Extranodal right-optic nerve Rosai-Dorfman disease: A rare localization case report

Abstract

Background:Rosai–Dorfman is a rare disease that usually occurs in young adults. It is characterized with massive painless cervical lymphadenopathy and histiocyte proliferation. Isolated intracranial involvement is extremely rare. Our aim is to present a new rare case of extranodal Rosai–Dorfman disease that involved the right optic nerve in a 4-year-old boy.Case Description:A 4-year-old boy with right-sided convergent strabismus and amblyopia lasting for 1 year was treated at the Department of pediatric ophthalmology. Initial optical fundus examination was normal. Examination repeated after 1 year noted the atrophy of the optic nerve papilla. Visual evoked potentials of the right eye showed normal findings of prechiasmatic visual pathway with severe dysfunction of the right optic nerve. Magnetic resonance imaging (MRI) of the brain and orbits showed expansive changed and elongated right optic nerve with contrast enhancement, and smaller lesion in the right temporal operculum region visible in T2 and fluid-attenuated inversion recovery sequence. Through small eyebrow “keyhole” osteoplastic frontoorbital craniotomy the fusiform enlarged (to 2 cm) right optic nerve was identified, resected between the eyeball and optic chiasm, and transferred for pathohistological analysis. Early postoperative course had no complications. Histological, immunohistochemical, and ultrastructural analyses revealed extranodal Rosai–Dorfman disease. Right periorbital edema was verified on the 7th postoperative day and regressed to supportive therapy. Control multi slice computed tomography (MSCT) and MRI of endocranium and orbits showed total tumor removal with no signs of complications.Conclusion:Although rare, extranodular intracranial Rosai–Dorfman disease should be taken into account in the differential diagnosis of intracranial and intraorbital lesions, especially in the pediatric age group.

Orbital Rosai–Dorfman disease: A case report and literature review

“…[1314] The differential diagnosis also sometimes includes lymphoma, malignant histiocytosis, and monocytic leukemia. [67] The differential diagnoses may also include granulomatous inflammatory diseases, inflammatory idiopathic pseudotumors. [15] In the presence of numerous plasma cells, as in our case, the pathological differential diagnosis may also include IgG4-related disease.…”

“…[2] Orbit and ocular glove involvement have been reported, usually as a retro-orbital mass and proptosis. [67] We report a case of extranodal RDD in a 53-year-old man who presented with right orbital mass.…”

Extranodal Intracranial Rosai-Dorfman-Destombes Disease in Children: a Literature Review

“…Surgical treatment of RDD is indicated for extranodal forms of disease that are affecting vital structures; however, remissions with surgery alone have been reported in CNS-only disease [14,30]. Total resection of the intracranial lesion may be curative [3,20]. The majority of patients remain disease-free for prolonged periods of time [14].…”

“…There are only few cases of extranodal intracranial disease described in pediatric population so far. Recently intracranial RDD in pediatric patient was also successfully treated in our country [20]. We aimed to provide updated comprehensive review of reported extranodal intracranial RDD in children.…”