Localized Primary Extranodal Hodgkin's Disease

Cases with extranodal Hodgkin's disease located in sites outside those defined as lymphatic structure are quite rare. Even rarer are cases with disease completely limited to those extranodal sites. The exact incidence of this localized form of Hodgkin's disease is unknown, but it is estimated to be one fourth of 1% of the overall incidence of Hodgkin's disease. World literature was reviewed for well-documented cases of localized, primary extranodal Hodgkin's disease, to define its prognosis. Localized primary extranodal Hodgkin's disease seems to have an excellent prognosis in some cases, including skin, tongue, lung, stomach, small bowel, and thyroid. Data are insufficient for specific conclusions about most other areas of localized Hodgkin's disease. Hodgkin's disease can arise and may be localized in surprisingly diverse sites and may well be related to small fossi of lymphatic tissue within these extralymphatic organs that undergo malignant transformation.