Involvement of the Central Nervous System in Non-Hodgkin's Lymphoma

Abstract

Fifteen of 146 (10%) adult patients with non-Hodgkin's lymphoma showed clinical and pathologic evidence of involvement of the central nervous system (CNS): in 6 patients, the CNS lymphoma was present at the onset of disease, in 3 of them it was the only sign detected. In the remaining 9 cases, CNS involvement appeared during the course of systemic disease. In all cases symptoms related to infiltration of the CNS were associated with advanced disease (stage IV); bone marrow or bone involvement was found in 9 patients (60%). The histologic subtypes were mostly of high-grade malignancy according to the Kiel classification: immunoblastic (3), centroblastic (3), Burkitt type (2), lymphoblastic (1), LP immunocytoma in polymorphic variant (3), unclassifiable (3). The prominent signs and symptoms of CNS lymphoma are listed: the cranial nerve palsies are the most common finding. The principal means of detecting CNS involvement are discussed: cerebrospinal fluid cytology, brain scan and CAT scan were the most useful diagnostic procedures. The reported data allow identification of patients at high risk of CNS lymphoma: this includes histologies of high-grade malignancy, advanced stage of disease, and bone marrow or bone infiltration. Therefore, either intensive systemic chemotherapy or CNS prophylaxis are recommended for patients with high risk of CNS disease.

Secondary Involvement of the Central Nervous System in Malignant Non-Hodgkin’s Lymphoma

“…1975;Herman et al, 1979;Young et al, 1979], Our results are consistant with those of Johnson and Oken [1984], Isolated CNS relapses were generally followed shortly by a relapse of lymphoma [Bunn et al, 1967], CNS involvement is associated with ad vanced stages of disease [Bunn et al, 1967;Young et al, 1979;Cetto et al, 1981;Harousseau et al, 1983]. In our series.…”

“…63.3% of the patients had stages 111 or IV at the time of lymphoma diagnosis. Bone marrow infiltration was recognized in 50% of patients in our series before or at the time of CNS involvement, 60% in others [Cetto et al, 1981], There is probably a direct spread of lymphoma from the marrow through the dura and into the arachnoid space, just as in acute lymphocytic leukemia [Weil et al, 1973], In 8 patients, visceral parietal involvement could explain a con tiguous extension. So, just as in acute lymphocytic leukemia, some varieties of NHL need prophylactic treatment of CNS.…”

“…But CNS disease usually arises in uncontrolled peripheral lymphomas. Our results and those of Cetto et al [1981] allow precise identification of groups of patients who arc at high risk of develop ing CNS lymphoma. Prophylactic treatment of CNS lymphoma should be employed in unfavorable histol ogy NHL (lymphoblastic and Burkitt type), diffuse undifferentiated lymphoma according to Rappaport's classification [Johnson and Oken, 1984] or more pre cisely in lymphoblastic lymphoma (convoluted and Burkitt types, according to the Kiel classification).…”

“…Intrathecal chemotherapy plus cranial irradiation were used by us and many others, but the results are poor [Cetto et al, 1981;Harousseau et al, 1983;Johnson and Oken, 1984], Death occurred 2.5 and 3.5 months after diagnosis of CNS involvement and half of the patients died of CNS involvement. It is suggest ed that in the high-risk group of patients, prophylac tic treatment could be employed when complete remission has been achieved.…”

Central nervous system‐directed preventative therapy in adults with lymphoma

“…This article will attempt to identify, according to the WHO classification, those groups where specific CNS‐directed therapy to prevent later relapse within the CNS is justified, but it must be stated at the outset that, unfortunately, there are no randomised prospective trials available which specifically address this decision‐making process. CNS relapse of lymphoma may occur rarely in a wide range of different low‐grade histology's, for example the Bing–Neel syndrome reported in lympho‐plasmacytoid lymphoma (Pennacchio & Orlandini, 1969; Cetto et al , 1981), however the incidence of CNS relapse is insufficient to justify prophylactic therapeutic intervention for any of the broad range of low‐grade NHL subtypes. Paediatric NHL and primary CNS lymphoma (including ocular lymphoma) will not be discussed in this annotation.…”