Isolated cranio-spinal involvement of Rosai-Dorfman disease: case report

Ambekar, Sudheer; Somanna, Sampath; Bhat, Dhananjaya I; Ranjan, Manish

doi:10.3109/02688697.2010.508849

Cited by 11 publications

(7 citation statements)

References 2 publications

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“…[9] A review of the literature showed that to date there are about 50 reported cases of isolated extranodal CNS RDD described in 6 other articles. [13810131618] Most cases have a florid presentation with seizures or headaches and neurological deficits like visual loss and limb weakness or numbness being most common.…”

Section: Discussionmentioning

confidence: 99%

Benign scalp lump as an unusual presentation of extranodal Rosai-Dorfman Disease

et al. 2014

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Background:Rosai–Dorfman disease (RDD) is a rare benign histioproliferative disease. It is typically characterized by benign histiocyte proliferation with lymphadenopathy, fever, and leukocytosis and was first described in 1969 by Rosai and Dorfman. Extranodal involvement has been reported in approximately up to 43% of the cases with isolated central nervous system (CNS) manifestations being even rarer.Case Description:We report our management of a 41-year-old female with extranodalpurely CNS RDD presenting as a benign scalp lump. Her lump progressed from an asymptomatic benign lesion to one causing localized cerebral edema. Treatment was surgical excision of both the cervical and CNS lesions achieving complete removal of the lesions and resolution of her symptoms.Conclusion:RDD is a rare condition and isolated CNS RDD is even less common. Benign scalp lumps have a myriad of differential diagnoses, but RDD should be a consideration in the presence of preexisting RDD lesions at other sites given its potential to progress and result in morbidity. It is imperative to be aware that symptoms may be especially deceiving as the absence of lymphadenopathy may point away from RDD as the diagnosis.

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Section: Discussionmentioning

confidence: 99%

Benign scalp lump as an unusual presentation of extranodal Rosai-Dorfman Disease

et al. 2014

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“…After injection of gadolinium contrast agent, the lesion shows homogeneously or inhomogeneously intense enhancement, and the dural tail sign can often be found. [4,11] In our present case, brain MRI revealed a well-defined homogenous mass which is closely related to the dura mater in the foramen magnum extending toward the cervical spinal canal; the lesion was isointense on T1WI, hypointense on T2WI, and intensely enhanced with gadolinium. These above radiological findings are difficult to preoperatively distinguish RDD from meningioma.…”

Section: Discussionmentioning

confidence: 47%

Isolated Rosai–Dorfman disease with craniocervical junction involvement in the foramen magnum

Wang¹,

Zou

Zeng³

et al. 2019

Medicine

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Rationale:Rosai–Dorfman disease (RDD) is a rare benign histiocytic proliferative disease. RDD with cranio-spinal involvement in the foramen magnum is extremely rare. To the best of our knowledge, only 4 cases of RDD with craniocervical junction involvement have been reported so far. Herein, we present the fifth case of RDD with craniocervical junction.Patient concerns:A 26-year-old female presented with a sudden headache, accompanied by nausea and vomiting several times during the past half-month.Diagnoses:Magnetic resonance imaging (MRI) showed a well-defined, lobulated, homogenous mass in the left foramen magnum. The lesion was isointense on T1-weighted images (T1WI) and hypointense on T2-weighted images (T2WI), and showed homogeneously obvious enhancement following the intravenous administration of gadolinium. It was dural based and extending inferiorly along the spinal dura up to the cervical spinal canal. The brainstem was compressed and deflected to the right side. Initial diagnosis of meningioma with craniocervical junction involvement in the foramen magnum was made according to MRI findings. Final diagnosis of RDD was confirmed by histopathological and immunohistochemical examinations after subtotally surgical resection.Interventions:The bulk of lesion in the foramen magnum was removed surgically with suboccipital craniectomy because of brainstem compression.Outcomes:The patient recovered well and was discharged 17 days after the surgery.Lessons:RDD should be considered in patients with dural-based, extra-axial, well-circumscribed, hypo- to isointense on T1WI, hypo- to isointense on T2WI, enhancing intracranial or spinal lesions or both.

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“…CNS involvement is rare in RDD, and it usually presents in the form of a well-defined, solitary, extraparenchymal supratentorial dura-based lesion resembling a meningioma in neuroimaging scans. 3 Lesions located in the posterior fossa and extending to the cervical spinal canal, as in our case, are rare.…”

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confidence: 54%

“…To the best of our knowledge, only three cases of RDD displaying spinal cord compression secondary to craniocervical junction involvement have been reported since the disease was first described in 1969. 2 3 4 …”

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confidence: 99%

“…In all three previously reported cases of RDD with craniocervical junction involvement, the most frequent neurological symptoms were motor deficits (in two of the three patients) and cervical pain. 2 3 4 The patients described by Ambekar et al 3 and Sandoval-Sus et al 4 presented prolonged insidious symptoms that had developed for more than 1 year before they were assessed by a neurosurgery department. 3 4 In contrast, the severity of our case (tetraparesis and right-sided sensory alterations developing within 1 week) resulted in an early diagnosis of spinal cord compression; our patient was treated surgically less than 1 month after the diagnosis.…”

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confidence: 99%

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Extra-Axial Mass in the Foramen Magnum Causing Cervical Compressive Myelopathy as a Complication of Rosai-Dorfman Disease

et al. 2017

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Isolated cranio-spinal involvement of Rosai-Dorfman disease: case report

Cited by 11 publications

References 2 publications

Benign scalp lump as an unusual presentation of extranodal Rosai-Dorfman Disease

Benign scalp lump as an unusual presentation of extranodal Rosai-Dorfman Disease

Isolated Rosai–Dorfman disease with craniocervical junction involvement in the foramen magnum

Extra-Axial Mass in the Foramen Magnum Causing Cervical Compressive Myelopathy as a Complication of Rosai-Dorfman Disease

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