Primary central nervous system lymphomas: retrospective analysis of 10 cases.

Abstract

Purpose: Primary central nervous system (CNS) lymphoma, a rare form of non-Hodgkin's lymphoma (NHL), is defined as high-grade B cell neoplasm of aggressive extranodal CNS. The incidence of CNS lymphoma has been increasing in recent years. In general, it has a poor prognosis. The aim of this study is to evaluate the clinical, pathological, immunohistochemical and radiological features of patients with primary CNS lymphoma in the light of literature.

Materials and methods: Between May 2011 and January 2017, 10 patients with pathologic confirmation of primary CNS lymphoma were retrospectively reviewed.

Results: Of 10 patients with CNS lymphoma, 7 (70%) were female and 3 (30%) were male. The mean age of all patients at diagnosis was 59.1±13.98 years. The most common settlements; eight patients (80%) had basal ganglia and 3 patients (30%) had multiple intracranial masses. Sterotaxic brain biopsy was performed in 8 patients (80%) and tumor resection was performed by craniotomy in 2 patients (20%). All of the patients with non-Hodgkin B cell lymphoma had diffuse large B cell lymphoma in 10 (100%). All cases had diffuse and strong positivity for CD20. None of the patients had immunodeficiency.

Conclusion: Primary CNS lymphoma with a poor prognosis is frequently seen over 55 years of age. Diffuse large B-cell lymphoma is the most common subtype and basal ganglia is the most common site.

Keywords

• Non-Hodgkin lymphoma
• large B cell lymphoma
• Central nervous system
• primary lymphoma

Primer santral sinir sistemi lenfomaları: 10 vakanın retrospektif analizi.

Öz

Amaç: Non-Hodgkin lenfoma (NHL)’ nın nadir bir formu olan primer santral sinir sistemi (SSS) lenfoması, agresif ekstranodal SSS’nin yüksek gradlı B hücre neoplazmı olarak tanımlanır. SSS lenfoma insidansı son yıllarda giderek artmaktadır. Genel olarak kötü prognoza sahiptir. Bu çalışmada; primer SSS lenfoması tanısı alan hastaların klinik, patolojik, immünhistokimyasal ve radyolojik özellikleri literatür eşliğinde değerlendirmesi amaçlanmıştır.

Gereç ve yöntem: Mayıs 2011 ile Ocak 2017 tarihleri arasında primer SSS lenfoma tanısı patolojik olarak doğrulanmış 10 hasta retrospektif olarak incelendi.

Bulgular: SSS lenfomalı 10 hastanın 7 (%70)’si kadın 3 (%30)’ü erkekti. Tüm hastaların tanı konulduğundaki ortalama yaşları 59,1±13,98 idi. En sık yerleşim yeri; 8 hastada (%80) bazal ganglia bölgesi olup, 3 hastanın (%30) çoklu intrakranyal kitleleri vardı. 8 hastada (%80) sterotaksik beyin biyopsisi, 2 hastada (%20) kranyotomi le tümör rezeksiyonu yapıldı. Tümü non-Hodgkin B hücreli lenfoma olan hastaların 10’u da (%100) diffüz büyük B hücreli lenfoma idi. Tüm vakalarda CD20 için diffüz ve kuvvetli pozitiflik görüldü. İmmün yetmezlik hiçbir hastada yoktu.

Sonuç: Kötü prognoza sahip olan primer SSS lenfoması sıklıkla 55 yaşın üzerinde görülür. Diffüz büyük B-hücreli lenfoma en sık alt tip olup bazal ganglia bölgesi en sık yerleşim yeridir.

Anahtar Kelimeler

• Non-Hodgkin lenfoma
• santral sinir sistemi
• primer lenfoma
• büyük B hücreli lenfoma

Kaynakça

1. 1. Wang CC, Carnevale J, Rubenstein JL. Progress in central nervous system lymphomas. British journal of haematology. 2014 Aug;166(3):311-25. DOI: 10.1111/bjh.12938
2. 2. Bhagavathi S, Wilson JD. Primary central nervous system lymphoma. Archives of pathology & laboratory medicine. 2008 Nov;132(11):1830-4. DOI: 10.1043/1543-2165-132.11.1830
3. 3. Brastianos PK, Batchelor TT. Primary central nervous system lymphoma: overview of current treatment strategies. Hematology/oncology clinics of North America. 2012 Aug;26(4):897-916. DOI: 10.1016/j.hoc.2012.05.003
4. 4. Haque S, Law M, Abrey LE, Young RJ. Imaging of lymphoma of the central nervous system, spine, and orbit. Radiologic clinics of North America. 2008 Mar;46(2):339-61, ix. DOI: 10.1016/j.rcl.2008.04.003
5. 5. Nayak L, Batchelor TT. Recent advances in treatment of primary central nervous system lymphoma. Current treatment options in oncology. 2013 Dec;14(4):539-52. DOI: 10.1007/s11864-013-0252-6
6. 6. Perini GF, Campregher PV, Santos FP, Hamerschlak N. Primary central nervous system lymphoma: what a neurologist/neurosurgeon should know? Arquivos de neuro-psiquiatria. 2013 Apr;71(4):254-7. DOI: 10.1590/0004-282x20130011
7. 7. Phillips EH, Fox CP, Cwynarski K. Primary CNS lymphoma. Current hematologic malignancy reports. 2014 Sep;9(3):243-53. DOI: 10.1007/s11899-014-0217-2
8. 8. Rubenstein J, Ferreri AJ, Pittaluga S. Primary lymphoma of the central nervous system: epidemiology, pathology and current approaches to diagnosis, prognosis and treatment. Leukemia & lymphoma. 2008;49 Suppl 1:43-51. DOI: 10.1080/10428190802311441

9. 9. Yoon JH, Kang HJ, Kim H, Lee JW, Park JD, Park KD, et al. Successful treatment of primary central nervous system lymphoma without irradiation in children: single center experience. Journal of Korean medical science. 2012 Nov;27(11):1378-84. DOI: 10.3346/jkms.2012.27.11.1378
10. 10. Gallop-Evans E. Primary central nervous system lymphoma. Clinical oncology. 2012 Jun;24(5):329-38. DOI: 10.1016/j.clon.2012.02.009
11. 11. Liu HL, Liu T, Ma C, Zhao Y, Zhou Y. [Clinical characteristics and prognosis of 20 cases of primary central nervous system lymphoma]. Zhongguo shi yan xue ye xue za zhi. 2015 Feb;23(1):111-8. DOI: 10.7534/j.issn.1009-2137.2015.01.021
12. 12. Bathla G, Hegde A. Lymphomatous involvement of the central nervous system. Clinical radiology. 2016 Jun;71(6):602-9. DOI: 10.1016/j.crad.2016.02.006
13. 13. Olson JE, Janney CA, Rao RD, Cerhan JR, Kurtin PJ, Schiff D, et al. The continuing increase in the incidence of primary central nervous system non-Hodgkin lymphoma: a surveillance, epidemiology, and end results analysis. Cancer. 2002 Oct 1;95(7):1504-10. DOI: 10.1002/cncr.10851
14. 14. Gasser O, Bihl FK, Wolbers M, Loggi E, Steffen I, Hirsch HH, et al. HIV patients developing primary CNS lymphoma lack EBV-specific CD4+ T cell function irrespective of absolute CD4+ T cell counts. PLoS medicine. 2007 Mar 27;4(3):e96. DOI: 10.1371/journal.pmed.0040096
15. 15. Gopal S, Patel MR, Yanik EL, Cole SR, Achenbach CJ, Napravnik S, et al. Temporal trends in presentation and survival for HIV-associated lymphoma in the antiretroviral therapy era. Journal of the National Cancer Institute. 2013 Aug 21;105(16):1221-9. DOI: 10.1093/jnci/djt158
16. 16. Beiko J, Suki D, Hess KR, Fox BD, Cheung V, Cabral M, et al. IDH1 mutant malignant astrocytomas are more amenable to surgical resection and have a survival benefit associated with maximal surgical resection. Neuro-oncology. 2014 Jan;16(1):81-91. DOI: 10.1093/neuonc/not159
17. 17. Weller M, Martus P, Roth P, Thiel E, Korfel A, German PSG. Surgery for primary CNS lymphoma? Challenging a paradigm. Neuro-oncology. 2012 Dec;14(12):1481-4. DOI: 10.1093/neuonc/nos159