Low grade cerebellar T-cell lymphoma: a novel response to treatment; a case report

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Acta Neurochir (2010) 152:1075–1077 DOI 10.1007/s00701-009-0555-3

CASE REPORT

Low grade cerebellar T-cell lymphoma: a novel response to treatment; a case report Robert Corns & Matthew Crocker & Atul Kumar & Jon Salisbury & Christos Tolias & Gill Sadler & Mark Hill

Received: 18 November 2008 / Accepted: 19 October 2009 / Published online: 20 November 2009 # Springer-Verlag 2009

Abstract Low-grade primary T-cell lymphoma of the central nervous system is extremely rare. We present a case developing in a previously fit young woman presenting with symptoms of raised intracranial pressure and found on CT to have a cerebellar mass. Biopsy of this lesion revealed features of non-Hodgkin’s lymphoma with histochemical analysis confirming T-cell phenotype and a Ki67 proliferation index of only 1%. Contrary to the prevailing view in the literature, the patient’s clinical condition deteriorated following high-dose intravenous methotrexate and improved after a short course of whole-brain radiotherapy.

between 1.7 and 6.6% of all primary brain neoplasms, although the incidence is increasing [2, 8, 12]. Over 90% of PCNSL is B cell in origin [2], with immunohistochemistryconfirmed T-cell lymphoma making up the majority of the remainder. There are no large or phase III studies of this entity, the world literature being limited to case reports and small case series [5, 7, 10, 11, 13, 14]. It has an estimated incidence of less than four cases worldwide per annum. We present here a case of T-cell lymphoma arising in the cerebellum that had unusual histopathological findings and an apparently atypical response to treatment, as well as a brief review of the current literature.

Keywords Primary central nervous system lymphoma . T-cell lymphoma . Brain . Low grade . Treatment Case report Introduction Primary CNS lymphoma (PCNSL) is an uncommon form of extranodal non-Hodgkin’s lymphoma. It represents No financial support was received in the preparation of this report. R. Corns (*) : M. Crocker : A. Kumar : C. Tolias Departments of Neurosurgery, King’s College Hospital, Denmark Hill, London SE5 9RS, UK e-mail: [email protected] J. Salisbury Department of Pathology, King’s College Hospital, Denmark Hill, London SE5 9RS, UK G. Sadler : M. Hill Lymphoma Unit, Kent Oncology Centre, Maidstone Hospital, Hermitage Lane, Maidstone, Kent ME16 9QQ, UK

A 37-year-old right-handed otherwise healthy female presented with a 4-week history of persistent occipital headache associated with nausea and vomiting. A week prior to admission, she had noted unsteadiness on her feet. On examination, she was alert and orientated with intact cranial nerves and peripheral nervous system. Examination of cerebellar function revealed a slightly broad-based gait. She was afebrile with no lymphadenopathy; no abnormality was detected in the chest or abdomen. A CT of the head was performed that showed a midline, heterogenously enhancing lesion in the posterior fossa with surrounding oedema and minimal mass effect. MRI scan of the brain (Fig. 1) confirmed a 3 × 3 × 2-cm enhancing lesion within the cerebellar vermis, which was isointense on T1- and T2-weighted sequences. CT of the chest, abdomen and pelvis was normal. The patient was commenced on high-dose dexamethasone. A lumbar puncture showed no atypical or malignant cells. A repeat MRI showed some resolution of the mass in response to dexamethasone.

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Fig. 1 Axial T1 pre- and post-contrast MRI showing 3 × 2 × 2-cm enhancing cerebellar lesion

Stereotactic biopsy of the cerebellar lesion was conducted and dexamethasone gradually reduced. Histological examination of the biopsy specimens revealed fragmented cerebellar tissue infiltrated by a dense infiltrate of small lymphoid cells. The infiltrate showed some perivascular accentuation. Immunohistochemical staining demonstrated strong positivity for CD2, CD3, CD4, CD5, CD7 and CD8. CD20, CD30, CD56 and CD57 were negative. The features were those of a low-grade Tcell non-Hodgkin's lymphoma. Of note, the ki67 immunostaining revealed that the proliferation index was very low at only 1%. Blood tests confirmed her to be HIV negative. The patient underwent four monthly cycles of high-dose intravenous methotrexate (MTX); however, she deteriorated despite additional high-dose dexamethasone, becoming wheelchair bound and requiring admission to a hospice. The decision was made to proceed with whole-brain radiotherapy. Upon last review, 1 year post-diagnosis, she had improved to being able to walk short distances and was living at home with only occasional help with her activities of daily living.

Discussion Low-grade T-cell lymphoma is an extremely rare sub-type of PCNSL [5]. Diagnosis is made by histopathological and immunohistochemical analysis of malignant brain tissue. The patient presented here is of particular interest because the Ki67 proliferation index was so low at only 1%. The largest published series of 45 patients identified by the International PCNSL Collaborative Group covering North America, Europe and Australia from 1983 to 2000 [13] makes no reference to proliferation index. Although now a standard part of an immunohistological panel, Ki67 has

only become routine over recent years, following its introduction in 1986 [4]. The patient described above presented with progressive neurological symptoms, which is typical of patients with PCNSL [2]. Presentation with “B” symptoms (such as fever and night sweats) is rare, especially in the T-cell sub-group of PCNSL. The diagnosis of lymphoma is often suggested from radiological examination, with lesions isointense to hypointense on T2-weighted MRI images; they also enhance with contrast. Lesions are multiple in 35% of all PCNSL cases and 29% of the T-cell sub-group. The most common sites are adjacent to the ventricles in the deep white matter of the cerebral hemispheres [9], with only 9% of all PCNSL cases affecting the cerebellum; we have identified only four cases in total reported in the literature of T-cell PCNSL of the cerebellum [7, 13]. The median disease-specific survival for patients with Tcell PCNSL is 22 months [13]. Prognosis is significantly better in patients with higher performance status scores. Because of the paucity of cases, there is little evidence for what constitutes optimal therapy for T-cell PCNSL. There are a number of therapeutic studies in PCNSL as a whole, which mostly favour treatment with methotrexate (MTX) [1, 3, 6, 13]. Ferreri et al. [3] conducted a multicentre study looking at the treatment of all types of PCNSL of all grades. They found that chemotherapy (with high dose MTX) followed by radiotherapy provided the greatest increase in survival, with radiotherapy alone providing the least. The only study to look specifically at T-cell PCNSL also found that MTX provided a significant survival benefit, although there is no mention of proliferation index [13]. Our experience with this single case of T-cell PCNSL with very low proliferation index would suggest that radiotherapy alone may be the treatment of choice in some individuals with tumours of

Low grade cerebellar T-cell lymphoma: a novel response to treatment; a case report

this type, with dexamethasone not providing any additional benefit.

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