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1994-09-19
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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).