Primary Adenocarcinoma in Peristomal Skin: A Case Study

Abstract

Primary adenocarcinoma at an ileostomy site is an exceedingly rare occurrence but has been documented at the peristomal skin of patients with a long-standing ileostomy. Chronic irritation and resultant metaplasia is thought to be a key underlying mechanism for this phenomenon.

Biopsy of newly developing lesions in the peristomal area of long-standing stomas is essential in order to avoid delayed diagnosis and limit complications. A 37-year-old man with a history of ulcerative colitis and ileostomy surgery 18 years prior presented with an asymptomatic polypoid lesion at the mucocutaneous junction. Initially diagnosed as pyogenic pranuloma, the lesion was treated using topical silver nitrate. This did not resolve the lesion but ulceration and bleeding were observed. A biopsy showed evidence of primary adenocarcinoma arising from the ileostomy site. The lesion was removed surgically, an ileo-anal J pouch was created, and the patient is currently receiving long-term follow-up and monitoring for any possible future complications. This case study is one of several in the literature suggesting that a high index of suspicion is warranted when ileostomy patients, especially those with a history of ulcerative colitis, present with unusual peristomal lesions.

Please address correspondence to: Firas Al-Niaimi, Department of Dermatology, Salford Royal Hospital, Stott Lane, Manchester, UK M50 3TZ; email: firas55@hotmail.com.

     Peristomal dermatoses such as contact dermatitis, psoriasis, and pyoderma gangrenosum are well recognized by most dermatologists. Primary adenocarcinoma at an ileostomy site is exceedingly rare, with fewer than 50 cases reported worldwide,¹ predominantly in the literature concerning gastroenterology. To enhance recognition, understanding, and management of this phenomena, a case of primary adenocarcinoma arising at the mucocutaneous junction of an ileostomy in a patient who is under follow-up with a dermatologist for peristomal irritant dermatitis is presented.

Case Report

     Mr. H is a 37-year-old man with a history of ulcerative colitis (UC). He presented to the dermatology department with an asymptomatic polypoid lesion at the mucocutaneous junction 18 years following an ileostomy. His subtotal colectomy with an excluded rectal segment was performed when he was 17 years old. The peristomal lesion initially was diagnosed as a pyogenic granuloma and treated with topical silver nitrate with no response. Approximately 10 weeks later, the lesion began to ulcerate and bleed and developed some areas of overgranulation (see Figure 1, arrow), at which point a biopsy was performed that showed a well-differentiated invasive adenocarcinoma of intestinal type with focal areas of colonic metaplasia, indicating a dysplastic change from ileal mucosa. A subsequent biopsy from the rectal stump showed no evidence of dysplasia or adenocarcinoma to suggest a possible metastasis. Mr. H experienced no abdominal pain, increased stomal effluent, or intestinal bleeding. Physical examination was unremarkable and computed tomography imaging of the chest, abdomen, and pelvis showed no evidence of a visceral malignancy.

     Surgical excision was performed. An ileo-anal J pouch was fashioned with good outcome and the patient is on long-term follow-up for any possible recurrences.

Discussion

     Ileostomy as a treatment for UC was first suggested by Brown² in 1913; however, this method of treatment did not gain popularity in Britain until the 1940s³ and is increasingly used for other conditions such as familial adenomatous polyposis (FAP) and Crohn’s disease (CD). Mechanical complications include stomal retraction, prolapse, stenosis, abscess, and fistula formation.⁴

     Adenocarcinoma at an ileostomy site is a very rare late complication, with an estimated incidence of two to four per 1,000 ileostomies⁵; however, long-term follow-up studies are required to validate these data, reported predominantly in patients with UC.^5-7 Sigler and Jedd⁶ reported the first case in 1969 in a woman with UC that presented 18 years after ileostomy. The relatively higher incidence among UC patients may be related to the fact that more UC patients have an ileostomy than patients with other conditions, such as Crohn’s disease.^3,4,6

     Adenocarcinoma arising at an ileostomy site may present clinically as finger-like projections or polypoid lesions, pyogenic granuloma-like lesions, excess granulation tissue, or ulcerations.^5-8 The limited number of reported cases may hamper identification of common characteristics; however, the presence of overgranulation in a localized area of the peristomal skin is the most frequently noted sign in the reported cases^7-9 and was also observed in Mr. H’s case.

     Neoplasms typically occur long after ileostomy surgery (on average, more than 25 years), but one was reported as early as 9 years after surgery in a patient with FAP.⁹ The exact etiology of the neoplasm remains unclear but the long latent period between ileostomy construction and the appearance of neoplasm suggests that long-standing regenerative epithelial hyperproliferation caused by chronic irritation at the mucocutaneous junction is relevant. The chronic irritation may occur as a result of the constant exposure of the ileal mucosa to physical and chemical agents such as adhesives and irritants, some of which might be carcinogenic.^5,6 Another hypothesis is that skin, which is constantly exposed to fecal material, may develop metaplasia comparable to the development of Barrett’s esophagus with chronic gastro-esophageal reflux.¹⁰

     The pathogenesis of metaplasia and its possible risk regarding the development of neoplasm have been further supported by the presence of dysplasia in histological specimens from the area adjacent to a primary ileostomal adenocarcinoma; thus, it is conceivable that this mechanism is instrumental in the development of these neoplastic lesions.5,9 Attanoos et al11 reviewed 60 polyps in the peristomal area and found the majority to be inflammatory polyps; however, four were neoplastic, showing adenocarcinoma with focal areas of dysplasia, suggestive of a primary focal transformation. More importantly, these researchers found that neoplastic and non-neoplastic lesions could not be differentiated clinically. This further highlights the importance of obtaining histological tissue from suspect lesions arising in the peristomal area before implementing potentially destructive treatment methods, as demonstrated in the current study.

     Primarily, adenocarcinoma of the ileostomy is treated by surgical excision and careful long-term monitoring for any recurrence or complication because metastasis from such neoplasms have been reported.³ The authors assert that healthcare professionals involved in the care of patients with a stoma should be aware of such risk and have a high index of suspicion, particularly among patients with a long-standing ileostomy of more than 8 years, and consider regular monitoring in case of malignant change.

     Further research in this area is needed both to establish the exact etiology of this phenomenon and to help identify individuals at possible risk. This will require the collaboration of various facilities and clinicians involved in the care of patients with a stoma.

Conclusion

     As illustrated in the case of a 37-year-old man with an 18-year-old ileostomy who developed adenocarcinoma of the peristomal skin, clinicians need to be vigilant regarding the development of stoma-related lesions. Misdiagnosis can be destructive but appropriate management involving surgery and long-term follow-up can provide positive outcomes in this rare but treatable condition.

Acknowledgement

     The authors thank Mr. N. Scott from Royal Preston Hospital for his involvement with the care of the patient.

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