JNS-13766; No of Pages 2 Journal of the Neurological Sciences xxx (2015) xxx–xxx

Contents lists available at ScienceDirect

Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns

Letter to the Editor Differentiating primary central nervous system (CNS) lymphoma from “butterfly glioma”: A case report Keywords: Primary central nervous system lymphoma Glioblastoma Brain biopsy

1. Introduction While in immunocompetent patients the butterfly appearance of a brain tumor is highly suggestive of glioblastoma multiforme, primary CNS lymphoma (PCNSL) can mimic this pattern in immunocompromised patients. We present a case of PCNSL proven by brain biopsy in a patient with acquired immunodeficiency syndrome (AIDS).

periventricular and callosal location, is one of the rare exceptions that may mimic the neuroimaging characteristics of “butterfly glioma” [2,3]. Whereas in T2 imaging there is typical butterfly glioma presentation, contrast enhancement is not as diffuse as in glioma which, generally, is of more disruptive nature. Especially in immunocompromised patients there is a characteristic ring enhancement as the tumor grows rapidly, outstripping its blood supply and becoming necrotic centrally [4]. Left posterior lateral ventricle also exhibits ependymal enhancement, a further element in favor of PCNS. CSF EBV PCR is also useful as in AIDS patients PCNSL is almost always associated to EBV [5]. However, stereotactic brain-biopsy is the only diagnostic modality that can reliably differentiate the two tumors and exclude metastatic disease as well as non-neoplastic masses such as toxoplasmosis, especially in AIDS patients, or tumefactive demyelination in immunocompetent patients [2,3]. Treatment consists of whole brain irradiation, methotrexate-based chemotherapy and antiviral drugs that can lower viral load [6].

Authorship contribution statement 2. Case report A previously healthy 51-year-old man presented with progressive dizziness and gait instability. Neurologic examination revealed ataxic gait and gazed-evoked nystagmus more accentuated when looking down with upward gaze limitation. Laboratory examination showed moderate thrombocytopenia (104,000; normal values 150– 440,000/μL) and lymphocytopenia (860/μL; normal values 1200– 3500/μL). Brain CT scan was normal but brain-MRI findings (Fig. 1; Panels A & B) were consistent with “butterfly glioma”. Lumbar puncture showed marked lymphocytosis (2224/μL; normal values 0–5/μL), increased protein (2.42 g/L; normal values 0.15–0.45 g/L), while PCR for Epstein–Barr virus (EBV) showed 62,700/mL copies of the virus in the CSF. Blood serology was positive for HIV-1. CD4 count was low (132/μL; normal values 410–1590/μL). Pulmonary CT scan was normal and careful clinical examination excluded lymphadenopathy that would suggest non-Hodgkin lymphoma and secondary CNS involvement. One week after admission the patient deteriorated rapidly and showed signs of intracranial hypertension on brain CT and had an external ventricular drain. Stereotactic brain-biopsy revealed a B-cell cerebral lymphoma (Fig. 1; Panels C & D). The patient continued to deteriorate despite IV treatment with corticosteroids and ganciclovir, went into a coma and deceased from diffuse cerebral edema a week after biopsy. Autopsy was declined by the patient's family. 3. Discussion Diffuse invasion of neoplastic cells among nerve fibers in the white matter of the brain is almost unique in aggressive gliomas and gives the characteristic appearance of “butterfly glioma” when the tumor crosses the midline via the corpus callosum [1]. PCNSL, with its common

Andrey Bragin: Data collection and critical comments during manuscript revision. Apostolos Safouris: Study design, data collection, drafting and revising the manuscript. Bram Bourgonjon: Critical comments during manuscript revision. Alex Michotte: Data collection, critical comments during manuscript revision. Nikos Triantafyllou: Critical comments during manuscript revision. Georgios Tsivgoulis: Study design, drafting and revising the manuscript.

Study funding Dr Georgios Tsivgoulis has been supported by European Regional Development Fund — Project FNUSA-ICRC (No. CZ.1.05/1.1.00/02.0123).

Disclosures Dr Bragin reports no disclosures. Dr Safouris reports no disclosures. Dr Bourgonjon reports no disclosures. Dr Michotte reports no disclosures. Dr Triantafyllou reports no disclosures. Dr Tsivgoulis reports no disclosures.

Acknowledgments None.

http://dx.doi.org/10.1016/j.jns.2015.04.037 0022-510X/© 2015 Elsevier B.V. All rights reserved.

Please cite this article as: Bragin A, et al, Differentiating primary central nervous system (CNS) lymphoma from “butterfly glioma”: A case report, J Neurol Sci (2015), http://dx.doi.org/10.1016/j.jns.2015.04.037

2

Letter to the Editor

Fig. 1. Brain-MRI showing a confluent periventricular hyperintense lesion involving the genu of the corpus-callosum (A, axial FLAIR-sequence) with gadolinium-enhancement (B, axial gadolinium-enhanced T1-sequence). Brain-biopsy with CD20 immunohistochemical staining (original magnification ×400) showing strong CD-20 immunopositivity of tumoral cells. (D) Brain-biopsy with CD3 immunohistochemical staining (original magnification ×400) showing scattered inflammatory lymphocytes.

References [1] Claes A, Idema AJ, Wesseling P. Diffuse glioma growth: a guerilla war. Acta Neuropathol 2007;114:443–58. [2] Yap KK, Sutherland T, Liew E, Tartaglia CJ, Pang M, Trost N. Magnetic resonance features of primary central nervous system lymphoma in the immunocompetent patient: a pictorial essay. J Med Imaging Radiat Oncol 2012;56:179–86. [3] Ricard D, Idbaih A, Ducray F, Lahutte M, Hoang-Xuan K, Delattre JY. Primary brain tumours in adults. Lancet 2012;379:1984–96. [4] Tang YZ, Booth TC, Bhogal P, Malhotra A, Wilhelm T. Imaging of primary central nervous system lymphoma. Clin Radiol 2011;66:768–77. [5] Cinque P, Brytting M, Vago L, et al. Epstein–Barr virus DNA in cerebrospinal fluid from patients with AIDS-related primary lymphoma of the central nervous system. Lancet 1993;342:398–401. [6] Bossolasco S, Falk KI, Ponzoni M, et al. Ganciclovir is associated with low or undetectable Epstein–Barr virus DNA load in cerebrospinal fluid of patients with HIVrelated primary central nervous system lymphoma. Clin Infect Dis 2006;42: e21–5.

Andrey Bragin Stroke Unit, Brugmann University Hospital, Brussels, Belgium Apostolos Safouris⁎ Stroke Unit, Brugmann University Hospital, Brussels, Belgium Second Department of Neurology, University of Athens, School of Medicine, “Attikon” University Hospital, Athens, Greece

Corresponding author at: Place Van Gehuchten 4, 1020 Bruxelles. Tel.: + 32 4773281; fax: + 32 4773467. E-mail address: [email protected] Bram Bourgonjon Department of Neurosurgery, UZ Brussel, Brussels, Belgium Alex Michotte Department of Neuropathology, UZ Brussel, Brussels, Belgium Nikos Triantafyllou First Department of Neurology, University of Athens, School of Medicine, “Eginition” University Hospital, Athens, Greece Georgios Tsivgoulis Second Department of Neurology, University of Athens, School of Medicine, “Attikon” University Hospital, Athens, Greece International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic 5 March 2015 Available online xxxx

Please cite this article as: Bragin A, et al, Differentiating primary central nervous system (CNS) lymphoma from “butterfly glioma”: A case report, J Neurol Sci (2015), http://dx.doi.org/10.1016/j.jns.2015.04.037

Differentiating primary central nervous system (CNS) lymphoma from "butterfly glioma": A case report.

Differentiating primary central nervous system (CNS) lymphoma from "butterfly glioma": A case report. - PDF Download Free
726KB Sizes 0 Downloads 12 Views