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1 General Rules for the Study of Pancreatic Cancer April 2009 (The 6th Edition) Japan Pancreas Society - 1 -

2 I 1 II 2 III IV PCM 13 2 BCM 13 3 DPM V VI

3 VII TNM UICC - 3 -

4 I UICC TNM

5 II T N M X Stage T N M clinical findings surgical findings pathological findings final findings c,s,p,f final findings f pt3,pn1,sm0,f Stage III f CH DU S RP PV A PL OO p.5 pch pdu ps prp ppv sa ppl soo p.13 s p ppcm pbcm pdpm 1 clinical findings surgical findings pathological findings final findings - 5 -

6 III portion 1 2 Phb,Pbht Ph Pb Pt PV SMA SMV UP 1 portion 2 SMV PV - 6 -

7 2 cm TS tumor size TS1 2.0cm TS1 2.0cm TS2 2.0cm 4.0cm 2.0cm TS2 4.0cm TS3 4.0cm 6.0cm 4.0cm TS3 6.0cm TS4 6.0cm TS4 6.0cm p masked type nodular type infiltrative type cystic type - 7 -

8 ductectatic type mixed type 2 unclassifiable type 4 T CH DU S RP PV A PL OO Tis 1 T1 2cm 2 T2 2cm T3 CH DU S RP T4 3 PV A PL 4 OO TX 1 carcinoma in situ 2 2cm T RP3 4 S3 1 CH CH CHX 2 DU DU DUX 3 S S SX 4 RP RP RPX - 8 -

9 5 PV PV PVX PVp PVsm PVsp 6 A A AX Ach Asm Asp Ace 7 PL PL PLX PL 3 8 OO OO OOX 4 RP3 4 S3 3a 3b PL phi I PL phii II PL sma PL cha PL hdl PL spa PL ce - 9 -

10 M a 2 13b 3 14p 4 14d a1 a1 7 16a2 a2 8a 16b1 b1 8p 16b2 b2 9 17a 10 17b 11p 18 11d 12a 12p 12b a 14b 14c 14d 5 14a 14p 14d 14p 14d v 17b 4 12c 12b a 13b 17a 17b 8a 8p 10 11p 11d a 8p 12a 12b 12p 14p 14d p 14d p 11d a 12b 12p 13a 13b 15 16a2 16b a 17b 16a2 16b

11 膵癌取り扱い規約第6版案 N0 リンパ節転移 N1 1 群リンパ節のみに転移 N2 2 群リンパ節まで転移 N3 3 群リンパ節まで転移 NX リンパ節転移の程度が不明図 4 膵臓に関連したリンパ節番号図 5 肝十二指腸間膜内リンパ節の部位と境界右の図は左の図の A の部位における横断図を示す

12 6 7 2 M0 M1 MX M1 HEP PER LYM PUL PLE OSS MAR BRA SKI ADR OTH 1 N3 M

13 3 Stage 4 4 Stage M0 M1 N0 N1 N2 N3 Tis 0 T1 I II III T2 II III III T3 III III IVa T4 IVa IVb

14 IV (Hand-Assisted Laparoscopic Surgery ) PHR: pancreatic head resection PD: pancreaticoduodenectomy PPPD: pylorus-preserving PD SSPPD: subtotal stomach-preserving PD DPPHR: duodenum-preserving PHR II PHRSD pancreatic head resection with segmental duodenectomy DP : distal pancreatectomy DP(tail) DP(body-tail) DP(subtotal) TP total pancreatectomy TP total pancreatectomy PPTP pylorus-preserving total pancreatectomy spleen-preserving total pancreatectomy PPSPTP pylorus-preserving spleen-preserving total pancreatectomy duodenum-preserving total panceatectomy II TPSD total pancreatectomy with segmental duodenectomy MP middle pancreatectomy PR partial resection EN enucleation subtotal resection 2 portion PD subtotal DP subtotal

15 PD PPPD PD,PPPD I PD I, PPPD I, SSPPD I II PD II, PPPD II, SSPPD II III PD III, PPPD III, SSPPD III a b IV PD IV, PPPD IV, SSPPD IV A) B) C) D) PD- IIA- 1 PPPD- IVB- 2 DPPHR- C- 1 MP- A- 2 MP- D 3 1 pancreatic cut end margin PCM PCM PCM PCMX 2 bile duct cut end margin BCM BCM BCM BCMX 3 dissected peripancreatic tissue margin DPM

16 DPM DPM DPMX 4 D0 1 D1 1 D2 1 2 D residual tumor R R0 R1 R2 RX

17 V * 6 * Kaplan Meier generalized Wilcoxon

18 VI

19 9 2 1 Kerckring 5mm 10a 1 10b 10a 1 2 5mm 10a 2 10b

20 10a

21 10b CBD MPD APD Common bile duct Main pancreatic duct Duct of Santorini (accessory pancreatic duct)

22 3-22 -

23 VII 1 1 Epithelial neoplasms Exocrine neoplasms Serous cystic neoplasms (SCNs) a Serous cystadenoma (SCA) b Serous cystadenocarcinoma (SCC) Mucinous cystic neoplasms (MCNs) a Mucinous cystadenoma (MCA) b Mucinous cystadenocarcinoma(mcc) i) ii) non-invasive minimally invasive iii) invasive Intraductal papillary-mucinous neoplasms (IPMNs)* a Intraductal papillary-mucinous adenoma (IPMA) b Intraductal papillary-mucinous carcinoma (IPMC) i) ii) non-invasive minimally invasive iii) invasive * with mucin-hypersecretion without c) mucin-hypersecretion Intraductal tubular neoplasms(itns) etc atypical epithelium(ae) and carcinoma in situ(cis) Invasive ductal carcinoma i) Papillary adenocarcinoma (pap) ii) Tubular adenocarcinoma (tub) well differentiated (tub1) moderately differentiated (tub2) iii) Poorly differentiated adenocarcinoma (por) iv) Adenosquamous carcinoma (asc) v) Mucinous carcinoma (muc)

24 vi) Anaplastic carcinoma 1) giant cell type 2) osteoclast-like giant cell type/giant cell carcinoma of osteoclastoid type 3) pleomorphic type 4) spindle cell type vii others 6. Acinar cell neoplasms a) Acinar cell adenoma b) Acinar cell carcinoma B Endocrine neoplasms C Combined neoplasms D Epithelial neoplasms of uncertain differentiation a Solid-pseudopapillary tumor b Pancreatoblastoma c Undifferentiated carcinoma E Unclassifiable F Miscellaneous 2 Non-epithelial neoplasms Hemangioma Lymphangioma Leiomyosarcoma Malignant fibrous histiocytoma Malignant lymphoma Others Paraganglioma 2 medullary type intermediate type scirrhous type

25 3 INF INF INF INF INF 4 ly0 ly1 ly2 ly3 5 v0 v1 v2 v3 6 ne0 ne1 ne2 ne

26 mpd mpd mpd b b borderline 11 p

27 8 1 epithelial neoplasms A exocrine neoplasms 1 serous cystic neoplasms (SCNs) a serous cystadenoma microcystic cystadenoma, glycogen-rich cystadenoma 1 3 mm macrocystic serous cystadenoma b serous cystadenocarcinoma 2 mucinous cystic neoplasms MCNs a mucinous cystadenoma MCA 4 7 ovarian-type stroma b mucinous cystadenocarcinoma MCC 8 10 carcinoma in mucinous cystadenoma ovarian-type stroma non-invasive minimally invasive (invasive)

28 3 intraductal papillary-mucinous neoplasms IPMNs intraductal papillary tumors with mucin-hypersecretion without mucin-hypersecretion high-papillary growth low-papillary growth completely flat growth CIS a intraductal papillary-mucinous adenoma IPMA oncocytic type b intraductal papillary-mucinous carcinoma IPMC 18-21, , >22 53->25 loss of polarity minimally invasive

29 c intraductal tubular neoplasms ITNs intraductal tubular adenoma ITA intraductal tubular carcinoma ITC 4 atypical epithelium (AE) and carcinoma in situ CIS mm (5mm) low-papillary growth completely flat growth IPMN 5 PanIN Squamous metaplasia Epidermoid metaplasia, multilayered metaplasia. PanIN 1A Pyloric gland metaplasia, goblet cell metaplasia, mucinous hypertrophy, flat duct

30 lesion without atypia, mucinous ductal hyperplasia, simple hyperplasia, mucinous cell hyperplasia, flat ductal hyperplasia, nonpapillary epithelial hypertrophy. PanIN 1B Papillary hyperplasia, papillary duct lesion without atypia, and ductal hyperplasia. Mucinous metaplasia and pyloric gland metaplasia commonly involve small branch ducts or extend into lobules surrounding PanIN in ducts such involvement has been called adenomatoid or adenomatous hyperplasia, especially when the change dominates involvement of ductal epithelium and is regarded as part of the spectrum of PanIN 1. PanIN 2 Atypical hyperplasia, papillary duct lesion with atypia, low-grade dysplasia, and some cases of moderate dysplasia. PanIN 3 Carcinoma in situ, intraductal carcinoma, high-grade dysplasia, severe dysplasia, and some cases of moderate dysplasia. 5 invasive ductal carcinomas duct epithelium 1 4 a papillary adenocarcinoma b tubular adenocarcinoma desmoplasia 3 1 well differentiated type 33,34 35,36 2 moderately differentiated type 35,36 37,38 c) poorly differentiated adenocarcinoma 37,38 39,40 PAS alcian blue d adenosquamous carcinoma 39,40 41,

31 mucoepidermoid carcinoma 30 e mucinous carcinoma colloid carcinoma mucinous non-cystic carcinoma mucus nodule 50 signet ring cell carcinoma f anaplastic carcinoma undifferentiated carcinoma 1 undifferentiated carcinoma giant cell type pleomorphic type spindle cell type 2 3 giant cell carcinoma of osteoclastoid type so-called carcinosarcoma 6 acinar cell neoplasms a acinar cell adenoma acinar cell cystadenoma b acinar cell carcinoma B endocrine neoplasms

32 Grimelius chromogranin, synaptophysin WHO 1999 well differentiated endocrine tumor well differentiated endocrine carcinoma, poorly differentiated endocrine carcinoma a) 2cm 10 2 MIB1/Ki-67 2% benign behavior b) 2cm MIB1/Ki-67 2% uncertain behavior HPF 1 -oma Insulinoma gastrinoma, glucagonoma, somatostatinoma -oma -oma 2 Multiple endocrine neoplasia type 1 MEN1 Wermer's syndrome C combined neoplasms duct-islet cell carcinoma duct-islet-acinar cell carcinoma

33 D epithelial neoplasms of uncertain differentiation a solid-pseudopapillary tumor (solid cystic tumor) papillary cystic tumor solid cystic tumor xanthoma cell cholesterol granuloma 1 antitrypsin NSE b pancreatoblastoma 66,67 64,65 10 squamoid corpuscle organoid structure AFP c undifferentiated carcinoma E unclassifiable F miscellaneous other rare epithelial neoplasms 2 non-epithelial neoplasms hemangioma lymphangioma leiomyosarcoma malignant fibrous histiocytoma malignant lymphoma, paraganglioma

34 1 mm 2 PAS

35 a b c 12 Wirsung Santorini alcian blue alcian blue loss of polarity

36 mm PanIN back to back PanIN PanIN back to back PanIN PanIN

37 mucoepidermoid carcinoma ( ) ( ) bizarre giant cell ( ) osteoclast-like giant cell type/giant cell

38 carcinoma of osteoclastoid type ( ) perineural invasion cm

39 Solid pseudopapillary tumor Solid pseudopapillary tumor Solid pseudopapillary tumor 1 antitrypsin squamoid corpuscle N C PanIN 1A

40 71 69 PanIN 1A

41 TNM UICC 2002 ICD O C25 T N M T N M 1 1 C C C TNM T TX T0 Tis

42 T1 T2 T3 T4 2cm 2cm N NX N0 N1 M MX M0 M1 ptnm pt pn pm T N M pn0 10 pn0 G GX G1 G2 G3 G4 R R R RX R0 R1 R2-42 -

43 0 Tis N0 M0 IA T1 N0 M0 IB T2 N0 M0 IIA T3 N0 M0 IIB T1 N1 M0 T2 N1 M0 T3 N1 M0 III T4 N M0 IV T N M1 T1 T2 T3 T4 N1 2cm 2cm UICC TNM L. H. Sobin Ch. Wittekind

膵癌超音波診(案)

膵癌超音波診(案) 1 2 3 4 5 6 7 8 9 10 2009 7 6 2 a. invasive ductal carcinoma * 1 b. endocrine neoplasm c. Solid pseudopapillary neoplasm d. tumor producing pancreatitis * 2 * 1 invasive ductal carcinomas papillary adenocarcinoma