Recurrent intracranial Rosai-Dorfman disease: Management of a challenging case

Abstract

Rosai-Dorfman disease (RDD) is a rare, idiopathic, benign histioproliferative disorder. Extranodal involvement is seen in around 25–40% of patients. Central nervous system manifestation of RDD is uncommon and suprasellar location of the lesion is a distinct rarity. Surgery is the cornerstone of management of intracranial RDD. However, tumor recurrence or regrowth is a potential problem. Hence, low dose conformal radiotherapy (RT) should be considered in patients undergoing sub-total resection or having unresectable recurrent disease. Though cranial RT usually leads to satisfactory improvement of symptoms and long-term disease stabilization or regression, in few patients there may be an eventual progression of disease for which systemic chemotherapy may be considered. We have highlighted the salient features of this enigmatic disease by citing a case of a 50-year-old male patient with suprasellar RDD treated by maximal safe surgery and deferred radiation therapy on progression.

Staging surgery for intraventricular bilateral giant Rosai–Dorfman disease in children

“…The large size of the lesions leads us to believe that the hemorrhage may be caused by a rupture of proliferating blood vessels. 3,5,10 Zhang et al 18 also reported a case of RDD with necrosis and calcification on CT scans. 18 Hemorrhage, necrosis, and calcification on CT scans cannot help rule out the diagnosis of RDD.…”

“…Complete excision is ideal, because no recurrence after complete resection has been reported in intracranial RDD, while the recurrence rate with subtotal resection is about 14%. 3,8 The curative effects of postoperative radiotherapy, chemotherapy, or steroid for RDD vary individually and they all have certain side effects. [1][2][3][4][5]7,12,20,[22][23][24][25][26] We therefore recommend active follow-up with MRI.…”

“…elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. [3][4][5] About 25%-40% of patients have extranodal involvement, and those with intracranial lesions constitute approximately 5% of all RDD cases. [1][2][3][4]6 Most of these cases are associated with the endocranium or skull base and the condition is usually misdiagnosed as meningioma.…”

Intracranial Rosai-Dorfman Disease: pathophysiology, diagnosis and treatment

“…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] . Therefore patients after surgical treatment should be clinically and radiologically observed.…”

“…This non-Langerhans histiocytosis is a rare disorder with a prevalence about 1:200,000 people [2] . 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] .…”