조영증강되지 않은 원발중추신경계림프종

Non-Enhancing Primary Central Nervous System Lymphoma

Article information

J Korean Neurol Assoc. 2019;37(4):440-441
Publication date (electronic) : November 1, 2019
doi : http://dx.doi.org/10.17340/jkna.2019.4.25
Department of Neurology, Veterans Health Service Medical Center, Seoul, Korea
윤운규, 박재영, 배희원, 강상준, 하상원, 이지혜, 이미지, 김은주
중앙보훈병원 신경과
Address for correspondence: Sang-Won Ha, MD Department of Neurology, Veterans Health Service Medical Center, 53 Jinhwangdo-ro 61-gil, Gangdong-gu, Seoul 05368, Korea Tel: +82-2-2225-4305 Fax: +82-2-2225-1324 E-mail: hippocam@naver.com
received : June 26, 2019 , rev-recd : August 23, 2019 , accepted : August 23, 2019 .

74세 남자가 일주일 전부터 집을 못 찾고, 비밀번호를 잊어 내원하였다. T2강조영상에서 왼쪽 내측두엽에 고신호강도 병변이 있었고 이 병변은 조영증강되지 않았다. 뇌척수액검사는 단백질 상승(51.1 mg/dL) 이외에는 정상이었고, 면역기능 저하를 유발할 만한 특이한 병력은 없었다. Acyclovir를 2주간 정맥 주사하였으나 추적 뇌영상에서 병변 크기가 증가하였고, 각종 신생물딸림항체검사는 모두 정상이었다. 흉복부 computed tomography상 원발암은 확인되지 않았으나 신생물딸림증후군이 배제되지 않아 면역글로블린을 투약하였다. 4주후 시행한 뇌영상에서 뇌섬엽, 기저핵주변백질에도 병변이 확산되었으나 여전히 조영증강은 되지 않았다(Fig. A-F). 확산강조영상에서 경미한 고신호강도 병변이 확인되었고(Fig. G), 기울기에코영상은 정상이었다(Fig. H, I). 조직검사를 시행하였고 원발중추신경계림프종(diffuse B cell lymphoma)으로 진단하였다(Fig. J, K). 환자는 혈액종양내과 전과 이후 한 달 만에 사망하였다.

Figure.

(A-C) Brain magnetic resonance images (MRI) at 4 weeks after symptom onset of a rapidly progressive dementia. MRI T2-weighted flair images show hyperintensities in the left internal temporal lobe, basal ganglia and thalamus. T2 hyperintensities were extended to the frontal lobes and parietal lobes on the follow-up brain MRI which are performed two months later. (D-F) T1-weighted enhenced images show contrast enhancement at one month after cognitive impairment. (G) Diffusion weighted images show high signal intensity in the left internal temporal lobe in the emergency department. (H, I) The patient has no abnormal findings in the gradient echo imaging taken at a same time. (J) Histopathologically, atypical lymphocytes are diffusely infiltrated in brain parenchyma (hematoxylin and eosin stain, ×200). (K) The atypical lymphocytes are positive for CD20 on immunohistochemistry, which supports the diagnosis of diffuse large B cell lymphoma (×100)

병변이 magnetic resonance imaging에서 조영증강을 보이지 않는 원발중추신경계림프종은 극히 드물다[1,2]. 322명의 원발중추신경계림프종 환자를 분석한 한 연구에 따르면 그중 한 명만 조영증강을 보이지 않았다. 조영증강이 되지 않는다고 하여 원발중추신경계림프종을 완전히 배제할 수는 없다.

References

1. Lachenmayer ML, Blasius E, Niehusmann P, Kovacs A, Stuplich M, Eichler O, et al. Non-enhancing primary CNS lymphoma. J Neurooncol 2011;101:343–344.
2. Jahnke K, Schilling A, Heidenreich J, Stein H, Brock M, Thiel E, et al. Radiologic morphology of low-grade primary central nervous system lymphoma in immunocompetent patients. AJNR Am J Neuroradiol 2005;26:2446–2454.

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Figure.

(A-C) Brain magnetic resonance images (MRI) at 4 weeks after symptom onset of a rapidly progressive dementia. MRI T2-weighted flair images show hyperintensities in the left internal temporal lobe, basal ganglia and thalamus. T2 hyperintensities were extended to the frontal lobes and parietal lobes on the follow-up brain MRI which are performed two months later. (D-F) T1-weighted enhenced images show contrast enhancement at one month after cognitive impairment. (G) Diffusion weighted images show high signal intensity in the left internal temporal lobe in the emergency department. (H, I) The patient has no abnormal findings in the gradient echo imaging taken at a same time. (J) Histopathologically, atypical lymphocytes are diffusely infiltrated in brain parenchyma (hematoxylin and eosin stain, ×200). (K) The atypical lymphocytes are positive for CD20 on immunohistochemistry, which supports the diagnosis of diffuse large B cell lymphoma (×100)