CASE PRESENTATION 2014.06.11 전임의윤종민
M/59 C.C.: SMT on stomach, duodenum P.I.: 2013. 12월외부병원검진위내시경상 cardia 및 2 nd portion of duodenum의 SMT 관찰되어 EUS 등 F/E 위해내원
HISTORY P.Hx) HT / DM / Hepa / Tbc - / - / - / - S.Hx) N-S F.Hx) N-S
PHYSICAL EXAMINATION GA / GC Well-looking appearance HEENT Not anemic conjunctivae Anicteric sclera Not dehydrated tongue Not engorged neck v. No CLN, SCLN enlargement Chest CBS /s r RHB /s m Abdomen Not distended No tender point Normoactive BS Extremities Grossly free No Pitting edema
REVIEW OF SYSTEM Fever/chilling/wt loss/wt gain -/-/-/- General weakness /Fatigue -/- Headache/dizziness/tinnitus -/-/- Cough/sputum/dyspnea -/-/- Chest pain/palpitation -/- A/N/V/D/C -/-/-/-/- Dysuria/frequency/dark urine -/-/-
Initial Lab. finding CBC LRFT WBC 5750 /mm 3 AST/ALT 32/35 IU/L Neut 32.1 % ALP/LDH 60/150 IU/L Hb 14.2 g/dl PLT 313 10 3 /mm 3 TB/DB 0.57/0.16 mg/dl Electrolyte Na 140.6 mmol/l K 4.18 mmol/l Cl 102.3 mmol/l Viral marker HBs Ag/Anti-HBs Ab - / + Anti HCV Ab VDRL Anti HIV Ab Negative Non Reactive Negative TP/Alb 7.5/4.8 gm/dl BUN/Cr 8.7/0.83 gm/dl TC/UA 262/5.8 gm/dl Ca/P 9.3/4.3 gm/dl CRP 0.04 mg/dl PT(INR) 1.01 aptt 29.0 Sec ESR 2 mm/hr
Endoscopic finding (2014.1.29)
Endoscopic finding (2014.1.29)
Endoscopic finding (2014.1.29)
EUS finding (2014.1.29) 1.6cm
EUS finding (2014.1.29)
2-phase abd. CT (2014.1.29)
EMR (2014.3.26)
EMR (2014.3.26)
EMR (2014.3.26)
Pathologic finding
HE 40x mucosa mm submucosa
HE 40x
HE 400x
HE 400x
Ki-67 400x
Chromogranin 100x
Synaptophysin 100x
Synaptophysin 400x
CD56 100x
CD56 400x
Pathologic finding Small intestine, duodenum, second portion, endoscopic mucosal resection : Carcinoid tumor, well-differentiated neuroendocrine tumor (grade 2 by WHO 2000) Neuroendocrine neoplasm (grade I by WHO 2010) 1. Size: 2.2x1.4cm 2. Location: mainly at submucosa (pt2, by AJCC), without mucosa invasion 3. Growth pattern: type A (solid) 4. Nuclear atypia: moderate 5. Mitosis: 1/10HPFs, Ki-proliferation index: less than 1% [CD56(+)/synaptophysin(+)/chromogranin-A(-)/p53(-)] 6. Histologic grade: G1 (by AJCC) 7. Status of resection margins: LM(-)/VM(-) 8. Lymphatic invasion: not identified 9. Venous invasion: not identified 10. Perineural invasion: not identified.
3-phase abd. CT (2014.4.7) stomach cardia 에존재하는 1.6cm 의 submucosal tumor 는 stomach 이 collapse 되어관찰되지않음. EMR 시행부위에 local tumor recurrence 소견이나 significant lymphnode 는관찰되지않는다. Gallbladder 내에 stone 이관찰되고, right kidney 에 well defined, hypodense, hemogeneous 하게 water concentration 을보이는 lesion 이관찰되고이것은 renal cyst 로생각된다. CONCLUSION 1. S/P Neuroendocrine carcinoma at duodenum post EMR state --> No evidence of local tumor recurrence and significant lymph node 2. No visible submucosal tumor at stomach cardia 3. GB stone 4. Right renal cyst(1.2cm)
PET-CT (2014.4.8) Brain : Not performed Head & neck : 정상적인 glucose metabolism 을보이고있으며, 비정상적인 hypo 또는 hypermetabolic area 는관찰되지않는다. Chest & breast : 정상적인 glucose metabolism 을보이고있으며, 비정상적인 hypo 또는 hypermetabolic area 는관찰되지않는다. Abdomen & pelvis : Hepatoduodenal LN (SUVmax 1.7) 에 mild FDG uptake 관찰됨. Extremities : 정상적인 glucose metabolism 을보이고있으며, 비정상적인 hypo 또는 hypermetabolic area 는관찰되지않는다. CONCLUSION 1. No evidence of abnormal glucose metabolism 2. Benign reactive hepatoduodenal LN, more likely
Diagnosis Carcinoid tumor, well-differentiated neuroendocrine tumor, grade 2 by WHO 2000 Neuroendocrine neoplasm, grade I by WHO 2010
Review Duodenal Neuroendocrine tumor
Duodenal Carcinoid Tumor 유암종은위장관, 담도계, 췌장, 폐, 난소등의장크롬친화성세포에서기원하며, 그중위장관에서가장흔하게발생한다. 국내보고 : 직장 (71.7%) 에서호발, 위 (13.6%), 십이지장 (8.6%) 의순서로발생하였다. 원발성십이지장유암종은 57% 가십이지장의구부에서발생하고팽대부주위에서 23%, 십이지장제3부에 3% 의빈도로발생한다. 평균발생연령은 50세전후이며성별에따른발생률차이는없음.
Duodenal Carcinoid Tumor ( 증상 ) 대부분의경우에서무증상으로다른복부질환에의한검사나수술중우연히발견. 종양자체에의한물리적영향으로발생하는복부동통, 장폐색, 황달등의증상. 종양에서분비하는생화학물질 ( 세로토닌, 히스타민, 타키키닌등 ) 에의해설사및안면홍조, 기관지수축, 심내막의섬유화, 모세관확장증등의유암종증후군으로나타난다.
Duodenal Carcinoid Tumor 내시경상흔히초기에는정상점막으로덮여있는황색의부드러운타원형의병변 -> 크기가커지면발적이나중심부의함몰이나타남 그러나육안적으로는다른용종과구별이어렵고, 국내유암종의내시경육안적진단율은 62% 에불과하다는보고 유암종의전이와관련된인자들로크기가 10 mm이상, 표면의중심함몰이나궤양, 고유근층의침범, 림프또는혈관침범, 유사분열수 3개이상, Ki-67 표지지수 3이상등이있다. 예후는특히간으로의전이유무가가장중요한것으로알려져있다.
Duodenal Carcinoid Tumor ( 치료 ) 십이지장유암종의치료는전이가없을때는수술적절제가원칙이었으나최근내시경치료가점차증가추세 결론적으로십이지장유암종은위치가시술가능하고, 크기가 10 mm 이하이며, 전이가없고, 근층으로침범이없고점막하층에국한되었을경우내시경절제술을할수있다고알려져있다. 내시경적절제술후에는 6 개월간격으로주의깊은추적관찰이추천.
Duodenal Carcinoid Tumor ( 치료 )
Duodenal Carcinoid Tumor ( 치료 )
Duodenal Carcinoid Tumor ( 치료 ) 전이가있는경우에는증상완화를위한치료를할수있다. 약물치료로서지속성소마토스타틴유사체를쓸수있고, 면역요법 으로인터페론을사용함으로써악성유암종환자에서종양의크기 감소및생존율을증가시킨다고알려져있다. 또한증상의완화에부분절제, 간동맥색전술 / 항암색전술, 경피고주파소작술도기대할수있는치료이다.