Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy

Abstract

Sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman. The typical clinical characteristics of this disease include painless cervical lymphadenopathy, fever, and weight loss. The condition can present with an extranodal mass in about 25% of patients, and isolated masses without lymph node involvement occur rarely. The authors describe a 5-year-old boy with cavernous sinus syndrome due to an isolated extranodal form of sinus histiocytosis with massive lymphadenopathy in the temporal fossa. Several cases of this disease involving the central nervous system are reviewed. The histopathological and magnetic resonance imaging characteristics are discussed.

Spinal epidural Rosai–Dorfman disease preceding by relapsing uveitis: a case report with literature review

“…Extranodal RDD, including central nervous system, has been noted to have fewer typical histiocytes and less evidence of emperipolesis. 6,7 The histiocytes in this case are positive for the dendritic cell-associated protein, S-100.…”

Isolated intracranial rosai-dorfman disease: report of 2 cases and a review of the literature

“…Seizures were among the symptoms in that case and were the only symptom at presentation for our second case. Massive and painless cervical lymphadenopathy was noticed in 90% of reported cases (35). None of our patients had lymphadenopathy.…”

“…Isolated central nervous system (CNS) involvement is extremely rare (2,6,4,10,13,15, [17][18][19][21][22][23]28,33,35,36,38,46). In a large series of RDD including 200 cases there were only 3 cases with intracranial manifestations (9).…”

“…The average age of patients at admission is in the second decade. Only one patient at the young age of 5-years was treated for clinical manifestations of RDD (35). Clinically, patients with intracranial RDD usually present with headaches, seizures, numbness, and paraplegia (4).…”

Isolated Intracranial Rosai-Dorfman Disease Without Dural Attachment -Case Report-

“…20) Moreover, some patients received corticosteroid treatment, anticancer agents, or radiotherapy, because of aggressive courses. 5,8,10,25) The mortality of patients with intracranial Rosai-Dorfman disease is approximately 7%. 14) Low dose radiotherapy led to resolution of the residual mass after surgical resection.…”