J Neurosurg 74:668-672, 1991
Primary central nervous system T-cell lymphoma Case report MARTIN M. BEDNAR, M.D., Ph.D., ANTHONY SALERNI, M.D., MARTIN E. ELANAGAN, M.D., AND WILLIAM W. PENDLEBURY, M.D.
Departments of Surgery (Division of Neurosurger)~9and Pathology, University of Vermont, Burlington, Vermont o- Primary central nervous system (CNS) T-cell lymphoma is extremely rare. The present case report provides immunocytochemical evidence for a cerebellar CNS T-cell lymphoma. The patient underwent surgery followed by radiation therapy and is alive and well 36 months postoperatively. The clinical and pathological features of primary CNS T-cell lymphoma as well as diagnostic measures and treatment options are discussed, together with a compilation of all previous case reports of primary CNS T-cell lymphomas. KEY WORDS
9
T-cell lymphoma
ucn attention has been focused on primary central nervous system (CNS) lymphoma since the first case was reported in 1929. ~This rare neoplasm accounts for less than 2% of all malignant lymphomas 3"~~.,5.3, and less than 1.5% of all intracranial tumors. ~'-~4,~5.3~ Although its association with congenital and acquired immunological deficits 2,67.J3.,7. 2,.2s.2~ and autoimmune diseases ~2~ is thought to be significant, the pathogenesis of primary CNS lymphoma is not well understood. In fact, the cell of origin of primary CNS lymphomas is controversial. ~2 Transplant recipients 6'-'4"25'-~7and patients with the acquired immune deficiency syndrome (AIDS) ~2~ now account for many of the cases of primary CNS lymphoma; in fact, this tumor is the second most common CNS mass lesion in the population positive for human immunodeficiency virus (HIV). ~-~Thus, with the spread of AIDS, the incidence of primary CNS lymphoma is expected to rise dramatically. It is essential that the correct antemortem diagnosis be made, since specific treatment of primary CNS lymphoma is effective) m.~,.14 In the non-immunosuppressed population, large-cell non-Hodgkin's lymphoma is the most common primary CNS lymphoma and is of B-cell origin. Small noncleaved-cell and immunoblastic lymphomas are common forms of CNS and non-Hodgkin's lymphomas in AIDS and other immunosuppressed patients. Testing with contemporary immunocytochemical methods has shown that this concept is generally valid. ~~s-~s-3~In-
M
668
9
immunosuppression
9
lymphoma
deed, primary CNS lymphomas of T-cell origin are a distinct rarity. To date, there have been only 14 reported cases of primary CNS T-cell lymphoma. 48'9'j6'~9'22'23'26 The present report describes a primary CNS lymphoma of T-cell origin in the cerebellum and provides a compilation of all reported primary CNS T-cell lymphomas. Case Report
This 32-year-old white man was healthy until 2 months prior to his first hospital admission when he noted the onset of a constant, dull occipital headache. The pain was initially relieved with ibuprofen but he sought further medical attention when nausea and vomiting developed. Examination. He denied any difficulty with balance, coordination, vision, or weight loss. Except for a slightly wide-based gait, the general and neurological examinations were unremarkable. Social history revealed the patient was a nonsmoker and used ethanol rarely. His family history was significant in that his mother died of "cancer" with possible metastases to the brain. Computerized tomography (CT) of the brain revealed two densely enhancing mass lesions, one in the right cerebellar hemisphere measuring 2 cm and one in the left cerebellar hemisphere measuring 4 cm (Fig. 1 left). The patient was placed on a course of high-dose dexamethasone (10 mg orally every 6 hours). A full
J. Neurosurg. / Volume 74/April, 1991
P r i m a r y c e n t r a l n e r v o u s s y s t e m T-cell l y m p h o m a
FI(;. I. Preoperative contrast-enhanced computerized tomography scans. L~/l. Scan obtained on admission showing enhancing mass lesions within both right and left eerebellar hemispheres. Right. Scan obtained I week later demonstrating marked reduction in the size of the left cerebellar lesion and the replacement of the enhancing lesion in the right cerebellar hemisphere by a region of low attenuation.
metastatic workup failed to demonstrate any systemic neoplastic process. The cerebrospinal fluid (CSF) was also negative for any malignant cells and HIV serology was negative. A vertebral angiogram demonstrated avascular mass effects in both cerebellar hemispheres without evidence of neovascularity or blush, Follow-up CT scanning 1 week after admission revealed that the cerebellar masses had decreased in size, and CT scanning 3 days later showed that the fight cerebellar mass was no longer evident. By 2 months after presentation, the left cerebellar mass was approximately 1 cm in size (Fig. I right). The patient remained asymptomatic.
Operation. For diagnosis and treatment, the patient underwent a left suboccipital craniectomy with gross total removal of the left cerebellar mass. At surgery, the
FI~. 2. Low-power photomicrograph of the left cerebellar mass showing sheets of small round cells without evidence of necrosis. H & E. bar = 100 u.
leptomeninges appeared normal; however, the cerebellar cortex overlying the lesion was relatively avascular and the lesion itself appeared tannish-gray.
Pathological Examination. Histological study of the specimen revealed that the CNS parenchyma and leptomeninges were partly invaded by masses of lymphocytes (Fig. 2) with a fairly significant number of admixed plasma cells. These took the form of prominent perivascular (Fig. 3 left) and intra-adventitial cellular collections that spread out from these locations to form fairly large sheet-like masses (Fig. 3 right). Astrocytic gliosis was present, with some of the astrocytes showing large, bizarre, hyperchromatic nuclei. The lymphocytes appeared as very monotonous small cells with scant cytoplasm and dark nuclei. There were no mitotic figures or necrosis. The cells identified as lymphocytes were positive to common leukocyte antigen (Fig. 4 left) and stained with antibodies directed against T cells (Fig.
FI6. 3. Photomicrographs from other areas of the tumor showing both a perivascular (left) and intra-adventitial (right) distribution &cells. H & E, bar = 100 u (lt:[)) and 25 u (right).
J. Neurosurg. / Volume 74/April, 1991
669
M. Bednar, et aL
FIG. 4. Left. Immunocytochemical staining using an antibody directed against common leukocyte antigen (DAKO mouse monoclonal antileukocyte common antigen; clone po 7/26 and 2BI I). Essentially all of the tumor cells were immunolabeled by this antibody. Avidin biotin method, bar = 20 Ix. Right: Immunocytochemical staining using an antibody directed against T lymphocytes (DAKO monoclonal mouse anti-human T cell; clone UCH1). Greater than 90% of the tumor cells were immunolabeled by this antibody. Avidin biotin method, bar = 25 u.
FIG. 5. Photomicrograph showing prominent perivascular ventricular proliferation reticulin stain, bar = 25 u.
4 right). Reticulin stain showed prominent reticulum proliferation in a perivascular distribution (Fig. 5). Postoperative Course, The patient did welt postoperatively and remained neurologically intact. He was discharged home on postoperative Day 6. He underwent radiation therapy with 4600 rad directed to the operative site and a 1000-rad cranial boost in the 2 months following his discharge. He tolerated the radiation therapy well. He is now 3 years postsurgery, working full time. He denies any complaints. His neurological examination is unremarkable and both his last chest x-ray film and CT brain scan were negative for any lesions (Fig, 6).
Discussion Primary CNS lymphoma of T-cell origin is a rare lesion. 4'~'9'16"19'23'26Recently, Morgello, et a l . Y and C 670
FIG. 6. Postoperative contrast-enhanced computerized tomography scan demonstrating regions of low density in both cerebellar hemispheres without evidence of tumor enhancement.
Rao (unpublished data) treated an additional four patients with primary CNS T-cell lymphomas, We describe a case of primary T-cell lymphoma of the cerebellum and discuss the 15 cases reported to date (Table 1). The need for brain biopsy to establish an accurate diagnosis in our case reinforces the experience of others who have demonstrated the lack of reliability of CSF cytology,22 even with repeated lumbar punctures. Indeed, a confirmatory biopsy was typically performed even in those cases in which the presumptive diagnosis was made by CSF cytology.4'19'2~ The present case report is similar in its presentation to that of CNS lymphomas in general. Our patient presented with general symptoms referable to increased intracranial pressure and focal symptoms referable to the site of involvement. Steroid therapy had a marked J. Neurosurg. / Volume 74/April, 199I
Primary central nervous system T-cell lymplnoma TABLE [ Summary 0/15 ca.~es qf yrimat3' CNS T-eel! ]ymphoma* .Authors & Year
.Age(yrs). CNS S i t e Cvtochemistr', Treatment Outcome Sex " " Schmitt-Graff& Pfitzer. 1983 52, M leptomeninges acid phosphatase MTX died (1~ mos) Grant. et al., 1986 63, M midline subfalcine pan T-cell & T-helper AB's biopsy; RT; chemotherapy died (11 mos) mass Marsh. et al.. 1983 20. M leptomeninges OKT-3:acid phosphatase biopsy:thiotepa, MTX: remission(2 mos) RT O'Neill. et al., 1987 38, F thalamus CLA (negativeIg light biopsy NR chain) 17, M cerebellum leu-l, leu-2a, leu-3a(nega- surgery NR tire Ig light chain) Bogdahn, et al., 1986 2, F superior vermin acid phosphatase RT: chemotherapy; sur- remission(24 mos) gery (gross total) 51, M lateralventricle acid phosphatase Ara-C, MTX: RT partial remission(16 mos) Morgello, et al., 1989 30, M frontalmass, lep- pan T-cell AB, T-helper & biopsy;RT; MTX alive (14 mos), minimal tomeninges suppressorAB's (negahemiparesis tire Ig light chains) 28, M temporallobe & same biopsy; RT; MTX, Ara-C died (20 mos) brain stem 60, F cerebellarhemi- CLA; pan T-cell AB biopsy: RT died (21 mos) sphere Grant & yon Deimling, 1990 64. M cerebellarhemi- T-cell markers RT; surgery (gross total) alive& well (8 mos) sphere Kuwata, et al., 1987 50, M temporoparietal T-cell markers RTI chemotherapy; sur- alive(14 mos) get3' Rao, et aL, 1989 biopsy died 30, F cerebellar vermis pan T-cell AB pan T-cell AB biopsy NR 36, M temporal lobe Bednar, eta[., 1991 CLA, immunoperoxidase RT; surgery(gross total) alive& well (36 mos) 32, M cerebellum * Abbreviations:CNS = central nervous system; AB = antibody; MTX = methotrexate; RT = radiation therapy; NR = not recorded; CLA = common leukocyteantigen; Ig = immunoglobulin;Ara-C = cytosine arabinoside.
effect, with transient relief of the CNS symptoms. Our patient demonstrated full resolution of his symptoms shortly after steroid therapy was initiated. Various other modalities have been used in the treatment of primary, CNS T-cell lymphoma: surgery, radiation therapy, and chemotherapeutic regimens. Both radiation therapy 4~9~622 and c h e m o t h e r a p y ~~6~97-226 are considered to be beneficial, although the small n u m b e r of cases reported prohibits a definitive assessment of any adjuvant therapy. Our patient has completed a course of radiation therapy and remains neurologically intact after more than 3 years from the time of his diagnosis, despite the observation that the prognosis for CNS nonHodgkin's l y m p h o m a is generally poor. In comparing the features of the present case to those of other primary CNS T-cell lymphomas, it is not clear why our patient has survived longer than other reported patients. It is of interest that most other patients underwent surgical biopsy instead of an attempt at gross total removal as in the present case. Indeed, the longest survivor recorded so far also underwent a gross total removal with the patient remaining in remission 2 years following surgery? The rarity of T-cell cerebellar l y m p h o m a prohibits a definitive statement regarding its management. However, an aggressive surgical approach used in the present case enabled both a confirmative tissue diagnosis and a substantial reduction in the t u m o r burden. This may, J. Neurosurg. / V o l u m e 74~April. 1991
when combined with adjuvant therapy, result in remission or p e r m a n e n t cure as seen in the present case. References
I. Bailey P: Intracranial sarcomatous tumors of leptomeningeal origin. Arch Sure 18:1359-1402, 1929 2. Beckstead JH: Optimal antigen localization in human tissues using aldehyde-fixed plastic-imbedded sections. J Histochem Cytochem 33:954-958, 1985 3. Berry MP, Simpson WJ: Radiation therapy in the management of primary malignant lymphoma of the brain. Int J Radiat Oncol Biol Phys 7:55-59, 1981 4. Bogdahn U, Bogdahn S, Mertens HG, et al: Primary nonHodgkin's lymphoma of the CNS. Acta Neurol Seand 73: 602-614, 1986 5. Fudenberg HH: Genetically determined immune deficiency as the predisposing cause of "autoimmunity" and lymphoid neoplasia. Am J Med 51"295-298, 1971 6. Fflipovich H, Spector BD, Kersey J: Immunodeficiency in humans as a risk factor in the development of malignancy. Wislott Aldrich syndrome. Prey Med 9:252-259, 1980 7. Gallo RC, Wong-Staal F: Current thoughts on the viral etiology of certain human cancers: The Richard and Hinda Rosenthal Foundation Award Lecture. Cancer Res 44:2743-2749, 1984 8. Grant JW, Gallagher PJ, Jones DB: Primary cerebral lymphoma. A histologic and immunohistochemical study of six cases. Arch Pathol Lab Med 110:897-901, 1986 9. Grant JW, yon Deimling A: Primary T-cell lymphoma of the central nervous system. Arch Pathol Lab Med 114: 671
M. Bednar, et a/. 24-27, 1990 10. Helle TL, Britt RH, Colb~ TV: Primar~ l~mphoma of the central nervous system. Clinicopathological study of the experience at Sianford J Neurosurg 60:94-103, 1984 11. Henry JM, Hcffner RR Jr, Dillard Slf, ct al: Primary malignant lymphomas of the central nervous system. Cancer 34:1293-1302, 1974 t2. Hochberg FH, Miller DC: Primary central nervous system lymphoma. J Neurosurg 68:835-853, t 988 13. Hoover R, Fraumeni JF Jr: Risk of cancer in renaltransplant recipients. Lancet 2:55-57, 1973 14. Jellinger K, Radaskiewicz TH, Slowik F: Primary malignant lymphomas of the central nervous system in man. Acta Neuropathol Suppl 6:95-102, 1975 15. Jellinger K, Slowik F, Sluga E: Primary intracranial malignant lymphomas. A fine structural, cytochemical and CSF immunological study. Clin Neurol Neurosurg 83: 173-186, 1979 16. Kuwata T, Funahashi K, ltakura T, et al: [T cell type primary lymphoma of the central nervous system: a case report.] No Shinkei Geka 15:657-66t, 1987 (Jpn) 17. Levine AM, Meyer PR, Begandy MK, et al: Development of B-cell lymphoma in homosexual men. Clinical and immunologic findings. Ann Intern Med 100:7-13, 1984 18. Levy RM, Bredesen DE, Rosenblum ML: Neurological manifestations of the acquired immunodeficiency syndrome (AIDS): experience at UCSF and review of the literature. J Neurosurg 62:475-495, 1985 19. Marsh WL Jr, Stevenson DR. Long HJ Ill: Primary leptomeningeal presentation of T-cell lymphoma. Cancer 51:1125-1131, 1983 20. Mellors RC: Autoimmune disease in NZB/B~ mice. II. Autoimmunity and malignant lymphoma. Blood 27: 435-448, 1966 21. Mendenhall NP, Thor TL, Agee OF, et al: Primary' lymphoma of the central nervous system. Computerized tomography scan characteristics and treatment results in 12 cases. Cancer 52:1993-2000, 1983
672
22. Morgello S, Maiese K, Petito CK: T-cell lymphoma in the CNS. Clinical and pathologic features. Neurology 39: 1190-1196, 1989 23. O'Neill BP. Kelly PJ, Earle JD, et al: Computer-assisted stereotaxic biopsy for the diagnosis of primal, central nervous system lymphoma. Neurology 37:1160-1164, 1987 24. Penn I: Development of cancer as a complication of clinical transplantation. Transplant Proc 9:1121-1127, 1977 25. Penn 1. Hammond W, Brettschneider L, et al: Malignant lymphomas in transplantation recipients. Transplant Proc 1:106-I 12, 1969 26. Schmitt-Grfiff A, Pfitzer P: Cytology of the cerebrospinal fluid in primary, malignant lymphoma of the central nervous system. Acta Cytol 27:267-272, 1983 27. Schneck. SA, Penn 1: De-novo brain tumors in renal transplant recipients. Lancet 1:983-986, 1971 28. So YT, Bcckstead JH, Davis RL: Primary central nervous system lymphoma in acquired immune deficiency syndrome: a clinical and pathological study. Ann Neurol 20: 566-572, 1986 29. Taylor CR, Russell R, Lukes RJ, et al: An immunohistological study of immunoglobulin content of primary central nervous system lymphomas. Cancer 41: 2197-2205, 1978 30. Varadachari C, Palutke M, Climie ARW, et al: Immunoblastic sarcoma (histioeytic lymphoma) of the brain with B-cell markers. Case report. J Neurosurg 49: 887-892, 1978 31. Woodman R, Shin K, Pineo G: Primary non-Hodgkin's lymphoma of the brain. Medicine 64:425-430, 1985
Manuscript received May 16, 1990. Accepted in final form September 24, 1990. Address reprint requests to." Martin E. Flanagan, M.D., Division of Neurological Surgery,, 1 South Prospect Street, Burlington, Vermont 05401.
3,. Neurosurg. / Volume 74/April, 1991
Primary central nervous system T-cell lymphoma Case report MARTIN M. BEDNAR, M.D., Ph.D., ANTHONY SALERNI, M.D., MARTIN E. ELANAGAN, M.D., AND WILLIAM W. PENDLEBURY, M.D.
Departments of Surgery (Division of Neurosurger)~9and Pathology, University of Vermont, Burlington, Vermont o- Primary central nervous system (CNS) T-cell lymphoma is extremely rare. The present case report provides immunocytochemical evidence for a cerebellar CNS T-cell lymphoma. The patient underwent surgery followed by radiation therapy and is alive and well 36 months postoperatively. The clinical and pathological features of primary CNS T-cell lymphoma as well as diagnostic measures and treatment options are discussed, together with a compilation of all previous case reports of primary CNS T-cell lymphomas. KEY WORDS
9
T-cell lymphoma
ucn attention has been focused on primary central nervous system (CNS) lymphoma since the first case was reported in 1929. ~This rare neoplasm accounts for less than 2% of all malignant lymphomas 3"~~.,5.3, and less than 1.5% of all intracranial tumors. ~'-~4,~5.3~ Although its association with congenital and acquired immunological deficits 2,67.J3.,7. 2,.2s.2~ and autoimmune diseases ~2~ is thought to be significant, the pathogenesis of primary CNS lymphoma is not well understood. In fact, the cell of origin of primary CNS lymphomas is controversial. ~2 Transplant recipients 6'-'4"25'-~7and patients with the acquired immune deficiency syndrome (AIDS) ~2~ now account for many of the cases of primary CNS lymphoma; in fact, this tumor is the second most common CNS mass lesion in the population positive for human immunodeficiency virus (HIV). ~-~Thus, with the spread of AIDS, the incidence of primary CNS lymphoma is expected to rise dramatically. It is essential that the correct antemortem diagnosis be made, since specific treatment of primary CNS lymphoma is effective) m.~,.14 In the non-immunosuppressed population, large-cell non-Hodgkin's lymphoma is the most common primary CNS lymphoma and is of B-cell origin. Small noncleaved-cell and immunoblastic lymphomas are common forms of CNS and non-Hodgkin's lymphomas in AIDS and other immunosuppressed patients. Testing with contemporary immunocytochemical methods has shown that this concept is generally valid. ~~s-~s-3~In-
M
668
9
immunosuppression
9
lymphoma
deed, primary CNS lymphomas of T-cell origin are a distinct rarity. To date, there have been only 14 reported cases of primary CNS T-cell lymphoma. 48'9'j6'~9'22'23'26 The present report describes a primary CNS lymphoma of T-cell origin in the cerebellum and provides a compilation of all reported primary CNS T-cell lymphomas. Case Report
This 32-year-old white man was healthy until 2 months prior to his first hospital admission when he noted the onset of a constant, dull occipital headache. The pain was initially relieved with ibuprofen but he sought further medical attention when nausea and vomiting developed. Examination. He denied any difficulty with balance, coordination, vision, or weight loss. Except for a slightly wide-based gait, the general and neurological examinations were unremarkable. Social history revealed the patient was a nonsmoker and used ethanol rarely. His family history was significant in that his mother died of "cancer" with possible metastases to the brain. Computerized tomography (CT) of the brain revealed two densely enhancing mass lesions, one in the right cerebellar hemisphere measuring 2 cm and one in the left cerebellar hemisphere measuring 4 cm (Fig. 1 left). The patient was placed on a course of high-dose dexamethasone (10 mg orally every 6 hours). A full
J. Neurosurg. / Volume 74/April, 1991
P r i m a r y c e n t r a l n e r v o u s s y s t e m T-cell l y m p h o m a
FI(;. I. Preoperative contrast-enhanced computerized tomography scans. L~/l. Scan obtained on admission showing enhancing mass lesions within both right and left eerebellar hemispheres. Right. Scan obtained I week later demonstrating marked reduction in the size of the left cerebellar lesion and the replacement of the enhancing lesion in the right cerebellar hemisphere by a region of low attenuation.
metastatic workup failed to demonstrate any systemic neoplastic process. The cerebrospinal fluid (CSF) was also negative for any malignant cells and HIV serology was negative. A vertebral angiogram demonstrated avascular mass effects in both cerebellar hemispheres without evidence of neovascularity or blush, Follow-up CT scanning 1 week after admission revealed that the cerebellar masses had decreased in size, and CT scanning 3 days later showed that the fight cerebellar mass was no longer evident. By 2 months after presentation, the left cerebellar mass was approximately 1 cm in size (Fig. I right). The patient remained asymptomatic.
Operation. For diagnosis and treatment, the patient underwent a left suboccipital craniectomy with gross total removal of the left cerebellar mass. At surgery, the
FI~. 2. Low-power photomicrograph of the left cerebellar mass showing sheets of small round cells without evidence of necrosis. H & E. bar = 100 u.
leptomeninges appeared normal; however, the cerebellar cortex overlying the lesion was relatively avascular and the lesion itself appeared tannish-gray.
Pathological Examination. Histological study of the specimen revealed that the CNS parenchyma and leptomeninges were partly invaded by masses of lymphocytes (Fig. 2) with a fairly significant number of admixed plasma cells. These took the form of prominent perivascular (Fig. 3 left) and intra-adventitial cellular collections that spread out from these locations to form fairly large sheet-like masses (Fig. 3 right). Astrocytic gliosis was present, with some of the astrocytes showing large, bizarre, hyperchromatic nuclei. The lymphocytes appeared as very monotonous small cells with scant cytoplasm and dark nuclei. There were no mitotic figures or necrosis. The cells identified as lymphocytes were positive to common leukocyte antigen (Fig. 4 left) and stained with antibodies directed against T cells (Fig.
FI6. 3. Photomicrographs from other areas of the tumor showing both a perivascular (left) and intra-adventitial (right) distribution &cells. H & E, bar = 100 u (lt:[)) and 25 u (right).
J. Neurosurg. / Volume 74/April, 1991
669
M. Bednar, et aL
FIG. 4. Left. Immunocytochemical staining using an antibody directed against common leukocyte antigen (DAKO mouse monoclonal antileukocyte common antigen; clone po 7/26 and 2BI I). Essentially all of the tumor cells were immunolabeled by this antibody. Avidin biotin method, bar = 20 Ix. Right: Immunocytochemical staining using an antibody directed against T lymphocytes (DAKO monoclonal mouse anti-human T cell; clone UCH1). Greater than 90% of the tumor cells were immunolabeled by this antibody. Avidin biotin method, bar = 25 u.
FIG. 5. Photomicrograph showing prominent perivascular ventricular proliferation reticulin stain, bar = 25 u.
4 right). Reticulin stain showed prominent reticulum proliferation in a perivascular distribution (Fig. 5). Postoperative Course, The patient did welt postoperatively and remained neurologically intact. He was discharged home on postoperative Day 6. He underwent radiation therapy with 4600 rad directed to the operative site and a 1000-rad cranial boost in the 2 months following his discharge. He tolerated the radiation therapy well. He is now 3 years postsurgery, working full time. He denies any complaints. His neurological examination is unremarkable and both his last chest x-ray film and CT brain scan were negative for any lesions (Fig, 6).
Discussion Primary CNS lymphoma of T-cell origin is a rare lesion. 4'~'9'16"19'23'26Recently, Morgello, et a l . Y and C 670
FIG. 6. Postoperative contrast-enhanced computerized tomography scan demonstrating regions of low density in both cerebellar hemispheres without evidence of tumor enhancement.
Rao (unpublished data) treated an additional four patients with primary CNS T-cell lymphomas, We describe a case of primary T-cell lymphoma of the cerebellum and discuss the 15 cases reported to date (Table 1). The need for brain biopsy to establish an accurate diagnosis in our case reinforces the experience of others who have demonstrated the lack of reliability of CSF cytology,22 even with repeated lumbar punctures. Indeed, a confirmatory biopsy was typically performed even in those cases in which the presumptive diagnosis was made by CSF cytology.4'19'2~ The present case report is similar in its presentation to that of CNS lymphomas in general. Our patient presented with general symptoms referable to increased intracranial pressure and focal symptoms referable to the site of involvement. Steroid therapy had a marked J. Neurosurg. / Volume 74/April, 199I
Primary central nervous system T-cell lymplnoma TABLE [ Summary 0/15 ca.~es qf yrimat3' CNS T-eel! ]ymphoma* .Authors & Year
.Age(yrs). CNS S i t e Cvtochemistr', Treatment Outcome Sex " " Schmitt-Graff& Pfitzer. 1983 52, M leptomeninges acid phosphatase MTX died (1~ mos) Grant. et al., 1986 63, M midline subfalcine pan T-cell & T-helper AB's biopsy; RT; chemotherapy died (11 mos) mass Marsh. et al.. 1983 20. M leptomeninges OKT-3:acid phosphatase biopsy:thiotepa, MTX: remission(2 mos) RT O'Neill. et al., 1987 38, F thalamus CLA (negativeIg light biopsy NR chain) 17, M cerebellum leu-l, leu-2a, leu-3a(nega- surgery NR tire Ig light chain) Bogdahn, et al., 1986 2, F superior vermin acid phosphatase RT: chemotherapy; sur- remission(24 mos) gery (gross total) 51, M lateralventricle acid phosphatase Ara-C, MTX: RT partial remission(16 mos) Morgello, et al., 1989 30, M frontalmass, lep- pan T-cell AB, T-helper & biopsy;RT; MTX alive (14 mos), minimal tomeninges suppressorAB's (negahemiparesis tire Ig light chains) 28, M temporallobe & same biopsy; RT; MTX, Ara-C died (20 mos) brain stem 60, F cerebellarhemi- CLA; pan T-cell AB biopsy: RT died (21 mos) sphere Grant & yon Deimling, 1990 64. M cerebellarhemi- T-cell markers RT; surgery (gross total) alive& well (8 mos) sphere Kuwata, et al., 1987 50, M temporoparietal T-cell markers RTI chemotherapy; sur- alive(14 mos) get3' Rao, et aL, 1989 biopsy died 30, F cerebellar vermis pan T-cell AB pan T-cell AB biopsy NR 36, M temporal lobe Bednar, eta[., 1991 CLA, immunoperoxidase RT; surgery(gross total) alive& well (36 mos) 32, M cerebellum * Abbreviations:CNS = central nervous system; AB = antibody; MTX = methotrexate; RT = radiation therapy; NR = not recorded; CLA = common leukocyteantigen; Ig = immunoglobulin;Ara-C = cytosine arabinoside.
effect, with transient relief of the CNS symptoms. Our patient demonstrated full resolution of his symptoms shortly after steroid therapy was initiated. Various other modalities have been used in the treatment of primary, CNS T-cell lymphoma: surgery, radiation therapy, and chemotherapeutic regimens. Both radiation therapy 4~9~622 and c h e m o t h e r a p y ~~6~97-226 are considered to be beneficial, although the small n u m b e r of cases reported prohibits a definitive assessment of any adjuvant therapy. Our patient has completed a course of radiation therapy and remains neurologically intact after more than 3 years from the time of his diagnosis, despite the observation that the prognosis for CNS nonHodgkin's l y m p h o m a is generally poor. In comparing the features of the present case to those of other primary CNS T-cell lymphomas, it is not clear why our patient has survived longer than other reported patients. It is of interest that most other patients underwent surgical biopsy instead of an attempt at gross total removal as in the present case. Indeed, the longest survivor recorded so far also underwent a gross total removal with the patient remaining in remission 2 years following surgery? The rarity of T-cell cerebellar l y m p h o m a prohibits a definitive statement regarding its management. However, an aggressive surgical approach used in the present case enabled both a confirmative tissue diagnosis and a substantial reduction in the t u m o r burden. This may, J. Neurosurg. / V o l u m e 74~April. 1991
when combined with adjuvant therapy, result in remission or p e r m a n e n t cure as seen in the present case. References
I. Bailey P: Intracranial sarcomatous tumors of leptomeningeal origin. Arch Sure 18:1359-1402, 1929 2. Beckstead JH: Optimal antigen localization in human tissues using aldehyde-fixed plastic-imbedded sections. J Histochem Cytochem 33:954-958, 1985 3. Berry MP, Simpson WJ: Radiation therapy in the management of primary malignant lymphoma of the brain. Int J Radiat Oncol Biol Phys 7:55-59, 1981 4. Bogdahn U, Bogdahn S, Mertens HG, et al: Primary nonHodgkin's lymphoma of the CNS. Acta Neurol Seand 73: 602-614, 1986 5. Fudenberg HH: Genetically determined immune deficiency as the predisposing cause of "autoimmunity" and lymphoid neoplasia. Am J Med 51"295-298, 1971 6. Fflipovich H, Spector BD, Kersey J: Immunodeficiency in humans as a risk factor in the development of malignancy. Wislott Aldrich syndrome. Prey Med 9:252-259, 1980 7. Gallo RC, Wong-Staal F: Current thoughts on the viral etiology of certain human cancers: The Richard and Hinda Rosenthal Foundation Award Lecture. Cancer Res 44:2743-2749, 1984 8. Grant JW, Gallagher PJ, Jones DB: Primary cerebral lymphoma. A histologic and immunohistochemical study of six cases. Arch Pathol Lab Med 110:897-901, 1986 9. Grant JW, yon Deimling A: Primary T-cell lymphoma of the central nervous system. Arch Pathol Lab Med 114: 671
M. Bednar, et a/. 24-27, 1990 10. Helle TL, Britt RH, Colb~ TV: Primar~ l~mphoma of the central nervous system. Clinicopathological study of the experience at Sianford J Neurosurg 60:94-103, 1984 11. Henry JM, Hcffner RR Jr, Dillard Slf, ct al: Primary malignant lymphomas of the central nervous system. Cancer 34:1293-1302, 1974 t2. Hochberg FH, Miller DC: Primary central nervous system lymphoma. J Neurosurg 68:835-853, t 988 13. Hoover R, Fraumeni JF Jr: Risk of cancer in renaltransplant recipients. Lancet 2:55-57, 1973 14. Jellinger K, Radaskiewicz TH, Slowik F: Primary malignant lymphomas of the central nervous system in man. Acta Neuropathol Suppl 6:95-102, 1975 15. Jellinger K, Slowik F, Sluga E: Primary intracranial malignant lymphomas. A fine structural, cytochemical and CSF immunological study. Clin Neurol Neurosurg 83: 173-186, 1979 16. Kuwata T, Funahashi K, ltakura T, et al: [T cell type primary lymphoma of the central nervous system: a case report.] No Shinkei Geka 15:657-66t, 1987 (Jpn) 17. Levine AM, Meyer PR, Begandy MK, et al: Development of B-cell lymphoma in homosexual men. Clinical and immunologic findings. Ann Intern Med 100:7-13, 1984 18. Levy RM, Bredesen DE, Rosenblum ML: Neurological manifestations of the acquired immunodeficiency syndrome (AIDS): experience at UCSF and review of the literature. J Neurosurg 62:475-495, 1985 19. Marsh WL Jr, Stevenson DR. Long HJ Ill: Primary leptomeningeal presentation of T-cell lymphoma. Cancer 51:1125-1131, 1983 20. Mellors RC: Autoimmune disease in NZB/B~ mice. II. Autoimmunity and malignant lymphoma. Blood 27: 435-448, 1966 21. Mendenhall NP, Thor TL, Agee OF, et al: Primary' lymphoma of the central nervous system. Computerized tomography scan characteristics and treatment results in 12 cases. Cancer 52:1993-2000, 1983
672
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Manuscript received May 16, 1990. Accepted in final form September 24, 1990. Address reprint requests to." Martin E. Flanagan, M.D., Division of Neurological Surgery,, 1 South Prospect Street, Burlington, Vermont 05401.
3,. Neurosurg. / Volume 74/April, 1991
