Original Articles
Idiopathic tumefactive chronic pancreatitis: Clinical profile, histology, and natural history after resection,☆☆

https://doi.org/10.1053/cgh.2003.50016Get rights and content

Abstract

Background & Aims: Little is known about subjects with idiopathic tumefactive chronic pancreatitis (TCP), that is, chronic pancreatitis whose clinical presentation, usually with a mass or obstructive jaundice, suggests cancer. Methods: We independently reviewed clinical data and histology of 45 TCP (27 idiopathic, 18 alcohol induced) resected at Mayo Clinic (January 1985-March 2001). Follow-up data were obtained from medical records and mailed questionnaires. Results: Compared with alcoholic subjects, idiopathic TCP patients were older (58 ± 2 vs. 48 ± 3 yr, P < 0.001), had shorter symptom duration (median 3 vs. 24 wk, P < 0.001), were more likely to have no or mild abdominal pain (70% vs. 17%, P = 0.001), and were more often jaundiced (67% vs. 33%, P = 0.02). Three distinct histologic patterns were identified in TCP. Typical CP (n = 19) showed lobular atrophy, fat necrosis, and ductal changes (dilatation, protein plugs, and stones). Lymphoplasmacytic sclerosing pancreatitis (LPSP) (n = 14) was characterized by periductal lymphoplasmacytic infiltration, obliterative phlebitis, and cholangitis with edema. Idiopathic duct-centric CP (IDCP) (n = 12) had neutrophil-predominant lobular inflammation, without phlebitis. On correlation of clinical and histologic diagnosis, 17 of 18 (94%) patients with alcohol-induced TCP had typical CP, and 25 of 27 (93%) with idiopathic TCP had LPSP or IDCP. LPSP and IDCP were indistinguishable clinically except for higher incidence of jaundice in LPSP (93% vs. 42%, P = 0.005). In idiopathic TCP no recurrence of symptoms was observed after resection (median follow-up 49 mo). Conclusions: Idiopathic TCP is clinically and histologically distinct from alcohol-induced TCP. It is unclear whether LPSP and IDCP, 2 unique patterns of histologic injury observed in idiopathic TCP, are part of the spectrum of the same disease or represent 2 or more different entities. Resection of mass prevents recurrence of symptoms in idiopathic TCP.

Section snippets

Methods

The Mayo Foundation Institutional Review Board approved the study and the contact of consenting patients, either by telephone or mail. In accordance with a Minnesota state statute,13 2 individuals who declined to authorize the use of their medical records in research were excluded from further review.

Clinical presentation

Of 45 TCP patients who underwent pancreatic resection, 27 were classified as idiopathic and 18 as alcohol-induced TCP. Two patients with inflammatory bowel disease (one each with Crohn's disease and ulcerative colitis) were included in the idiopathic TCP group. None of the TCP patients had a personal or family history of autoimmune disorder. Compared with alcohol-induced TCP, idiopathic TCP patients were older (58 ± 2 vs. 48 ± 3 yr; P < 0.001), had a shorter duration of symptoms (median 3 vs.

Discussion

In this study we show that idiopathic TCP is clinically and histologically distinct from alcohol-induced TCP. The histologic findings of lymphoplasmacytic infiltration seen in idiopathic TCP resemble those described previously by a variety of different names including autoimmune and sclerosing pancreatitis.4, 5, 6, 7, 8, 9, 10, 11, 12 In idiopathic TCP, pancreatic resection prevented recurrence of symptoms.

To our knowledge, the term tumefactive chronic pancreatitis has not been used in the

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