Key result
Endoscopic resection improves facial paralysis but shows 6-month tumor recurrence in intracranial Rosai-Dorfman disease.
Why the study?
Intrasellar Rosai-Dorfman disease is extremely rare and easily confused with pituitary adenoma, presenting diagnostic challenges and management questions.
Case Report (n=1)
No
Intrasellar Rosai-Dorfman disease can mimic pituitary adenoma and present with facial paralysis, requiring surgical resection for definitive diagnosis and close follow-up due to recurrence risk.
Consider Rosai-Dorfman disease in sellar masses mimicking adenoma with facial paralysis requiring surgery for diagnosis and close follow-up; hypothesis-generating for recurrence risk after endoscopic resection.
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is an extremely rare non-Langerhans cell histiocytosis. The diagnostic histiocytes in RDD are characterized by the immunophenotypic markers S100 (+), CD68 (+), and CD1a (−) (Abla O, Blood 131: 2877, 2018),(Emile JF, Blood 127: 2672, 2016) , and exhibit phagocytosis with varying frequency. To our knowledge, this is the first reported case of isolated Intracranial Rosai-Dorfman Disease Mimicking Sellar Pituitary Adenoma, with peripheral facial paralysis as the initial presentation. By integrating imaging findings, histopathological features, and treatment response, we explore the diagnostic challenges and management strategies, aiming to enhance clinicians’ awareness of RDD involving the pituitary. We report the case of a 50-year-old Han Chinese female who presented with left-side peripheral facial paralysis and tender enlargement of the left cervical lymph nodes. Pituitary MRI revealed an intrasellar mass with homogeneous enhancement, initially diagnosed as a pituitary adenoma. The patient underwent transnasal transsphenoidal endoscopic resection of the pituitary adenoma. But histopathology and immunohistochemistry (S100+, CD68+, CD1a−) confirmed the diagnosis of RDD. Postoperatively, the facial paralysis improved, Unfortunately, 6 months follow-up MRI showed tumor recurrence. The clinical manifestations of intrasellar RDD are insidious and lack specificity, making it easy to confuse with common pituitary space-occupying lesions. Currently, surgical resection remains the gold standard for definitive diagnosis and is the primary therapeutic intervention, long-term follow-up is crucial for monitoring recurrence.
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Huang et al. (2026) conducted a case report in 50-year-old Han Chinese female with isolated intracranial Rosai-Dorfman Disease mimicking sellar pituitary adenoma presenting with left peripheral facial paralysis (n=1). Neuroendoscopic endonasal transsphenoidal surgical resection was evaluated on Tumor recurrence assessed by MRI at 6 months post-surgery. Neuroendoscopic endonasal transsphenoidal resection improved facial paralysis but tumor recurrence was observed by MRI at 6 months in a patient with isolated intracranial Rosai-Dorfman disease mimicking pituitary adenoma.
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