai-Dorfman Disease of the Central Nervous System

Extensive Multifocal Rosai‐Dorfman Disease Involving the Central Nervous System and Paranasal Sinuses

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