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1 Kor J Oral Maxillofac Pathol 2009;33(2): 신성골이영양증의구강내증상으로나타난하악골의국소적증대 - 1 례보고 - 유미현 1), 김현실 2), 지유진 3), 송현철 4), 김진 2),5) 남서울대학교치위생학과 1), 연세대학교치과대학구강종양연구소 2), 경희대학교동서신의학병원치대병원구강악안면외과 3), 가톨릭대학교성빈센트병원치과 / 구강악안면외과 4) 연세대학교치과대학구강병리학교실 5) ABSTRACT Localized Jaw Enlargement as Oral Manifestation in Renal Osteodystrophy - Report of a case and review of the literature - Mi Heon Ryu 1), Hyun Sil Kim 2), Yu Jin Jee 3), Hyun Chul Song 4) 2), 5), Jin Kim Department of Dental Hygiene, Namseoul University 1), Oral Cancer Research Institute, Yonsei University College of Dentistry 2), Deptartment of Oral and Maxillofacial Surgery, Dental Hospital, East-West Neo Medical Center, Kyunghee University 3), Divison of Oral & Maxillofacial Surgery, Department of Dentistry, St. Vincent's Hospital, The Catholic University of Korea 4), Department of Oral Pathology, Yonsei University College of Dentistry 5) Renal osteodystrophy is the complex disease of the skeleton that occurs in patients with chronic renal failure. A 29-year-old female with chronic renal failure and a history of hemodialysis during 10 years showed localized jaw enlargement in the anterior mandible. She also revealed increased serum PTH level, osteosclerosis in her skull base and facial bone, chronic pain in the both knee joint, and gastrointestinal disorder which are symptoms of renal osteodystrophy. The patient was diagnosed hyperparathyroidism associated with chronic renal failure. Parathyroidectomy was performed, however, serum PTH level increased again after the parathyroidectomy, the affected area of the mandible was enlarged as the serum PTH level increased. The enlarged area seemed like as peripheral lesion clinically. We reported a case of localized jaw enlargement as oral manifestation in renal osteodystrophy. Key words : Renal osteodystrophy, Hyperparathyroidism, Chronic renal failure, Oral manifestation, Brown tumor, Osteitis fibrosa 만성신부전증은심각한신장기능소실에의해일어날수있는다인자적증후군이며산업화된환경하에서지난 10년간점차적으로발생률이증가하고있다 1). 만성신부전증이있는환자는대부분말기신장질환으로이행하게되며여러가지합병증을동반할수있는데골격계에나타날수있는합병증으로는신성골이영양증이있다 2). 신성골이영양증에는이차성부갑상선기능항진증과동반되어일 * Correspondence : Jin Kim, Department of Oral Pathology, Yonsei University College of Dentistry, 134 Shinchondong, Seodaemungu, Seoul , Tel : , jink@yuhs.ac 어나는섬유성골염 (osteitis fibrosa), 낭종섬유성골염 (osteitis fibrosa cystica) 이주병변인고전환골이영양증 (high turnover disease) 과이차성부갑상선기능항진증없이골연화증 (osteomalacia), 무력성골질환 (adynamic bone disease) 등을보이는저전환골이영양증 (low turnover disease) 의두가지병변이있다 2). 고전환골이영양증은늑골, 쇄골, pelvic girdle, phalangeal tuft 등을침범하는것으로알려져있으나악골을침범하여국소적증대가일어나는경우는드문것으로보고되어있다 3-5). 본증례는만

성신부전증환자에서이차성부갑상선항진증의결과로서신성골이영양증을동반하였으며악골에국소적증대를초래하였다. 저자들은본증례를통하여신성골이영양증이악골의국소적증대로나타나는증례에대한문헌고찰과함께악골의다른질환들과의감별진단등을고찰해보고자한다. I.

이환자의치과병력은특이사항이없었으며전신적병력에 서는 1996년만성신부전증 (chronic renal failure) 으로진단받아가톨릭대학교성빈센트병원내과에서투석시행중이었다.

골스캔에서는전신적으로 bone uptake가증가하여신성골이영양증의가능성이있는것으로진단되었다 (Fig. 2). 이환자의부갑상선호르몬과생화학적검사소견은 Table 1, 2에나타나있다.

Panoramic view showing a welldefined radiolucent lesion in the anterior mandible (arrow).

2 성신부전증환자에서이차성부갑상선항진증의결과로서신성골이영양증을동반하였으며악골에국소적증대를초래하였다. 저자들은본증례를통하여신성골이영양증이악골의국소적증대로나타나는증례에대한문헌고찰과함께악골의다른질환들과의감별진단등을고찰해보고자한다. I. 증례 2003년 7월 25일, 29세여환이하악전방부의국소적증대를주소로가톨릭대학교성빈센트병원치과 / 구강악안면외과에내원하였다. 이환자는약 3~4 개월전부터하악전방부위가커진것을인지하였다 (Fig. 1). 이환자의치과병력은특이사항이없었으며전신적병력에 서는 1996년만성신부전증 (chronic renal failure) 으로진단받아가톨릭대학교성빈센트병원내과에서투석시행중이었다. 1998년신경외과에서뇌하수체의미세선종 (microad enoma) 으로진단받았으며 2003년 11월에부갑상선의선종 (adenoma) 으로부갑상선적출술시행예정이었다. 이외에도결핵, 무월경, 두통, 출혈성위염, 오심 / 구토, 발작, 양무릎관절의퇴행성관절염등으로치료받은기왕력이있었다. 골스캔에서는전신적으로 bone uptake가증가하여신성골이영양증의가능성이있는것으로진단되었다 (Fig. 2). 이환자의부갑상선호르몬과생화학적검사소견은 Table 1, 2에나타나있다. 초진시환자의구강내소견은하악좌측측절치, 견치, 제 1 소구치설측부위에 dome shape의외향성종괴가관찰되었으며촉진시경결감을보였다. 해당부위의협측골 Fig 3. Panoramic view showing a welldefined radiolucent lesion in the anterior mandible (arrow). The loss of trabeculation and cortical borders, and loss of lamina dura were observed. Fig. 1. Intraoral photograph showing a domeshaped exophytic lesion in the anterior left mandible. Fig. 2. Bone scan showing increased bone uptakes in whole bones and, especially skull and mandible without kidney and bladder activity. Fig. 4. Computed tomography showing an exophytic lesion in the anterior left mandible (arrow). The thinning of cortical bone and blurring of bone trabecular pattern were observed. 98

3 과이환부위의치아는이상소견을보이지않았으며이학적검사상이상소견은없었다. 환자는 2003년 11월부갑상선적출술을시행받고 intact-pth(parathyroid hormone) 가감소하였으나다시증가하기시작하였다. 2004년 4월 28일치과 / 구강악안면외과에 2차내원시 1 차내원시보다종괴의크기가증가하는소견을보여 ( cm) 조직생검을시행하였다. 파노라마사진에서는하악전치부치근부위에경계가분명한방사선투과성병소를보였으며 (Fig. 3) 치조백선과골소주소실이관찰되었다. 전산화단층촬영사진에서는좌측하악부위에서골침식소견이있었으며두개골기저부와안면골에미만성경화성골변화가관찰되었다. 침범된골에서는피질골의비박소견과골소주패턴의 blurring 도있었다 (Fig. 4). 2004년 10월전신마취하에종괴적출술을시행하였다. 종괴는피막으로둘러싸여있었으며종괴제거후접시모양의골파괴병변과종물주변의피질골화가관찰되었으나치근노출은없었다. 적출된종괴는한조각의단단한조직이었으며저배율소견에서혈관이풍부한섬유성조직의증식과새로형성된다량의골 양조직, 소수의다핵거대세포가관찰되었다. (Fig. 5. A, B, C). 고배율소견에서는골양조직의외형을따라둘러싸고있는다수의골모세포양세포와파골세포, 출혈소견등이있었다. 이상의임상소견과생화학적검사소견, 병리조직학적소견을바탕으로만성신부전증에동반된신성골이영양증의구강내증상으로발생한국소적인섬유성골염 ( 갈색종양 ) 으로진단하였다. II. 고찰신성골이영양증은조직학적, 병인론적으로고전환골이영양증과저전환골이영양증으로분류된다. 1, 2, 6) 이러한골질환의유형은투석방법에따라달라지는것으로보고되어있는데, 혈액투석환자의경우는부갑상선기능항진증의빈도가 38%, 저전환골이영양증의빈도는 36% 이며복막투석환자의경우부갑상선기능항진증이있는경우는 10%, 저전환골이영양증의빈도는약 2/3 정도를차지한다. 두가지유형의병소가함께나타나는경우도있어혈액투석환자의 10%, 복막투석환자의 5% 에서복합성요독증골이영양증 (mixed uremic osteodystrophy) 이라불리는복합된병소를보인다 2). Table 1. Intact PTH values according to parathyroidectomy Parathyroidectomy Before After Normal value pg/ml Table 2. Biochemical studies according to parathyroidectomy Parathyroidectomy Before After Test Normal value Protein, total (g/dL) Sugar, fasting (mg/dL) ALK. Phosphatase (IU/L) Urea Nitrogen 8-23(mg/dL) Creatinine (mg/dL) Sodium (mEq/L) Potassium (mEq/L) Phosphorus (mg/dL) Calcium (mg/dL)

Fig. 5. A The mass showed fibroblastic prolif- eration with multinucleated giant cells(h/e stain, 200). B.

The mass showed the spicules of woven bone with osteoblastic rim- ming and osteoclastic multnucleated giant cells (H/E stain, 200). 신성골이영양증에서악골과두개골에변화를나타내는경우는질병의초기에서부터관찰할수있다.

Damm 등은 7명의신성골이영양증환자에서악골의증대소견을보고하였는데, 5명의환자는미만성악골증대를보였고 2 명의환자에서는부정교합이나안면변형없이국소적악골증대를보여본경우와비슷한경우를보고하였다 5). 부갑상선기능항진증과연관되어병변이발생하는경우대부분섬유성골염이나낭성섬유성골염의형태로발생한다 6).

4 Fig. 5. A The mass showed fibroblastic prolif- eration with multinucleated giant cells(h/e stain, 200). B. The mass showed the trabeculae of woven bone within a background of cellular fibrous tissue(h/e stain, 100). C. The mass showed the spicules of woven bone with osteoblastic rim- ming and osteoclastic multnucleated giant cells (H/E stain, 200). 신성골이영양증에서악골과두개골에변화를나타내는경우는질병의초기에서부터관찰할수있다. 그러나신성골이영양증과연관되어악골국소적증대소견을보이는경우는드물며 Kalyvas 등의보고에서는투석환자중 16명에서악골증대소견을보고하였다고하였고 1), Michiwaki 등은신부전증과이차성부갑상선기능항진증이동반된경우에서악골의미만성증대를보고하였다 7). Damm 등은 7명의신성골이영양증환자에서악골의증대소견을보고하였는데, 5명의환자는미만성악골증대를보였고 2 명의환자에서는부정교합이나안면변형없이국소적악골증대를보여본경우와비슷한경우를보고하였다 5). 부갑상선기능항진증과연관되어병변이발생하는경우대부분섬유성골염이나낭성섬유성골염의형태로발생한다 6). 본예에서는병소의위치, 종괴를둘러싼골조직이없는점, 파노라마에서는하악전치부치근부위에방사선투과성병변을보였으나교합사진에서골내병소로관찰되지않는점등임상적으로주변성에가까운소견을나타내었다. Shannon과 Rapport 는부갑상선기능항진증과연관하여발생한갈색종양이피질골을천공하면서성 장하여 epulis 와같은병소를형성한예를보고하였으며, Burkes 와 White 도일차성부갑상선기능항진증에서주변성 giant-cell granuloma를보고하였다 8). 본예에서는이환된부위의치근흡수, 치근전위나치아의동요도가관찰되지않았으며하악협측피질골은정상적인소견을나타내었다. 이는발생한악골내병소가하악의설측피질골과치아사이에위치하여성장하여피질골을뚫고성장하여임상적으로주변성처럼관찰되는것이라고생각된다. 보고된바에의하면신성골이영양증과부갑상선기능항진증이동반된경우에는 3가지주요한조직학적소견을보일수있다고하였다 7). 비광화골양기질 (unmineralized bone matrix) 증가, 파골세포수와골흡수공간 (reporption lacunae) 증가, 골내골소주주위섬유화 (endosteal peritrabecular fibrosis), tunnelling marrow fibrosis 등의소견을보여골합성과골흡수가동시에증가하는소견을보인다고하였고, 무층골소주 (woven bone spicule) 가평행한주행을하면서증식하는소견및섬유아세포가밀집하여증식하는소견등이신성골이영양증에서관찰할수있는소견이라고하였다 9-11). 본예에서는혈관 100

5 이풍부한섬유성조직의증식, 골모세포양세포와파골세포로둘러싸인새로형성된골양조직이다수관찰되는반면섬유성세포증식부분과동반되어나타나는다핵거대세포들의수는비교적적은조직학적소견을보여만성신부전증에동반된신성골이영양증에서발생한국소적인섬유성골염의조직학적소견과일치하였다. 악골에발생하는병변중다핵거대세포들이나타나는중심성거대세포육아종 (central giant cell granuloma), 중심성거대세포종양 (central giant cell tumor), 초기단계의체루비즘 (early stage cherubism) 등의질환은갈색종양과감별진단이필요하다. 조직학적으로이들질환은단핵구성기질세포 (mononuclear stromal cell) 와다핵성거대세포 (multinucleated giant cell) 의 2가지세포성분을포함하고있으며조직학적소견이매우유사하나서로다른임상적소견을나타낸다. 조직학적소견만으로는감별진단이어렵고임상적소견과생화학적검사소견을함께고려할때이들병소를감별진단하는데도움이될수있다 10, 12, 13). 혈중칼슘농도, 인산농도, intact PTH 농도등이도움이될수있다. 중심성거대세포육아종은공격적이지않은병소에서는유골과무층골형태의신생골형성이관찰되나공격적인병소에서는단핵세포와거대세포가현저히증가하고결합조직의감소와골형성은미미한조직소견을보인다 10). 중심성거대세포육아종의조직소견은섬유성골염과매우비슷하여조직학적소견만으로는감별진단하기어렵고혈중칼슘농도, 부갑상선호르몬농도등임상적소견과방사선적소견을종합하여야한다 14). 중심성거대세포종양의조직학적소견역시섬유성골염의소견과매우비슷하나매우크기가큰거대세포가나타나고거대세포의핵의수가 3~100 개정도로증가하며세포의중앙부로핵이군집되어있는것이특징이다. 중심성거대세포종양에서보이는거대세포는병소에미만성으로분포하여있으며기질세포충실도가매우높고괴사소견도나타날수있다 14). 초기단계의체루비즘의조직학적소견에서나타나는단핵구성기질세포는섬유성골염에서는나타나지않으며체루비즘에서주로관찰되는소견이다. 또한골소주주위섬유화소견은섬유성골염에서만관찰된다 15). 면역조직화학염색소견이감별진단에도움이될수있는데, 섬유성골염의경우대부분의다핵거대세포에 CD 68에서양성반응을보이며 vimentin, lysozyme에서는약양성을나타낸다. 반면, Proliferating cell nuclear antigen (PCNA), α1-antitrypsin, α1-antichymotrypsin, CD 34, factor VIII, S-100 에서는음성반응을보인다. 악골에발생하는중심성거대세포육아종에서는 α1-antitrypsin, α1-antichymotrypsin, S-100 단백에양성반응을보여섬유성골염과감별진단하는데도움이될수있다 12, 16). III. 참고문헌 1. Kalyvas D, Tosios KI, Leventis MD, Tsiklakis K, Angelopoulos AP. Localized jaw enlargement in renal osteodystrophy : Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;97: 대한내과학회역. 해리슨내과학. 도서출판 MIP. 서울. 제 15판. pp Guney E, Yigitbasi OG, Bayram F, Ozer V, Canoz. Brown tumor of the maxilla associated with primary hyperparathyroidism. Auris Nasus Larynx 2001;28: Apaydin S, Sariyar M, Erek E, Yiǧitbaş R, Hamazaoǧlu İ, ǖlkǖ U. Brown tumor as manifestation of parathyroid hyperplasia in two cases with end-stage renal failure. Nephron 1999;81: Damm DD, Neville BW, McKenna S, Jones AC, Freedman PD, Anderson WR, Allen CM. Macrognathia of renal osteodystrophy in dialysis patients. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;83: Rubins pathology clinicopathologic foundations of medicine. Lippincott Williamsd & Wilkins, Philadelphia, 4th ed. 2005, pp Michiwaki Y, Michi K, Yamaguchi A. Marked enlargement of the jaws in secondary hyperparathyroidism-a case report. Int J Oral Maxillofac Surg 1996;25: Burkes EJ, White Jr. RP. A peripheral giant-cell granuloma manifestation of primary hyperparathyroidism : report of case. JADA 1989;118:

6 9. Neville BW, Damm DD, Allen CM, Bouqout JE. Oral and Maxillofacial Pathology. 2nd ed. Philadelphia: W.B. Saunders, 2002; Sapp JP, Eversole LR, Wysocki GP. Contemporary Oral and Maxillofacial Pathology. 2nd ed. St. Louis: Mosby, 2004; Slatopolsky E, Gonzalez E, Martin K. Pathogenesis and treatment of renal osteodystrophy. Blood Purif 2003;21: Okada, Davies, Yamamoto. Brown tumor of the maxilla in a patient with secondary hyperpar athyr oidism : A case study involving immunohistochemistry and electron microscopy. J Oral Maxillofacial Surg 2000;58: Yamazaki H, Ota Y, Aoki T, Karayuki K. Brown tumor of the maxilla and manbible: Progressive mandibular brown tumor after removal of parathyroid adenoma. J Oral Maxillofacial Surg 2003;61: Regezi JA. Odontogenic cysts, odontogenic tumors, fibrosseous, and giant cell lesions of the jaws. Mod Pathol 2002;15: Southgate J, Sarma U, Townendd JV, Barron J, Flanagan AM. Studdy of the cell biology and biochemistry of cherubism. J Clin Pathol 1998;51: Shang ZJ, Li ZB, Chen XM, Li JR, McCoy JM. Expansile lesion of the mandible in a 45-year-old woman. J Oral Maxillofac Surg 2003;61:

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05.Kaes001.hwp 혈액투석환자에서삼차성부갑상선기능항진증으로부갑상선절제술후발생한 Hungry Bone Syndrome 1 예 A Case of Hungry Bone Syndrome after Parathyroidectomy in Patient with Tertiary Hyperparathyroidism and Hemodialysis Patient Myoung Won Son, M.D.,