Sclerosing encapsulating peritonitis (abdominal cocoon) associated with liver cirrhosis and diffuse large B‐cell lymphoma: Autopsy case

Abstract

A case of sclerosing encapsulating peritonitis (SEP) associated with liver cirrhosis (LC) and complicated by diffuse large B-cell lymphoma (DLBCL) is reported herein. A49-year-old Japanese man had undergone peritoneovenous shunt against refractory ascites due to hepatitis C virus-positive uncompensated LC for 2 years. After he received a diagnosis of DLBCL of the left neck lymph node 3 months before his death, palliative care was given because of his poor general condition. He developed severe abdominal distention and pain over 1 week and was found to have marked ascites and whole bowel lumped together on abdominal CT. At autopsy, the peritoneum was covered with a thick white membrane and the bowel could not be distinguished, which was macroscopically characterized by a cocoon-like appearance. Histology indicated a proliferation of diffusely thickened or hyalinized fibrocollagenous tissue in the entire peritoneum with a slight chronic inflammatory infiltrate and without remarkable change of mucosa. A diagnosis of SEP, also known as abdominal cocoon, was established based on these features. Additionally, in the abdominal cavity, a large amount of serous ascites and multiple peritoneal nodules or masses involved by DLBCL were recognized. To the authors' knowledge this is the first case report of SEP associated with LC and complicated by the invasion of DLBCL in the abdominal cavity.

Abdominal cocoon: idiopathic sclerosing encapsulating peritonitis

“…Many hypotheses have been formulated to try to explain the primary aetiology, including viral peritonitis due to gynecological infection from retrograde menstruation. However, a number of cases have been reported in males and other age groups, which go against the support for these theories 1 3–5 7. Another theory is that the condition is an embryological abnormality.…”

“…Sclerosing encapsulating peritonitis is a rare complication whose incidence increases the longer the patient is receiving dialysis treatment. Other cases have been found to be associated with prior abdominal surgery, practolol (β-blocker) treatment, abdominal tuberculosis, sarcoidosis, cirrhosis and malignancy to name a few 1 6 7. There has also been a case report that describes a patient with a peritoneovenous shunt and diffuse large B-cell lymphoma that later developed sclerosing encapsulating peritonitis 7…”

Sclerosing encapsulating peritonitis

“…SEP can be classified either as idiopathic or secondary to conditions such as peritoneal dialysis (PD), lupus, sarcoidosis, familiar Mediterranean fever, and use of beta blockers. 1 SEP has been reported in cirrhotic patients with [2][3][4] or without peritoneal-venous shunt (PVS). [5][6][7] It can also develop after liver transplantation (LT).…”

A problem encapsulated – role of CT

“…In the appropriate clinical setting, recognition of the entire dilated small bowel at the center of the abdomen and encased within a thick fibrocollageneous membrane, as though it were in a ‘cocoon,’ on a CT image is diagnostic of SEP. Yamada et al reported a case of SEP associated with liver cirrhosis and complicated by diffuse, large B cell lymphoma (1). There are reports of perforation of cocoon, particularly in immune-compromised patients which carries a high risk of mortality.…”