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Case Reports
. 2018 Aug 21;12(2):390-395.
doi: 10.1159/000490659. eCollection 2018 May-Aug.

Adenocarcinoma Arising from Perianal Fistulizing Crohn's Disease

Rosario Fornaro  1 , Marco Frascio  1 , Michela Caratto  1 , Elisa Caratto  1 , Rita Bianchi  2 , Andrea Razzore  1 , Giuseppe Caristo  1 , Camilla Sticchi  3 , Marco Casaccia  1
Affiliations
Case Reports

Adenocarcinoma Arising from Perianal Fistulizing Crohn's Disease

Rosario Fornaro et al. Case Rep Gastroenterol. .

Abstract

Perianal fistula is a very debilitating event and a cause of morbidity in patients with Crohn's disease (CD). Its malignant transformation is very rare with an incidence of around 0.004-0.7$. Presence of disease in the colon and rectum is the major risk factor for the development of a perianal fistula. In this report we show a case of adenocarcinoma arising from a perianal fistulizing CD. This type of tumor is highly aggressive, difficult to diagnose, and has a rather poor prognosis. The different neoplastic transformations and the different types of tumors that may appear in patients with CD, especially at the colorectal level or at the level of an eventual anastomosis, are to date well documented and described in the literature, while there is a lack of information and of treated cases concerning the occurrence of cancer at the level of a fistula. Due to the rarity of cases, we tried to identify the most frequent and important risk factors: sex, duration of disease, age at diagnosis, and the history of the fistula.

Keywords: Adenocarcinoma; Crohn's disease; Perianal fistula.

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Figures

Fig. 1
Fig. 1
a Lower magnification showing diffuse infiltration of a scarcely differentiated tumor in the muscular walls of the intestine and the dysplasia of the mucosa from which it derives. Hematoxylin and eosin, ×200. b Together with the neoplasia an extensive inflammatory status can be observed in the intestinal wall, with pseudofollicular lymphocytic aggregates and multiple granulomas. Hematoxylin and eosin, ×400. c At higher magnification, multiple granulomas with multinucleated cells can be observed. Hematoxylin and eosin, ×1,000. d Immunohistochemical reaction for cytokeratin AE1–AE3, an epithelial marker, showed a single cell infiltration of the neoplasia in the intestinal wall. Cytokeratin AE1–AE3, ×400.
Fig. 2
Fig. 2
a Nuclear expression of CDX-2 in a portion of adenocarcinomatous glands. CDX-2, ×200. b Another part of the same lesion showed loss of CDX-2 expression. CDX-2, ×200. c, d Expression of microsatellites MSH-6 (c) and PMS-2 (d) was maintained by neoplastic glands. c MSH-6, ×100. d PMS-2, ×200.
See this image and copyright information in PMC

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