Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): report of a case with widespread nodal and extra nodal dissemination

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Abstract
A fatal case of sinus histiocytosis with massive lymphadenopathy (SHML) is described in a male patient who presented at 11 yr of age with swelling of the malar region and nasal obstruction due to the disease and who died at age 28 in uremic coma following renal involvement. At autopsy SHML tissue extended into the hilum of both lungs and from the retroperitoneal tissue into the pancreas and kidneys. The appearances suggest that the extension of the disease into these organs may have been due to retrograde lymphatic spread from involved lymph nodes. Replacement of the atypical sinus histiocyte by fibrous tissue was seen in the cervical lymph nodes and this may be the usual mode of resolution of the lesion. The nature of SHML is discussed in relation to a possible infectious etiology and defects in the host immune system. The term massive lymphadenopathy is not applicable to all cases and sinus histiocytosis is not appropriate for the now frequently recognized extranodal manifestations of the disease. It is proposed that the term lymphophagocytic histiocytosis syndrome should be adopted or that the eponymous title Rosai-Dorfman disease should be used.