Case Capsule

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PANCREATIC MASS

Moderator: Dr K.R.PalaniswamySenior consultant,

Dept of Gastroenterology,Apollo Hospitals, Chennai

TNISGCON 2015PANEL DISCUSSION

Panelists

• Dr Anil Arora• Dr G.N.Ramesh• Dr R.Surendran• Dr Rochita• Dr Ashok Parameswaran

History• 47 y, male• Diabetic : 7 yrs

-recent worsening in last 3 months

• Left upper abdominal pain: 6 months-intermittent , each episode lasting for 15 min

• Back pain: 15 days; continuous, moderate• Weight loss -3 kgs in 3 month• No h/o anorexia, vomiting

Examination

• Moderately built• No pallor/ icterus or lymphadenopathy• Abdomen: Soft, non tender. No palpable mass

Questions

• Dr Anil Arora: What are the possibilites?• Dr Ramesh: How will you proceed?

Previous investigations

• CBC-N• LFT,RFT-N• Amylase, Lipase: Normal; CA 19-9: Normal• USG abdomen:

– Ill defined echopoor mass of 27mm in body of pancreas.

– Gallbladder wall thickened with a 5mm polypoid mucosa

CECT ABDOMEN

Questions

• Dr Rochita: Comment on CT abomen

CECT abdomen

• Ill defined hypodense mass lesion 3.9 x 2.3 cms seen in uncinate process with minimal fat stranding

• Another mass in body of pancreas 2.3 x 2.5 cms seen in body of pancreas abutting splenic artery

• Both lesions show contrast enhancement• MPD mildly prominent in body with parenchymal

atrophy in body and distal tail• Small peripancreatic nodes • Imp: Possiblity of pancreatic malignancy to be

considered

Questions

Dr Anil Arora: How to proceed?

Dr Ramesh: Role of EUS in pancreatic malignancy?

Gress et al. GI Endoscopy 1999

EUS diagnostic accuracy

• Staging sensitivity with EUS has consistently been reported to be over 90%.

• EUS is also more accurate in the assessment of vascular invasion that might preclude surgical resection (compared to h-CT/MRI)

Rösch et al. Gastrointest Endosc Clin N Am 1995;5:735-9.Yasuda et al. Endoscopy 1993;25: 151-5.Palazzo et al. Endoscopy 1993;25:143-50.

Brugge et al. GI Endosc 1996;43:561-7.

Pancreatic CancerPancreatic CancerEndoscopic US

Recent evidence suggests that EUS is similar to CT in diagnosis and staging of pancreatic cancer. EUS requires special endoscopic skills and expertise, and it is less readily available worldwide.

A 2.5cm round, hypoechoic tumor is identified in the the

region of the genu. The superior mesenteric vein can be seen separate from the

tumor.

Invasion of the dilated CBD by a large irregular

hypoechoic tumor located in the head of pancreas.

A large hypoechoic tumor is seen to invade the portal vein (arrow), with loss of tumor-vessel interface and tumor extension into vessel lumen.

The dilated CBD contains echogenic sludge.

FNA for solid pancreatic mass• All areas of pancreas including uncinate process and

tail, diameter >5mm• Low complication rate ~ 1-2% (pancreatitis,

haemorrhage)• ? Risk of peritoneal seeding

Sensitivity Sensitivity specificityspecificity PPVPPV NPVNPV

75-93%75-93% 95-100%95-100% 100%100% 25-85%25-85%

Vilmann, Wiresma, Giovannini, Chang, Gress, Bhutani, Hawes, Williams, Palazzo

Suspected pancreatic mass

Ultrasound

H-CT

No MassMass

EUS +/- FNA

Bx (CT, EUS or ERCP)

Palliation (biliary stent/CPN, oncology)

ERCP/Stent

Surgery

Cholangitis OR delayed

resection + jaundice/pruritu

s

Unresectable

Resectable

Unresectable

Resectable

Unresectable

Dr Surendran: Is tissue diagnosis required before surgery?

Surgery

• Laparotomy, locally advanced hard pancreatic mass- uncinate (7cms) and body (5cms) of pancreas, encasing SMA and SMV, hepatic artery and infiltrating serosa of posterior stomach.

• Trial dissection, trucut biopsy of pancreatic tumours and hepatic artery node excision done.

• Laparotomy, trial dissection, trucut biopsy of pancreatic tumours and hepatic artery node excision done

• Dr Surendran’s comments:

Histopathology pictures

HISTOPATHOLOGY

HISTOPATHOLOGY

HISTOPATHOLOGY

HISTOPATHOLOGY

Histopathology

• Pancreatic core biopsies: chronic inflammatory lesion with eosinophilia and dense fibrosis, ductular proliferation and granuloma-no malignancy

• Node- reactive inflammatory changes

• Dr Ashok Parameswaran’s comments:

Dr Rochita: Role of PET-CT in pancreatic malignancy?

Questions

• Dr Anil Arora: What are the possibilities… DD for inflammatory mass of pancreas?

• Dr Ramesh: Can this still be malignant mass? How to proceed?

Two weeks later

• Yellowish discolouration of eyes• Itching• Palpable ill defined mass in epigastrium• Bilirubin: 5mg/dl (Direct: 3.7 mg/dl)• ALP:560 U/L• ALT/AST: 78/60

• Dr Anil Arora: How to proceed?

• Dr Anil Arora: How to proceed?

CECT ABDOMEN

CECT ABDOMEN

Our case Case for comparison

• Dr Rochita’s comments

CECT abdomen• The uncinate process of pancreas is enlarged. However, it

reveals normal enhancement in the venous phase. A similar smaller area is seen in the mid body pancreas. The adjacent main pancreatic duct is narrowed.In the tail and head of pancreas, the main pancreatic duct is dilated. A rim of hypodensity is seen around the lesion in the uncinate process and body of pancreas. Peripancreatic fat planes are hazy

• Few small enhancing peripancreatic nodes are seen.

• The terminal common bile duct is compressed. Proximal to this, the biliary tree is mildly dilated

CECT abdomen• Enhancing soft tissue is seen along side the lesion in

body of pancreas and adherent to the common hepatic artery

• IMPRESSION- CT findings could be suggestive of autoimmune pancreatitis involving uncinate process and body. The soft tissue along the body of pancreas can be post operative changes.– However, the possibility of an atypical neoplasm

cannot be ruled out. A close follow up would be worthwhile.

Questions

• Dr Ramesh: What next? Diagnostic criteria for autoimmune pancreatitis?

HISORt CRITERIA OF AIPCategory CriteriaA. Histology 1. Diagnostic (any one):

a) Pancreatic histology showing periductal lymphoplasmacytic infiltrate with obliterative hlebitis (LPSP) b) Lymphoplasmacytic infiltrate with abundant (>10 cells/hpf) IgG4 positive cells in the pancreas2. Supportive (any one) a) Lymphoplasmacytic infiltrate with abundant (>10 cells/hpf) IgG4 positive cells in involved extra-pancreatic organ b) Lymphoplasmacytic infiltrate with fibrosis in the pancreas

B. Imaging Typical imaging features:1. CT/MR: diffusely enlarged gland with delayed (rim) endhancement2. ERCP: Diffusely irregular, attenuated main pancreatic duct

Atypical Imaging Features: Pancreatitis, focal pancreatic mass, focal pancreatic duct stricture, pancreatic atrophy, pancreatic calcification

C. Serology Elevated serum IgG4 level (normal 8-140 mg/dl)

D. Other Organ involvement

Hilar/intrahepatic biliary strictures, persistent distal biliary stricture, Parotid/lacrimal gland involvement, Mediastinal lymphadenopathy, Retroperitoneal fibrosis

E. Response to steroid therapy

Resolution/marked improvement of pancreatic/extrapancreatic manifestation with steroid therapy

CLINICAL DIAGNOSTIC CRITERIA FOR AIP 2006

1. Diffuse or segmental narrowing of the MPD with irregular wall and diffuse or localized enlargement of the pancreas by imaging studies, such as abdominal US, CT, and magnetic resonance2. High serum γ-globulin, IgG, or IgG4, or the presence of autoantibodies such as antinuclear antibodies and rheumatoid factor3. Marked interlobular fibrosis and prominent infiltration of lymphocytes and plasma cells in the periductal area, occasionally with lymphoid follicles in the pancreas

Diagnosis of AIP is established when criterion 1 and criterion 2 and/or 3 are fulfilled. However, it is necessary to exclude malignant diseases.

AUTOIMMUNE PANCREATITIS

23,0% FOCAL FORM(LIKE MALIGNANT LESION)23,0% FOCAL FORM(LIKE MALIGNANT LESION)

DIFFUSE FORM 77,0%(LIKE ACUTE PANCREATITIS) DIFFUSE FORM 77,0%(LIKE ACUTE PANCREATITIS)

Dr Anil Arora: Sensitivity of Ig G4?

Dr Ashok Parameswaran: Histological criteria for autoimmune

pancreatitis?

Histopathological Features of IgG4-Related Disease-Type I AIP(Predominantly Lobular)

1. Dense lymphoplasmacytic infiltrate

2. Fibrosis, arranged at least focally in a storiform pattern

3. Obliterative phlebitis

Consensus Statement on the Pathology of IgG4-Related Disease Mod Pathol. 2012;25(9):1181-1192

Fibrosis, arranged in a storiform pattern

Dense lymphoplasmacytic infiltrate

Obliterative phlebitis

Histopathological Features of Type II AIP (Predominantly ductal)

• Periductal lymphoplasmacytic infiltrate• Granulocytic epithelial lesion( Neutrophils in

ducts)• Neutrophils within acini

• No storiform fibrosis• No increased IgG4 plasma cells

1.Presence of epithelioid granulomas

2.Prominent neutrophilic infiltrate

Consensus Statement on the Pathology of IgG4-Related Disease

Mod Pathol. 2012;25(9):1181-1192

Histopathological Features Inconsistent With a Diagnosis of IgG4-

Related Disease

Vikram Deshpande, Mari Mino-Kenudson, William Brugge, and Gregory Y. Lauwers (2005) Autoimmune Pancreatitis: More Than Just a Pancreatic Disease?A

Contemporary Review of Its Pathology. Archives of Pathology & Laboratory Medicine: September 2005, Vol. 129, No. 9, pp.

1148-1154.

• Epithelioid cell granulomas have been reported only rarely in association with AIP. However, in a review of 19 pancreatectomy specimens we identified granulomas in 6 cases (32%). In 3 of these cases, granulomas were identified in almost every section examined. In all cases, the granulomas showed an exquisite ductocentric location The lack of nodal involvement and the periductal distribution help exclude pancreatic sarcoidosis and other granulomatous pathologies, including mycobacterial infections.

Types of AIP

AIP TYPE IFeatures of IgG4 disease

Older patients ( 70s)

Serum IgG4 increased

Tissue IgG4 positive plasma cells

AIP TYPE IINo features of IgG4 disease

Younger patients ( 50s)

No Serum IgG4 increase

IgG4 positive plasma cellsnot noted

• IgG4 : Elevated• Started on steroids

Questions• Dr Ramesh: What are the types of autoimmune

pancreatitis?• Dr Rochita: Imaging features differentiating

autoimmune pancreatitis and malignancy.• Dr Surendran: What proportion of Whipple’s surgery

specimen are autoimmune pancreatitis? Indian experience?

• Dr Ashok Parameswaran: Histological findings in other organs involved in IgG4 associated pancreatitis.

• Dr Anil Arora: What proportion of autoimmune pancreatitis respond to steroids? Do they need maintenance treatment? What is the relapse rate?

Types of autoimmune pancreatitis

COMPARISON OF TYPE 1 AND TYPE 2 AIPType 1 AIP Type 2 AIP

Mean age Sixth decade Fourth decade

Gender distribution Predominantly male Equal

Histological pattern Lymphoplasmacytic sclerosing pancreatitis

Duct-destructive pancreatitis

Histological hallmarks Periductal lymphoplasmacytic infiltrateSwirling fibrosisObliterative venulitis

Lymphoplasmacyic infiltrateGranulocyte epithelial lesion with partial/complete duct obstruction

IgG4 cells on immunostaining

Moderate-severe (98%) Moderate (40%) in one study

Serum IgG4 levels Elevated Normal

Other organ involvement Chronic sclerosing sialadenitis, IgG4-associated cholangitis, retroperitoneal fibrosis, IgG4-associated tubulointerstitial nephritis

Inflammatory bowel disease

AIP,autoimmune pancreatitis, IgG4, immunoglobulin G4

CLINICAL PRESENTATIONS OF TYPE 1 AUTOIMMUNE PACREATITIS

Clinical presentations of type I AIP

Pancreatic Predominantly extra-pancreatic

Biliary stricture, sclerosing cholangitis

Interstitial nephritis, renal failure

Retroperitoneal fibrosiswith complications

(e.g., ureteral obstruction)

Acute Post-acute/late

Obstructive jaundice

Pancreatitis

Steatorrhea

Persistent mass

Steatorrhea

Calcification,atrophy

Park, D.H. 2009

Repeat CECT abdomen pictures

• Dr Rochita’s comments:

Follow up

• On review after 4 weeks, significant reduction in size of pancreatic mass; jaundice resolved.

• Repeat CECT abdomen after 3 months, no mass lesion noted.

THANK YOU