Summary
A retrospective histologic study of 145 consecutive autopsy cases of systemic malignant lymphomas (including lymphatic leukemias) was performed. The classification followed the Kiel Classification (Gérard-Marchant et al., 1974). There was an overall secondary CNS involvement in 26.2% of the total or in 30.4% of the non-Hodgkin's lymphomas including ALL, with intracranial lesions in 21.4 and 26.1%, respectively, and spinal epidural spread in 5.5 (5.1)%. Peripheral nerve involvement was seen in almost 40% of the examined cases. Ten further cases were isolated (“primary”) intracranial lymphomas without evidence of extraneural deposits or systemic lymphatic disease. The CNS complications in non-Hodgkin's lymphomas were diffuse meningeal and/or perivascular infiltration with or without invasion of the nervous parenchyma, and did not differ from those in CNS leukemia. Isolated solid mass lesions in the brain were only present in 7% of the secondary CNS lymphomas, but were seen in all instances of “primary” cerebral lymphomas. The incidence of CNS complications was highest in lymphoblastic lymphomas including ALL (39%), CLL (31%), immunocytic lymphoma (29%), less frequent in immunoblastic (18.7%), and centrocytic lymphomas (16.6%). No intracranial lesion was observed in centroblastic-centrocytic and centroblastic lymphomas which only produced epidural spread. Bone marrow involvement was present in 92.8% of the cases with secondary CNS lesions, and in 83.2% of the epidural lymphomas. Leukemic conversion, present in 44% of the total (52% with ALL), was demonstrated in 83.3% of the cases with secondary brain lesions, but was hardly combined with epidural spread. The histologic pattern of CNS lesions in non-Hodgkin's lymphomas and their frequent association with leukemic conversion suggest the importance of hematogenous dissemination rather than of direct spread from bone marrow or local manifestation in multisystem disease. Isolated (“primary”) lymphomas of the CNS which are morphologically identical with the extraneural lymphomas may represent a primary, often lethal manifestation of a multisystem disease with or without secondary generalization.
Similar content being viewed by others
References
Adams, J. H.: The classification of microgliomatosis with particular reference to diffuse microgliomatosis. Acta neuropath. (Berl.), Suppl. VI, 119–123 (1975)
Adams, R. D.: Certain notable clinical attributes of the histiocytic sarcomas of the central nervous system. Acta neuropath. (Berl.), Suppl. VI, 177–180 (1975)
Aur, R. J. A., Simone, J. V., Hustu, H. O., Verzosa, M. S.: A comparative study of central nervous system irradiation and intensive chemotherapy early in remission of childhood acute lymphocytic leukemia. Cancer (Philad.) 29, 381–391 (1972)
Banks, P. M., Arseneau, J. C., Gralnick, H. R., Canellos, G. P., DeVita, V. T., Jr., Berard, C. W.: American Burkitt's lymphoma: A clinicopatholgoic study of 30 cases. Amer. J. Med. 58, 322–329 (1975)
Barnard, R. D., Scott, T.: Patterns of proliferation in cerebral lymphoreticular tumours. Acta neuropath. (Berl.), Suppl. VI, 125–130 (1975)
Barnett, L. B., Schwartz, E.: Cerebral reticulum cell sarcoma after multiple renal transplants. J. Neurol. Neurosurg. Psychiat. 37, 966–970 (1974)
Billingham, M. E., Rawlinson, D. G., Berry, P. F., Kempson, R. L.: The cytodiagnosis of malignant lymphomas and Hodgkin's disease in cerebrospinal, pleural and ascitic fluids. Acta cytol. (Philad.) 19, 547–556 (1975)
Buerger, L. F., Monteleone, P. N.: Leukemic-lymphomatous infiltration of skeletal muscle. Cancer (Philad.) 19, 1416–1422 (1966)
Bunn, P. A., Jr., Schein, P. S., Banks, P. M., DeVita, V. T., Jr.: Central nervous system complications in patients with diffuse histiocytic and undifferentiated lymphoma: leukemia revisited. Blood 47, 3–10 (1976)
Calvo, W., Hoelzer, D.: Involvement of the central nervous system in guinea pigs with L2/NB leukemia. Acta haematol. (Basel) 55, 28–35 (1976)
Clausen, R. E., Lincoln, A. F., Silberman, H. K.: Diffuse lymphosarcomatosis of the central nervous system simulating infectious polyneuritis. Amer. J. Med. 20, 292–300 (1956)
Cravioto, H.: Human and experimental reticulum cell sarcoma (microglioma) of the nervous system. Acta neuropath. (Berl.), Suppl. VI, 135–140 (1975)
Ebels, E. J.: Reticulosarcomas of the brain presenting as butterfly tumors. Europ. Neurol. 8, 333–338 (1972)
Flament-Durand, J., Ketelbant-Balasse, P., Maurus, R., Regnier, R., Spehl, M.: Intracerebral calcifications appearing during the course of acute lymphocytic leukemia treated with methotrexate and X-rays. Cancer (Philad.) 35, 319–325 (1975)
Friedman, M., Kim, T. H., Panahon, A. M.: Spinal cord compression in malignant lymphoma. Cancer (Philad.) 37, 1485–1491 (1976)
Gendelman, S., Rizzo, F., Mones, R. J.: Central nervous complications of leukemic conversion of the lymphomas. Cancer (Philad.) 24, 676–682 (1969)
Gérard-Marchant, R., Hamlin, I., Lennert, K., Rilke, R., Stansfeld, A. G., Van Unnik, J. A. M.: Classification of non-Hodgkin's lymphomas. Lancet 1974 II, 406–408
Gerstenbrand, F., Jellinger, K., Lutz, D., Maida, E.: Zerebrale Beteiligung bei Sézary-Syndrom. J. Neurol. 212, 55–64 1976
Glick, A. D., Leech, J. H., Waldron, J. A., Flexner, J. M., Horn, R. G., Collins, R. D.: Malignant lymphomas of follioular center origin in man. II. Ultrastructural and cytochemical studies. J. nat. Cancer Inst. 54, 23–36 (1975)
Globus, J. H., Meltzer, T.: Metastatic tumors of the brain. Arch. Neurol. Psychiat. (Chic.) 48, 163–226 (1942)
Graham, P. J., Willoughby, M. L.: Leukemia in the nervous system: Factors in pathogenesis. In: C. Pochedly (ed.): Acute childhood leukemia, pp. 59–79. Basel: S. Karger 1975
Griffin, J. W., Thompson, R. W., Mitchinson, J. M., Kiewief, J., Welland, F. H.: Lymphomatous leptomeningitis. Amer. J. Med. 51, 200–208 (1971)
Gunderson, C. H., Henry, J., Malamud, N.: Plasma globulin determination in patients with microglioma. J. Neurosurg. 35, 406–415 (1971)
Henry, J. M., Heffner, R. R., Jr., Dillard, S. H., Earle, K. M., Davis, R. L.: Primary malignant lymphomas of the central nervous system. Cancer (Philad.) 34, 1293–1302 (1974)
Henry, K.: Electron microscopy in the non-Hodgkin's lymphomata. Brit. J. Cancer 31, Suppl. II, 73–93 (1975)
Herdin, B., DeVivo, D. C., Torack, R., Lell, M.-E., Ragab, A. H., Vietti, T. J.: Parenchymatous degeneration of the central nervous system in childhood leukemia. Cancer (Philad.) 33, 468–482 (1974)
Hirano, A., Ghatak, N. R., Becker, N. H., Zimmerman, H. M.: A comparison of the fine structure of small blood vessels in intracranial and retroperitoneal malignant lymphomas. Acta neuropath. (Berl.) 27, 93–104 (1974)
Horvath, B., Peña, C., Fisher, E. R.: Primary reticulum cell sarcoma (microglioma) of the brain. Arch. Path. (Chic.) 87, 609–616 (1969)
Hubert, J. W. A.: Tumours of the reticuloendothelial system. In: P. J. Vinken, G. W. Bruyn (eds.), Handbook of clinical neurology, Vol. 18, pp. 233–267. Amsterdam: North Holland 1975
Hyman, C. B., Bogle, J. M., Brubaker, C. A., Williams, K., Hammon, D.: Central nervous system involvement by leukemia in children. I. Relationship to systemic leukemia and description of clinical and laboratory manifestations. Blood 25, 1–12 (1965)
Ishida, Y.: Fine structure of primary reticulum cell sarcoma of the brain. Acta neuropath. (Berl.), Suppl. VI, 147–153 (1975)
Jänisch, W., Gerlach, H., Remus, I.: Neoplastic involvement of the central nervous system in generalized lymphomas. Acta neuropath. (Berl.), Suppl. VI, 81–84 (1975a)
Jänisch, W., Gerlach, H., Remus, I.: Malignant lymphomas with neoplastic affliction of the brain. Abstr. 5th Congr. Europ. Soc. Path., p. 38, Vienna, Oct. 6–11, 1975 (b)
Janota, I.: Involvement of the nervous system in malignant lymphoma in Nigeria. Brit. J. Cancer 20, 47–61 (1969)
Jellinger, K., Radaszkiewicz, Th.: Befall des Nervensystems bei malignen Lymphomen (abstr.) Blut 28, (1975)
Jellinger, K., Radaszkiewicz, Th., Slowik, F.: Primary malignant lymphomas of the central nervous system in man. Acta neuropath. (Berl.) Suppl. VI, 98–102 (1975 a)
Jellinger, K., Sluga, E., Slowik, F.: Fine structure of primary intracranial malignant lymphomas. Abstr. 51th Ann. Meetg. Amer. Assn. Neuropath., pp. 55–56, May 30–June 1, 1975(b)
John, H. T., Nabarro, J. D. N.: Intracranial manifestation of malignant lymphoma. Brit. J. Cancer 9, 386–400 (1955)
Johnson, P. C.: Ultrastructural study of two central nervous system lymphomas. Acta neuropath. (Berl.), Suppl. VI, 155–160 (1975)
Jones, S. E., Rosenberg, S. A., Kaplan, H. S.: Non-Hodgkin's lymphomas. I. Bone marrow involvement. Cancer (Philad.) 29, 954–967 (1972)
Kernohan, J. W., Uihlein, A.: Sarcomas of the brain. Springfield, Ill.: C. Ch. Thomas 1962
Lambert, C. D., Trewby, P. N.: Microglioma with paraproteinaemia. J. Neurol. Neurosurg. Psychiat. 37, 835–840 (1974)
Law, I. P., Dick, F. R., Blom, J., Bergevin, P. R.: Involvement of the central nervous system in non-Hodgkin's lymphoma. Cancer (Philad.) 36, 225–231 (1975)
Leder, L. D.: Der Blutmonocyt. Berlin-Heidelberg-New York: Springer 1967
Lennert, K.: Morphology and classification of malignant lymphomas and so-called reticuloses. Acta neuropath. (Berl.), Suppl. VI, 1–16 (1975)
Littman, P., Wang, C. C.: Reticulum cell sarcoma of the brain. A review of the literature and a study of 19 cases. Cancer (Philad.) 35, 1412–1420 (1975)
Ljungdahl, I., Strang, R. R., Tovi, D.: Intracerebral Hodgkin's granuloma. Report of a case and review of the literature. Neurochirurgia (Stuttg.) 12, 113–118 (1965)
Magrath, I. T., Mugerwa, J., Bailey, I., Olweny, C., Kiryabwire, Y.: Intracerebral Burkitts' lymphoma. Pathology, clinical features and treatment. Quart. J. Med., N.S. 43, 489–508 (1974)
Mathé, G., Pouillart, P., Schwarzenberg, L., Hayat, H., Amiel, J. L., Schlumberger, J. R., Misset, J. L., Musset, M., Jasmin, C., De Vassal, F.: Leukaemic conversion of non-Hodgkin's malignant lymphomata. Brit. J. Cancer 31, Suppl. II, 96–101 (1975)
Michotte, Y., Smeyers-Verbeke, J., Ebinger, G., Maurus, R., Pelsmaekers, J., Lowenthal, A., Massart, D. L.: Brain calcification in a case of acute lymphoblastic leukaemia. J. neurol. Sci. 25, 145–152 (1975)
Möbius, W., Hennekeuser, H. H., Schmidt, D., Noetzel, H.: Der Befall des Nervensystems und des Auges bei akuten und chronischen myeloischen Leukämien. In: Stacher, A., Höcker, P., Hrsg., Erkrankungen der Myelopoese, S. 338–340. Wien-Berlin-München: Urban & Schwarzenberg 1976
Moore, E. W., Thomas, L. B., Shaw, R. K., Freireich, E. J.: The central nervous system in acute leukemia. A postmortem study of 117 consecutive cases, with particular reference to hemorrhages, leukemic infiltrations, and the syndrome of meningeal leukemia. Arch. intern Med. 105, 141–158 (1960)
Nadel, E. M., Nelson, J. S.: The pathologic characteristics of leukostasis and leukemic nodules in the central nervous system of guinea pigs with L2C/NB leukemia. J. Neuropath. exp. Neurol. 35, 75–89 (1976)
Norrell, H., Wilson, C. B., Slagel, D. E., Clark, D. B.: Leukoencephalopathy following the administration of methotrexate into the cerebrospinal fluid in the treatment of primary brain tumors. Cancer (Philad.) 33, 923–932 (1974)
Olson, M. E., Chernik, N. L., Posner, J. B.: Infiltration of the leptomeninges by systemic cancer. A clinical and pathologic study. Arch. Neurol. (Chic.) 30, 122–137 (1974)
Olvera Rabiela, J. E., López, S. P., Saavedra, J. A.: Linfomas primarios del encefalo. Informe de 10 cases. Patologia (Mex. City) 13, 325–339 (1975)
Plafker, J., Martinez, A. J., Rosenblum, W. I.: A neoplasm of the reticulo-endothelial system involving the brain (microglioma) and viscera (reticulum cell sarcoma). Sth. med. J. (Bgham, Ala.) 65, 385–389 (1972)
Pochedly, C.: Neurologic manifestations in acute leukemia. N.Y. St. J. Med. 75, 575–580, 715–721, 878–882 (1975)
Price, R. A., Johnson, W. W.: The central nervous system in childhood leukemia. I. The arachnoid. Cancer (Philad.) 31, 520–533 (1973)
Rawlinson, D. G., Billingham, M. E., Berry, P. F., Kempson, R. L.: Cytology of the cerebrospinal fluid in patients with Hodgkin's disease and malignant lymphoma. Acta neuropath. (Berl.), Suppl. VI, 187–191 (1975)
Reznik, N.: Pathology of primary reticulum cell sarcoma of the human central nervous system. Acta neuropath. (Berl.) Suppl. VI, 91–94 (1975)
Rubinstein, L. J.: Tumors of the central nervous system. Atlas of tumor pathology, 2nd ser., Fasc. 6. Washington, D. C.: Armed Forces Institute of Pathology 1972
Rubinstein, L. J., Herman, M. M., Long, T. F., Wilbur, J. R.: Disseminated necrotizing leukoencephalopathy: a complication of treated central nervous system leukemia and lymphoma. Cancer (Philad.) 35, 291–305 (1975)
Russell, D. S., Marshall, A. H. E., Smith, F. B.: Microgliomatosis: a form of reticulosis affecting the brain. Brain 71, 1–14 (1948)
Schaumburg, H. H., Plank, C. R., Adams, R. D.: The reticulum cell sarcom-microglioma group of brain tumours. Brain 95, 199–212 (1972)
Scholtze, P., Jänisch, W.: Zur Häufigkeit und Einordnung neoplastischer Retikulosen des Zentralnervensystems. Zbl. allg. Path. path. Anat. 114, 159–167 (1971)
Sparling, H. J., Jr., Adams, R. D., Parker, F., Jr.: Involvement of the nervous system by malignant lymphomas. Medicine (Balt.) 26, 285–332 (1947)
Stefanko, S., Moffie, D.: Primary reticulum-cell-sareoma of the brain. A clinical-pathological study. Clin. Neurol. Neurosurg. (Amst.) 77, 96–109 (1974)
Stein, H., Kaiserling, E., Lennert, K.: Evidence for B-cell origin of reticulum cell sarcoma. Virchows Arch. Abt. A 364, 51–67 (1974)
Thomas, L. B.: Pathology of leukemia in the brain and meninges: post mortem studies of patients with acute leukemia and of mice given inoculation of L 1210 leukemia. Cancer Res. 25, 1555–1571 (1965)
Valsamis, M. P., Levine, P. H., Rapin, I., Santorineou, M., Shulman, K.: Primary intracranial Burkitt's lymphoma in an infant. Cancer (Philad.) 37, 1500–1507 (1976)
Verity, G. L.: Neurologic manifestations and complications of lymphoma. Radiol. Clin. N. Amer. 6, 97–106 (1968)
Vuia, O.: Primary reticulum cell sarcoma of the brain. Neuropath. appl. Neurobiol. 1, 357–366 (1975)
Vuia, O., Mehraein, P.: Cerebral reticuloplasmacytosis with hyperglobulinaemia. Europ. Neurol. 7, 469–482 (1972)
Willis, R. A.: The spread of tumors in the human body. London: Butterworths 1952
Zimmerman, H. M.: Malignant lymphomas. In: J. Minckler, ed.: Pathology of the nervous system, Vol. II, pp. 2165–2178. New York: McGraw-Hill 1971
Zimmerman, H. M.: Malignant lymphomas of the nervous system. Acta neuropath. (Berl.), Suppl. VI, 69–74 (1975)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Jellinger, K., Radaszkiewicz, T. Involvement of the central nervous system in malignant lymphomas. Virchows Arch. A Path. Anat. and Histol. 370, 345–362 (1976). https://doi.org/10.1007/BF00445779
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00445779