이화여자대학교의과대학목동병원이비인후과학교실신승호 J Korean Skull Base Society 12 권 2 호 : 13~17, 2017 Treatment strategy of modified Fisch class A and B paragangliomas 종설1 종설2 원저1 원저2 증례1 증례2 증례3 증례4 증례5 증례6 증례7 증례8 증례9 Department of Otorhinolaryngology, College of Medicine, Ewha Womans University, Seoul, Republic of Korea Seung-Ho Shin Though paragangliomas are benign tumors which originate from the jugular bulb or tympanic 교신저자 Seung-Ho Shin 논문접수일 : 2017 년 8 월 5 일논문완료일 : 2017 년 8 월 25 일주소 : Department of Otorhinolaryngology, Ewha Womans University Mokdong Hospital, 1071, Anyangcheon-ro, Yangcheon-gu, Seoul 07985, Korea Tel : +82-2-2650-2942 Fax : +82-2-2648-5604 E-mail : drshinsh@gmail.com cavity, it is not easy to remove them due to their vascularity. Because the treatment for tympanic paragangliomas is not well-established, their classification and treatment strategy will be described. Tympanic paragangliomas are classified as A1, A2, B1, B2 and B3 through modified Fisch classification. A transcanal approach is recommended for A1 tumor. A retroauricular transmastoid approach is preferred for A2 tumor. For B1 tumor, a canal wall up mastoidectomy and facial recess approach supplies a sufficient surgical field. For B2 tumor, an extended facial recess approach is required. For B3 tumor, subtotal petrosectomy is essential. Key Words Ear Paraganglioma Vascular neoplasms 13
INTRODUCTION 부신경절종 (paraganglioma) 은측두골의사구소체에서기원한양성종양으로경정맥구상단에위치한다. [1, 2] 이런사구소체는고실에도존재하는데고실에서기원한부신경절종은박동성이명을유발할수있다. 이학적검사상고막안쪽에붉은색의덩어리로관찰된다. 이종양은서서히자라하고실의함기화세포를침습하게되며유양동으로진행되기도하며앞쪽으로는내경동맥관을침범하게된다. Sanna 등 [1] 은이종양의분류를제시하였다 (Table 1 and Fig. 1). 이분류는 Fisch [3] 에의해부신경절종이 4가지로분류가되었는데그것은 Class A, B, C, D이며이를세분화한것이 Sanna의 modified Fisch classification이다. Class A의종양은 하고실의침범없이종양이중이내에국한된경우이며 A1는이경으로완전히관찰되는경우, A2는이관이나후중고실로종양이커져서이경으로종양전체가보이지않는경우를말한다. Class B의경우종양이경정맥구의침범없이하고실이나유양동으로진행된경우로 B1은종양이하고실에침습된경우이며, B2는하고실과유양동이침습된경우이며 B3는종양에의해내경동맥관에미란이생긴경우이다. MAIN SUBJECT Class A와 B 종양은 Class C의종양처럼종양의범위가광범위하지않기때문에 Table 1의분류에따른각기다른접근법을이용 Table 1. Modified Fish classification of tympanic and tympanomastoid paragangliomas Class A B A1 A2 B1 B2 B3 Description Tumors limited to the middle ear cleft without invasion of the hypotympanum Tumors completely visible on otoscopic examination Tumor margins are not visible on otoscopy. Tumor may extend anteriorly to the Eustachian tube and/or to the posterior mesotympanum Tumors limited to the tympanomastoid compartment of the temporal bone without erosion of the jugular bulb Tumors confined to the middle ear cleft with extension to the hypotympanum Tumors involving the middle ear cleft with extension to the hypotympanum and the mastoid Tumors confined to the tympanomastoid compartment with erosion of the carotid canal [Reprinted from "Microsurgery of skull base paragangliomas", by Sanna M, Piazza P, Shin SH, Flanagan S, and Mancini F, Thieme, Stuttgart, DE, Copyrihgt 2013 by the Thieme. Reprinted with permission]. A Fig. 1 B C D E Illustrations of modified Fish classification of tympanic and tympanomastoid paragangliomas. (A) Class A1, (B) A2, (C) B1, (D) B2, and (E) B3. FN: facial nerve, ICA: internal carotid artery, JB: jugular bulb, SS: sigmoid sinus, T: tumor. 14 JOURNAL OF KOREAN SKULL BASE SOCIETY SEPTEMBER Vol. 12 No. 2
해종양의제거를할수있다. 특히종양이크기않기때문에안면신경기능이나내이의기능을보존하면서적절한시야를유지하여출혈성경향이있는부신경절종의제거가가능한접근법의선택이필요하다. 1. Class A1 Tumor 종양전체가이경에보이는경우로대개경외이도접근법 (transcanal approach) 으로종양제거가가능하다. 부분마취하에가장큰이경 (ear speculum) 을넣고외이도피판을 U자형으로만들어거상후중이를노출하게됨. 외이도가작다면후이개접근법을이용한외이도확장술을같이시행할수있다. 종양의노출 이충분히이루어지기전에출혈은서지셀 (Surgcel ; Johnson & Johnson, Somerville, NJ, USA) 과솜 (cottonolids) 을이용하여조절하게된다. 종양을이소골이나고삭신경그리고고막으로부터분리를조심스럽게한후충분한노출이이루어지면, 소작기 (bipolar coagulator) 를이용하여종양의맥관질 (vascularity) 을줄여출혈을방지한다 (Fig. 2). 종양을하고실에서완전히분리하여제거후남은공간에는젤폼 (Gelfoam ; Pharmacia & Upjohn Company, Kalamazoo, MI, USA) 으로채우게된다. 2. Class A2 Tumor Class A2 종양은크기로인하여경외이도접근법을통해제거하 Fig. 2 Fig. 3 Illustration of electrocauterization for class A1 tumor via transcanal approach. Illustration of retroauricular approach for class A2. Fig. 4 A B C Illustration of class B1 and B2 tumor removal. (A) Tumor removal of posterior tympanostomy, (B) extended posterior tympanostomy, and (C) tumor removal of extended posterior tympanostomy. 15
기어렵다. 이에후이개접근법을이용하여제거하는것이바람직하다. 대개 Class A2 종양은크기로인하여이소골을침범할수있다. 후이개절개를한후외이도절개를원형으로한후고실외이도피판 (tympanomeatal flap) 과고막을골부외이도와골성고실륜, 추골로부터분리하여통째로떼어내어 (Fig. 3) 생리식염수에보관을하고, 필요시외이도확장술을시행한다. 충분한시야확보후에종양의하연부터소작기를이용하여소작하면서둔적박리 (blunt dissection) 를시행하여종양을수축시키고조금씩가동시킨다. 종양을떼기적당할때까지수축시킨후중이에서종양을꺼낸후빈공간은젤폼으로채운후분리했던고실외이도피판과고막을원위치시킨후젤폼으로고정하면수술이종료된다. 3. Class B1 and B2 Tumor Class B1과 B2 종양은하고실과고실동 (tympanic sinus) 을침범하기때문에종양제거에후이개경유양동접근법 (retroauricular transmastoid approach) 이필요하다. Class B1 의경우일반적인후고실개방술 (posterior ) 로종양의소작이가능하지만, Class B2의경우확장된후고실개방술 (extended posterior ) 를통해종양의제거가가능하다 (Fig. 4A). 외이도와안면신경와 (facial recess) 양쪽을통해적절히종양을소작하고가동시켜크기가제거에적당할때꺼내게된다 (Fig. 5). 다만 Class B2 종양의경우하고실로의확장으로인해안면신경와를조금더크게열어수술시야를확보하는것이필요하게된다 (Fig. 4B, C). 안면신경와와안면신경밑으로그리고외이도를통해종양을소작할수있게된다. 만약경정맥구로의종 양의확장이관찰되는경우, 종양을다떼어내지못해도수술을종 료해야한다. 4. Class B3 Tumor Class B3 종양의경우, 종양에의해내경동맥관의미란이유발 된경우로, 대개안면신경밑과유양동에도종양이침범된다. 종양 의광범위한침범이있고경정맥구나내경동맥의시야확보를위해 추체아전절제술즉, 외이도피부와고막을제거하고외이도골벽을 내려개방성유양동절제술을시행한후에외이도를꿰매서막는방 법이필요하다 (Fig. 5). 물론추골과침골은제거하고필요에따라 등골의상부구조도제거될수있다. 종양은소작을통한수축과섬 세한절제술을통해주요혈관과안면신경의손상없이제거하는것 이포인트라고할수있다. 종양이제거되면빈공간은복부의지방 조직으로채우게된다. Fig. 6 Class A1 Transcanal approach Class A Class A2 Retroauricular-transcanal approach Surgical strategy for class A paragangliomas. [Reprinted from "Microsurgery of skull base paragangliomas", by Sanna M, Piazza P, Shin SH, Flanagan S, and Mancini F, Thieme, Stuttgart, DE, Copyrihgt 2013 by the Thieme. Reprinted with permission]. Fig. 5 Fig. 7 Class B Class B1 Class B2 Class B3 Illustration of approach for class B3. SS: sigmoid sinus, JB: jugular bulb, TMJ: temporomandibular joint, ICA: internal carotid artery. Canal wall up mastoidectomy with Posterior Canal wall up mastoidectomy Posterior Subfacial recess Subtotal petrosectomy with mastoid obliteration Surgical strategy for class B paraganglioma. [Reprinted from "Microsurgery of skull base paragangliomas", by Sanna M, Piazza P, Shin SH, Flanagan S, and Mancini F, Thieme, Stuttgart, DE, Copyrihgt 2013 by the Thieme. Reprinted with permission]. 16 JOURNAL OF KOREAN SKULL BASE SOCIETY SEPTEMBER Vol. 12 No. 2
5. Treatment strategy of Class A and B Tumor Class A와 B의치료전략에대해서 Figure 6과 7과같다. Class A1인경우경외이도접근법을 Class A2인경우에는후이개경유양동접근법을통해종양의적출이가능하며 Class B1인경우, 폐쇄형유양동절제술및후고실개방술을이용하여그리고 Class B2인경우 Class B1의접근법에후고실개방술을확장하여종양의제거가가능하다. Class B3인경우주요혈관을보존하고안정하게종양을제거하기위해추체아전절제술을통해종양을제거할수있다. References 1. Sanna M, Piazza P, Shin SH, Flanagan S, Mancini F. Microsurgery of skull base paragangliomas. Stuttgart, DE: Thieme; 2013. 2. O'Leary MJ, Shelton C, Giddings NA, Kwartler J, Brackmann DE. Glomus tympanicum tumors: a clinical perspective. Laryngoscope 1991;101:1038-43. 3. Fisch U. Infratemporal fossa approach for glomus tumors of the temporal bone. Ann Otol Rhinol Laryngol 1982;91:474-9. CONCLUSION 부신경절종중 Class C와달리 Class A와 Class B의경우종양의침습정도가적기때문에 modified Fisch 분류에맞는적절한접근법선택을한다면합병증없이안전하고완벽한종양의제거가가능할것이다. 17