gms | German Medical Science

Objective: Sinus histiocytosis with massive lymphadenopathy is a synonym for the Rosai-Dorfman disease. It describes a benign, non Langerhans-cell, histiocytic proliferative disorder that primarily affects the lymph nodes. This case presents a rare isolated extranodal involvement of the central nervous system in the context of this disease. So far over fifty cases have been reported.

Methods: A thirty-nine year-old male had been suffering from retroorbital headache for four months, additionally from left-sided hypoacusis as well as from hypoesthesia in the region of the left tongue, palate, and lip. A cranial MRI showed a contrast enhancing hyperdense, meningioma-like petroclival tumor with a spatial extension from the foramen magnum to the optic chiasm with compression of the brain stem. Besides, two further supratentorial lesions were found. The radiological preoperative diagnosis was a petroclival meningioma.

Results: With the suspected diagnosis of meningioma the patient underwent surgery with total mass resection. According to the first histopathology the tumor was described as a lymphoplasmocytic meningioma WHO I°. Reference pathology on the basis of a recurrence after eighteen months initially stated a chronic reactive inflammation and in the end came up with the diagnosis of a non-Langerhans-cell histiocytosis of the Rosai-Dorfman type. It showed a dense infiltrate of lymphocytes, plasma cells, and histiocytes. Neither eosinophilic granulomas nor Langerhans giant cells were seen. The patient underwent a staging for other typical localizations like the lymph nodes, the skin, the upper respiratory tract or the orbit, but without any findings. A standard therapeutic concept does not exist, but after initial surgery corticosteroids, antimetabolites like vinblastin or methotrexate, and interferon alpha may be applied. The latest drug under investigation is imatinib.

Conclusions: Rosai-Dorfman histiocytosis belongs to the rheumatic disease spectrum and is altogether rarely seen, especially in this isolated intracranial variant. Its ability to radiologically mimic meningioma should put this disease into the list of differential diagnoses.