Oral Biology Research, 2017; September 30, 41(3): Copyright c 2017, Oral Biology Research Institute DOI: /obr Case Repo

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1 Oral Biology Research, 2017; September 30, 41(3): Copyright c 2017, Oral Biology Research Institute DOI: /obr Case Report ORAL BIOLOGY RESEARCH Numb chin syndrome with metastatic malignancy in hyperparathyroidism: Case report Byung-Yoon Roh 1, Jong-Mo Ahn 2,4, Jin-Soo Kim 3,4, and Yo-Seob Seo 3,4 * 1 Medical examiner's office, National forensic service, 10, Ipchun-ro, Wonju-si, Gangwon-do 26460, Republic of Korea 2 Department of Oral Medicine, School of Dentistry, Chosun University, Gwangju 61452, Republic of Korea 3 Department of Oral and Maxillofacial Radiology, School of Dentistry, Chosun University, Gwangju 61452, Republic of Korea 4 Oral Biology Research Institute, Chosun University, Gwangju 61452, Republic of Korea (Received Jul 31, 2017; Revised version received Aug 28, 2017; Accepted Aug 28, 2017) ABSTRACT Numb chin syndrome (NCS) is sensory neuropathy characterized by numbness in the chin and lower lip, involving distribution of the mental nerve. Causes of NCS may be of regional origin, a systemic disease, and malignancy. In this study, we report a case of NCS occurring as a first symptom of malignant metastasis in a 57-year-old female patient diagnosed with hyperparathyroidism. KEY WORDS: Hyperparathyroidism, Hypesthesia, Neoplasm Metastasis 서 론 턱저림증후군 (numb chin syndrome, NCS) 은이부의신 경병증 (mental neuropathy) 이라고도하며, 이신경과그가 지의지배영역에발생한감각신경의신경병증을지칭한다. 이신경이분포하는하순과이부의지각감퇴, 감각이상이 발생할수있고드물게는통증이나타나기도한다 [1]. NCS 의원인은국소적원인, 전신적원인, 악성종양으로 분류될수있다. 치성감염, 국소마취, 안면부의외상, 치 아의외상, 골수염, 악골및안면부에발생한양성종양등 의국소적원인이가장흔하지만아밀로이드증, 겸상적혈 구빈혈, 당뇨등과같은전신적원인이나유방암, 악성림 프종, 폐암등과같은악성종양의전이에의해서도발생할 수있다. NCS 는악성종양의첫번째증상일수있으며악 성종양의전이에의한 NCS 환자는치료에잘반응하지 않고, 생존기간이매우짧으므로 NCS 환자에서전신질환 의병력청취및구강내임상검사, 진단학적영상검사 등을통해 NCS 를유발할수있는요인을감별하는것은 *Corresponding author: Yo-Seob Seo Department of Oral and Maxillofacial Radiology, School of Dentistry, Chosun University, 309 Pilmun-daero, Dong-gu, Gwangju 61452, Republic of Korea Tel.: , Fax: moresys@chosun.ac.kr 매우중요하다 [2-4]. 부갑상선항진증은혈청내부갑상선호르몬이과도하게존재하는내분비이상으로부갑상선종양이나과형성으로인하여발생하는경우를원발성부갑상선항진증 (primary hyperparathyroidism) 이라하고, 저칼슘혈증이발생하고이에대한반응으로부갑상선호르몬의분비가증가하는것을속발성부갑상선항진증 (secondary hyperparathyroidism) 이라고한다 [5,6]. 부갑상선항진증은악성종양발생과관련이있다고알려져있으며, Pickard 등 [7] 은 2425명의부갑상선항진증환자에서 219 례의악성종양을관찰하여부갑상선항진증환자의악성종양위험이남성보다여성에서더높다고하였고, 원발성부갑상선항진증환자에서는다발골수종과같은조혈성종양이증가하지만, 속발성부갑상선항진증환자에서는모든악성종양의발생위험이증가한다고보고하였다. 저자들은부갑상선항진증으로진단한 57세의여자환자에서악성종양의전이의첫번째증상으로 NCS가발생한증례를보고하고자한다. 증례보고 57 세여자환자가왼쪽턱부위에감각이이상하다는것

Byung-Yoon Roh et al. 을주소로 OO 대학교치과병원에내원하였다. 일주일전부 터왼쪽턱부위에마취한듯한느낌이시작되었고, 가끔 저린느낌도들었으며증상이약간더심해지는것같다 고하였다. 혀가말라서그런지 2 일전쯤자고일어났을 때혀끝의느낌이좀이상했다가괜찮아졌으며, 전반적인 혀의느낌이나맛은정상이라고하였다.

Quantitative sensory testing Test site Touch (%) Pressure (%) Pin Prick (%) Chin Left 50 50 130 Lip Left 70 100 80 Labial mucosa Left 80 100 120 Buccal gingiva Left 100 100 110 One hundred percent

2 Byung-Yoon Roh et al. 을주소로 OO 대학교치과병원에내원하였다. 일주일전부 터왼쪽턱부위에마취한듯한느낌이시작되었고, 가끔 저린느낌도들었으며증상이약간더심해지는것같다 고하였다. 혀가말라서그런지 2 일전쯤자고일어났을 때혀끝의느낌이좀이상했다가괜찮아졌으며, 전반적인 혀의느낌이나맛은정상이라고하였다. 뇌쪽의이상이 의심되어병원에문의하니치과에먼저가보라고했다고 하였다. 환자는 7 년전위암 (adenocarcinoma) 수술을, 4 년전 Table 1. Quantitative sensory testing Test site Touch (%) Pressure (%) Pin Prick (%) Chin Left Lip Left Labial mucosa Left Buccal gingiva Left One hundred percent means normal sensation 갑상선암 (papillary thyroid carcinoma) 수술을받았고, 류마 티스관절염약을복용하고있었다. 2 년전암수술부위 검진후, 최근까지검사를받지않았고근래에치과치료 를받은적이없었다. 빈혈이있고, 귀가울리며머리가아 프지만이에대하여는따로약을복용하지않았다. 딸이 루푸스로입원하여스트레스를많이받으며본인도루푸 스가발생할지모른다는불안감을가지고있었고, 수면시 간은 10 시간정도이나불규칙하고깊이잠들지못한다고 하였다. 구강내임상검사에서하악좌측중절치의미약한타진 Fig. 1. Panoramic radiograph shows thinning of mandibular cortex and irregular radiodensity within mandible. Fig. 2. CBCT reveals the loss of normal cortical boundary and internal ground glass appearance of the bones in head and neck, including cervical vertebra, occipital bone, temporal bone, mandible, and sphenoid bone. Bone fusion of atlas and occipital bone (A) and bone expansion of sphenoid bone with almost loss of cortical boundary (B and C) are observed. Panoramic reconstruction shows ground glass appearance and heterogenic radiodensity in mandible (D). Cortical continuity is relatively remained in maxilla, mandible and zygomatic bone. Note that moderate defined radiolucency with internal faint radiopacity at left mandibular ramus (white arrows in B and D) and right greater wing of sphenoid bone (black arrow in C)

3 NCS with Malignancy in Hyperparathyroidism Fig. 3. Lateral skull radiograph (A) and posteroanterior skull radiograph (B) show internal ground glass bone pattern and generalized lack of defined outer cortical boundary of the osseous structure in head and neck. Note the lack of normal cortical bone at the inner and outer tables of the skull. 반응이있었고하악좌측제1대구치부위의경미한치은부종을제외하고특별한이상이관찰되지않았다. 정량적감각검사에서좌측이부, 아래입술, 구강점막부위의감각이저하되어있었고침통각검사에서부분적으로과감작되어나타났다. 정중부는비교적명확하게구분하였으나후방부위는명확하게표시되지않았다 (Table 1). 파노라마방사선영상에서하악각하연의피질골이얇아져있었고, 하악지및하악체가전반적으로균일하지않은방사선불투과상을보였으며하악관의경계가불명확하였다 (Fig. 1). 하악관주위의병소여부를확인하기위해서콘빔전산화단층장치 ( 콘빔CT, cone beam computed tomography; CBCT) 검사를시행하였다. CBCT 영상에서경추, 후두골, 접형골, 측두골, 설골등대부분의골에서피질골이소실되고내부가간유리모양의골로채워져있었다. 접형골은피질골대부분을소실하고약간팽융되어두드러져보였고상악골, 하악골과관골은다른골에비해피질골이비교적건전하였다. 상악골은과립상의골양상을보였고, 하악골은하악지와우측하악체부위에간유리모양의골양상을보였다. 좌측하악지부위와우측안와외하측의접형골에간유리모양의골내에서경계가비교적명확하고내부에희미한방사선불투과상이혼재되어있는방사선투과상이관찰되었고, 하악좌측병소는좌측하악관과인접해있었다 (Fig. 2). 두개골의변화를관찰하기위해추가로두부방사선촬영을하였고, 측방두부방사선영상과후전방두부방사선영상에서두개골내판과외판의피질골소실과, Table 2. Results of blood test Result Normal range Unit HGB (hemoglobin) low ~16.0 g/dl HCT (hematocrit) low ~47 % RBC (red blood cell) low ~ /µl WBC (white blood cell) low ~ /µl Rheumatoid factor high ~15 IU/mL Intact PTH high ~68.3 pg/ml Alk-phosphatase high ~123 U/L Albumin ~5.2 g/dl Creatinine ~1.3 mg/dl Total Ca low ~10.2 mg/dl Ionized Ca ~4.8 mg/dl Inorg. Phosphorus (P) ~4.5 mg/dl 판사이공간에간유리모양의골변화가관찰되었다 (Fig. 3). 영상검사에서부갑상선항진증이의심되어환자에게추가 로문진을실시하였고, 과거신장질환병력을확인하였다. 환자의혈액검사상 hemoglobin, hematocrit, red blood cell, white blood cell 의수치가정상보다낮았고 Rheumatoid factor 는정상보다높은수치를보였다. Parathyroid hormone 이 132 pg/ml( 정상 : 15~68.3 pg/ml), alkaline phosphatase 가 929 U/L( 정상 : 35~123 U/L) 로정상보다높았고, total calcium 은 7.94 mg/dl( 정상 : 8.4~10.2 mg/dl) 로정상보다 낮았다 (Table 2). 기존암의재발을확인하기위해시행한양전자방출컴퓨터

4 Byung-Yoon Roh et al. Fig. 4. Positron emission tomography-computed tomography (PET-CT) reveals hypermetabolism in multiple osteolytic lesions in right sphenoid bone (A), left mandible (B), sternum (C), and both femurs and humeri (D). 단층촬영 (positron emission tomography-computed tomography, PET-CT) 에서 fluorodeoxyglucose (FDG) 의섭취가증가된과대사종괴들이좌측하악지, 우측안와외측의접형골, 흉골, 양측상완골과대퇴골에서관찰되었다 (Fig. 4). 흉골부위병소의조직검사결과전이성선암종 (metastatic adenocarcinoma) 으로진단되었고이전의위암 (adenocarcinoma) 과갑상선암 (papillary thyroid carcinoma) 의과거력을고려하였을때위암기원의골내전이로생각되었다. 항암치료가시행되었으나 NCS 발생으로부터 3개월후사망하였다. 고찰 속발성부갑상선항진증의주요원인은신부전증으로알려져있다 [8]. 속발성부갑상선항진증이장기간지속되었을때부갑상선호르몬이자율적으로과도하게분비되는 이상상태가될수있는데이를삼차성부갑상선항진증 (tertiary hyperparathyroidism) 이라고한다 [9]. 본증례에서부갑상선호르몬, alkaline phosphatase가정상보다높았고, 혈중의 ionized calcium은 4.14 mg/dl로정상범주에속했으나, total calcium은 7.94 mg/dl로정상보다낮았다. 속발성부갑상선항진증에서삼차성부갑상선항진증으로의이행이의심된다 (Table 2). 부갑상선호르몬은혈청내칼슘농도를높이는역할을하는호르몬으로파골세포를활성화하여골을흡수시키고, 신장에서칼슘의재흡수를촉진시키며, 신장에서비타민D 의활성화를촉진시킨다. 부갑상선항진증환자는골흡수가촉진되고골개조가증가하므로진단영상에서전반적인골의탈회, 치조백선의소실, 피질골의비박과소실, 과립상또는간유리모양, 낭성섬유골염 (osteitis fibrosa cystica), 갈색종양 (brown tumor) 등의골변화가관찰될수있다 [1,10]. 본증례에서파노라마방사선영상과치근단방사선영상에서는특이한진단정보를얻지못하였다. CBCT 영상과

5 NCS with Malignancy in Hyperparathyroidism 두부방사선영상에서경추, 접형골, 두개골등악골이외의 골에서피질골소실과간유리모양의골양상을관찰하여 부갑상선항진증을의심하였고추가로문진하여과거신장 질환병력을확인하였다 (Fig. 2 and 3). 또한제 1 경추와후 두골의융합과같이환자의관절염병력과연관된병적상 태도확인할수있었다 (Fig. 2A). 치과에서 CBCT 등의진 단영상활용시악골이외의골도면밀히관찰하면환자의 전신질환에대한정보를얻을수있을것으로생각된다. 부갑상선항진증에서나타날수있는갈색종양은 PET- CT 영상에서과대사종괴로나타나악성종양으로오인될 수있다. 전이성암종과는달리갈색종양은부갑상선호르 몬이정상화되면퇴행할수있기때문에두병소를구분 하는것이필요하고이를위해조직검사를시행하여야한 다 [11,12]. 본증례에서는하악지, 접형골, 흉골, 양측상완 골과대퇴골에방사선투과성의병소가있었고, PET-CT 영 상에서과대사양상을보였는데, 이중흉골부위에서만 조직검사가시행되었다. 하악지부위의병소는조직검사 가시행되지않았으나갈색종양의활성형태라고보기어 려웠고, NCS 의발생과연관지었을때갈색종양보다는전 이성암종일가능성이높을것으로사료된다. NCS 와연관된전신적요인으로는다발성경화증, HIV, 당뇨, 매독, 겸상적혈구빈혈증, 혈관염, 아밀로이드증등 이있으며쇼그렌증후군, 류마토이드관절염, 루프스, 전신 성경화증과같은결합조직질환도신경병증을일으킬수 있다고보고되었다 [5-7,13]. 부갑상선항진증도 NCS 를유 발할수있지만근래에는통상적인혈액검사에서조기에 발견되어처치를받게되므로임상증상을나타내는경우 는거의없다 [2,9,14]. 본증례에서는류마토이드관절염과 부갑상선항진증이동반되었으나 NCS 의직접적인원인으 로보기는어려웠다. 부갑상선항진증과같은내분비장애는 multiple endocrine neoplasia type 1, type 2a, hyperparathyroidism-jaw tumor syndrome 등의유전적질환에의해서도발생할수있고종양과연관될수도있다 [2]. 본증례의환자는여성이었고, 류마티스관절염, 위암, 갑상선암, 부갑상선항진증이발생하였고, 딸이전신에영향을끼치는자가면역질환인루프스를앓고있었던점을고려할때유전적소인의가능성도배제할수없다. 국소적원인을제외하면 NCS의가장흔한원인은악성종양이다 [5]. Gil 등 [15] 은 16개의연구에포함된 NCS 136 례를분석하여 NCS와연관된악성종양은유방암, 림프종, 전립선암, 백혈병순으로빈도가높고, NCS가악성종양의 27.7% 에서, 재발성병소의 37.7% 에서첫번째증상으로나타났다고보고하였다. Lossos 등 [16] 은 42명의 NCS 환자에서 89% 가전이에의해 NCS가발생하였으며, 그중 50% 는하악골에, 14% 는두개저에, 22% 는연수막에발생 하였다고하였다. 한편, Sanchis 등 [17] 은 NCS를일으키는구강내원발성악성종양 11례중편평세포암종이 10례로가장높은빈도를보였다고보고하였다. 본증례는위암이재발하였고, 골내전이가일어났으며그첫번째증상으로 NCS가발생한것으로보인다. 본증례에서와같이 NCS 환자는일차적으로치과로의뢰될수있으므로치과의사는치성요인을감별할수있어야하고, 전신질환을파악하기위해노력하며, 악성병소전이의가능성을고려할수있어야한다. 감사의글 이논문은 2014학년도조선대학교학술연구비의지원을받아연구되었음. Conflict of Interest The authors declare that they have no competing interests. ORCID Byung-Yoon Roh Jong-Mo Ahn Jin-Soo Kim Yo-Seob Seo References 1. Zaheer F, Hussain K, Rao J. Unusual presentation of numb chin syndrome as the manifestation of metastatic adenocarcinoma of the lung. Int J Surg Case Rep 2013; 4: doi: /j.ijscr Jung JK, Hur YK, Choi JK. A Case Report of Numb Chin Syndrome with Facial Pain Caused by Diffuse Large B-Cell Lymphoma. J Oral Med Pain 2011;36: Marinella MA. Numb chin syndrome: a subtle clue to possible serious illness. Hospital physician 2000;36: Oh D, Woo K, Kim ST, Ahn HJ, Choi JH, Kwon JS. Numb Chin Syndrome as the First Symptom of Diffuse Large B-cell Lymphoma. J Oral Med Pain 2016;41: doi: /jomp Khan AA. Medical management of primary hyperpara

6 Byung-Yoon Roh et al. thyroidism. J Clin Densitom 2013;16: doi: / j.jocd Neville BW, Damm DD, Chi AC, Allen CM. Oral and maxillofacial pathology. 4th ed. St. Louis: Elsevier Health Sciences; Pickard AL, Gridley G, Mellemkjae L, Johansen C, Kofoed-Enevoldsen A, Cantor KP, Brinton LA. Hyperparathyroidism and subsequent cancer risk in Denmark. Cancer 2002;95: doi: /cncr Cunningham J, Locatelli F, Rodriguez M. Secondary hyperparathyroidism: pathogenesis, disease progression, and therapeutic options. Clin J Am Soc Nephrol 2011; 6: doi: /CJN Selvi F, Cakarer S, Tanakol R, Guler S, Keskin C. Brown tumour of the maxilla and mandible: a rare complication of tertiary hyperparathyroidism. Dentomaxillofac Radiol 2009;38: doi: /dmfr/ White SC, Pharoah MJ. Oral Radiology: Principles and Interpretation 7th ed. St. Louis: Elsevier Health Sciences; Andersen KF, Albrecht-Beste E. Brown tumors due to primary hyperparathyroidism in a patient with parathyroid carcinoma mimicking skeletal metastases on 18F- FDG PET/CT. Diagnostics 2015;5: doi: / diagnostics Lee JH, Chung SM, Kim HS. Osteitis fibrosa cystica mistaken for malignant disease. Clin Exp Otorhinolaryngol 2013;6: doi: /ceo Dumas M, Pérusse R. Trigeminal sensory neuropathya study of 35 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87: doi: /S (99)70137-X. 14. Krishnan U, Moule AJ. Mental nerve paraesthesia: A review of causes and two endodontically related cases. Saudi Endodontic Journal 2015;5:138. doi: / Gil SG, Diago MP, Diago MP. Malignant mental nerve neuropathy: systematic review. Med Oral Patol Oral Cir Bucal 2008;13:E616-E Lossos A, Siegal T. Numb chin syndrome in cancer patients Etiology, response to treatment, and prognostic significance. Neurology 1992;42: Sanchis JM, Bagán JV, Murillo J, Díaz JM, Poveda R, Jiménez Y. Mental neuropathy as a manifestation associated with malignant processes: its significance in relation to patient survival. J Oral Maxillofac Surg 2008;66: doi: /j.joms

Lumbar spine

Lumbar spine CT 32 111 DOI : 10.3831/KPI.2010.13.2.111 Lumbar Spine CT 32 Received : 10. 05. 23 Revised : 10. 06. 04 Accepted : 10. 06. 11 Key Words: Disc herniation, CT scan, Clinical analysis The Clinical