Faculty Bibliography

Rosai-Dorfman disease is a rare disorder of unknown etiology caused by the proliferation of histiocytes within lymph nodes, predominantly in the cervical chain. Extranodal sites may be involved as well, with involvement of nearly every organ system described in the literature. Abdominal and pelvic manifestations of Rosai-Dorfman disease are extremely rare. We present four cases of abdominal and pelvic involvement by Rosai-Dorfman disease, specifically within the biliary tract, pancreas, spleen, and presacral space. Of note, a presacral mass was present in three of the four patients, and although not previously reported in Rosai-Dorfman disease, may suggest the diagnosis in the proper clinical scenario.

Since the recognition of the anaplastic large-cell lymphomas in the 1980s, much has been learned about the diagnosis, clinical presentation, and treatment of these malignant conditions. The systemic and primary cutaneous types of anaplastic large cell lymphomas have been differentiated on clinical and immunophenotypical findings, but further research is required to elucidate their exact etiologies and pathogeneses. Primary cutaneous anaplastic large-cell lymphoma has a 95% disease-specific 5-year survival, owing partly to the relatively benign course of the disease and partly to the variety of effective treatments that are available. As with many other oncological diseases, new drugs are continually being tested and developed, with immunotherapy and biological response modifiers showing promise.