Pancreatic cancer classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2]

Overview

Pancreatic cancer can be classified into malignant and borderline malignant. Duct cell carcinoma is the most common type of pancreatic cancer.

Classification

• Cellular Classification of Pancreatic Cancer

• Pancreatic cancer includes the following carcinomas:

• Malignant

• Duct cell carcinoma (90% of all cases)
• Acinar cell carcinoma
• Adenosquamous carcinoma
• Cystadenocarcinoma (serous and mucinous types)
• Giant cell carcinoma
• Invasive adenocarcinoma associated with cystic mucinous neoplasm or intraductal papillary mucinous neoplasm
• Mixed type (ductal-endocrine or acinar-endocrine)
• Mucinous carcinoma
• Pancreatoblastoma
• Papillary-cystic neoplasm (Frantz tumor). This tumor has lower malignant potential and may be cured with surgery alone
• Papillary mucinous carcinoma
• Signet ring carcinoma
• Small cell carcinoma
• Unclassified
• Undifferentiated carcinoma

• Borderline Malignancies

• Intraductal papillary mucinous tumor with dysplasia
• Mucinous cystic tumor with dysplasia
• Pseudopapillary solid tumor

• Types of Pancreatic Cancers:

Types of Pancreatic Cancers
Pancreatic Exocrine Cancers
Adenocarcinoma
Acinar cell carcinoma
Adenosquamous carcinoma
Giant cell tumour
Intraductal papillary mucinous neoplasm (IPMN)
Mucinous cystadenocarcinoma
Pancreatoblastoma
Serous cystadenocarcinoma
Solid and pseudopapillary tumours
Pancreatic Endocrine Cancers (Pancreatic Neuroendocrine Tumours)
Gastrinoma
Glucagonoma
Insulinoma
Nonfunctional islet cell tumour
Somatostatinoma
Vasoactive intestinal peptide releasing tumour (VIPoma)

• Types of Pancreatic Intraepithelial Neoplasia (PanIN):

Types of Pancreatic Intraepithelial Neoplasia (PanIN)
PanIN 1 (low grade)
Minimal degree of atypia
Subclassified into PanIN 1A: absence of micropapillary infoldings of the epithelium; and 1B, presence of micropapillary infoldings of the epithelium
PanIN 2 (intermediate grade)
Moderate degree of atypia, including loss of polarity, nuclear crowding, enlarged nuclei, pseudostratification, and hyperchromatism
Mitoses are rarely seen
PanIN 3 (high grade/carcinoma in situ)
Severe atypia, with varying degrees of cribriforming, luminal necrosis, and atypical mitoses
Contained within the basement membrane

• TNM classification for pancreatic cancer:

TNM Classification for Pancreatic Cancer:
Primary tumor
TX	Primary tumor cannot be assessed
T0	No evidence of primary tumor
Tis	Carcinoma in situ
T1	Tumor limited to the pancreas, ≤2 cm in greatest dimension
T2	Tumor limited to the pancreas, >2 cm in greatest dimension
T3	Tumor extends beyond the pancreas but without involvement of the celiac axis or the superior mesenteric artery
T4	Tumor involves the celiac axis or the superior mesenteric artery (unresectable primary tumor)
Regional lymph nodes
NX	Regional lymph nodes cannot be assessed
N0	No regional lymph node metastasis
N1	Regional lymph node metastasis
Distant metastases
M0	No distant metastasis
M1	Distant metastasis

• Stage grouping of pancreatic cancer:

Stage grouping of pancreatic cancer:
Stage	T	N	M
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	Any N	M0
IV	Any T	Any N	M1

• Functional Pancreatic Neuroendocrine Tumors and their Characteristics are mentioned in the following table:

Functional Pancreatic Neuroendocrine Tumors and their Characteristics
Tumor type and syndrome	Location in pancreas	Signs and symptoms	Circulating biomarkers
Insulinoma (Whipple’s triad)	Head, body, tail (evenly distributed)	Hypoglycemia, dizziness, sweating, tachycardia, tremulousness, confusion, seizure	CgA and CgB, insulin inappropriate for blood glucose level, proinsulin, C-peptide
Gastrinoma (Zollinger–Ellison)	Gastrinoma triangle Often extrapancreatic (duodenal); can be found anywhere in gland	Gastric acid hypersecretion, peptic ulcer, diarrhea, esophagitis, epigastric pain	CgA, gastrin, PP (35%)
VIPoma (Verner– Morrison syndrome, WDHA)	Distal pancreas (body and tail) Often spread outside pancreas	Watery diarrhea, hypokalemia, achlorhydria (or acidosis)	CgA, VIP
Glucagonoma	Body and tail of pancreas Often large and spread outside pancreas	Diabetes (hyperglycemia), necrolytic migratory erythema, stomatitis, glossitis, angular cheilitis	CgA, glucagon, glycentin
Somatostatinoma	Pancreatoduodenal groove, ampullary, periampullary	Gallstones, diabetes (hyperglycemia), steatorrhea	CgA, somatostatin
Ppoma	Head of pancreas	None	CgA, PP