1. Surgical Pathology Conference 一般外科 : CR 吳柏鋼 / VS 伍超群 2005-07-26

2. Case Presentation Name: 陳 x 賢 ID: J121713949 Gender: male Age: 29 y/o Date of admission: 2005-07-04 Date of operation: 2005-07-07 Date of discharge: 2005-07-14

3. Chief Complaint Abdominal pain over LUQ for 1 month

4. Present Illness This 29 y/o man suffered from LUQ pain since 2005-05-31 and he visited GI OPD at 2005-06-07. His appetite was good and stool passage was normal. Abdominal ultrasonography was performed and revealed 7cm intra-abdominal cystic tumor with calcification. PES was arranged and showed hiatal hernia with esophagitis, superficial gastritis, and duodenal ulcer. Under the impression of pancreatic cystic neoplasm, he was admitted for surgical treatment.

5. Personal History No DM, no HTN, no surgical history Smoking (+) 1/3 PPD Alcohol consumption (+)

6. Family History Esophageal cancer

7. Physical Examination Consciousness: clear Vital signs: BP: 118/77 mmHg, TPR: 37/87/18 HEENT: grossly normal 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

8. Lab. Data CBC: WBC: 9900 /uL, Hb: 15.5 g/dL, PLT: 216000 /uL DC: N.seg: 71.5%, Lym: 23.1%, Mono: 2.9% PT: 10.7” (control: 10.1”), APTT: 30.1” (control: 28.3”) GOT: 15 IU/L, GPT: 14 IU/L TBI: 0.48 mg/dL, DBI: 0.06 mg/dL BUN: 14.7 mg/dL, CRE: 0.73 mg/dL Na: 137 mmol/L, K: 4.46 mmol/L Amylase: 69 IU/L, Lipase: 45.5 IU/L GLU: 115 mg/dL

9. CT

15. UGI

16. Preoperative Diagnosis Pancreatic tumor at the tail R/o microcystic adenoma R/o pancreatic carcinoma R/o GIST

17. Operation Operative Finding: An 6x5x4 cm stony hard mass at pancreatic body with mild adhesion An 6x5x4 cm stony hard mass at pancreatic body with mild adhesionProcedure: Subtotal pancreatectomy + dissection of regional LNs Subtotal pancreatectomy + dissection of regional LNs Splenectomy Splenectomy

19. Pathology Solid pseudopapillary tumor

20. Discussion

21. Benign Exocrine Tumors Most benign pancreatic exocrine tumors are cystic, but not all cystic tumors are benign Serous (microcystic) cystadenoma Serous (microcystic) cystadenoma Mucinous (macrocystic) cystadenoma Mucinous (macrocystic) cystadenoma Intraductal papillary mucinous tumor (IPMT) Intraductal papillary mucinous tumor (IPMT) Solid-pseudopapillary tumor of the pancreas Solid-pseudopapillary tumor of the pancreas Sabinston Textbook of Surgery, 17th ed., 1665- 1667.

22. Serous Cystadenoma 20% to 40% of cystic pancreatic neoplasm No malignant potential

23. Mucinous Cystadenoma 20% to 40% of cystic tumors Have malignant potential

24. Intraductal Papillary Mucinous Tumor IPMT is believed to follow an adenoma- carcinoma sequence (Pan-IN classification)

25. Solid PseudopapillaryTumor A relatively uncommon tumor, often occurs in young women and follows a benign course Large, round, and well-demarcated masses that can occur in any part of the pancreas Local resection is usually curative Incomplete removal can result in local recurrence Incomplete removal can result in local recurrence Malignant varieties have been described Malignant varieties have been described Solid and consists of monomorphous eosinophilic or clear cells that demonstrate a pseudopapillary architecture

26. Solid Pseudopapillary Tumor of the Pancreas: Review of 718 Patients Reported in English Literature Papavramidis T and Papavramidis S. J Am Coll Surg 2005; 200(6) 965-972

27. Methods Review 718 well-documented cases in 210 articles since 1933 to 2003 Database included (1)age, (2)gender, (3)symptoms, (4)localization, (5)size of the tumor, (6)metastasis or invasion of adjacent tissues, (7)treatment, and (8)follow-up Database included (1)age, (2)gender, (3)symptoms, (4)localization, (5)size of the tumor, (6)metastasis or invasion of adjacent tissues, (7)treatment, and (8)follow-up

28. Results

29. Age Distribution

30. Results Mean age: 21.97 years (range 2~85) M:F = 64:626 = 1:9.78 Localization: tail(35.9%); head(34%); body(14.8%); body & tail(10.3%) Mean diameter: 6.08 cm (range 0.5~34.5 cm)

31. Size of Tumor

33. Metastasis or Invasion 97/497 (19.52%) patients were positive Liver: 27 Liver: 27 Portal vein: 26 Portal vein: 26 Spleen: 17 Spleen: 17 Other: duodenum, omentum, colon, lung, peritoneum, and vessels Other: duodenum, omentum, colon, lung, peritoneum, and vessels

35. Follow-up F/U <5 years: 309/467 (66.17%) F/U 5~10 years: 89/467 (19.6%) F/U > 10 years: 53/467 (11.35%) Recurrence: 31/467 (6.63%) Overall survival rate: 2 years: 97% 2 years: 97% 5 years: 95% 5 years: 95%

37. Acceptable Names in the Past Papillary cystic neoplasm Solid and papillary epithelial neoplasm Papillary and solid neoplasm Papillary and cystic tumor Papillary cystic carcinoma Solid and papillary neoplasm Papillary cystic tumor Solid and cystic tumor Solitary cystic tumor Solid and cystic papillary epithelial neoplasm Frantz’s tumor

38. Discussion SPTs have been incorrectly diagnosed as adenocarcinomas, cystadenomas or cystadenocarcinomas, papillary cystadenomas or cystadenocarcinomas, and islet cell tumors Lichtenstein (1934): papillary cystadenocarcinoma of the pancreas Lichtenstein (1934): papillary cystadenocarcinoma of the pancreas Frantz (1959): Tumors of the pancreas (Frantz’s tumor) Frantz (1959): Tumors of the pancreas (Frantz’s tumor) Kloppel (1981): solid and cystic acinar cell tumors of the pancreas Kloppel (1981): solid and cystic acinar cell tumors of the pancreas WHO (1996): solid pseudopapillary tumors WHO (1996): solid pseudopapillary tumors

39. Epidemiology SPTs account for 6% of all exocrine pancreatic tumors Cubilla(1979): 1/645 (0.17%) Cubilla(1979): 1/645 (0.17%) Morohoshi(1987): 7/264 (2.7%) Morohoshi(1987): 7/264 (2.7%) Warshaw(1990): 3/67 (4.48%) Warshaw(1990): 3/67 (4.48%) Koito(1997): 5/52 (10%) Koito(1997): 5/52 (10%) Lam(1999): 8 cases (2.5%) in a 24-year period Lam(1999): 8 cases (2.5%) in a 24-year period Sheeham(2003): 5/336 (1.48%) in a 9-year period Sheeham(2003): 5/336 (1.48%) in a 9-year period In children: 8%~16.6%

40. Pathogenesis Unclear β-catenin mutations Tanaka(2001), Abraham(2002) Tanaka(2001), Abraham(2002)

41. Clinical Features Presenting features of SPT are nonspecific Abdominal pain (the most common symptom) Abdominal pain (the most common symptom) Slowly enlarging, non-tender, upper abdominal mass Slowly enlarging, non-tender, upper abdominal mass Some are completely asymptomatic Some are completely asymptomatic

42. Differential Diagnosis Disoncogenetic cysts Retention cysts Pseudocysts Hydatid cysts Cystic tumors Cystadenoma, cystadenocarcinoma, microcystic adenoma, lymphangioma, hemangioma, various forms of sarcomas, mucinus cystic tumors, cystic islet cell tumors, acinar cell cystadenocarcinoma Cystadenoma, cystadenocarcinoma, microcystic adenoma, lymphangioma, hemangioma, various forms of sarcomas, mucinus cystic tumors, cystic islet cell tumors, acinar cell cystadenocarcinoma Secondary pancreatic involvement of tumors Neuroblastoma, leukemia, lymphoma, lymphoproliferative disorder Neuroblastoma, leukemia, lymphoma, lymphoproliferative disorder

43. Diagnostic Techniques Barium meal examinations: Deformity of stomach; enlargement of duodenal loop; narrowing of duodenal lumen Deformity of stomach; enlargement of duodenal loop; narrowing of duodenal lumenUltrasonography: Well-defined hypoechoic solid masses Well-defined hypoechoic solid masses CT scan: (most helpful) Identify a cystic mass or huge mass with cystic component; determine size, configuration; define pancreatic anatomy; recognize invasion Identify a cystic mass or huge mass with cystic component; determine size, configuration; define pancreatic anatomy; recognize invasionMRI: Provide information of resectability Provide information of resectability

44. Diagnostic Techniques ERCP: Displacement and rarely disruption of the main pancreatic duct Displacement and rarely disruption of the main pancreatic duct Percutaneous fine-needle cytology Endoscopic ultrasonography scan with fine-needle aspiration (EUS-FNA): Popular and useful in identifying the tumor Popular and useful in identifying the tumor

46. Treatment Surgical procedure is the main treatment Invasion to portal vein or SMA should not be included as a criterion for nonresectability Invasion to portal vein or SMA should not be included as a criterion for nonresectability Extensive lymphatic dissection or more radical local approaches are no indicated Extensive lymphatic dissection or more radical local approaches are no indicated Surgical debulking for metastasis Surgical debulking for metastasisChemotherapy Successful in some patients Successful in some patientsRadiotherapy Suggested in unresectable SPT (radiosensitive) Suggested in unresectable SPT (radiosensitive)

47. Conclusion SPT is an uncommon pancreatic tumor that mainly affects young women Preoperative diagnosis is difficult Surgical procedure is the main treatment Prognosis is good (low grade malignancy) Unresectable tumors: survival > 10 years Unresectable tumors: survival > 10 years Treated liver metastasis: survival > 5 years (range 6 months ~17 years) Treated liver metastasis: survival > 5 years (range 6 months ~17 years)