Appendix: WHO Classifcation of Tumors of the 2019

(Modifed from Lokuhetty D, White VA, • Mucinous cystic neoplasm with low-grade Watanabe R, Cree IA (eds). Digestive system dysplasia tumors. WHO classifcation of tumors 5th edi- • Mucinous cystic neoplasm with high-grade tion. Lyon: IARC Press; 2019, p. 296) dysplasia • Mucinous cystic neoplasm with associated invasive carcinoma Benign Epithelial Tumors and Precursors Malignant Epithelial Tumors • Serous cystadenoma NOS –– Macrocystic (oligocystic) serous cysta­ • Ductal adenocarcinoma NOS denoma –– Colloid carcinoma –– Solid serous adenoma –– Signet-ring (poorly cohesive cell) –– Von Hippel-Lindau syndrome-associated carcinoma serous cystic neoplasm –– Medullary carcinoma –– Mixed serous-neuroendocrine neoplasm –– Adenosquamous carcinoma • Serous cystadenocarcinoma NOS –– Hepatoid carcinoma • Glandular intraepithelial neoplasia, low grade –– Invasive micropapillary carcinoma • Glandular intraepithelial neoplasia, high –– Carcinoma, undifferentiated, NOS grade –– Undifferentiated carcinoma with • Intraductal papillary mucinous neoplasm with osteoclast-­like giant cells low-grade dysplasia • Acinar cell carcinoma • Intraductal papillary mucinous neoplasm with –– Acinar cell cystadenocarcinoma high-grade dysplasia –– Mixed acinar cell carcinoma-neuroendo- • Intraductal papillary mucinous neoplasm with crine carcinoma associated invasive carcinoma –– Mixed acinar cell carcinoma-ductal adeno- • Intraductal oncocytic papillary neoplasm carcinoma-neuroendocrine carcinoma NOS –– Mixed acinar-ductal carcinoma • Intraductal oncocytic papillary neoplasm with • Pancreatoblastoma associated invasive carcinoma • Solid pseudopapillary neoplasm of the • Intraductal tubulopapillary neoplasm pancreas • Intraductal tubulopapillary neoplasm with –– Solid pseudopapillary neoplasm with high-­ associated invasive carcinoma grade carcinoma

© Springer Nature Switzerland AG 2021 F. Campbell, C. S. Verbeke, Pathology of the Pancreas, 433 https://doi.org/10.1007/978-3-030-49848-1 434 Appendix: WHO Classifcation of Tumors of the Pancreas 2019

Pancreatic Neuroendocrine –– Gastrinoma Neoplasms –– VIPoma –– Glucagonoma • Pancreatic neuroendocrine microadenoma –– Somatostatinoma • Neuroendocrine tumor NOS –– ACTH-producing tumor –– Neuroendocrine tumor, grade 1 –– Enterochromaffn-cell carcinoid –– Neuroendocrine tumor, grade 2 –– Serotonin-producing tumor –– Neuroendocrine tumor, grade 3 • Neuroendocrine carcinoma NOS • Pancreatic neuroendocrine tumor, –– Large cell neuroendocrine carcinoma nonfunctioning –– Small cell neuroendocrine carcinoma –– Oncocytic neuroendocrine tumor, • Mixed neuroendocrine—non-neuroendocrine ­nonfunctioning pancreatic neoplasm (MiNEN) –– Pleomorphic neuroendocrine tumor, –– Mixed ductal adenocarcinoma-neuroendo- ­nonfunctioning pancreatic crine carcinoma –– Clear cell neuroendocrine tumor, –– Mixed ductal adenocarcinoma-­ ­non­functioning pancreatic neuroendocrine tumor –– Cystic neuroendocrine tumor, –– Mixed acinar cell carcinoma-­ ­nonfunctioning pancreatic neuroendocrine carcinoma • Functioning pancreatic neuroendocrine tumors –– Mixed acinar cell carcinoma-ductal adeno- –– Insulinoma carcinoma-neuroendocrine carcinoma Index

A Alpha-cell hyperplasia, 357 Acinar cell, 11 Alpha-fetoprotein, 185, 209, 212 carcinoma, 175, 203–214, 302, 341 Ampulla, 9 carcinoma cytology, 413–417 minor, 10, 21–22, 44, 274 cystadenocarcinoma, 210, 308 oozing mucin, 140, 274 cystadenoma, 305–308 swollen, 115 nodule, 67 vallecula, 21 Acinar cystic transformation, 305–308 of Vater (major), 9, 19–21, 44 Acinar dilatation (ectasia), 67 Ampullary cancer, 177 Acinar to ductal metaplasia, see Metaplasia Amylase, 12, 207 Acini, 11 Amyloid, 73, 132, 332 Acute pancreatitis, 87–92 Anastomosis, 25, 30 complications, 92 Anatomy, 5–11 etiology, 88 Angiolipoma, 217, 219 fbrosis, 96 Angiomatoid pattern (PanNET), 327 infectious, see Infection Angiomyolipoma, 219 mild (edematous/interstitial), 87, 90 Anterior surface (pancreas), 39, 47, 170 severe (hemorrhagic), 87, 90, 91 Artery recurrent, 93, 95, 102, 104, 235 gastroduodenal, 10, 47 Adenocarcinoma of pancreas, see Ductal hepatic, 25, 194 adenocarcinoma pancreatic, 10 Adenomyoma(tosis), 124, 126 pancreatoduodenal,10, 47 Adenomyosis, 238 splenic, 7, 10, 55 Agenesis and hypoplasia, 236 superior mesenteric (SMA), 6, 10, 26, 27, 47, 194 Alcohol abuse, 105, 124, 238 Ataxia telangiectasia, 82 Allograft (pancreas), 31, 127, 363, 364, 367, 371, 380 Atrophy, 95 Allograft rejection, 127 acinar, 95, 100, 110 acute cellular, 367, 371–375 lobulocentric, 70, 71, 83–84, 96, 140 arteritis, 373 ATRX, 321 ductitis, 373 Audit, 63 grading, 374–375 Autoimmune isletitis, 382 venulitis, 371 Autoimmune pancreatitis (AIP), 105–120, 123, 127, 129, antibody-mediated, 368–371 132, 394 acute, 369–370 cytology, 419–421 chronic active, 370 diagnostic algorithm, 118–120 C4d, 364, 370, 378, 379 differential diagnosis, 116–118 chronic, 367, 375–378 distinction from follicular pancreatitis, 129 staging, 376–377 eosinophils, 109, 127 chronic active cell-mediated, 375 etiology, 107 chronic allograft arteriopathy, 377–378 focal, 107 differential diagnosis, 379–380 frozen section, 394 hyperacute, 368–369 granulocytic epithelial lesion, 112 Alpha-1-antitrypsin, 208 idiopathic duct-centric pancreatitis, 107 Alpha-cell, 15, 376 IgG immunohistochemistry, 114

© Springer Nature Switzerland AG 2021 435 F. Campbell, C. S. Verbeke, Pathology of the Pancreas, https://doi.org/10.1007/978-3-030-49848-1 436 Index

Autoimmune pancreatitis (AIP) (cont.) Case review, 63 IgG4+/IgG+ ratio, 115 Castleman disease, 123 in children, 120, 132–133 Celiac trunk, 27, 194 lymphoid follicles, 111 Central fbrous scar, 251 lymphoplasmacytic sclerosing pancreatitis, 107 Central pancreatectomy, 28–29, 56 not otherwise specifed, 113 Centroacinar cell, 13 obliterative phlebitis, 111 Choledochal cyst, 245, 305, 310 periductal chronic infammation, 109 Chronic pancreatitis, 78, 93–104, 146, 393 storiform fbrosis, 111 calculus, see Stone type 1, 106–108 cancer risk, 81, 127–128, 147 type 2, 106–108 complications, 102, 103 unusual features, 112 cytology, 421 Autolytic change, 74 early, 72, 95, 102 etiology, 88, 89 focal, 94 B frozen section, 393–398 BCL10, 207 Churg-Strauss syndrome, see Eosinophilic Beckwith-Wiedemann syndrome, 236 granulomatosis with polyangiitis Beger procedure, 30, 58 Chymotrypsin, 12, 207 Beta-cell, 15, 376, 380, 382 Clear cell, 346 Beta-cell hyperplasia, 357 acinar cell carcinoma, 209 Bile duct ductal adenocarcinoma, see Ductal adenocarcinoma adenoma, 390–391 ectopic adrenal cortical nodule, 240, 342 brush cytology, 403, 405, 406, 421 intraductal tubulopapillary neoplasm, 288 cancer, 177 metastatic renal cell carcinoma, 228, 258 common (ductus choledochus), 8, 22, 44–47 pancreatic neuroendocrine neoplasm, 228, 240, 256, hamartoma, 390 258, 328, 346 Biliary IPMN, see Intraductal papillary neoplasm of the solid pseudopapillary neoplasm, 301 bile duct sugar tumor, 219 Biobanking, 33, 34 tumors (differential diagnosis), 258 Block key, 54 Collagen vascular disease, 130–132 Bone, 188 Common channel, 20 Branch-duct IPMN, see Intraductal papillary mucinous long, 237 neoplasm (IPMN) Congenital (developmental) anomaly, 235–242 Brush cytology, 403, 405, 406 Core biopsy in pancreas transplantation, 365–368 Bursa omentalis, see Lesser sac Cullen’s sign, 90 Cushing’s syndrome, 325 Cyst, 124, 305 C acinar, 305–308 Calcifcation, 266, 278, 332 choledochal, see Choledochal cyst eggshell, 262 classifcation, 245–246 sunburst, 250 cytology, 422–423 Cancer registry, 63 daughter, 262–263 Cancerization of ducts, 140, 141, 164 dermoid (mature cystic teratoma), 236, 305, 311, 312 Carcinoid syndrome, 325 endometriotic, 315 Carcinoma epidermoid, 305, 313 adenosquamous, 181, 182, 268 foregut (duplication), 236, 315, 316 colloid, 151, 182 hamartoma, 315 hepatoid, 185 honeycomb, 251 invasive micropapillary, 185 lymphoepithelial, 305, 310–312 medullary, 184 mucinous, 305, 308–309 mucinous, 145, 151 mucinous nonneoplastic, see Simple mucinous cyst mucinous noncystic, 145, 150, 182 pancreatic neuroendocrine tumor, 326 signet ring cell (poorly cohesive cell), 183 pancreatobiliary, 305, 309–310 squamous cell, 181 paraduodenal (groove) pancreatitis, 123–127 undifferentiated, 186, 267 parasitic, 316 anaplastic, 186 pseudocyst, see Pseudocyst carcinosarcoma, 186 retention, see Retention cyst sarcomatous, 186 sampling, 246–248, 264, 280 undifferentiated with osteoclast-like giant cells, simple mucinous, see Simple mucinous cyst 187–190, 267–268 squamous, 305, 310–315 Index 437

Cystic dystrophy of the duodenum, 124 carcinogenesis, 179 Cystic fbrosis, 73, 77–78, 82 clear cell pattern, 157, 228 Cystic lesions, 245, 305–317 concomitant, see Intraductal papillary mucinous Cytology, 403–432 neoplasm (IPMN) acinar cell carcinoma, 413–417 cystic papillary pattern, 158, 159, 285 algorithmic approach, 404 cytology, 408–412 autoimmune pancreatitis, 419–421 desmoplastic stroma, 151 bile duct brushings, 403, 405, 406, 421 differential diagnosis, 116, 170–179, 340, 343, 393 chronic pancreatitis, 421 epidemiology, 145–146 contaminants, 407, 408 etiology, 146–147 cyst fuid, 406 familial forms, 77, 82, 83, 140, 274 cystic lesions, 422–423, 425 foamy gland pattern, 156, 228 metastases, 431 frozen section, 393 molecular testing, 431 grading, 154–155, 192 mucinous cystic lesion, 423–427 immunohistochemical profle, 159–160 nonmucinous cysts, 427 intestinal type, 153 pancreatic ductal adenocarcinoma, 408–412 large duct pattern, 158 pancreatic neuroendocrine tumor, 412–413 major criteria, 394–397 Papanicolaou Society, 407 metastatic, 389, 392–393 paraduodenal (groove) pancreatitis, 421 minor criteria, 397–398 rapid on-site evaluation (ROSE), 404, 405 molecular diagnostics, 197–198 sample preparation, 405 neoadjuvant treatment, 55, 181, 191–197 serous cystic neoplasms, 427 pancreatobiliary type, 150–153 solid lesions, 406–408 staging, 164–168, 194 solid pseudopapillary neoplasm, 417–419 subtypes, 181–190 triple approach, 425 treatment and prognosis, 179–181 Ductulo-insular complex, 16, 73, 99 Duodenal adenocarcinoma, 177 D Duodenum-preserving pancreatic resection, 27–28 Daughter cyst, 262 Dysplasia, 266–267, 276, 281–282 DAXX, 321 Delta-cell, 15 Denuded epithelium, 246–247 E Dermoid cyst, see Cyst Ectopia, 237–242 Desmoplastic small round cell tumor, 216, 221, 343 Ectopic adrenal cortical nodule, 240, 342 Diabetes mellitus, 73, 147, 361 Ectopic hormone, 325 Digestive enzymes, 12 Ectopic (heterotopic) pancreatic tissue, 124, 238 Dissection technique, 36–59 Ectopic (heterotopic) spleen, 239, 305, 313 axial slicing, 37, 41, 43 Elastase, 12 bivalving/multivalving, 36 Embolization coil, 41 bread loaf slicing, 36 Embryology, 3–5 sagittal slicing, 55 Endocrine cell hyperplasia, 350, 355–358 for total pancreatectomy specimens, 56 Endocrine microadenoma(tosis), 350, 355 for other specimen types, 56–58 Endoscopic ultrasound (EUS) Distal pancreatectomy, 26–27, 55 fne-needle aspiration, 404 Diverticulum, 236, 245, 315 tissue acquisition, 404–405 Double duct sign, 147 Endoscopic retrograde cholangiopancreatoscopy Duct (pancreas) (ERCP) tissue branch, 44 acquisition, 405 dilatation in chronic pancreatitis, 96 Enucleation, 29, 58 ectasia, 72 Eosinophilic granulomatosis with polyangiitis, 127, 131 epithelial metaplasia, 69, 70 Eosinophilic hyaline globules, 297, 302, 331 intercalated, 13 Epidermoid cyst, 305 interlobular, 13 in intrapancreatic heterotopic spleen, 313 intralobular, 13 Epithelial subtype in IPMN, see Intraductal papillary main (Wirsung’s), 3, 4, 12, 14, 43 mucinous neoplasm (IPMN) rupture, 96, 285 Epsilon-cell, 15 Santorini’s, 4, 8, 21 Erythroblastosis fetalis, 236, 355 stricture, 96 Examination of the failed allograft, 364 system, 12–14, 43–47 External quality assurance, 63 Ductal adenocarcinoma, 145–201, 246 Extrainsular endocrine cell, 16 438 Index

F endocrine cell, 350, 355–358 Familial adenomatous polyposis (FAP), 82, 274, 282 periductal glands, 70 Familial atypical multiple mole melanoma (FAMMM), PP-cell, 357 82 Hypoglycemia, 323, 325, 355 Familial pancreatic cancer (FPC), 77, 82, 83, 140, 274 Fatty replacement, 72–73 Fibrous pseudotumor, 117 I Floating pancreas, 236 IgG4-related systemic disease, 107, 120–123 Follicular cholangitis, 129 Immunohistochemistry (differential diagnosis tables), Foregut (duplication) cyst, 236, 245, 305, 315, 316 230, 339 Frey procedure, 29, 56 Inborn errors of metabolism, 77 Frozen section, 389–400 Incipient IPMN, see Intraductal papillary mucinous major criteria, 394–397 neoplasm (IPMN) minor criteria, 397–398 Incomplete lumina, 396 pancreatitis, 393–398 Infection pancreatitis, 88, 92, 93, 127 pancreas allograft, 380 G Infammatory myofbroblastic tumor, 117, 123, 127, 217 Gangliocytic paraganglioma, 341, 343 Infammatory pseudotumor, 117, 123 Gastrinoma, 323, 343, 346 Inherited pancreatic cancer, see Ductal adenocarcinoma Gastrointestinal stromal tumor, 216 Inherited syndromes, 77, 78, 84, 346 Gerota´s fascia, 7, 27 Inking, 35, 37–39 , 15 Inspissated secretion, 95–97, 105, 126 Globules, see Eosinophilic hyaline globules , 15 Glucagon, 15 Insulin gene enhancer protein (ISL1), 337 Glucagon cell hyperplasia and neoplasia (GCHN), 347 Insulinoma, 323, 357 Glucagonoma, 324 Insulinomatosis, 323, 325, 348, 350, 357 Glucagonoma syndrome, 324, 348 Interstitial cell of Cajal, 17 Glue, see Sealant Interstitium, 16 Grading Intestinal mimicry, 154 ductal adenocarcinoma, see Ductal adenocarcinoma Intraductal (tumor) growth, 209, 212, 228, 285–286, 326 dysplasia, 266–267, 276, 281–282 Intraductal/intraepithelial neoplasm of low grade, either pancreatic endocrine neoplasia, 334, 335 PanIN or IPMN, 141, 284, 399 Granular cell tumour, 216 Intraductal oncocytic papillary neoplasm (IOPN), 273, Granulocytic epithelial lesion (GEL), see Autoimmune 286–287 pancreatitis Intraductal papillary mucinous neoplasm (IPMN), 83, Grey Turner’s sign, 90 273–286 Groove pancreatitis, see Pancreatitis associated invasive carcinoma, 245–246, 276–278, Gyriform pattern (PanNET), 327 280, 282–283 branch-duct type, 275, 278 classifcation, 273, 275 H concomitant ductal adenocarcinoma, 278, 285 Hamartoma, 124, 216, 245, 305, 315 cytology, 423–427 Hemangioma, 218 differential diagnosis, 140–141, 212, 258, 269, 283–285 Hemochromatosis (hereditary), 77–79 dysplasia, 276, 281–282 Hemorrhage, 90–92, 103 epithelial subtype, 275–276, 281 Hemosuccus pancreaticus, 104 extension into smaller ducts, 284–285 Hepatoid variant (PanNET), 330 frozen section, 399 Hereditary breast and ovarian cancer (HBOC) gastric type, 281 syndrome, 82 immunohistochemistry, 283 Hereditary exocrine disorders, 77 incipient, 284 Hereditary tumor predisposition syndromes, 82 intestinal type, 281 Histiocytosis X, 127 main-duct type, 275, 278 Honeycomb-like, 251 mixed-duct type, 275, 279 Human immunodefciency virus (HIV), 88, 89, 130 mucus extravasation, 285 Hydatid cyst, 305, 316 oozing mucin, 274 Hyperplasia pancreatobiliary type, 281 alpha-cell, 357 sampling, 280 beta-cell, 357 screening, 83–84 Brunner’s gland, 125, 126 skip lesions, 280, 399 Index 439

Intraductal papillary neoplasm of the bile ducts, 285 M Intraductal tumor propagation, see Cancerization Macrocystic serous cystadenoma, see Serous cystadenoma of ducts Macroscopic description, 52, 53 Intraductal tubulopapillary neoplasm (ITPN), 273, 287 Macroscopic examination, 33, 35 Intraoperative pancreatoscopy, 290 MAFA, 348 Intrathoracic pancreas, 236 Mahvash syndrome, 347 Intratumor heterogeneity, 154 Main-duct IPMN, see Intraductal papillary mucinous Ischemia, 364–365, 379 neoplasm (IPMN) Islet (of Langerhans), 14–16, 99 Major criteria, see Frozen section aggregates, 99 Malakoplakia, 129–130 amyloid, 73 Margin, see Resection margin compact, 14 Mature cystic teratoma, see Cyst cystic dilatation, 74 Meckel-Gruber syndrome, 236 diffuse, 15, 350 Melanin in solid pseudopapillary neoplasm, 301 transplantation, 362, 363 MEN1, see Multiple neuroendocrine neoplasia type 1 (MEN1) MEN4, see Multiple neuroendocrine neoplasia type 1 J (MEN4) Japan Pancreas Society (JPS), 36, 49 Mesenchymal neoplasia, 215 Johanson-Blizzard syndrome, 73 Metaplasia acinar to ductal, 51, 52, 68, 95, 140 goblet cell (intestinal), 69 K mucinous, 69, 137 Ki67 index, 208, 322, 334, 336, 412 oncocytic, 69, 96 squamous, 69, 96, 265, 305, 315 Metastasis, 225–234, 246, 389 L adrenal cortical carcinoma, 342 Left-sided pancreatectomy, 27 breast cancer, 228 Leiomyosarcoma, 217 colorectal cancer, 228 Lesser sac, 6, 39 cystic, 247 Li Fraumeni syndrome, 82 cytology, 431 Lipase, 12, 207 frozen section, 389, 392 Lipase hypersecretion syndrome, 203 hepatocellular carcinoma, 185, 343 Lipid-rich variant (PanNET), 328, 346 immunohistochemistry, 228, 230 Lipofuscin, 301, 332 malignant melanoma, 229, 343 Lipoma, 217–218 renal cell carcinoma, 157, 228–229, 240, 258, 341–342 Lipomatosis, see Fatty replacement Microcystic serous cystadenoma, see Serous cystadenoma Liposarcoma, 218 Minor criteria, see Frozen section Liver Mitotic count, 334 biopsy, 389 Mixed acinar-ductal carcinoma, 191, 210 focal nodular hyperplasia, 391 Mixed acinar cell carcinoma-ductal adenocarcinoma-­ metastasis, 392–393 neuroendocrine carcinoma, 210 reactive ductular proliferation, 391 Mixed acinar cell carcinoma-neuroendocrine Lobular architecture, 99, 172, 339, 394 carcinoma, 209, 210 Lobule, 4, 11, 72 Mixed-duct IPMN, see Intraductal papillary mucinous Lobulocentric atrophy, see Atrophy neoplasm (IPMN) Lupus erythematosus, 130 Mixed neuroendocrine-non-neuroendocrine neoplasm Luteinization (ovarian-type) stroma, 265 (MiNEN), 176, 191, 210, 322, 344 Lymphangioma, 218, 258 Mixed serous-neuroendocrine neoplasm, 250, 257 Lymphatic tumor propagation, 162, 338 Mucin profles (IPMN), 276 Lymph node, 11, 27, 49, 53 Mucinous cystic neoplasm (MCN), 247, 258, 261–271, glandular inclusion, 46, 168, 241 283, 305–308 IgG4-related disease, 123 associated invasive carcinoma, 261, 267–268 metastasis, 167–168 differential diagnosis, 258, 269–270, 308 sampling, 54 high-grade dysplasia, 261, 267 station, 49 involving the main pancreatic duct, 269 yield, 168, 195 low-grade dysplasia, 261, 266–267 Lymphoepithelial cyst, see Cyst mesenchymal overgrowth, 269 Lymphoma, 118, 221–223, 226, 343 sampling, 262, 264 Lynch syndrome, 82, 184 sarcomatous differentiation, 269 440 Index

Mucinous nonneoplastic cyst, see Simple mucinous cyst hereditary pancreatitis, 80, 140 Mucus extravasation, 285 high grade, 138–139 Multidisciplinary team, 61–63 intestinal variant, 139 Multiple endocrine neoplasia (MEN), 321, 323, 346, 347 low grade, 138 differential diagnosis, 355 oncocytic variant, 139 MEN1, 321, 346–347, 350, 355 screening, 83–84 MEN4, 346–347 Pancreatic neck, 5 surgical procedure, 27 Pancreatic neuroendocrine carcinoma (PanNEC), 176, Multivisceral resection, 28, 51, 53, 56, 194 210, 332–334 Muscular blood vessels, 28, 51, 53, 56, 150, differential diagnosis, 176, 210, 220–221, 343 158, 172 194, 396 large cell, 332 Mycobacterial infection, 88 prognosis, 346 Myeloproliferative disease, 226 small cell, 220, 322 Myoepithelial hamartoma, 124, 238 Pancreatic neuroendocrine neoplasia, 210, 321–322 Pancreatic neuroendocrine tumor (PanNET), 327–332 classifcation, 334–335 N cystic, 246, 247, 326 Naked tumor glands, 152, 394 cytology, 421 Necrolytic migratory erythema, 324 differential diagnosis, 176, 210, 219, 302, 339–344 Necrosis, 90, 112, 148, 204, 205, 373, 379, 392 frozen section, 389 fat, 89, 90, 379 functioning/nonfunctioning, 322–325 Needle-based confocal laser endomicroscopy, 245, 250, grading, 327 253, 274 immunohistochemistry, 335–337 Nesidioblastosis, 357 liver metastasis, 337 Neuroendocrine carcinoma (PanNEC), see Pancreatic microscopy, 327–332 neuroendocrine carcinoma (PanNEC) prognosis, 345 Neuroendocrine neoplasms of the ampulla, common bile proliferative activity, 334–337 duct, and duodenum, 343–344 staging, 337 Neuroendocrine tumor (PanNET), see Pancreatic variants, 328–331 neuroendocrine tumor (PanNET) Pancreatic polypeptide, 15 Neurofbromatosis (NF), 221, 324, 343, 346 Pancreatic primordia, 3, 4 Non-insulinoma-pancreatogeneous hypoglycemia Pancreatic stellate cell, 17 (NIPH), 357 Pancreatic tail, 5 Nucleoli, 206, 397 Pancreatitis acute, see Acute pancreatitis alcohol-related, 104–105 O autoimmune, see Autoimmune pancreatitis Oligocystic serous cystadenoma, see Serous cystadenoma cancer risk, 81, 127–128 Oncocytic variant (PanNET), 328 chronic, see Chronic pancreatitis Osteoid, 188 cytology, 421 Ovarian-type stroma, 261, 265–266, 268 eosinophilic, 127 follicular, 128–129 hereditary, 77, 79–82, 105, 128, 140, 237 P in children, 132 Pacinian corpuscle, 18 infectious, see Infection Pancreas annulare, 235 obstructive, 105, 149 Pancreas divisum, 132, 235–237 paraduodenal (groove), 123–127, 238 Pancreas transplantation, 361–384 peritumoral, 102, 149 Pancreatic anlage, 3 recurrent, 93, 95, 102, 104 Pancreatic body, 5 tropical, 105 Pancreatic duct, see Duct (pancreas) vasculogenic, 130 Pancreatic ductal adenocarcinoma, see Ductal Pancreatobiliary maljunction, 237 adenocarcinoma Pancreatoblastoma, 182, 211–212, 341 Pancreatic head, 5 Pancreatoduodenal crevice, 47, 49, 164, 170, 177 cancer, 145, 176 Pancreatoduodenal groove, 6, 123, 125 Pancreatic intraepithelial neoplasia (PanIN), 137–143, 178 Pancreatoduodenectomy, 25 chronic pancreatitis, 96 Papilla differential diagnosis, 140–142, 284 minor (lesser), 4, 10, 21–22 familial pancreatic cancer, 140 of Vater (major), 9 foamy variant, 139 Papillae, 209, 252 frozen section, 398, 399 Paraganglioma, 218–219, 341 Index 441

Paraganglion, 17 Sarcoidosis, 132 PEComa, see Perivascular epithelioid cell neoplasm Schwannoma, 221 Periampullary cancer, 150, 176 Screening, 82–84 Peribiliary gland hamartoma, 390–391 Sealant, 41 Perineural tumor propagation, 161, 338 Second opinion, 51, 63 Periodic acid-Schiff (PAS), 207, 216, 255, 340 Segmental pancreas transplant, 362 Peritoneal biopsy, 389 Serotonin, 16, 325 Peritoneal lining, 6 Serous cystadenocarcinoma, 250, 257 Perivascular epithelioid cell neoplasm (PEComa), Serous cystadenoma, 249 219–220, 228, 259, 342 macrocystic, 247, 249–251, 270, 283, 308 Persistent hyperinsulinemic hypoglycemia (PHIH), 236, 357 microcystic, 210, 249–252, 257 Peutz-Jeghers syndrome, 82, 274 microcystic with subtotal cystic degeneration, 257 Photodocumentation, 51 oligocystic, 249 Pigmented variant, 301, 331 Serous cystic neoplasm, 218, 249–260 Plasma cell granuloma, 117 classifcation, 250 Pleomorphic variant (PanNET), 328 cytology, 427 Polycystic disease, 236 differential diagnosis, 258 PP-cell, 15 locally aggressive growth, 257 PP-cell hyperplasia, 357 occurring within intrapancreatic heterotopic spleen, 313 Precursor lesion, 137, 178, 261, 273 with complex forid papillary architecture, 257 Primitive neuroectodermal tumor (PNET), 220–221, 343 Shwachman-Diamond syndrome, 73 Protein plug, 96, 126 Simple mucinous cyst, 269, 283–284, 305, 308–309 Psammoma body, 323, 324, 331, 340, 343 Situs inversus, 236 Pseudoaneurysm, 103 Sjögren’s syndrome, 132 Pseudocyst, 92, 102, 245, 246, 258, 270, 302 Smoking, 146 Pseudopapillae, 297, 306 Solid pseudopapillary neoplasm (SPN), 176, 210, Puestow procedure, 30 295–304, 340 cytology, 417–419 high-grade malignant transformation, 301 Q prominent microcystic pattern, 301 Quality monitoring, 51, 63 Solid serous adenoma, 220, 250, 255, 342 Solitary fbrous tumor, 221 Somatostatin, 15 R Somatostatinoma, 324, 343, 346 RB1/p16 pathway inactivation, 321, 335 Somatostatinoma (SSToma) syndrome, 324, 325 Reactive ductular proliferation, 391 Specimen handling, 33 Resection margin, 38, 168, 195, 338–339 Specimen type, 25–31 frozen section, 170, 398–399 Sphincter of Oddi, 19, 237 macroscopic involvement, 170 Spindle cell variant (PanNET), 331 microscopic involvement, 169–171 Splanchnoptosis, 236 neoadjuvant treatment, 195–197 Splenunculus, 239 pancreatic neuroendocrine neoplasia, 338 Sponge-like cut surface (serous microcystic adenoma), 251 periductal (radial bile duct), 39 Squamoid cyst of pancreatic duct, 305, 313 posterior, 39, 48, 170 Squamoid nest (morule), 211 SMA, 39, 48, 170 Stent, 39, 149 SMV, 38, 170 Stone, 94, 105 transection, 38, 55, 56, 170, 398 Sugar tumor, 219 venous resection, 170 Superior mesenteric vein (SMV) groove, 6, 38, 47 Retention cyst, 141, 269, 284, 305, 309–310 Synovial sarcoma, 221 Retroperitoneal mucinous cystic tumor, 270 Syphilis, 123 Rhabdoid variant (PanNET), 330 Systemic mastocytosis, 127 Rheumatoid arthritis, 130 Rosai-Dorfman disease, 117, 123 T Teratoma, 236, 312 S Thrombosis, 103, 363–364 Sacculi of Beale, 14, 22 Total pancreatectomy, 27, 56 Salt and pepper chromatin, 328 TP53, 321, 335 Sampling, 34, 53, 205 Transplantation pancreas, 27, 361–384 Sampling of cystic lesions, 246–248, 252, 264, 280 calcineurin inhibitor toxicity, 382 Saponifcation, 90 core biopsy, handling, 367 442 Index

cytomegalovirus infection, 380 V examination of failed allograft, 364 Vascular tumor propagation, 162, 338 failed allograft, 363 Vasculitis, 127, 130 indications, 362 Vasculogenic pancreatitis, 130 infections, 380 Vein ischemia/reperfusion, infection, 364–365 portal, 7, 25, 27, 122, 165, 194 islet cell, 362, 363 splenic, 7, 55 living donor, 362 superior mesenteric (SMV), 6, 25, 27, 47, 122, 165, 194 recurrent diabetes, 382 vv. pancreaticae, 11 rejection, 368–380 Vein resection, 38, 55, 170, 194 segmental, 362 Verner-Morrison syndrome, 323 surgery, 362 VIPoma, 323 surgical complications, 363 von Hippel-Lindau (VHL) syndrome, 228, 236, 245, surrogate donor duodenum biopsy, 367 250, 255–256, 258, 259, 324, 328, 342, 346, surrogate kidney biopsy, 367 350, 355 surveillance biopsies, 367 vascular thrombosis, 363–364 transverse mesocolon, 6 W Trisomy 13, 236, 240 WDHA syndrome, 323 Trypsin, 12, 207 Whipple’s resection, 25 Tuberous sclerosis complex (TSC), 324, 346 WHO classifcation of tumors of the pancreas, 433 Tumor regression, 191, 192, 196 Whole-mount sample, 54 grading of, 196–197

Z U Zollinger-Ellison syndrome, 323, 344 Uncinate process, 5 Zymogen granules, 12, 205