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1 검안및콘택트렌즈학회지 2017 년제 16 권제 4 호 Ann Optom Contact Lens 2017;16(4): ISSN (Print) ISSN (Online) Case Report 전체눈근육마비로처음진단된원발성뇌하수체림프종 1 예 Primary Pituitary Lymphoma with Complete Ophthalmoplegia as the 1st Manifestation 송석현ㆍ염혜리 Seok Hyeon Song, MD, Hae Ri Yum, MD 건양대학교의과대학안과학교실 Department of Ophthalmology, Konyang University College of Medicine, Daejeon, Korea Purpose: To report a patient with total ophthalmoplegia as the 1st manifestation due to a primary pituitary lymphoma (PPL). Case summary: A 62-year-old woman visited the ophthalmology clinic with 2-week history of right upper eyelid ptosis and binocular diplopia. Ophthalmologic evaluation showed a 7 mm dilated pupil in the right eye, relative afferent pupillary defect (RAPD), exotropia of 70 prism diopters in primary position and eye movement limitation in adduction (-4), elevation (-4), depression (-4), and abduction (-2) of the right eye. The patient suffered from right facial pain and headache, but no definite other systemic symptoms were found. Brain magnetic resonance imaging showed the pituitary macroadenoma. Trans-sphenoidal surgery was performed and the pathology confirmed an infiltration of the pituitary gland by a diffuse large B-cell lymphoma. A bone marrow biopsy, abdomen, pelvis, chest computed tomography, and total body positron emission tomography computed tomography confirmed the absence of systemic involvement and thus the diagnosis of PPL was posed. The postoperative course was without complications. The patient underwent treatment with systemic chemotherapy. By 2 months postoperatively, the patient had complete resolution of the ptosis, relative afferent pupillary defect, pupil dilatation, diplopia, exotropia and eye movement limitation, and the best corrected visual acuity was approved. Conclusions: Although primary pituitary lymphoma hardly occurs, ophthalmic symptoms like ophthalmoplegia, diplopia, and ptosis can be the first sign of primary pituitary lymphoma. The patient can show recovery from ophthalmic symptoms and nervous symptoms with appropriate diagnosis and treatment. Ann Optom Contact Lens 2017;16(4): Key Words: Diffuse large B-cell lymphoma; Ophthalmoplegia; Primary pituitary lymphoma 원발성뇌하수체림프종 (primary pituitary lymphoma) 은전신적인침범없이뇌하수체및안장주변에국한된림프종을말하며, 이는안장에생긴종양의 1% 미만을차지하는매우드문질환으로알려져있다. 1,2 위험인자로는후천면 Received: Revised: Accepted: Address reprint requests to Hae Ri Yum, MD Department of Ophthalmology, Konyang University Hospital, #158 Gwanjeodong ro, Seo-gu, Daejeon 35365, Korea Tel: , Fax: smile-ri@hanmail.net 역결핍증후군과면역억제제의투여, 항암치료및방사선치료로인한면역력의저하, 뇌하수체호르몬의과다분비, 뇌하수체염, 뇌하수체선종등이알려져있다. 1,3-6 원발성뇌하수체림프종은병변의발생위치및침범범위에따라두통, 안와통증, 눈운동장애, 시력저하, 시야장애, 동공반응이상, 뇌하수체기능저하등의다양한증상을보인다. 1,7 저자들은우안의눈꺼풀처짐과두눈복시를주소로내원한 62 세여자환자에서진단된원발성뇌하수체림프종 1예를경험하였기에이를보고하고자한다. Copyright 2017, The Korean Optometry Society The Korean Contact Lens Study Society Annals of Optometry and Contact Lens is an Open Access Journal. All articles are distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 154

- 송석현ㆍ염혜리 : 원발성뇌하수체림프종 1 예 - 증례 62세여자환자가내원 2주전부터시작된우안의눈꺼풀처짐과두눈복시를주소로내원하였다. 환자는고혈압이외의기저질환및과거력은없었다. 최대교정시력은우안 0.5, 좌안 1.0이었고, 우안의동공이 7 mm로커진상태로동공대광반사가감소되어있었으며, 상대구심동공운동장애가관찰되었다.

2 - 송석현ㆍ염혜리 : 원발성뇌하수체림프종 1 예 - 증례 62세여자환자가내원 2주전부터시작된우안의눈꺼풀처짐과두눈복시를주소로내원하였다. 환자는고혈압이외의기저질환및과거력은없었다. 최대교정시력은우안 0.5, 좌안 1.0이었고, 우안의동공이 7 mm로커진상태로동공대광반사가감소되어있었으며, 상대구심동공운동장애가관찰되었다. 교대프리즘가림검사에서 70프리즘디옵터의우안외사시소견과함께안구운동검사에서우안의 -4의내전, 상전, 하전장애및 -2의외전장애소견을보였다 (Fig. 1). 안저검사에서망막및시신경에특이소견은관찰되지않았다. 우측안면통증및두통을호소하였으나의식은명료하였고, 그외전신적이상소견은보이지않았다. 뇌자기공명영상및뇌혈관조영술에서우측해면정맥동및속목동맥을침범한뇌하수체거대선종이관찰되었다 (Fig. 2). 수술전시행한뇌하수체자극검사 (cocktail test) 에서혈중프로락틴이 ng/ml로증가되어있었고, 일반혈액검사에서 Lactate dehydrogenase가 1174 IU/L로증가되어있었다. 후천면역결핍증후군바이러스검사를포함한다른혈액검사에서특이사항은없었다. 이후본원신경외과에서경접형동접근법으로뇌하수체선종제거술을시행하였다. 조직검사결과면역조직화학염색에서 B림프구표지자인 CD20에양성반응을보였고 (Fig. 3), Revised European American Lymphoma (REAL) 분류상미만성거대 B세포림프종 (diffuse large B-cell lymphoma, DLBCL) 으로진단하였다. 항암치료전병기설정을위한흉부, 복부및골반컴퓨터단층촬영과뇌척수액검사, 양전자방출단층촬영검사상신체의다른부위에서종양의침범소견은보이지않아원발뇌하수체림프종으로진단하였고, rituximab, methotrexate, procarbazine, vincristine 전신항암치료를시행하였다. 수술 2개월후우안의눈꺼풀처짐, 동공산대, 상대구심동공운동장애및눈운동장애가완전히회복되었고 (Fig. 4), 최대교정시력은우안 0.8로호전되었다. 고찰 원발성뇌하수체림프종은전신적인침범없이뇌하수체및안장주변에국한된림프종을말하며, 지난 20년동안약 20예만보고되었을정도로발생률이매우낮은질환이다. 1,2 호발연령은 50세전후이며, 발생률이여성보다남성에서약 2배더높은것으로알려져있다. 1,4,7 조직학적으로대부분비호지킨성림프종이며 REAL 분류상대부분이 CD20 양성을보이는미만성거대 B세포림프종 (DLBCL) 이다. 1,2,8,9 원발성뇌하수체림프종은주로반구의뇌실주변백색질에서발생하며, 뇌신경에서기원하여발생하기도한다. 10 원발성뇌하수체림프종의발생원인은아직정립된이론은없으나여러가지가설이제시되고있다. 후천면역결핍증후군, 면역억제제투여, 항암치료및방사선치료에의한면역 Figure 1. At the first visit, the patient had a severe ptosis and eye movement limitation in adduction (-4), elevation (-4), depression (-4), and abduction (-2) of the right eye. 155

- 검안및콘택트렌즈학회지 2017 년제 16 권제 4 호 - 저하시바이러스의중추신경계침범, 성장호르몬, 프로락틴, 난포자극호르몬과같은뇌하수체호르몬, 만성염증, 뇌하수체선종에의한림프구의증식이그원인으로제시되고있다. 1,3-6 원발성뇌하수체림프종의약 70% 에서뇌하수체기능저하소견을보이며, 30% 에서는뇌하수체줄기를압박하는덩이효과로고프로락틴혈증을보인다고알려져있다.

3 - 검안및콘택트렌즈학회지 2017 년제 16 권제 4 호 - 저하시바이러스의중추신경계침범, 성장호르몬, 프로락틴, 난포자극호르몬과같은뇌하수체호르몬, 만성염증, 뇌하수체선종에의한림프구의증식이그원인으로제시되고있다. 1,3-6 원발성뇌하수체림프종의약 70% 에서뇌하수체기능저하소견을보이며, 30% 에서는뇌하수체줄기를압박하는덩이효과로고프로락틴혈증을보인다고알려져있다. 7 원발성뇌하수체림프종은뇌하수체주변조직으로병변이침범하여안장, 해면정맥동, 안와첨부까지영향을주는경우가많으며증상은병변의발생위치및침범범위에따라다양하게나타난다. 원발성뇌하수체림프종이안장에한정된경우뇌하수체기능저하와시야장애를일으키며, 해면정맥동을침범한경우에는뇌신경마비와이로인한눈운동장애 소견을보이며, 안와첨부로침범한경우에는시력저하, 눈꺼풀처짐과안와통증을동반한다. 7 원발성뇌하수체림프종과관련된뇌신경마비는시신경, 동안신경, 외전신경에서흔하게나타나고, 삼차신경, 활차신경, 안면신경의마비는드문것으로알려져있다. 1,7 본증례의환자에서는뇌영상검사상병변이우측해면정맥동과속목동맥을침범하여우측동안신경을누르고있었다. 우측동안신경마비증상으로우안의눈꺼풀처짐, 눈운동장애및외사시가관찰되었고, 동안신경마비에의한속눈근육마비가동반되어우안의동공이 7 mm로커진상태로동공대광반사가감소된소견을보였다. 우안의시력저하와상대구심동공장애는뇌하수체림프종의시신경침범에의한증상일것으로생각되며, 병변의덩이효과로인하여혈중프로락틴수치가상승 A B C D Figure 2. Brain magnetic resonance imaging of the patient. Coronal T2-weighted images show a mass in the right pituitary gland with involvement of right cavernous sinus, encasement of right distal internal carotid artery (A) (arrow), compression and displacement of right third cranial nerve (B) (arrow). A coronal T1-weighted image shows a lesion occupying the right pituiray gland and infilitrating the right cavernous sinus with lateral and downward displacement of right carotid artery (C) (arrow). A sagittal image shows a lesion within pituitary gland enhanced by contrast (D) (arrow). 156

- 송석현ㆍ염혜리 : 원발성뇌하수체림프종 1 예 - A B C Figure 3. HE stain shows a diffuse infiltration of atypical lymphoid cells in pituitray gland parenchyma (A) (HE stain, 200), (B) (HE stain, 400).

4 - 송석현ㆍ염혜리 : 원발성뇌하수체림프종 1 예 - A B C Figure 3. HE stain shows a diffuse infiltration of atypical lymphoid cells in pituitray gland parenchyma (A) (HE stain, 200), (B) (HE stain, 400). Immunohistochemical stain against pan B-cell marker, the tumor cells shows diffuse strong immunoreactivity for CD20 (C) ( 400). 되었으나, 유즙분비의증상은보이지않았다. 원발성뇌하수체림프종의진단에중요한검사는뇌자기공명영상과조직검사이다. 7,11 환자가내원시두통, 안와통증, 눈운동장애, 시력저하, 시야장애, 동공반응이상등의증상을보이는경우눈병변뿐만아니라뇌병변의가능성을고려하여야하며특히, 동공반응이상을보이는경우뇌종양, 뇌동맥류, 뇌수막염등의뇌병변을평가하기위하여뇌자기공명영상을촬영하여야한다. 원발성뇌하수체림프종은뇌자기공명영상에서 T1 영상에서등강도, T2 영상에서저강도에서등강도신호를보이며, 이를통해해면정맥동과그주위의침범여부도확인할수있다. 뇌자기공명영상에서뇌하수체병변이보이면전신림프종의뇌전이, 두개인두종, 수막종, 뇌교종등과의감별을위해확진검사인조직검사를반드시시행하여야하고, 조직검사상뇌하수체림프종으로진단되면원발과이차림프종의감별, 전이여부, 병기설정을위해흉부, 복부및골반컴퓨터단층촬영, 양전자방출단층촬영, 골수검사를시행하여야한다. 7,9,12 원발성뇌하수체림프종은이전까지보고된사례가적어아직명확한치료방침이없고, 현재는원발성중추신경계 림프종에근거하여치료를시행하고있다. 영상검사에서뇌하수체종양이보이면종양제거술및조직검사를시행하여확진하고, 컴퓨터단층촬영, 양전자방출단층촬영, 골수검사등을통해원발과이차림프종을감별하고병기를설정하여항암치료및방사선치료여부를결정하여야한다. 원발성뇌하수체림프종은종양제거술후항암치료가주된치료로알려져있으나방사선치료를병행하는것이생존율을높이는지에대해서는여러이견이있다. Weller et al 10 은조직검사만시행한군에비해전절제술또는아전절제술을시행한군에서생존율이더높다고하였고, Tarabay et al 7 은원발성뇌하수체림프종환자의평균생존기간은 14.4개월이며, 방사선치료만시행한경우 9.5개월, 항암치료만시행한경우 11.9개월, 방사선치료와항암치료를병용한경우 17.6 개월로결과를보고한바있다. DeAngelis et al 13 은항암제중 methotrexate가예후를향상시키는효과가있다고하였으며, 국내에서도안구를침범한원발성중추신경계림프종에서유리체절제술후 5 cycle의고용량 methotrexate 전신주사및유리체강내주사, 뇌전체및침범안에방사선치료후종양이사라지고, 두통, 구어장애, 기억장애등의신경학 157

- 검안및콘택트렌즈학회지 2017 년제 16 권제 4 호 - Figure 4. By 2 months postoperatively, the patient had complete resolution of the ptosis and eye movement limitation. 적증상이호전되었던사례가보고된바있다.

5 - 검안및콘택트렌즈학회지 2017 년제 16 권제 4 호 - Figure 4. By 2 months postoperatively, the patient had complete resolution of the ptosis and eye movement limitation. 적증상이호전되었던사례가보고된바있다. 14 원발성뇌하수체림프종은해부학적특성상환자가시력저하, 시야결손, 눈꺼풀처짐, 눈운동장애와두눈복시등의안과적증상으로처음병원에내원할가능성이높은질환중의하나이다. 본증례에서는정확한진단및치료를통해환자가안검하수, 눈운동장애, 시력저하의안과적증상과두통, 안면부통증의신경학적증상을회복할수있었기에이를보고하는바이다. REFERENCES 1) Giustina A, Gola M, Doga M, Rosei EA. Clinical review 136: primary lymphoma of the pituitary: an emerging clinical entity. J Clin Endocrinol Metab 2001;86: ) Johnson BA, Fram EK, Johnson PC, Jacobowitz R. The variable MR appearance of primary lymphoma of the central nervous system: comparison with histopathologic features. AJNR Am J Neuroradiol 1997;18: ) Popovic V, Damjanovic S, Micic D, et al. Increased incidence of neoplasia in patients with pituitary adenomas. The Pituitary Study Group. Clin Endocrinol (Oxf) 1998;49: ) Matera L, Geuna M, Pastore C, et al. Expression of prolactin and prolactin receptors by non-hodgkin s lymphoma cells. Int J Cancer 2000;85: ) O Neal KD, Schwarz LA, Yu-Lee LY. Prolactin receptorgene expression in lymphoid cells. Mol Cell Endocrinol 1991;82: ) Costa O, Bouthet C, Sauvage P, et al. Age-dependent LH and FSH effect on the proliferation of women s peripheral blood lymphocytes in vitro. Int J Immunopharmacol 1990;12: ) Tarabay A, Cossu G, Berhouma M, et al. Primary pituitary lymphoma: an update of the literature. J Neuroncol 2016;130: ) Yoshida S, Morii K, Watanabe M, Saito T. Characteristic features of malignant lymphoma with central nervous system involvement. Surg Neurol 2000;53: ) Kaufmann TJ, Lopes MB, Laws ER Jr, Lipper MH. Primary sellar lymphoma: radiologic and pathologic findings in two patients. AJNR Am J Neuroradiol 2002;23: ) Weller M, Martus P, Roth P, et al. Surgery for primary CNS lymphoma? Challenging a paradigm. Neuro Oncol 2012;14: ) Katz BJ, Jones RE, Digre KB, et al. Panhypopituitarism as an initial manifestation of primary central nervous system non-hodgkin s lymphoma. EndocrPract 2003;9: ) Famini P, Maya MM, Melmed S. Pituitary magnetic resonance imaging for sellar and parasellar masses: ten-year experiencein 2598 patients. J Clin Endocrinol Metab 2011;96: ) DeAngelis LM, Seiferheld W, Schold SC, et al. Combination chemotherapy and radiotherapy for primary central nervous system lymphoma: Radiation Therapy Oncology Group Study J Clin Oncol 2002;20: ) Lee SH, Kim DJ, Kim IT. A case of primary central nervous system lymphoma with ocular involvement. J Korean Ophthalmol Soc 2005;46:

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<283732372D3733312920B4D9C3CAC1A120BCD2C7C1C6AEC4DCC5C3C6AEB7BBC1EEC0C720B3EBBEC8C0C720BDC3B7C2BAB8C1A4BFA120B4EBC7D120C0AFBFEBBCBA20C6F2B0A1283035292E687770> 대한안과학회지 제 49 권 제 5 호 2008 J Korean Ophthalmol Soc 49(5):727-731, 2008 DOI : 10.3341/jkos.2008.49.5.727 다초점 소프트콘택트렌즈의 노안의 시력보정에 대한 유용성 평가 김현경 1 김효명 2 정성근 1 가톨릭대학교 의과대학 성모병원 안과학교실 1, 고려대학교 의과대학 안암병원 안과학교실