Artikel
Rosai-Dorfman disease involving the cranial base, paranasal sinuses and spinal cord
Rosai-Dorfman-Erkrankung. Fallbeispiel mit Raumforderungen im Bereich der Schädelbasis, Spinalkanal und den Nasennebenhöhlen
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Autoren
Veröffentlicht: | 4. Mai 2005 |
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Gliederung
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Objective
Rosai-Dorfman disease is a lymphoproliferative disorder that rarely involves the central nervous system. We present an uncommon case of an extensive Rosai-Dorfman disease with co-existing involvement of the cranial base, the paranasal sinuses and the spinal cord and discuss the differential diagnosis and the therapeutic strategy in such cases.
Methods
A 32-year-old man was admitted to hospital presenting with a one-month history of chronic nasal obstruction and a two-weeks history of left trigeminal pain. Radiological investigation showed an extensive process involving the petroclival region, cavernous sinuses, suprasellar region, anterior cranial fossa, paranasal sinuses, and nasal cavity. Two months before admission, a successful removal of a spinal lesion was performed. The patient underwent a transnasal biopsy and partial resection of the frontobasal lesion. Histological examination was that of Rosai-Dorfman disease.
Conclusions
Rosai-Dorfman lesions may be dura-based and located in the skull base. As these lesions may mimic meningiomas as well as other disorders clinically and radiologically, Rosai-Dorfman disease should be included in the differential diagnosis of extensive lesions involving the intracranial and spinal compartments, as meningiomatosis and inflammatory diseases. In these cases the management of such disorders may be challenging due to the extensive involvement of the cranial base and the neurovascular structures by these lesions.