Document 0462
 DOCN  M9490462
 TI    Pathology with clinical correlations of primary central nervous system
       non-Hodgkin's lymphoma. The Massachusetts General Hospital experience
       1958-1989.
 DT    9411
 AU    Miller DC; Hochberg FH; Harris NL; Gruber ML; Louis DN; Cohen H;
       Department of Pathology, Rita and Stanley Kaplan Comprehensive; Cancer
       Center, New York University Medical Center, New York; 10016.
 SO    Cancer. 1994 Aug 15;74(4):1383-97. Unique Identifier : AIDSLINE
       MED/94332795
 AB    BACKGROUND. Primary central nervous system non-Hodgkin's lymphoma
       (NHL-CNS) is an enigmatic disease of uncertain origin. At the
       Massachusetts General Hospital, 104 patients with NHL-CNS were seen from
       1958 through 1989. An impression of changes in the frequency of
       diagnosis, character of the tumors, and therapy for this disease
       prompted this study of the pathologic features, clinical data, and
       natural history of this tumor in these 104 patients. METHODS. Histologic
       slides (neurosurgical specimens and autopsy tissues) were available for
       99 patients. The tumors were classified by the Working Formulation
       classification. Immunostaining data and all clinical data were retrieved
       from the relevant offices and hospital charts. RESULTS. Primary central
       nervous system non-Hodgkin's lymphoma tripled in frequency (5.66 cases
       per year in 1978-89 versus 1.75 cases per year in 1958-77) and now
       represents 6.6% of all primary brain neoplasms (versus 3.3% before 1978;
       chi 2 = 17.52, P < 0.01). For the 99 tumors histologically classified,
       89% were high grade. Intermediate grade lymphomas, once the second most
       common subtype, have disappeared since 1983. All tumors had diffuse
       architecture; 77% (including all 11 patients with acquired immune
       deficiency syndrome) were large cell subtypes. Two cases were
       intravascular lymphoma. With one exception, all of the 41 tumors
       evaluated were B-cell types; 32 of 40 had monotypic surface
       immunoglobulin. There was 1 T-cell lymphoma. Of 64 tumor recurrences, 29
       were at the initially defined site; 12 were in the leptomeninges, 29
       were in other sites in the neuraxis, and 8 were in systemic sites.
       Systemic metastases have not occurred since 1984. Median survival for
       the 68 patients who survived after diagnostic surgery and for whom
       follow-up information could be obtained was 19 months; 9 months for
       those with high grade tumors and 30.5 months for those with intermediate
       grade tumors. This difference was not significant (P = 0.13). A separate
       set of seven patients had focal tumorlike lymphoid infiltrates composed
       of benign-appearing lymphocytes, which were associated with good long
       term survival. The differential histologic diagnosis of NHL-CNS was
       occasionally difficult, and the spectrum of this differential was
       broader than generally stated. CONCLUSIONS. Primary central nervous
       system non-Hodgkin's lymphoma has increased in frequency even in
       nonimunocompromised patient populations. This increase has been
       accompanied by the disappearance of intermediate grade histologic types,
       suggesting a fundamental shift in the biology of the neoplasms. The
       introduction of chemotherapeutic regimens appears to have altered the
       natural history such that systemic metastases outside the central
       nervous system no longer occur, and there are now some long term
       survivors of this formerly uniformly fatal disease.
 DE    Brain Neoplasms/PATHOLOGY  Central Nervous System
       Neoplasms/CLASSIFICATION/EPIDEMIOLOGY/  *PATHOLOGY  Combined Modality
       Therapy  Diagnosis, Differential  Female  Follow-Up Studies  Human
       Immunocompromised Host  Incidence  Lymphoma,
       AIDS-Related/EPIDEMIOLOGY/PATHOLOGY  Lymphoma,
       High-Grade/EPIDEMIOLOGY/PATHOLOGY  Lymphoma,
       Large-Cell/EPIDEMIOLOGY/PATHOLOGY  Lymphoma, Large-Cell,
       Immunoblastic/EPIDEMIOLOGY/PATHOLOGY  Lymphoma,
       Non-Hodgkin's/CLASSIFICATION/EPIDEMIOLOGY/*PATHOLOGY  Male
       Massachusetts/EPIDEMIOLOGY  Meningeal Neoplasms/PATHOLOGY  Middle Age
       Neoplasm Recurrence, Local/PATHOLOGY  Survival Rate  Treatment Outcome
       JOURNAL ARTICLE

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).