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May 2002: 38-year-old man and 69-year-old woman with dural based masses
Monika A Wrzolek1, David Zagzag
1Staten Island University Hospital, USA.
Abstract:
The May 2002 COM. A 38-year-old man presented with new onset seizures and a 69-year-old woman presented with bilateral headaches and episodes of syncope. Both were found to have extra-axial masses that were contrast-enhancing and thought to be meningiomas. Both had complete resection. Microscopic examination revealed an inflammatory lesion composed of plasma cells, scattered lymphocytes and numerous large histocytic cells, which exhibited emperi polesis and were CD1 a negative, but positive for CD68 and S100. The diagnosis of Destombes-Rosai-Dorfman Disease (DRDD) was rendered. Both cases had good long-term outcome. The differential diagnosis of inflammatory masses in the dura (plasmacytoma, lymphomas, plasma cell fibroma, angiofollicular hyperplasia [Castleman's-disease] and Langerhan's cell histiocytosis) are discussed.
Insights
Destombes-Rosai-Dorfman Disease (DRDD) can mimic meningiomas in the brain, presenting with symptoms like seizures and headaches. Early diagnosis and complete resection lead to good long-term outcomes for patients with this rare inflammatory condition.
Area of Science:
- Neurology
- Pathology
- Oncology
Background:
- Destombes-Rosai-Dorfman Disease (DRDD) is a rare, non-Langerhans cell histiocytosis characterized by the proliferation of histiocytes.
- Intracranial involvement, particularly in the dura mater, is uncommon but can present as extra-axial masses.
- Distinguishing DRDD from more common dural tumors like meningiomas is crucial for appropriate management.
Observation:
- Two patients, a 38-year-old man and a 69-year-old woman, presented with neurological symptoms suggestive of intracranial masses.
- Both patients were found to have contrast-enhancing extra-axial masses initially suspected to be meningiomas.
- Post-surgical histopathological examination revealed inflammatory lesions with characteristic histiocytic cells.
Findings:
- Microscopic analysis identified plasma cells, lymphocytes, and large histiocytic cells exhibiting emperipolesis.
- Immunohistochemistry showed these histiocytic cells were CD1a negative but positive for CD68 and S100.
- The final diagnosis for both cases was Destombes-Rosai-Dorfman Disease (DRDD).
Implications:
- This study highlights the importance of considering DRDD in the differential diagnosis of inflammatory dural masses, even when they mimic meningiomas.
- Complete surgical resection of DRDD masses appears to be an effective treatment, leading to favorable long-term outcomes.
- Accurate histopathological and immunohistochemical evaluation is essential for differentiating DRDD from other dural pathologies.