Pseudo-Malignant Necrotic Histiocytosis of Lymph Nodes in Children. A Report of 2 Cases 1

Abstract

Dorfman and Warnke (3) have previously described varieties of lymphadenopathy simulating malignant lymphomas, and they have emphasized the diagnostic difficulties encountered. At the Gustave-Roussy Institute, we have observed 2 cases of lymphadenopathy, in young boys, which mimicked lymphoma. No reference to the particular clinical and histological features of this disorder could be found in the literature. The boys, 2 and 3 years of age, presented with massive cervical adenopathy, hepatosplenomegaly and intermittent fever, associated with a chronic nasopharyngitis, anemia and eosinophilia. Specific adenitis could be eliminated on the basis of laboratory data. No definite diagnosis could be established upon microscopic evaluation of the nodes. None of the pathologists, asked in consultation, was able to determine whether the lesion was malignant or not. Under these circumstances, 1 of the patients was treated by methotrexate and steroids, and the other by radiotherapy, neither with complete success. Fever and adenopathy continued off and on over a period of 5 years. However, at 7 years follow-up, both patients are now without evidence of disease. Microscopically, the features of the lymph nodes of these 2 patients were quite similar. Lymphoid tissue was more or less replaced by a diffuse histiocytosis with necrotic areas. Giant cells and macrophages could be found in the vicinity of the necrotic areas. Plasma cells, eosinophils, mast cells and bizarre cells simulating Reed-Sternberg cells were also observed. It was necessary to rule out 4 major diagnoses: Letterer-Siwe disease, sinus histiocytosis of Rosai and Dorfman, malignant histiocytosis, and Hodgkin's disease of the epithelioid type. The possibility of a new entity, which appears to have a self-limited course, must be recognized, if inappropriate use of radical therapy is to be avoided. The etiology of this pseudo-malignant necrotic histiocytosis is not clear and, for the moment, no precise pathogenesis can be proposed.