Intracranial Rosai-dorfman Disease: a Report of Seven Cases with Review of Literature

Review Article

Austin J Clin Pathol. 2014;1(3): 1013.

Intracranial Rosai-dorfman Disease: a Report of Seven Cases with Review of Literature

Qiuping Gui*, Fang Li and Xin Song

Department of Pathology, General PLA Hospital, China

*Corresponding author: Qiuping Gui, Department of Pathology, General PLA Hospital, Central Hospital of China Aerospace Corporation, 28# Fuxing Rd, Haidian District, Beijing 100853, China

Received: June 20, 2014; Accepted: July 09, 2014; Published: July 10, 2014


Introduction: Rosai–Dorfman disease (RDD) commonly involves cervical lymph nodes. Although extranodal involvement has been reported in diverse sites, the central nervous system (CNS) manifestation, particularly in the absence of nodal disease is uncommon.

Methods: Specimens from 7 patients with intracranial RDD, diagnosed between 2004 and 2012, were analyzed using clinicopathological and immunohistochemical methods.

Results: These cases analyzed in the present study including six men and one woman, ranging in age from 4 to 60 years, mean age was 35.42 years. These patients presented with headaches, seizures, numbness, or fever. The lesions were multifocal or solitary in the intracranial area. Histologically, the lesions consisted of variable numbers of pale-staining histocytes with emperipolesis. By immunohistochemical analysis, the characteristic histiocytes were positive for S-100 protein and CD68 and negative for CD1a. Treatment consisted of surgical excision, radiotherapy and steroids administration. Follow-up available for 7 cases with intervals ranging from 3 months to 6 years (mean: 15 mo), disclosed one patient recurrence of operative two years after diagnosis and six patients with no evidence of disease progression.

Conclusion: Intracranial RDD is rare, the diagnosis of CNS RDD is challenging. Pathologists should include RDD in the differential diagnosis of dural-based lesions that clinically and radiologically resemble meningiomas and other histiocytosis and granulomatous disease. Therapy remains controversial, but surgical removal of CNS lesions is an effective treatment, and follow-up is necessary to avoid relapses.

Keywords: Rosai-Dorfman disease; Central nervous system; Extranodal sinus histiocytosis with massive lymphadenopathy


Rosai-Dorfman disease (RDD), originally called “sinus histiocytosis with massive lymphadenopathy” (SHML), was first described by Rosai and Dorfman in 1969 as a rare benign histiocytic proliferative disorder [1]. RDD generally presents with massive, painless cervical lymphadenopathy, fever, weight loss, and polyclonal hypergamma globulinemia [2]. Histologically, the disease is characterized by lymph node sinus dilatation and large histiocytes demonstrating emperipolesis [3].

Extranodal sites are involved in 30% to 40% of cases, usually affecting the skin, upper respiratory tract, orbit, and testes. Central nervous system (CNS) involvement is unusual. Isolated CNS RDD without other body involvement is even more exceptional, with fewer than 70 reported cases and the vast majority as single case reports. Because of its rarity and morphologic similarities to other benign or malignant disease, intracranial RDD without concurrent lymphadenopathy can be a diagnostic challenge. In this report, we analyzed the clinicopathologic features of 7 cases of extranodal, intracranial RDD. 4 cases of lesions were extraparenchymal and associated with dural involvement, 2 of isolated intraparenchymal lesion, other one both involved dural-based and intraparenchymal regions.

Materials and Methods

7 cases of intracranial RDD diagnosed between 2004 and 2012 were identified in a search of the General PLA Hospital (Beijing, China) surgical pathology files. One patient of 27 years old male occurring in the sellar region has been previously described by the referring pathologist in a case report [4]. Information about clinical manifestations, neuroimaging examinations and follow-up was obtained by reviewing the medical records or by correspondence with physicians.

Formalin-fixed, paraffin-embedded sections were stained with hematoxylin and eosin for routine histological evaluation. Additional 4-micrometer-thick sections were mounted on electrostatic slides and used for immunohistochemical studies. The slides were stained using the EnVision method (Dako, Glostrup, Denmark), an automated immunostainer (Autostainer, Dako), and commercially available monoclonal and polyclonal antibodies. The primary antibodies against the following antigens were used: glial fibrillary acid protein (GFAP; Dako, Glostrup, Denmark; polyclonal, 1 :200 dilution), S-100 protein (Dako, 1 : 2000 dilution), CD1a (Dako, 1 : 50 dilution), MIB-1 (Dako, 1 :200 dilution), CD3 (Dako, 1 : 400 dilution), CD20 (Dako, 1 :400 dilution); and CD68 (Dako, 1 :100 dilution). Special stains for bacteria and fungi including Ziehl-Neelsen strain, periodic acid- Schiff, and Grocott methenamine silver were also preformed in the study.


Clinical features

The clinical data were summarized in Table 1. There were seven men and one woman, ranging in age from 4 to 60 years (mean 35.4 years). The clinical symptoms included headaches (5/7), seizures (3/7), dizziness (1/7), vomiting (1/7) and fever (1/7). None of the patients demonstrated lymph node involvement at presentation.