Clinicopathological characteristics of multiple intracranial Rosai-Dorfman disease with increased IgG4-positive plasma cells: a report of two cases

Abstract

Rosai-Dorfman disease (RDD) is an uncommon condition characterized by the proliferation of histiocytes and multiple intracranial involvements and it is extremely rare. Here, we present two cases of multiple intracranial RDD mimicking meningioma. These patients underwent surgery for tumour resection and pathological findings revealed an increased number of IgG4-positive plasma cells in RDD. The radiographic appearance and histology may contribute to a diagnostic dilemma, and immunohistochemical and serological examinations are a necessary complement for definitive diagnosis. Treatment protocols pertaining to such types of RDD cases are reviewed. Currently, surgical resection is the most effective therapy, and steroid therapy, radiotherapy, or chemotherapy may be provided as adjuvant treatments in some selected patients.

Intracranial Rosai-Dorfman Disease: pathophysiology, diagnosis and treatment

“…Although RDD is considered a benign condition and almost 50% of cases of sporadic RDD demonstrate spontaneous recovery without application of any treatment, remission is not observed in the case of intracranial RDD [6] . Current therapeutic options for this condition include surgery, radiotherapy, chemotherapy, corticosteroids and immunotherapy.…”

“…It improves neurological symptoms and provides tissue for histopathological diagnosis. Gross-total resection (GTR) can be obtained in the majority of intracranial RDD cases and results in long-term satisfactory outcomes [6] . However, RDD lesions may be located in deep brain structures or surrounded by vital neurovascular structures.…”

“…Thus, GTR is difficult, sometimes even impossible to obtain in these cases [72,73] . Biopsy or subtotal resection performed for such cases significantly increase the risk of RDD recurrence [6] . About 14% of subjects relapse after subtotal resection after about 10 years from surgery [3,64] .…”

“…Different chemotherapeutic schemes were used for the treatment of RDD, although the best therapeutic approach for intracranial involvement of RDD has not been established yet [87] . Chemotherapeutic agents investigated in the literature for RDD treatment include methotrexate, anthracyclines, cytarabine, vinca alkaloids, 6-mercaptopurine, etoposide and 2chlorodeoxyadenosine [6,[88][89][90]. However, their various efficacy raises the question of the use of chemotherapy for RDD treatment.…”

“…Most commonly, RDD occurs during the first or second decade of life and is presented by bilateral painless cervical lymphadenopathy coexisting with fever, elevated erythrocyte sedimentation rate, polyclonal hypergammaglobulinemia, and neutrophilia [3][4][5] . The etiology and pathogenesis of RDD remains unclear [6] . In 43% of cases, RDD is connected with extranodal involvement and refers to skin, cavum sinuses, orbit, bones and upper respiratory tract [7] .…”