Malignant lymphoma of the gastrointestinal tract and mesentery. A clinico-pathologic study of the significance of histologic classification. NHL Study Group of the Comprehensive Cancer Center West.

A series of 92 malignant lymphomas of the gastrointestinal tract and mesentery obtained from a population-based registry was studied to assess whether the newly defined concept of mucosa-associated lymphoma has clinical relevance. The cases were grouped according to localization; gastric, intestinal, and mesenteric lymphoma. All cases were reviewed histologically, graded according to the Working Formulation, and reclassified according to the Kiel classification, which was modified to include the categories low- and high-grade mucosa-associated lymphoma. Clinical data, as well as staging and follow-up data, were related to both the original diagnosis and the diagnosis after reclassification. The results showed that the distribution of the types of lymphoma is related to site: centroblastic lymphoma was predominant in the stomach, lymphoblastic in the bowel, and follicular centroblastic-centrocytic in the mesentery. Gastrointestinal lymphoma was disseminated in approximately 50% of the patients at presentation. Survival analysis revealed that classification according to the original Kiel classification and grading according to the Working Formulation provided important prognostic information, whereas introduction of mucosa-associated lymphoma as an entity did not. It was concluded that modification of current classifications to include a separate category for mucosa-associated lymphoma is not useful.

Axonal dystrophy in a case of connatal thalamic and brain stem degeneration.

A neonate with a rapidly fatal disease characterized by connatal hypertonia and arthrogryposis multiplex is described. Neuropathological investigations revealed bilateral thalamus and brain stem degeneration, axonal degeneration of pyramidal and other tracts in the spinal cord, and axonal spheroids in areas of origin of lower motor neurons and in the brain stem reticular substance. Congenital thalamic and brain stem degeneration is generally assumed to be the result of intrauterine asphyxia. The widespread occurrence of axonal spheroids in the present neonate points to the possibility of a genetic or toxic origin for at least some of these cases.