Surgical Pathology Conference 一般外科 : CR 吳柏鋼 / VS 伍超群

Case Presentation Name: 宣 x 仁 ID: F Gender: male Age: 57 y/o Date of admission: Date of operation: Date of discharge:

Chief Complaint Loss of body weight for 5 kg in the past 2 weeks

Present Illness This 57 y/o man suffered from tea-color urine, yellowish skin and poor appetite for 2 weeks He visited 忠孝 hospital and took some medicine home He was brought to our ER on Under the impression of obstructive jaundice, he was admitted for further evaluation and treatment

Personal History No DM, no HTN PPU s/p operation 1 year ago Smoking: quit for 1 year Alcohol consumption (-)

Physical Examination Consciousness: clear Vital signs: BP: 116/72 mmHg, TPR: 36.4/72/20 HEENT: icteric sclera Neck: supple, no palpable lymph node Chest & lung: symmetric, clear breathing sound Heart: regular rhythm, no murmur Abdomen: soft & flat, no tenderness, no palpable mass Back & spine: no deformity, no limited motion, no knocking tenderness Extremities: no deformity, no limited motion, no pitting edema Neurologic exam: negative

Lab. Data (6/22) CBC: WBC: 11000/uL, Hb: 12.5 g/dL, PLT: /uL DC: N.seg: 77.1%, Lym: 16.6% PT: 9.9” (10.1”), INR: 0.98 GOT: 51 IU/L, GPT: 68 IU/L TBI: 20.72mg/dL, DBI: mg/dL BUN: 16.3 mg/dL, CRE: 0.39 mg/dL Na: 131 mmol/L, K: 4.18 mmol/L GLU: 238 mg/dL Albumin: 3.42 g/dL

CT

PTCCD

Cholangiography

Lab. Data (6/28) CBC: WBC: 26500/uL, Hb: 11.3 g/dL, PLT: /uL DC: N.seg: 95%, Lym: 3% PT: 24.8” (10.1”), INR: 2.50 APTT: 47.3” (28.3”) GOT: 26 IU/L, GPT: 16 IU/L TBI: mg/dL, DBI: mg/dL BUN: 10.7 mg/dL, CRE: 0.59 mg/dL Na: 129 mmol/L, K: 4.49 mmol/L ALP: 377 U/L, GGT: 125.4

Tumor Markers CEA: 4.57 ng/mL CA19-9: 91.9 U/mL

Lab. Data (7/04) CBC: WBC: 22000/uL, Hb: 9.8 g/dL, PLT: /uL DC: N. band: 2%, N.seg: 88%, Lym: 3% PT: 10.6”” (10.1”), INR: 1.05 GOT: 47 IU/L, GPT: 69 IU/L TBI: 9.42 mg/dL, DBI: 6.96 mg/dL BUN: 8.8 mg/dL, CRE: 0.79 mg/dL Na: 132 mmol/L, K: 4.38 mmol/L ALP: 309 U/L, GGT: 122.3

Preoperative Diagnosis Ampullary tumor with obstructive jaundice

Operation (7/14) Operative Finding: 1.5 x 1.5cm polypoid fragile tumor at the papilla of vater with obstruction of distal CBD Marked dilatation of CBD; GB wall thickening Procedure: Transduodenal excision of papilla tumor Transduodenal excision of papilla tumor Side-to-side choledochoduodenostomy Side-to-side choledochoduodenostomy

Pathology Adenoma with low grade dysplasia Tubulovillous pattern Tubulovillous pattern

T-tube Cholangiography (7/25)

Discussion

Ampullary Tumors Average age at diagnosis: 50 y/o With a peak at 70 y/o With a peak at 70 y/o No gender predilection No gender predilectionSymptoms jaundice (80%), abdominal pain, weight loss jaundice (80%), abdominal pain, weight loss Adenocarcinoma of ampulla Incidence: 3 per million Incidence: 3 per million Tends to be local invasive Tends to be local invasive

Ampullary Adenoma Adenoma-to-carcinoma sequence Associated with FAP and adenomatous polyposis coli (APC) gene Almost 80% of patient with FAP gene have adenomas of ampulla Almost 80% of patient with FAP gene have adenomas of ampulla Risk of ampullary cancer: 100-fold Risk of ampullary cancer: 100-fold

Diagnosis Abnormal liver enzymes Ultrasound (endoscopic US) CT scan Endoscopic biopsy

Treatment Pancreaticoduodenectomy (Whipple procedure) Standard recommended curative operation Standard recommended curative operation 5-year survival: 25~55% 5-year survival: 25~55% Surgical mortality: 0~10% Surgical mortality: 0~10% Morbidity: 25~65% Morbidity: 25~65% Local excision Transduodenal excision (TDE) Transduodenal excision (TDE) Endoscopic snare excision (ESE) Endoscopic snare excision (ESE) Chemotherapy or radiotherapy No proven benefit No proven benefit

Snare Ampullectomy Newer endoscopic excisional technique Mortality: 0~1% Mortality: 0~1% Morbidity: 12% Morbidity: 12% Recurrence rate: 30% Recurrence rate: 30%

Transduodenal Ampullectomy Safe and effective treatment for benign ampullary tumors Essentially nil mortality Essentially nil mortality Morbidity: 0~25% Morbidity: 0~25% Recurrence rate: 5~30% Recurrence rate: 5~30%

Transduodenal Ampullectomy The treatment of choice for villous adenomas and T1 adenocarcinomas, with 1cm of resection margin to avoid local recurrence Fraguela Marina JA. Transduodenal ampullectomy in the treatment of villous adenomas and adenocarcinomas of the Vater’s ampulla. Rev Esp Enferm Dig, Dec 2004; 96(12); Fraguela Marina JA. Transduodenal ampullectomy in the treatment of villous adenomas and adenocarcinomas of the Vater’s ampulla. Rev Esp Enferm Dig, Dec 2004; 96(12);

Reference Martin JA, Haber GB. Ampullary adenoma: clinical manifestations, diagnosis, and treatment. Gastrointest Endosc Clin N Am, Oct 2003; 13(4): Tran TC; Vitale GC. Ampullary tumors: endoscopic versus operative management. Surg Innov, Dec 2004; 11(4): Fraguela Marina JA. Transduodenal ampullectomy in the treatment of villous adenomas and adenocarcinomas of the Vater’s ampulla. Rev Esp Enferm Dig, Dec 2004; 96(12); Sa Cunha A et al. Value of surgical ampullectomy in the management of benign ampullary tumors. Ann Chir, Jan 2005; 130(1): 32-6 Goldman: Cecil textbook of Medicine, 22nd ed., 2004, 956 Kumar: Robbins and Cotran: pathologic basis of disease, 7th ed., 2005, 862