Isolated intracranial Rosai-Dorfman disease mimicking petroclival meningioma in a child: Case report and review of the literature.

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Isolated intracranial Rosai-Dorfman disease mimicking petroclival meningioma in a child: Case report and review of the literature.

Medicine (Baltimore). 2017 Nov;96(47):e8754

Authors: Yang X, Liu J, Ren Y, Richard SA, Zhang Y

Abstract
RATIONALE: Rosai -Dorfman disease (RDD) is a rare, idiopathic, and non-neoplastic histioproliferative disease with distinctive entity of unknown etiology. Central nervous system (CNS) RDD is uncommon, hence, isolated intracranial RDD is extremely rare. So far only 6 cases of CNS RDD with the lesions originating from petroclival region have been reported. We present a case of isolated intracranial RDD mimicking petroclival meningioma.
PATIENT CONCERNS: A 14-year-old girl was admitted at our hospital with a 3-month history of dizziness, slowly progressing headache, and 2-month history of instability in walking. Cranial nerve deficits, including left facial paralysis, left facial numbness and left hearing loss, were evident on examination.
DIAGNOSES: Initial diagnosis of petroclival meningioma was made according to preoperative magnetic resonance imaging.
INTERVENTIONS: The lesion was resected subtotally and pathology confirmed RDD. The patient received gamma-knife treatment for the residual lesion.
OUTCOMES: The patient recovered well and the residual lesion significantly retrogressed on follow-up images.
LESSONS: Preoperative diagnosis of petroclival RDD is full of challenges. Although surgical resection of lesions is an effective treatment option, total resection is not highly recommended because the surgery-related defect must be minimal. Patient with residual lesion can be put on steroid therapy and/or radiotherapy, especially for IgG4 positive subset of RDD.

PMID: 29381971 [PubMed - indexed for MEDLINE]

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