Fistula-associated Anal Adenocarcinoma in Crohn’s Disease
Igors Iesalnieks, MD,* Wolfgang B. Gaertner, MD, MS,†Heidi Glab, MD,* Ulrike Strauch, MD,‡
Matthias Hipp, MD,§Ayman Agha, MD,* and Hans J. Schlitt, MD, Prof.*
Background: Adenocarcinoma arising from perianal fistulae in
patients with Crohn’s disease (CD) is rare. The literature consists
mainly of case reports and small series making characterization of
this clinical entity difficult. We present 6 patients with CD and
fistula-associated anal adenocarcinoma (FAAA) and a systematic
review of published series.
Methods: Retrospective charts were reviewed of 6 consecutive
patients with FAAA in CD treated from 1992 through 2007. All
available variables of our patients and of all available published
cases were included for statistical analysis.
Results: All patients treated at our institution had severe perianal
CD at presentation. The average age at time of diagnosis was 45.5
years. All patients underwent abdominoperineal resection (APR)
and 4 received chemoradiation. Four patients died with metastatic
disease, 1 is alive with pelvic recurrence at 55 months, and 1 is
alive without evidence of disease at 19 months follow-up. A total
of 23 publications including 65 patients (37 female, mean age 53
years) with FAAA were reviewed in our systematic review. The av-
erage fistula duration was 14 years. Mean delay of cancer diagnosis
was 11 months. APR was performed in 56 patients with an overall
3-year survival rate of 54%. Thirteen of 15 patients with node-posi-
tive tumors died with recurrent disease following surgery.
Conclusions: Adenocarcinoma arising from long-standing peria-
nal CD fistulae is being increasingly reported. The outcome is
poor following operative treatment, especially if perirectal lymph
nodes are involved. Periodical cancer surveillance should be per-
formed in all patients with long-standing perianal CD fistulae.
(Inflamm Bowel Dis 2010;16:1643–1648)
Key Words: fistula, adenocarcinoma, Crohn’s disease, perianal
of gastrointestinal carcinoma occur more frequently in
patients with CD than in the regular population: small
bowel cancer, colorectal cancer,2,3and carcinoma arising
from perianal fistulae.4–6The presence of anal carcinoma
in CD is clearly associated with long-standing perianal fis-
(FAAA) and squamous cell carcinoma have been increas-
ingly reported in the past 25 years. Most reports consist of
single cases7–9or small series;4,5,10,11and both adenocarci-
noma and squamous cell carcinoma are frequently reported
together,4,5,12making the thorough description of each en-
tity difficult. The purpose of this study was to report 6
patients with FAAA in the setting of CD and to perform a
systematic review of reported
t has been well recognized that Crohn’s disease (CD)
carries an increased risk for malignancy.1,2Three types
cases withthis rare
PATIENTS AND METHODS
We searched the computerized database of the
Department of Surgery, University of Regensburg, Ger-
many for all cases of anal adenocarcinoma occurring
between 1992 and 2007. We excluded all cases not associ-
ated with anal fistulae or CD. A detailed review of each
patient’s medical record was undertaken, concentrating on
demographics, presentation, therapy, and outcome.
Tumors were classified using the 1976 World Health
which recognizes 3 variants: rectal, anorectal fistula, and
anal gland adenocarcinoma.
A comprehensive search was conducted using MED-
LINE and PubMed databases from January 1946 to Sep-
tember 2009. The keywords used were: ‘‘Crohn’s disease,’’
‘‘perianal fistula,’’ ‘‘anal fistula,’’ ‘‘adenocarcinoma,’’ ‘‘car-
cinoma,’’ ‘‘cancer,’’ and ‘‘rectal cancer,’’ either singly or in
combination. Reports describing histological types of
fistula-associated neoplasms other than adenocarcinoma
and cases of rectal adenocarcinoma without a history of
anal fistulae were excluded. The reference lists from the
relevant articles were also searched manually to identify
trials for possible inclusion. All available clinical and path-
ological variables from these reports were included.
Received for publication November 24, 2009; Accepted December 28,
From the *Department of Surgery, University of Regensburg, Germany;
Minnesota,‡Department of Internal Medicine I, University of Regensburg,
Reprints: Igors Iesalnieks, MD, Department of Surgery, University of
Regensburg, Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany
†Department of Surgery, University of Minnesota, Minneapolis,
§Department of Radiation Therapy, University of Regensburg,
C2010 Crohn’s & Colitis Foundation of America, Inc.
2010 inWiley OnlineLibrary
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