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Year : 2010  |  Volume : 47  |  Issue : 1  |  Page : 80--81

Ethmo-spheno-intracranial Rosai-Dorfman disease

MS Walid1, AA Grigorian2,  
1 Medical Center of Central Georgia, Macon, GA, USA
2 Georgia Neurosurgical Institute, Macon, GA, USA

Correspondence Address:
A A Grigorian
Georgia Neurosurgical Institute, Macon, GA

How to cite this article:
Walid M S, Grigorian A A. Ethmo-spheno-intracranial Rosai-Dorfman disease.Indian J Cancer 2010;47:80-81

How to cite this URL:
Walid M S, Grigorian A A. Ethmo-spheno-intracranial Rosai-Dorfman disease. Indian J Cancer [serial online] 2010 [cited 2022 Jan 28 ];47:80-81
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Rosai-Dorfman Disease, first described in 1969 as Sinus Histiocytosis with Massive Lymphadenopathy, by Rosai and Dorfman, is an idiopathic histoproliferative disorder that manifests itself with lymphadenopathy, systemic symptoms, and hypergammaglobulinemia. [1] The Histiocytosis Association of America estimates that the disease affects one in 200,000 children born each year in the United States. [2] However, from the Foucar, Rosai, and Dorfman 1990 review, the average age of onset of the disease is 20.6 years (423 cases), usually in the third to fifth decades, although the age range can be quite broad ( [3] The central nervous system disease has a mean onset age of 37 years. [3] Neurological manifestations are rare, occurring in 4% of one series. [4] The isolated intracranial form of the Rosai-Dorfman Disease is very rare and may be encountered once in a neurosurgeon's career. We had a 60-year-old African American patient who was admitted from homecare because of altered mental status and bizarre behavior. She had a history of bipolar disease and paranoia. An MRI of the brain showed an enhancing, relatively homogeneous, multilobulated, right frontotemporal, 74 x 51 mm mass [Figure 1]A, extending through the skull base into the right sphenoid sinus [Figure 1]B, involving the left ethmoid air cells, and possibly obstructing the frontal ethmoidal recess on the left [Figure 1]C and D. The mass was noted to completely encase the right orbital apex and the right supraclinoid internal carotid artery. Moderate vasogenic edema, with a right-to-left subfalcine shift of approximately 13 mm, at the level of the foramen of Monroe, was noted. The differential diagnosis included meningioma and brain tumor. The patient had right-sided craniotomy, microscopic dissection, and partial resection of the large frontotemporal tumor, with the assistance of an intraoperative microscope, electrophysiologic monitoring, and a navigational system. The tumor was accessed and the surface of the tumor was coagulated. Multiple specimens were obtained and submitted for frozen section. Although dissection around the tumor was performed, the tumor appeared to be moderately hemorrhagic with good separation from the surrounding brain parenchyma. The frozen section came back reporting the presence of extensive plasmocytes [Figure 2] rather than the presence of typical features of a meningioma. Resection was discontinued, the tumor was coagulated, and meticulous hemostasis was achieved. Additional immunohistochemical stains for CD138 and CD68 demonstrated the majority of the cells to be histiocytes rather than plasma cells. The unequivocal finding of emperipolesis confirmed the diagnosis of "Rosai-Dorfman Disease of the dura." Radiation Oncology was consulted. It was felt that the patient would need to return to the operating room for further debulking of the lesion. The patient was taken back to the operating room. She was subsequently arranged for radiation therapy and discharged.

Rosai-Dorfman Disease of the central nervous system (CNS) is rare. There are around 90 cases described in the literature. [4] The disease can mimic meningioma and can present with seizures. [5] It is important to consider Rosai-Dorfman in the differential diagnosis of meningioma-like lesions. Histiocytosis (Rosai-Dorfman) has historically been treated by oncologists as a malignancy, with chemotherapy and radiation. Unlike cancer, however, histiocytosis may occasionally go into remission without treatment. The optimum treatment has not been established, but some patients treated surgically or with radiation therapy can have long-term improvement. [4]


1Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.
2Disease Information. Histiocytosis Association of America. Accessed 12/04/2008. URL:
3Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 1990;7:19-73.
4Kidd DP, Revesz T, Miller NR. Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement. Neurology 2006;67:1551-5.
5Ghosal N, Murthy G, Visvanathan K, Sridhar M, Hegde AS. Isolated intracranial Rosai Dorfman disease masquerading as meningioma: a case report. Indian J Pathol Microbiol 2007;50:382-4.