Re: Pancreatic mass or cyst... Has anyone else had one of these?

[Guest guest]

Don't assume pancreatic malignancy! In PSC pancreatic masses can

occur, and those with these masses may actually be a sub-group of

patients with " Pancreatic pseudotumor with sclerosing pancreato-

cholangitis " or " Autoimmune pancreatocholangitis " :

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Am J Gastroenterol. 2004 Feb;99(2):377-82.

Pancreatic pseudotumor with sclerosing pancreato-cholangitis: is this

a systemic disease?

Toosi MN, Heathcote J

Department of Medicine, University Health Network, Toronto Western

Hospital, University of Toronto, Toronto, Ontario, Canada.

OBJECTIVE AND METHOD: Primary sclerosing cholangitis (PSC) is a

disease that predominantly affects the biliary tree, although the

pancreas may also be affected. A review of the presenting features of

all patients given a diagnosis of PSC at a single center was

conducted. The aim was to clarify the presentation of patients with

pseudotumor of the pancreas in this patient population. RESULTS:

Seventy-two patients were diagnosed with PSC either by ERCP (63/72 =

88%) or by liver biopsy (9/72 = 12%). The diagnosis of PSC was made

following referral for abnormal liver tests (67%), jaundice (17%),

and acute cholangitis (5%). Inflammatory bowel disease (IBD) (60%),

non-insulin-dependent diabetes mellitus (NIDDM) (13%), thyroid

disease (8%), and pancreatic disease (7%) were the major coexistent

extrahepatic diseases. Three patients, all with marked weight loss,

who presented with jaundice, abdominal pain, and/or diarrhea were

found to have a pancreatic mass at first presentation. Clinical and

radiological findings suggested pancreatic malignancy, and only later

was advanced sclerosing cholangitis identified. The biopsy of the

pancreas in two of these three patients revealed chronic

pancreatitis. The long-term follow-up and good clinical response to

medical therapy confirmed lack of pancreatic malignancy. These three

patients all had other evidence of systemic involvement:

submandibular gland fibrosis and urethral stricture in one,

fibromuscular dysplasia of the renal artery in another, and

retroperitoneal fibrosis in the third. None had IBD. CONCLUSION:

Pancreatic pseudotumor with sclerosing pancreato-cholangitis may be a

manifestation of a systemic disease characterized by nonmalignant

strictures and multifocal fibroinflammatory processes, unlike

classical PSC. PMID: 15046232.

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Scand J Gastroenterol Suppl. 2006 May;(243):70-8.

Autoimmune pancreatocholangitis: a series of ten patients.

van Buuren HR, Vleggaar FP, Willemien Erkelens G, Zondervan PE,

Lesterhuis W, Van Eijck CH, Puylaert JB, Van Der Werf SD

Department of Gastroenterology and Hepatology, Erasmus Medical

Centre, Rotterdam, The Netherlands. h.vanbuuren@...

BACKGROUND: During a 10-year period we observed 10 patients who

suffered from an inflammatory-fibrosing disease mimicking pancreatic

carcinoma and primary sclerosing cholangitis (PSC). METHODS: A review

of the presenting features, the clinical course and the relevant

literature. RESULTS: Ten male patients (mean age 55 years) presented

with weight loss, jaundice and pruritus. Pancreatic cancer was

suggested by imaging studies, which showed focal or generalized

pancreatic enlargement and compression of the distal common bile

duct. Cholangiography also demonstrated intrahepatic biliary stenoses

consistent with sclerosing cholangitis. None had evidence of IBD.

Exocrine pancreatic insufficiency was found in six cases and diabetes

in four. Pancreatic histology (n=3) showed fibrosis and extensive

inflammatory infiltrates. Immunosuppressive treatment was instituted

in five patients. Clinical and biochemical remission occurred in

three; in one other patient, previously documented intrahepatic

biliary strictures had disappeared after 3 months. One patient had

concomitant Sjögren's disease. The clinical features, pancreatic

involvement, age at presentation, absence of IBD and response to

steroids all plead against a diagnosis of " classical " PSC. The

natural course of the disease was highly variable. Thirty-five

comparable cases, with a largest series of three, have been reported

in the literature. The disease has been associated with Sjögren's

disease, retroperitoneal fibrosis and other fibrosing conditions, and

may be a manifestation of a systemic fibro-inflammatory disorder.

CONCLUSION: Autoimmune pancreatocholangitis is a distinct

inflammatory disorder involving the pancreas and biliary tree. The

disease may mimick pancreatic carcinoma and PSC and responds to

immunosuppressives. PMID: 16782625.

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You'd want to rule this out before agreeing to any pancreatic surgery

or chemotherapy because it responds well to immunosuppressive therapy.

Best regards,

Dave

(father of (23); PSC 07/03; UC 08/03)