제 75 회 소화기 내시경 학회 월례 집담회 나윤주, 심기남, 강민정, 정지민, 하창윤 정해선, 백수정, 송현주, 염혜정, 정성애, 유권 이화여자대학교 부속 목동 병원 소화기 내과

► Age/sex 54/F ► Chief complaint 건강검진상의 내시경 이상 소견 건강검진상의 내시경 이상 소견 ► Present illness ► 상기 54 세 여환은 특이병력 없던 분으로 06 년 11 월 건강검진으로 시행한 위내시경검사에서 식도 점막하 건강검진으로 시행한 위내시경검사에서 식도 점막하 종양이 발견되어 치료를 위해 입원하였다. 종양이 발견되어 치료를 위해 입원하였다. ► PHx and FHx N-C ► Drug history none ► Op Hx(-) ► Social history Alcohol (-) Smoking (-) Alcohol (-) Smoking (-) Case

Physical examination and Lab findings Physical examination and Lab findings ► ► P/EX BP 120/80 mmHg PR 86/min RR 12 /min not anemic conjunctiva, not icteric sclera soft & flat abdomen no lymphadenopathy, soft & flat abdomen ► ► LAB Hgb 12.2g/dl, WBC 4,900/  L(neutrophil 43%),Plt 141,000/  L AST/ALT 17/11 IU/L, glucose 15 mg/dl BUN 15 mg/dL Creatinine 1.0 mg/dL total protein 6.1 g/dL, albumin 3.9 g/dL, ALP 11 IU/L T.B 0.2 mg/d, CEA 2.3mg/dl

EGD and EUS findings

Enucleation of esophageal SMT

Mixed tubular and cribriform features (H&E, x200) Numerous ‘microcyst' containing homogenous materials (H&E, x400) Pathologic findings Pathologic findings

CHEST CT

PET–CT SCAN

Final diagnosis Primary adenoid cystic carcinoma of esophagus

Adenoid cystic carcinoma Adenoid cystic carcinoma ► Malignant tumor from the salivary glands, the lacrimal glands, the upper respiratory tract, the breast or the uterine cervix ► Rare and slow-growing epithelial tumor ► Salivary gland tumors: 5% to 10% ► Head and neck malignancies : 2% to 4% ► Most frequently located in : minor salivary glands (31%), submandibular gland (14%), the parotid(2%) : minor salivary glands (31%), submandibular gland (14%), the parotid(2%)

Primary ACC of Esophagus ► Uncommon : 0.1% of all esophageal malignancies : 0.1% of all esophageal malignancies : only 60 cases have been reported : only 60 cases have been reported ► Mean age : 65 years / male : female = 3.4 : 1 ► Progressive dysphagia ► Site : middle third of the oesophagus (63%) lower third (30%), upper third (7%) lower third (30%), upper third (7%) ► Gross morphology : fungating or polypoid lesions : fungating or polypoid lesions > ulcerative and infiltrative growth patterns > ulcerative and infiltrative growth patterns ► Metastasis : 76% of patients  highly aggressive : 76% of patients  highly aggressive Distant metastasis: dominate Distant metastasis: dominate Petursson et. Cancer 1986 Petursson et. Cancer 1986

► Primary therapy : surgery ► Secondary therapy : radiation ► Salivary gland / head/ neck origin 1. curative resection 2. radiation (palliation, not prolong survivor) 3. chemotherapy (option) Primary ACC of Esophagus

Three cases of esophageal ACC in SM layer SiteSizeMetsTreatment Kabuto T, et al Lower 1.4*1.0 cm None Esophageal resection Esophagogastrostomy (f/u 18 m) Blaauwgee rs JL, et al Middle 1.5*1.0 cm None Esophageal resection Esophagogastrostomy (f/u 5 m) (f/u 5 m) Kim JH, et al Lower 2.3*1.8 cm None Esophageal resection Esophagogastrostomy Combination chemotherapy (f/u 12 m)