Lymphoplasmacytic Sclerosing Pancreatitis With Pseudotumor Formation

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Abstract

Lymphoplasmacytic sclerosing pancreatitis (LPSP), also known as nonalcoholic, duct destructive chronic pancreatitis or autoimmune pancreatitis, increasingly is recognized as a form of pancreatitis associated with autoimmune and fibroinflammatory lesions. The process often simulates pancreatic carcinoma because of the tumor-like nature of the fibrosis and the co-existence of jaundice caused by bile duct involvement. A case is reported of a 51-year-old woman with a mass in the tail of the pancreas with histologic features of LPSP exhibiting fibrotic pseudotumor formation. Although there were no autoimmune or fibroinflammatory diseases in her history, after distal pancreatectomy the patient experienced bilateral periorbital fibroinflammatory lesions (consistent with inflammatory pseudotumor of the orbit) and subsequently had recurrence of the process in the head of the pancreas accompanied by jaundice. The jaundice responded to steroid therapy. The histologic features in this case were characteristic of LPSP, with dense periductal inflammation rich in lymphocytes and plasma cells, ductal epithelial damage, focally dense fibrosis, and perivascular inflammatory cell aggregates (periphlebitis). The possibility of nonoperative therapy for LPSP can be considered, although reliable preoperative diagnosis currently requires a compelling clinical history of associated conditions.

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