Case 568 -- A 53-year-old man with abdominal distention

Contributed by Timothy S Gorrill, MD and Larry Nichols, MD


CASE HISTORY:

A 53-year-old white male with a history of alcohol abuse and pancreatitis presented to the emergency department with a 6-month history of nausea, vomiting and weight loss. One month prior, he had been diagnosed with cirrhosis of the liver, and subsequently hospitalized twice with removal of two and then five liters of ascites fluid. He developed significant abdominal pain associated with nausea and vomiting. He also had difficulty tolerating oral intake and constipation. He was not eating but sustaining himself on fluids. On physical examination, the patient was not jaundiced, but his abdomen was distended with a positive fluid wave. A firm mass was noted in the left mid epigastrium judged consistent with splenomegaly. His total bilirubin was 0.5 (direct 0.1), alkaline phosphatase 102, ALT 27, AST 17, albumin 3.4, total protein 6.3, prothrombin time 9.4, INR 1, partial thromboplastin time 33.8, amylase 257 and lipase 1130. He was admitted with a diagnosis of acute pancreatitis. He was made n.p.o. and given IV fluids.

CT scan with contrast showed a large amount of ascites. The morphology of the liver was unremarkable, with no evidence of nodularity, portal hypertension or varices. The greater omentum had a globular, engorged and homogeneous appearance. There was small bowel wall thickening. The appearance of the greater omentum and the finding of small bowel wall thickening was judged likely secondary to a hypoproteinemic state. There was a non-obstructing 0.4 cm right renal calculus. The spleen, gallbladder, adrenal glands and pancreas were normal in appearance. The ascites fluid was in the greater and lesser sacs, but not free in the peritoneal cavity, and there was no blurring of fat planes.

Diagnostic paracentesis revealed no spontaneous bacterial peritonitis and a very low amylase level. Cytological examination of the peritoneal fluid showed a single cluster of atypical cells. The cytologist favored the diagnosis of reactive change in mesothelial cells.

The patient's pain was minimal by the time he was admitted and was virtually gone overnight. On hospital day 2, he was started on clear liquids and his diet was advanced without nausea, vomiting or other complaints. AMA and ANA were negative. Serum ceruloplasmin was normal, ruling out Wilson's disease. Iron studies did not indicate any hemochromatosis. Hepatitis B and hepatitis C serologies were negative. He was discharged on Protonix, Lasix, Aldactone, potassium chloride, Xanax and Prozac.

The patient was admitted to an outside hospital five weeks later with nausea, vomiting, decreased oral intake, abdominal pain and cramping, and worsening ascites. He had bilious emesis. His sodium was 124, lipase 116 and creatinine 1.5. Eight liters of ascitic fluid were drained.

Two days later the patient was transferred to UPMC Presbyterian Hospital. On admission his temperature was 36.3, pulse 82, blood pressure 72/51 and respiratory rate 20. He had pinpoint pupils and asterixis. His sodium was 124, potassium 4.8, lipase 116, albumin 4.7, alkaline phosphatase 114, ALT 14, AST 20, blood urea nitrogen 27, creatinine 1.5, white blood cell count 8.1, hemoglobin 15.8, platelets 616 and INR 1.

Shortly following admission, the patient collapsed on the bathroom floor, with vomiting of "coffee ground" emesis. He was resuscitated, intubated and transferred to an intensive care unit. He developed increasing vasopressor requirements over the next several hours, with mean arterial pressure in the 50s to 60s on norepinephrine and dobutamine. Chest x-ray showed diffuse bilateral pulmonary infiltrates, judged to represent probable ARDS. His mean arterial blood pressure remained in the 50s overnight despite increasing pressors. Due to the patient's dismal prognosis, the decision was reached to switch to comfort measures only. The patient's mean arterial pressure steadily declined. He died quietly shortly thereafter. An autopsy was performed.

GROSS FINDINGS

The abdominal cavity had extensive fibrous adhesions and contained 4250 mL of serous fluid. There were numerous, confluent, raised, firm, tan-white nodules on the peritoneum and bowel serosa. The omentum was firm and adherent to the transverse colon. The liver capsule was roughened with multifocal fibrous adhesions. Adjacent to the stomach and pancreas, there was a 9 x 5.5 x 5 cm mass of firm tan-white tissue which extended retroperitoneally into the hepatic hilum, falciform ligament, diaphragm, and into the mesenteric root. The small bowel serosa was matted together by dense fibrous adhesions and extensive firm tan-white nodules (Image1). Some loops of small bowel were dusky, with severe congestion and hemorrhagic mucosa. The anatomy of the ileocecal region was extremely distorted by firm, tan-white tissue. The appendix was difficult to identify and appeared to be replaced by the firm, tan-white tissue.

MICROSCOPIC FINDINGS

Image 2 is a low power view of cross section of the obliterated appendix. Histologic examination of a transverse colon tumor nodule showed collections of cells with a high degree of pleomorphism as well as individual cells, many with signet ring features, admixed with pools of mucin (Image 3). Immunostains were positive for CEA, CDX2, and CK20 were positive while CK7 was negative (Image 4, 5, 6, and 7 respectively).

FINAL DIAGNOSIS


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