Scientific Papers
Gastrointestinal malignancies in patients with celiac sprue

https://doi.org/10.1016/S0002-9610(98)00193-7Get rights and content

Abstract

BACKGROUND: Celiac sprue is a malabsorption disease, which carries an increased risk of gastrointestinal malignancy, often underestimated. The purpose of this study was to examine the management of patients with gastrointestinal neoplasms complicating celiac disease.

PATIENTS AND METHODS:

The pathology database at our institution was searched from 1986 to present; and the literature from 1966 to 1997 was reviewed to identify reports of celiac sprue complicated by malignancy. A total of 82 cases were available for analysis.

RESULTS:

Two thirds of patients had carried the diagnosis of celiac sprue for a mean of approximately 10 years. The remaining one third were diagnosed with celiac disease and gastrointestinal malignancy simultaneously. Jejunal T-cell lymphoma was the most common malignancy. There was also an increased frequency of small intestinal adenocarcinoma and squamous cell carcinoma of the esophagus. Prognosis was generally poor, related to the histologic type and stage of the disease.

CONCLUSIONS:

Gastrointestinal malignant neoplasms, especially small bowel lymphomas, can occur in patients with celiac sprue. Patients with known celiac disease who present with exacerbation of symptoms should be promptly investigated for occult gastrointestinal malignancies, and considered for early surgical exploration.

Section snippets

Patients and methods

The mainframe of the Department of Pathology at Rhode Island Hospital was searched from its inception in 1986 to April 1997, for all patients with celiac disease whose course was complicated by malignancy. Two patients were identified and their records reviewed. (Figure 1, Figure 2).

The world literature was also reviewed from 1966 to 1997 using Medline. Eight case series and 9 case reports of celiac disease complicated by malignancies were identified. The analysis included demographic data,

Demographics

A total of 82 patients were available for analysis. Half of the patients reviewed were men and half were women. The mean age at the time of diagnosis of malignancy was 56.9 years for men and 52 years for women.

Presenting signs and symptoms

In patients with known celiac disease, malignancy was most commonly characterized by an exacerbation of the malabsorption syndrome, with worsening anorexia, nausea, vomiting, diarrhea, and weight loss. A perforated viscus was often the initial complication leading to a cancer diagnosis,

Comments

Malignant lymphoma occurring in association with celiac disease was first described in 1937,5 and was long thought to be a form of malignant histiocytosis, before the identification of the T-lymphocyte as the malignant cell population.6, 7 The incidence of T-cell lymphoma involving the gastrointestinal tract in patients with celiac sprue ranges from 3.6% over 3 years to 40% over 16 years.8 The malignant cell population appears to arise from the chronic reactive inflammatory infiltrate of the

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