Abstract Rosai–Dorfman disease, originally described by Juan Rosai and Ronald F. Dorfman, is a rare benign histiocytic proliferative disorder, classically presenting with massive lymphadenopathy and a self-limiting clinical course.1 Isolated intracranial skull base involvement is extremely rare and often resembles meningiomas, schwannomas, or other benign skull base lesions.2 The disease is difficult to diagnose radiographically, and tissue diagnosis with open skull base approaches has significant perioperative risks.2,3 We present the case of a 48-yr-old Caucasian male presenting with progressively worsening headaches, giddiness, hearing difficulty, and diplopia. Magnetic resonance imaging of the brain revealed T1-weighted isointense, T2-weighted hypointense, and contrast-enhancing dural-based lesion in the left cerebellopontine angle. The patient underwent maximally safe resection of the lesion through the retromastoid approach with careful preservation of the lower cranial nerve complex. The intraoperative findings of a variegated and lobulated mass adherent to the skull base, the surgical strategy of safe resection, and eventual good outcome in this patient are depicted in this 3-dimensional video presentation. The majority of patients with skull base Rosai–Dorfman disease reported in literature have had stable or regression of disease (78%) after initial conservative surgical treatment and hence aggressive surgical resection is of unproven efficacy.3 The patient has consented to depiction of his surgical video and intraoperative images in this video manuscript. Skull base tumor, Rosai–Dorfman disease, Operative findings, Sinus histiocytosis, Retromastoid approach View largeDownload slide Watch now at https://academic.oup.com/ons/article-lookup/doi/10.1093/ons/opy121 View largeDownload slide Watch now at https://academic.oup.com/ons/article-lookup/doi/10.1093/ons/opy121 Retromastoid Approach for Posterior Fossa Skull Base Rosai–Dorfman Disease—A Rare Pathology: 3-Dimensional Operative Video Retromastoid Approach for Posterior Fossa Skull Base Rosai–Dorfman Disease—A Rare Pathology: 3-Dimensional Operative Video Close Disclosure The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article. REFERENCES 1. Rosai J , Dorfman RF . Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity . World Neurosurg. 1969 ; 87 ( 1 ): 661.e5 - 661.e12 . 2. Griauzde J , Lieberman AP , McKean E , Sullivan S , Parmar HA . Radiology-pathology case report: isolated extranodal Rosai-Dorfman disease of the skull base . Clin Imaging. 2013 ; 37 ( 6 ): 1146 - 1148 . Google Scholar Crossref Search ADS PubMed 3. Hollon T , Camelo-Piragua SI , McKean EL , Sullivan SE , Garton HJ . Surgical management of skull base Rosai-Dorfman disease . World Neurosurg. 2016 ; 87 : 661.e5 - 12 . Google Scholar Crossref Search ADS COMMENTS The authors present a video demonstrating the typical imaging appearance and pathology of Rosai-Dorfmandisease. This entity, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign disorder that may present as an intracranial lesion alone or in combination with other sites of disease – lymph nodes, skin, or soft tissue. In the case of isolated central nervous system disease, imaging alone is unlikely to be diagnostic as the lesions of Rosai Dorfman may bear significant similarities to meningiomas, schwannomas, or other benign lesions. Here the authors demonstrate surgical resection of cerebelloponine angle Rosai-Dorfman via a retromastoid approach. In addition to the typical imaging findings, the video also shows the characteristic pathology finding of this disease – emperipolesis, histiocytes containing other cells. Although Rosai-Dorfman is a rare disease, many neurosurgeons will encounter a case during their practice. It should remain a consideration in the differential diagnosis in the appropriate clinical setting. This video serves to remind neurosurgeons of this rare clinical entity and associated findings. Angela M. Richardson Ricardo J. Komotar Miami, Florida This video describes an interesting case of Rosai-Dorfman disease. Reporting this case, the authors would alert the neurosurgical community in considering this rare pathology in the differential diagnosis of the posterior fossa extraparenchimal lesions. Federico Giuseppe Legnani Milan, Italy Copyright © 2018 by the Congress of Neurological Surgeons This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)
Operative Neurosurgery – Oxford University Press
Published: Jan 1, 2019
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