DOI:10.5125/jkaoms.2011.37.1.72 신영민 1 최소영 1 김진욱 1 변기정 2 김진수 1 1 경북대학교치의학전문대학원구강악안면외과학교실, 2 울산대학교병원치과 Abstract (J Korean Assoc Oral Maxillofac Surg 2011;37:72-6) Polymorphous low-grade adenocarcinoma on hard palate: case report Young-Min Shin 1, So-Young Choi 1, Jin-Wook Kim 1, Ki-Jung Byeon 2, Chin-Soo Kim 1 1 Department of Oral and Maxillofacial Surgery, School of Dentistry, Kyungpook National University, Daegu, Korea 2 Department of Dentistry, Ulsan University Hospital, Ulsan, Korea Polymorphous low-grade adenocarcinomas (PLGA) are distinctive salivary gland neoplasms with a propensity to arise from the minor salivary glands. The most frequent location of PLGA is the palate, even though other locations have been described. Previously used terms for PLGA include lobular carcinoma and terminal duct carcinoma. Although the frequency of the tumor is unknown, the recognition of PLGA as an individual tumor has increased with the establishment of specific histopathological criteria characterizing the PLGA. The first choice of treatment is a wide surgical excision including the subjacent bone if necessary. The prognosis is generally good and the recurrence rate ranges from 17% and 22%. Distant metastases is unusual (9%) but occur mainly in the regional lymph nodes. This is a case report of a 67 year old female patient with PLGA who was treated with a wide excision by layers (2 stage) of the lesion including the surrounding bone. We present this case with a review of the relevant literature. Key words: Polymorphous low-grade adenocarcinomas, Excision by layers [paper submitted 2010. 9. 30 / revised 2011. 1. 12 / accepted 2011. 1. 28] Ⅰ. 서론 다형성저등급선암종 (polymorphous low-grade adenocarcinoma) 은주로소타액선에서발생하는타액선신생물이며, 다른위치에서발생하는경우도있으나, 주된부위는경구개부이다 1. 이병소는이전에는소엽성암종 (lobular carcinoma) 2, 종말관암종 (terminal duct carcinoma) 3 등의호칭으로불렸고, 예전엔이병소의발생빈도는잘알려져있지않았으나, 종양을특징짓는병리조직학적기준이확립되어이종양의발견이증가하고있으며, 여성에서자주발견되는양상을보인다 4. (2-4.6[ 여 ]:1[ 남 ]) 주로통증없이천천히자라는양상을보이며, 조직학적으로는변연이피막으로둘러싸여있지않은침윤성병소로알려져있다. 김진수 700-705 대구시중구삼덕동 2 가 188-1 번지경북대학교치의학전문대학원구강악안면외과학교실 Chin-Soo Kim Department of Oral and Maxillofacial Surgery, School of dentistry, Kyungpook National University 188-1 Samduk-dong 2ga, Jung-gu, Daegu, 700-705, Korea TEL: +82-53-600-7562 FAX: +82-53-426-5365 E-mail: cskim@knu.ac.kr 치료방법은필요한경우, 주위골을포함한광범위한외과적절제술이며수술후방사선치료를추천하기도한다. 예후는좋은편이며재발률은 17-22% 사이이다 5,6. 전이는 9% 정도로흔하진않으나주로국소적인림프절에전이되는것으로알려져있다 7. 본증례는 67 세여성환자에서구개부에발생한다형성저등급선암종병소를 1 차적으로먼저제거한후주위구개골을 2 차적으로제거하는수술법을이용하여비강및상악동내점막을용이하게보존함으로써구개부결손을예방하여양호한치료결과를얻었기에문헌고찰과함께보고하는바이다. Ⅱ. 증례보고 67 세여자환자로 2009 년 3 월 10 일본원에처음내원하셨으며, 입천장에생긴무통성의궤양성병소를호소하였다.(Fig. 1) 병소는상악구개부의우측에위치하고있었으며, 크기는직경 1 cm 정도로병소중간부위에궤양이있었다. 궤양의중심부는흰색을, 주변은붉은색을띠고있었으며, 촉진할때약간의통증을호소하였다. 구강외특이소견은관찰되지않았으며, 경부림프절의전이를의심할 72
만한경결감은찾을수없었다. 이병소는 5 개월전부터생기기시작하였고, 평상시에는통증은없었으나, 양치질하다가가끔씩피가나고불편감을느꼈다고하였다. 병소의크기변화는느끼지못하고계시다가, 내원 1 주일전에주위치과의원에서본원으로의내원을권유받아내원하셨다. 환자의체격및영양상태는양호하며, 골격이상, 피부질환등의소견도발견할수없었으며, 복용하는약물도없었다. 혈액이화학적검사에서의수치들도전부정상범주에속하였다. 가족력에서도특이한사항을발견할수는없었다. 술전파노라마및경부초음파검사에서병소와관련있는특이한소견은없었으며, 골스캔에서도구개골로의침윤을의심할만한소견은보이지않았다. Paranasal sinus-computed tomography (PNS-CT) 에서도특이한변화를보이는림프절및해부학적구조는없었고, positron emission tomography-computed tomography (PET-CT) 에서우측 Level II 부위에서전이가의심되는림프절이관찰되었으나원발병소가있는부위에는 fluorodeoxyglucose (FDG) 섭취는거의나타나지않았다.(Fig. 2) 임상소견에서악성종양으로잠정적진단후, 시행한절개생검술결과에서다형성저등급선암종으로확진하였다. 수술은구강내접근을통한병소주위의구개부의골을포함하는광범위한절제술을시행하기로결정하고, 구개점막부의결손이 2 차치유로회복이힘들다고예상될경우구개부회전피판술을시행하기로하였다. 2009 년 5 월 12 일비기관삽관을통해전신마취후구개부절개를시작으로, 병소경계에서 2 cm 거리를두고원형으로구개부연조직의절제후하방구개골을연조직절제경계를따라 2 차적으로절제하였다.(Figs. 3, 4) 절제부위를세척한후바셀린거즈를넣고상악에레진스플린트를장 Fig. 1. Ulcerative lesion on right palate was showed at first visit. Fig. 2. PET-CT: without significant focal FDG uptake in right palate.(pet-ct: positron emission tomography-computed tomography, FDG: fluorodeoxyglucose) Fig. 3. Excised mass of tumor and underlying bone. Fig. 4. Intraoral view of postoperation state. 73
J Korean Assoc Oral Maxillofac Surg 2011;37:72-6 Fig. 5. Ten months after surgery: operation site is unremarkable. Fig. 6. Histological overview.(h&e staining, original magnification 4 10) 착하여치유를유도하였다. 술후 10 개월이지난현재치유양상은양호하며, 다른조직으로의전이는발견되지않고있다.(Fig. 5) 조직병리학적소견으로는원형또는계란형의핵과작은핵소체를가진일정한세포들의군집들이증식되어있었고, 세포질은호산성을띠고있었다. 일부에선 adenoid 나 solid 한형태를띠고, 또다른일부에선 papillar, cribriform, ductal 형태를보였다. 신경주위로의침습양상이나, 골조직으로의침윤은관찰할수가없었다.(Fig. 6) Ⅲ. 고찰 다형성저등급선암종은소타액선에서생기는모든종양에서 7-11% 를차지하고, 악성종양중에 19-26% 를차지하고있으며, 구개부에서가장흔하게발생한다 8. 하지만입술, 혀, 협부, 구강저부위등에서도발생할수있으며, 아주간혹대타액선에서발생하기도한다 9-13. 수술부위에서의재발률은 9-33% 정도이며, 경부림프절로의전이는 6-35% 로알려져있으나, 원위부로의전이는 1% 이하로보고되었다 14. 매우드물게고등급암종으로의변형이추적검사기간에나타나기도한다 15-17. 해부조직병리학적으로이병소는저등급이며, solid, trabecular, ductal, tubular, cribriform, cystic, papillary-cystic 와같은다양한성장양상을보이나전반적으로일정한세포학적양상을나타낸다 8,18. 또한신경주위로의침윤양상이자주보이나, 이증례에서는침윤양상이보이지않았다. 다형성저등급선암종의진단은일반적으로어려운편은아니나, 다형성선종 (pleomorphic adenoma) 와의감별을확실히해야한다. 이는정확한감별진단이치료와예후에큰 영향을미치기때문이다. 다형성선종과의감별에서신경교원섬유산단백 (glial fibrillary acid protein) 이유용하게이용되는데이는다형성저등급선암종에서는이단백과의반응이거의나타나지않으나다형성선종에는반응이나타나기때문이다 9,19. 또한소타액선에서생기는다형성선종은다형성저등급선암종과달리신경조직주위로의침입이관찰되지않으며, 침윤적이지않은것으로알려져있다. 감별진단이필요한다른병소로는선양낭성암종 (adenoid cystic carcinoma) 이있는데, 선양낭성암종의세포는다형성저등급선암종에비해작고, 기저양 (basaloid) 이며, 높은핵세포질비를보이며, 다염색성핵과투명한세포질을보인다. 다형성저등급선암종은완전한절제술이필요하나예후는일반적으로양호한편이며, 절개생검에서확연하게판단하기는쉽지않으며, CT, magnetic resonance imaging (MRI) 등의다른방사선검사가적절한치료를위해필수적이다. 다형성저등급선암종의치료에있어서가장적절한초기치료는병소경계의정확한평가후에국소적으로광범위하게절제하는것이다. 임상적평가에서림프조직으로의전이가명확하지않다면, 예방적경부곽청술이필요없다. 이증례에서는병소경계에서 2 cm 거리를확보하여연조직의절제를시행하였고, 하방구개골을연조직절제경계를따라 2 차적으로절제하였다. 병소자체가크지않아절제후 2 차치유를통한충분한연조직회복이가능하리라생각되어, 구개점막회전피판술은시행하지않았으며, PET-CT 에서우측경부에전이가의심되는림프절이존재한다하였으나 PNS-CT 및경부초음파검사에서특이소견이보이지않아예방적경부곽청술은시행하지않았다. 구개부에발생한암종의절제술을시행할경우 monobloc 74
Fig. 7. Schematic view of monobloc excision. Fig. 8. Schematic view of excision by layers. 을용이하게보존할수있었다 20.(Fig. 8) 수술후방사선검사에서상악동의점막비후가나타나기는하였으나, 상악동의기능은수술전과다르게보이지않았다.(Fig. 9) 현재환자는수술후경과관찰중이며, 10 개월이지난지금까지재발양상은발견할수없었고, 수술부위의치유양상은매우양호한편이다. 경부에서관찰되었던전이가의심되는림프절은지금까지큰차이가없으며상악동의기능또한문제가없었으며, 불편감도호소하지않고있다. Ⅳ. 결론 Fig. 9. Postoperative Water s view. excision 을통해수술을하는데이경우종양과아래에위치한골을동시에제거한다.(Fig. 7) 이경우상악동점막과비점막골막의보존이어려워, 상악동및비강누공등을야기할수있기에, 본증례에선종양및주위점막을우선절제한후하방에위치한골을제거하는 2 단계절제술 (excision in layer) 을시행하였다. 물론선양낭성암종과같은종양에서는조직의 plane 을따라침습적으로자라는특징이있어이러한수술방법의선택이불가하나, 본증례에서는술전에시행한 PET-CT, 골스캔, PNS-CT 에서 bone invasion 이없는것으로판단하였으며, 수술시에도구개부의점막제거후구개골을확인한바, 골표면이매끄러운상태라골로의종양의침범이없다고진단하였기에이런방법을선택할수있었다고생각되며, 이방법을통해상악동과비점막 본증례에서는구개부에생긴무통성의궤양으로내원한 67 세여자환자에서임상, 방사선학적소견및절개생검을통해구개부우측에발생한다형성저등급선암종을 excision in layer 로 2 단계로절제함으로써상악및비점막골막을보존하여재발및상악동누공발생등의술후합병증이없는양호한결과를얻었기에문헌고찰과함께보고하는바이다. References 1.Furuse C, Tucci R, Machado de Sousa SO, Rodarte Carvalho Y, Cavalcanti de Arau jo V. Comparative immunoprofile of polymorphous low-grade adenocarcinoma and canalicular adenoma. Ann Diagn Pathol 2003;7:278-80. 2.Freedman PD, Lumerman H.Lobular carcinoma of intraoral minor salivary gland origin. Report of twelve cases. Oral Surg Oral Med Oral Pathol 1983;56:157-66. 3.Frierson HF Jr, Mills SE, Garland TA.Terminal duct carcinoma of minor salivary glands. A nonpapillary subtype of polymorphous low-grade adenocarcinoma. Am J Clin Pathol 1985;84:8-14. 4.Castle JT, Thompson LD, Frommelt RA, Wenig BM, Kessler HP. Polymorphous low grade adenocarcinoma: a clinicopatho- 75
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