Central nervous system Rosai-Dorfman disease: a diagnostic challenge
Italy 
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11 slide(s) – English – 2011-09-10
Introduction. Rosai-Dorfman disease, or “sinus histiocytosis with massive lymphadenopathy”, is a rare, benign, idiopathic histiocytic proliferative disease with patognomonic histological and immunohistochemical features. Central nervous system (CNS) involvement may be mistaken with a variety of other conditions.
Case report. A 37 year-old man presented with indolent enlarged lymphnodes in the left sub-mandibular region, low-grade fever, increased erythrocyte sedimentation rate (ESR) and intense headache. A cerebral MRI showed a left meningeal occipital-polar mass, which appeared thickened, with a little portion partly eroding the skull bone, with an intense and homogeneous enhancement after Gadolinium. Histological examination oriented towards a meningoencephalitis due to abundant lymphoplasmacellular infiltrate, vascular and meningeal proliferation. 6 months later, an MRI showed a new extra-assial mass, isointense in T1, hypointense in T2, associated to meningeal thickening and intense enhancement after Gadolinium at the medial side of the surgical breach. Over two months, a right lateral homonymous hemianopsia appeared in spite of corticotherapy introduction (methilprednisolone 500 mg/die i.v. for 5 days and oral prednisone 50 mg/die). A new histological examination showed histiocytes with characteristic CD68 and S100 positivity and emperipolesis phenomena, lymphocytes, plasma cells, and a fibrous web due to fibroblastic reaction, consistent with the diagnosis of Rosai-Dorfman disease. Chemotherapy with weekly vinblastine was initiated, with a striking clinical and radiological improvement.
Discussion. Central nervous system involvement in Rosai-Dorfman disease is extremely rare, with a total of 111 cases recognized up to now. On MRI, it typically present as a meningioma-like, extraparenchymal, dural-based mass localised at the convexity or the skull base. Pathogenesis is unknown. The clinical course may range from benign steroid-responsive cases to more aggressive ones, as occurred in our case. Interestingly, a near complete response was achieved with vinblastine-based chemotherapy, which could support the hypothesis of a proliferative component and can be considered a valid therapeutic alternative.
Conclusion. A high index of suspicion is needed in this rare disease, in order to formulate a timely diagnosis which in turn may lead to an effective treatment.
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