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OBJECTIVE. We present the largest case series in the English-language imaging literature, emphasizing the variety of presentations of Rosai-Dorfman disease in the head and neck.CONCLUSION. Rosai-Dorfman disease is a rare disorder with multiple sites of involvement in the head and neck.

Patients with systemic leukemia, especially the acute leukemias, are at an increased risk of developing disease of the CNS or head and neck. Such disease may take the form of direct leukemic involvement, may result from underlying leukemic effects on the immune or hematopoietic systems, or may result from antileukemic therapy. This review presents the imaging and clinical features of the spectrum of CNS and head and neck disease that may be encountered in patients with systemic leukemia. An understanding of the role of radiology in the management of these patients is important to the practicing radiologist who is confronted with a leukemic patient presenting with signs and symptoms of neurologic dysfunction.

Modern imaging techniques play a vital role in the diagnostic evaluation and follow-up of patients with neoplastic disease affecting the skull base. Many of these lesions have a high rate of recurrence if surgical removal is not complete. Newer, more aggressive surgical approaches can have a maximal effect only if the full extent of a lesion is known preoperatively. CT and MR imaging provide the surgeon with detailed information about every site of tumor involvement. Although these lesions are not common in clinical radiologic practice, the radiologist must be prepared to offer a reasonable differential diagnosis and a full evaluation of the extent of disease. This review presents some of the more commonly encountered tumors that can affect the skull base and describes their radiologic features, with emphasis on CT and MR imaging.