The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).

The available information on treatment of sinus histiocytosis with massive lymphadenopathy (SHML) was reviewed. Treatment is not necessary in most instances, but some patients may require surgery, radiation therapy, and/or chemotherapy because of severe disease manifestations. An ideal treatment has yet to be identified. Of the various chemotherapeutic regimens that have been tried so far, a combination of vinca alkaloid, alkylating agent, and corticosteroid appears to be the most effective.