BACKGROUND: Celiac sprue is a malabsorption disease, which carries an
increased risk of gastrointestinal malignancy, often underestimated. T
he purpose of this study was to examine the management of patients wit
h 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-celI lymphom
a was the most common malignancy. There was also an increased frequenc
y of small intestinal adenocarcinoma and squamous cell carcinoma of th
e esophagus. Prognosis was generally poor, related to the histologic t
ype and stage of the disease. CONCLUSIONS: Gastrointestinal malignant
neoplasms, especially small bowel lymphomas, can occur in patients wit
h celiac sprue. Patients with known celiac disease who present with ex
acerbation of symptoms should be promptly investigated for occult gast
rointestinal malignancies, and considered for early surgical explorati
on. (C) 1998 by Excerpta Medica, Inc.