review of literature on clinical pancreatology - The Pancreapedia

vascular invasion (14/15). Abnormal findings in the pancreas and urinary tract were detectedin eight <strong>of</strong> 15 patients. In 13 patients with adequate specimens, moderate to severelymphoplasmacytic infiltration associated with dense fibrosis was observed. Infiltration <strong>of</strong>IgG4-positive plasma cells was moderate or severe in nine patients and minimal or absent infour patients. It was concluded that lymphoplasmacytic sclerosing cholangitis exhibitsrelatively characteristic clinical and CT findings, although they are not sufficiently specific fordifferentiation from other biliary diseases [310].Retroperitoneal fibrosisIt was presented a case <strong>of</strong> retroperitoneal fibrosis (RPF) in a 72-year-old man who previouslyreceived pancreatectomy for autoimmune pancreatitis. He had earlier received colectomy forearly colon cancer. During the routine follow-up for colon cancer, a swollen pancreas tail wasdetected on enhanced CT. He received distal pancreatectomy under the diagnosis <strong>of</strong>pancreas cancer two years later. Pathological diagnosis revealed the autoimmunepancreatitis. Eight months later, right hydronephrosis was observed in an abdominalultrasonographic study, and at the same time, right hydroureterosis due to retroperitoneals<strong>of</strong>t tissue mass around the bifurcation was detected on enhanced CT. He was treated withpredonisolone aiming at the diagnosis and therapy. Twelve weeks later, right hydronephrosishad disappeared and retroperitoneal mass had shrunken. Now, it is thought that autoimmunepancreatitis is a systemic sclerosing disease accompanied with extra-pancreatic pathologicchanges such as RPF [311].A 74-year-old male patient presented with progressive anorexia, cholestatic liver functiontests, and a diffuse enlarged pancreas suggestive <strong>of</strong> a pancreatic carcinoma. There was amarked elevation <strong>of</strong> total immunoglobulin G4 (IgG4) in serum. Further investigation led to thediagnosis <strong>of</strong> IgG4-related sclerosing disease with involvement <strong>of</strong> the pancreas,retroperitoneal fibrosis, and bilateral focal nephritis. This was the first report on these threeclinical entities occurring in the same patient. A short <strong>review</strong> <strong>of</strong> the <strong>literature</strong> concerningautoimmune pancreatitis and retroperitoneal fibrosis is made, with special interest to theconcept <strong>of</strong> IgG4-related pathology. This systemic disease can have several clinicalmanifestations: IgG4-positivity not only can be found in the pancreas, but also at the level <strong>of</strong>extrahepatic biliary ducts, gallbladder, salivary glands, retroperitoneal tissue, kidneys,ureters, and lymph nodes [312].DiagnosticsCTIt was investigated the clinical and radiological features <strong>of</strong> focal mass-forming autoimmunepancreatitis (FMF AIP) to help physicians avoid performing unnecessary surgery because <strong>of</strong>an improper diagnosis. It was evaluated 23 patients with chronic inflammatory pancreaticmasses and who underwent pancreatectomy for presumed pancreatic cancer from 1995 to2005. These patients were distinguished into 8 FMF AIP patients and 15 ordinary chronicpancreatitis patients through a histological <strong>review</strong>, along with considering the immunoglobulinG4 staining. Twenty-six randomly selected pancreatic cancer patients were also evaluatedas a control group. On the portal venous phase <strong>of</strong> computed tomography, 6 (86 %) <strong>of</strong> 7 FMFAIP patients showed homogeneous enhancement, whereas only 3 chronic pancreatitispatients (25 %) and none <strong>of</strong> the pancreatic cancer patients showed homogeneousenhancement. None <strong>of</strong> the FMF AIP patients showed upstream main pancreatic ductdilatation greater than 5 mm or proximal pancreatic atrophy. It was concluded that forpatients with a pancreatic mass, if their radiological images show homogeneous