Hammad et al. Journal of Medical Case Reports 2011, 5:556 http://www.jmedicalcasereports.com/content/5/1/556 Page 3 of 4 Figure 3 A recurrent polypoid mass visible at a stoma. The recurrent polypoid mass is shown at an ileostomy site two years after an en bloc resection. A wide excision of the terminal ileum and refashioning of the stoma were undertaken. The specimen was reported as benign tubular adenoma. Many factors have been implicated in the malignant transformation of the ileostomy mucosa: colonic meta- plasia and dysplasia of ileal mucosa, chronic irritation due to either physical or chemical trauma, ileitis, and development of chronic inflammatory polyps and malignant transformation in a pre-existing benign ade- noma. In this case, we believe that the invasive adenocar- cinoma was secondary to malignant transformation in a pre-existing adenoma as evident by the pathology report. Table 1 summarizes the case reports that we were able to find. There were 13 patients (seven men and six women), and the median age was 57 years (range of 42 to 75). The median interval between stoma formation and presenting adenocarcinoma was 25 years (range of nine to 42). None of the patients had lymph node metastasis at the time of presentation. Three patients showed local skin invasion (23%). No deaths were recorded after three years of follow-up. However, two patients had a recurrence that was excised locally. The treatment was constant in all cases and included en bloc resection of the terminal ileum with its mesentery, sur- rounding stoma skin, and relocation of a new ileostomy. Our patient was followed up to 18 months without evi- dence of malignant recurrence. However, she developed a benign adenomatous polyp, which was locally excised with refashioning of the stoma. Conclusions The number of reported cases for primary adenocarci- noma following panproctocolectomy for FAP is small. Table 1 Primary adenocarcinoma of an ileostomy in familial adenomatous polyps Study Age, years Roth and Logio [8] 44 (1982) Ross et al. [9] (1987) 56 Sex Duration, Tumor description Lymph years nodes Male 9 Ulcerating polypoid mass N 2 × 3 cm Female 32 Raised mass 12 × 12 cm N Primrose et al. [10] 72 (1988) Suarez et al. [4] 40 (1988) Gilson and 69 Sollenberger [11] (1992) Johnson et al. [12] 65 (1993) Lux et al. [1] (1993) 42 Mimura et al. [13] 54 (1999) Iizuka et al. [14] 55 (2002) Hata et al. [15] 57 (2003) Shenoy and Cassim 60 [16] (2009) Matsushima [17] 75 (2009) Present case (2010) 59 Female 25 Male Male Male Male Male 29 40 25 15 21 Female 14 Female 42 Male 24 Female 34 Friable mass Polypoid mass Mass 2 × 5 cm Polypoid exophytic mass 3.5 × 3 cm Mass 12 × 12 cm Polypoid mass 6 × 4 × 2 cm Polypoid mass extending from 1 to 9 o’clock Circumferential cauliflower-like polyps Polypoid mass Circumferential polypoid neoplasm 4.5 cm N N N N N N N N N N N Female Not stated Fungating mass This table summarizes 13 case reports of primary adenocarcinoma of an ileostomy in familial adenomatous polyps. N, negative. Follow- up Not stated 22 months two years one year two years Pathology Moderately differentiated adenocarcinoma involving the ileostomy skin Mucinous adenocarcinoma Well-differentiated adenocarcinoma Moderately differentiated adenocarcinoma with mucocutaneous invasion Moderately differentiated mucinous adenocarcinoma Well-differentiated adenocarcinoma one year Well-differentiated adenocarcinoma Not stated Well-differentiated adenocarcinoma three years Well-differentiated adenocarcinoma 23 months Well-differentiated to moderately differentiated two adenocarcinoma in adenoma years Adenocarcinoma in tubulovillous adenoma Not stated Well-differentiated adenocarcinoma 32 months Moderately differentiated mucinous one adenocarcinoma infiltrating the deep dermis year