Summary
Hodgkin lymphoma (HL) is a malignant tumor characterized by pleomorphic lymphocytic and histiocytic infiltrate with multinucleated Reed-Sternberg cells. Childhood cancers represent only 2% of neoplasms affecting the general population and HL accounts for 5.5% of all pediatric cancers. The primary nodal site of HL is situated above the diaphragm in two-thirds of patients. The usual clinical presentation consists of painless cervical or supraclavicular adenopathy. Splenomegaly and hepatomegaly often indicate advanced disease. Systemic symptoms are typical of B symptoms, including fever, weight loss, and night sweats. Epstein-Barr Virus is likely to be pathologic agent in a HL subgroup, although it is not a necessary factor for the development of HL or supraclavicular adenopathy. Currently, almost all children receive combined chemotherapy with low dose irradiation (1500 - 2000 cGy) solely in the initially involved area. Overall survival is more than 90%. New therapeutic strategies targeting molecular mechanisms in HL are currently emerging. Children with HL should be referred to multidisciplinary teams in pediatric oncology units with experience in treating pediatric cancers. *Author: Dr J. Sánchez de Toledo (October 2004)*.
