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C O M M AY 2 0 0 9 C A S E 1
Hans Brunnström ; Michael Dictor1; Christer Nilsson2; Dag Gülich2; Elisabet Englund1
1
1
Department of Pathology
2
Department of Geriatric Psychiatry
PATHOLOGY
Macroscopically, a tumor-like mass, 5 ¥ 4 ¥ 3 cm in size with
poorly defined margins, was found in the centre of the left frontal
lobe within the white matter (Figure 2b; Luxol fast blue stain). No
other lesions were seen in the brain or the meninges.
Microscopic examination revealed a diffuse mononuclear cell
infiltrate with variable cell density infiltrating the neuropil rather Figure 1.
Figure 2.
A B
Figure 3.
REFERENCES
DISCUSSION
1. Blay JY, Conroy T, Chevreau C, Thyss A, Quesnel N, Eghbali H,
Primary CNS lymphomas constitute about 6% of all primary brain
Bouabdallah R, Coiffier B, Wagner JP, Le Mevel A, Dramais-Marcel
tumors and most are of B-cell phenotype. In Western countries only D, Baumelou E, Chauvin F, Biron P (1998) High-dose methotrexate
2–5% of CNS lymphomas are derived from T-cells (3). Primary for the treatment of primary cerebral lymphomas: analysis of survival
CNS lymphomas of T-cell type (T-PCNSL) may appear at any age, and late neurologic toxicity in a retrospective series. J Clin Oncol
with a median age of about 60 years at diagnosis (10). Most 16:864–871.
affected patients are immunocompetent, but T-PCNSL has been 2. Choi JS, Nam DH, Ko YH, Seo JW, Choi YL, Suh YL, Ree HJ (2003)
reported in patients with human immunodeficiency virus (HIV) Primary central nervous system lymphoma in Korea: comparison of
or human T-lymphotropic virus 1 (HTLV-1) infections (9, 10). B- and T-cell lymphomas. Am J Surg Pathol 27:919–928.
Epstein-Barr virus (EBV) has not been linked to T-PCNSL (5, 8). 3. Deckert M, Paulus W (2007) Malignant lymphomas In: WHO
In the present case, a serologic test for HIV was negative, but Classification of Tumors of the Central Nervous System Louis DN,
Ohgaki H, Wiestler OD, Cavenee WK (eds), pp. 188–192. IARC
HTLV-1 status was not determined. In situ hybridization for EBV
Press: Lyon.
on autopsy material was negative. 4. Dictor M, Warenholt J, Isinger A (2005) Resolving T-cell receptor
Patients with T-PCNSL are usually treated with chemotherapy clonality in two and genotype in four multiplex polymerase chain
followed by irradiation, with or without initial corticosteroid treat- reactions. Haematologica 90:1524–1532.
ment. The prognosis is poor, with a 5-year disease-specific survival 5. Dulai MS, Park CY, Howell WD, Smyth LT, Desai M, Carter DM,
of 17% and a median progression-free survival of 22 months(10). Vogel H (2008) CNS T-cell lymphoma: an under-recognized entity?
Several factors have been linked to a worse prognosis, such as Acta Neuropathol 115:345–356.
advanced age, tumor in deep brain structures (cerebellum, brain- 6. Ferreri AJ, Blay JY, Reni M, ET AL (2003) Prognostic scoring system
stem, corpus callosum, and basal ganglia), elevated serum lactate for primary CNS lymphomas: the International Extranodal
dehydrogenase, and elevated CSF protein levels (1, 6). In the Lymphoma Study Group experience. J Clin Oncol 21:266–272.
7. Kim EY, Kim SS (2005) Magnetic resonance findings of primary
present case, the approximate duration of the illness was 14–15
central nervous system T-cell lymphoma in immunocompetent
months. patients. Acta Radiol 46:187–192.
It may prove difficult to clinically diagnose a T-cell lymphoma of 8. Liu D, Schelper RL, Carter DA, Poiesz BJ, Shrimpton AE, Frankel
the CNS. As in this case, MRI often shows a non-specific solitary BM, Hutchison RE (2003) Primary central nervous system
mass located in one of the cerebral hemispheres (7, 8, 10), and cytotoxic/suppressor T-cell lymphoma: report of a unique case and
brain biopsy is required for diagnosis. However, in the CNS, T-cell review of the literature. Am J Surg Pathol 27:682–688.
lymphomas often present as infiltrating cells with small nuclei and 9. Marshall AG, Pawson R, Thom M, Schulz TF, Scaravilli F, Rudge P
bland morphology, although pleomorphic or large cell phenotypes (1999) HTLV-I associated primary CNS T-cell lymphoma. J Neurol
are not infrequent (10), which explains why T-PCNSL may be Sci 158:226–231.
mistaken for non-neoplastic lymphoid infiltrates. In addition, pre- 10. Shenkier TN, Blay JY, O’Neill BP, ET AL (2005) Primary CNS
lymphoma of T-cell origin: a descriptive analysis from the
viously administered corticosteroid treatment may further compli-
international primary CNS lymphoma collaborative group. J Clin
cate the histopatholoigcal diagnosis. Oncol 23:2233–2239.
Genotypic analysis for TCR gamma (5) and other (4) TCR gene
rearrangements, in order to detect monoclonality, may be neces-
sary to establish the diagnosis, as no immunohistochemical marker
ABSTRACT
to detect T-cell monoclonality exists. In the present case, in which a A 76-year-old man presented with cognitive symptoms, followed
tumor diagnosis was not anticipated, the whole brain was routinely by headache and weakness of the lower limbs and left arm. The
fixed for several days in formaldehyde solution before sectioning clinical course was progressive but fluctuating. On magnetic reso-
and staining, and genotypic analysis was therefore not possible. nance imaging (MRI), a contrast-enhancing lesion 1 cm in diam-
However, the autopsy could provide a more solid base for diagnosis eter was seen in the left temporal lobe. This lesion became attenu-
than a brain biopsy would have done in this case, especially in the ated and a new contrast-enhancing lesion 1 x 2 cm was seen in the
light of the rather loosely scattered tumor cells and their non- left frontal lobe on a subsequent MRI. Following additional tests,
neoplastic appearance. The immunophenotype of T-PCNSL varies. treatment with corticosteroids for presumptive neurosarcoidosis
Staining may be positive for either or both CD4 and CD8 or as in was started, however, he soon expired. At autopsy, there was a
the present case both may be negative (2, 5, 8). Also, lymphoma tumor-like mass in the left frontal lobe. Pathologic evaluation
cells may lose their expression of CD5 and CD7 (5), as seen in revealed a primary T-cell lymphoma of the central nervous system
many cells in the present case. Although alternative conditions (CNS). CNS T-cell lymphomas may be difficult to diagnose, even
(such as infection, granulomatous diseases and other inflamma- histologically, due to their frequent small cell morphology and lack
tion) may be considered, the presentation of unusually fluctuating of significant atypia.