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Pancreas

Pancreatitis

Chronic pancreatitis


Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 22 November 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

See also subtypes below

General
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● Repeated attacks of pancreatic inflammation with loss of pancreatic parenchyma and replacement with fibrosis, variable pain and symptoms of pancreatic insufficiency (malabsorption, diabetes)
● May simulate or coexist with pancreatic carcinoma
Complications: pseudocysts in 10%, also pseudoaneursyms, polyarthropathy, avascular bone necrosis; rarely causes widespread metastatic fat necrosis (from liberation of lipase) affecting legs, mediastinum, pleura, pericardium, bone marrow, liver, skin (erythema nodosum like lesions), localized portal hypertension due to fibrosis of splenic vein in alcoholic hepatitis (Arch Pathol Lab Med 1997;121:612)
● Attacks precipitated by alcohol, overeating, opiates, other drugs
● Biliary disease usually not a factor in chronic pancreatitis
Other risk factors: hypercalcemia, hyperparathyroidism, hyperlipoproteinemia, pancreas divisum (seen in 12%), pancreatic neoplasm, cystic fibrosis; cigarette smoking; no known risk factor in 30%
● Also associated with mumps, polyarteritis nodosa, sarcoidosis, malakoplakia, primary sclerosing cholangitis, HIV (mild changes)

Epidemiology
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● Men, 40+, often alcoholics (70%)

Diagnosis
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● Clinical triad of steatorrhea, diabetes and pancreatic calcification on radiographic studies requires high degree of suspicion
● Mildly elevated amylase during attacks
● CT scan shows calcifications
● Weight loss, intractable abdominal pain, hypoalbuminemia and associated edema due to pancreatic insufficiency

Treatment
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● Pancreatic duct drainage, Whipple resection (relieves pain in 50% of patients with pain)

Gross description
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● Hard, shrunken, dilated ducts, visible calcified concretions (protein plugs), pseudocysts common
● 5% have obstruction due to tumor or stones

Gross images
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Whipple resection specimen due to chronic pancreatitis: cut surface of pancreatic head shows diffuse sclerosis with small calculi in main pancreatic duct, suggesting a "rock hard" tumor mass. Note the tubular stenosis of the bile duct in the upper pancreatic head


Main pancreatic duct in head-body region is irregularly dilated and contains calculi of varying sizes

Micro description
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● Loss of acini and ductal tissue with relative sparing of islets, irregularly distributed bland periductal fibrosis, variable obstruction of pancreatic ducts of all sizes
● Chronic inflammation (including mast cells) around lobules and ducts; perineural and intraneural inflammation with hyperplasia of the nerves
● Dilated ducts with concretions
● Ductal epithelium is atrophic, hyperplastic or undergoes squamous metaplasia
● Islets may become sclerotic and disappear
● Associated with Brunner gland hyperplasia in duodenum
● May have islet cell proliferation with invasive-like pattern (Islet aggregation) in peripancreatic adipose tissue

Micro images
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Focal fibrosis (upper left) was thought to be carcinoma at surgery, although no tumor was actually present


Advanced chronic pancreatitis with replacement of large areas of acinar parenchyma by sclerotic tissue and lobular aggregation of irregularly sized remaining ducts


Sclerotic area of pancreas with dilated duct (left) and clusters of remaining islets (right)


Dilated interlobular ducts containing protein plugs; surrounding tissue shows sclerosis and inflammatory reaction; note small islet at the bottom


Left: ducts are irregularly sized, but lining lacks cellular atypia; right: ducts (top) have benign appearing nuclei, islet is at bottom


Squamous metaplasia: mucinous epithelial duct cells are partly replaced by squamous epithelium


Figures 3 and 4

Positive stains
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● Trichrome (fibrosis), actin

Differential diagnosis
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● Pancreatic xanthomatous neuropathy associated with hyperlipidemia (Hum Pathol 1993;24:1023)

Additional references
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Arch Pathol Lab Med 2001;125:1051, Arch Pathol Lab Med 2000;124:1302, Hum Pathol 2001;32:1174, Am J Surg Pathol 2003;27:110


Subtypes of chronic pancreatitis

Familial hereditary pancreatitis
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● Childhood onset, first decade, increased risk for pancreatic carcinoma
● Autosomal dominant with mutation at trypsinogen (PRSS1), codon 117 (80% cases) that removes a proteolytic cleavage site, causing persistent trypsin activation
● Other mutations in cystic fibrosis transmembrane conductance regulator (CFTR), or in the serine protease inhibitor Kazal type 1 (SPINK1)

Paraduodenal or groove pancreatitis
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● Also called adenomyoma/myoadenomatosis, cystic dystrophy of heterotopic pancreas, pancreatic hamartoma of pancreatic wall, or periampullary or periduodenal wall cyst
● Scar develops between head of pancreas and duodenum
● Involves an area between the pancreas, common bile duct, and duodenum, typically involves the minor papilla
● Associated with alcohol abuse, tobacco smoking, and hypertension
● Duodenal submucosa and muscularis show a prominent chronic inflammatory cell infiltrate extending to pancreas, myoid proliferation, Brunnerís gland hyperplasia

Non-alcoholic chronic tropical pancreatitis
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● Younger individuals, in India, southern Asia, central Africa, and Brazil
● Due to protein-calorie malnutrition, dietary cyanogen toxicity, antioxidant deficiency, genetic predisposition (mutations in pancreatic secretory trypsin inhibitor gene)
Gross description: shrunken, firm, nodular, gritty pancreas due to calculi present diffusely in dilated ducts
Micro description: pancreatic ducts with denuded epithelium, squamous metaplasia, periductal lymphoplasmacytic infiltrate, acinar and islets atrophy, fibrosis

Infectious pancreatitis
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● In immunocompromised patients

End of Pancreas > Pancreatitis > Chronic pancreatitis


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