Working Formulation

Abstract: Since the Rye classification for staging was produced in 1965, the significance of 2 important observations with major impact on staging has been appreciated. First, extralymphatic disease, if localized and related to adjacent lymph node disease, does not adversely affect the survival of patients. Patients with localized extralymphatic disease do as well as comparable patients of the same stage without extralymphatic spread. Secondly, laparotomy with splenectomy has been introduced as a method of obtaining more information on disease extent in the abdominal region. Thus, it has become necessary to reconsider the Rye classification and to recommend a modified scheme. Staging has 2 aims. The first is to facilitate communication and exchange information. This can be done only at the expense of a loss of some information, as it is necessary to condense in one number a considerable amount of data. Furthermore, intercomparison demands that all the staging procedures performed should be as similar as possible in each center to avoid bias in staging and interpretation of the therapeutic results. The second aim is to provide guidance of prognosis and to assist in therapeutic decisions. This latter aim is best achieved when the greatest amount of information is collected for each patient. It has been recognized that a single staging procedure cannot achieve these 2 purposes. For instance, it is obvious that laparotomy and splenectomy provide much information, but these procedures cannot yet be recommended for every patient. A staging classification based on information obtained by histopathological examination of the spleen and lymph nodes obtained at laparotomy cannot be compared with another done without such vigorous exploration. As a result, unless these factors are taken into account, either intercomparisons of therapeutic results become virtually impossible or much valuable information is excluded from the staging method. Therefore, 2 systems of classifications are presented. Clinical staging (CS). while recognized as incomplete, is easily performed and should be reproducible from one center to another. The second, called pathological staging (PS), takes into account all the extrapathological data obtained from vigorous staging procedures and has a higher degree of precision but is restricted in its application to relatively few centers.

Abstract: Burkitt's lymphoma is a highly aggressive lymphoma identified and described in the last century by Denis Burkitt in Africa, in areas endemic for malaria. Since its description in African children, it has been recognized outside areas with endemic malaria, frequently also in children as well as among individuals with an underlying immunodeficiency. Since its initial designation as Burkitt's lymphoma, this type of lymphoma and lymphomas closely resembling it have received a variety of names in different classifications of lymphomas and leukemias: undifferentiated lymphoma, Burkitt's and non-Burkitt's type in the modified Rappaport Classification, malignant lymphoma, small non-cleaved cell, Burkitt's type in the Working Formulation, Burkitt's lymphoma and high-grade B-cell lymphoma, Burkitt-like in the REAL Classification, and acute lymphoblastic leukemia, L3 type in the FAB Classification. With the publication of the WHO Classification of Haematopoietic and Lymphoid Tumors, the nomenclature of this lymphoma has come full circle, and it is once again known simply as Burkitt's lymphoma. In recent years, efforts have focused on improving therapy for this rapidly proliferating neoplasm while minimizing, to the extent possible, treatment-associated toxicity. These efforts have led to the development of high-intensity, short-duration combination chemotherapy that has proven extremely effective for a high proportion of Burkitt's lymphoma patients. The differential diagnosis of Burkitt's lymphoma is broad, and precise diagnosis based on histologic, immunophenotypic, and genetic features remains the critical first step in planning appropriate therapy.

Abstract: Background. Significant advances in the understanding of non-Hodgkin's lymphoma (NHL) have occurred in the past two decades, resulting in changes in terminology and classification practices, which may affect the analysis of the Surveillance, Epidemiology, and End Results (SEER) data. Methods. The incidence rates for each subgroup of NHL in the Working Formulation were reviewed from the National Cancer Institute's SEER data during three time periods: 1973-1977, 1978-1982, and 1983-1987. Results. NHL has significantly increased by 50% from 1973 to 1988. The most frequently occurring NHL is diffuse large cell lymphoma, constituting nearly 30% of all lymphomas. Increased incidence rates for large cell immunoblastic and small noncleaved cell NHL observed during the 1980s are attributable largely to the acquired immunodeficiency syndrome epidemic. Exposure to environmental agents such as herbicides and hair coloring dyes have recently been identified as factors that increase the risk of NHL, but the overall contribution of these factors remains to be identified. The increase in extranodal lymphomas is in part a consequence of the application of modern immunophenotypic and genotypic methods, which lead to the reclassification of pseudolymphomas as monoclonal B-cell neoplasms. The apparent dramatic decline in the incidence of diffuse small cleaved NHL appears to be artifactual, as well, secondary to changes in classification of NHL. Conclusions. With the enhanced ability of pathologists to delineate new clinicopathologic entities by immunophenotypic and molecular biologic studies, future modifications to the collection of SEER data may be appropriate. Such an approach will address the limitations of the Working Formulation and lead to a more accurate data base for the evaluation of epidemiologic trends.