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JPTM > Volume 48(1); 2014 > Article
Kang, An, Chung, and Cho: Adenocarcinoma Arising in a Colonic Duplication Cyst: A Case Report and Review of the Literature
Duplications in the alimentary tract are uncommon congenital abnormalities that may occur anywhere from the oral cavity to the anus, with the ileum being the most common site.1 Malignant change in a duplication is extremely rare.
Here, we report a case of adenocarcinoma arising in a colonic duplication cyst in a 23-year-old female and provide a review of the relevant literature.

CASE REPORT

A 23-year-old female patient was admitted with a palpable abdominal mass. The mass had been noted three years previously. On physical examination, a nontender and movable mass measuring approximately 7 cm was observed at the right lower quadrant.
The patient's serum carcinoembryonic antigen (CEA) level was 6.51 ng/mL (normal range, <5.0 ng/mL), and that of cancer antigen 19-9 was 47.71 U/mL (normal range, <37 U/mL).
Ultrasonography disclosed a hypoechoic cystic mass with an internal echogenic dot in the right lower quadrant. The echogenic dot was thought to be either an intraluminal secretion or necrotic debris (Fig. 1A). Computed tomography (CT) revealed a nonenhancing cystic mass measuring 7 cm, with linear calcification located adjacent to the medial side of the ascending colon (Fig. 1B). No enlarged regional lymph nodes, ascites, or other abnormalities were observed.
A laparoscopic excision of the mass was performed. During the operation, the mass was observed to be attached to the ascending colon mesentery, having the blood supply by the right colic artery.
On gross examination, the cystic mass measured 8.6×6.4×2.9 cm, and its outer surface was smooth; mesenteric fat adhered to the outer surface. The maximum thickness of the cystic wall was 1.5 cm, and focal calcification was noted within the wall. The cyst contained brownish-colored mucoid material (Fig. 2A).
Microscopically, the cyst was found to have two well-organized layers of smooth muscle with an infiltrating adenocarcinoma forming irregular tubules (Fig. 2B, C). The tumor had invaded the pericystic mesenteric soft tissue and metastasized to eight of 16 mesenteric lymph nodes. Perineural and lymphovascular invasions were noted. Non-neoplastic glandular epithelium was not found; instead, a focal area of atypical columnar epithelium, which was compatible with low-grade dysplasia, was observed (Fig. 2D). On immunohistochemical study, tumor cells were positive for cytokeratin 7 (1:100, OV-TL 12/30, Dako, Glostrup, Denmark), cytokeratin 20 (1:100, KS20.8, Dako), CEA (prediluted, II-7, Dako), CDX2 (1:50, AMT 28, Novocastra, Newcastle upon Tyne, UK), and p53 (1:100, DO-7, Dako). The dysplastic epithelium was also positive for p53 (Fig. 2E).
Positron emission tomography-CT was performed after surgery and revealed no hypermetabolic areas other than the previous operation site. Two months after surgery, the patient started adjuvant chemotherapy.

DISCUSSION

Duplications are rare congenital anomalies that may occur anywhere along the alimentary tract.1 The most common site of involvement is the ileum. Colonic duplications comprise 4% to 18% of all duplications, and occur most often in the cecum. Strict morphologic criteria for the diagnosis of duplication have been established: 1) attachment to the alimentary tract, 2) presence of smooth muscle layers, and 3) presence of lining epithelium resembling that of the alimentary tract. Our case met all of these criteria.
Malignant change in a duplication is very rare, and adenocarcinoma is the most common histologic type of malignancy found in these unusual cases.1-10 However, squamous cell carcinoma, carcinoid tumor, gastrointestinal stromal tumor, and leiomyosarcoma have also been reported.
Thirteen cases of adenocarcinoma arising in a duplication of the colon have been reported to date in the English literature (Table 1).1-10 Among the 14 cases, including the current case, four patients were male and 10 patients were female. The mean age at diagnosis was 48.8 years (range, 23 to 72 years) and our patient was the youngest. Some of the patients, including our patient, were relatively young, suggesting that the epithelium of the duplication has a high risk of carcinogenesis. The most common symptoms were abdominal pain and a palpable mass, and the mean size was 10.2 cm (range, 3 to 20 cm). Calcifications in the cystic wall or calculi in the lumen were common findings and were present in six of the eight cases where relevant data were available. Peripheral calcifications were present in three cases and calculi were found in four of six cases. Calcification in a duplication can be a worrisome finding because calcification is extremely rare in benign duplications.6 The preoperative serum CEA level was available for five cases and was found to be elevated in four cases in which patients presented with advanced stage disease at the time of initial diagnosis. According to our survey, except for one case without relevant data, all cases had invasion beyond the muscular wall. Metastasis to a regional lymph node or to a distant organ was reported in three cases.3,9 Although there was insufficient data on the duration of follow-up, five of six cases involved uneventful clinical courses. One patient died 41 months after diagnosis due to extensive metastasis.9 No follow-up data was provided for the remaining eight patients. Due to its rarity and nonspecific symptoms, we thought that, in the present case, the diagnosis was made at an advanced stage. Calcification within the mass and elevated serum CEA level appear to be useful for preoperative prediction of malignant change in duplications.
In conclusion, intestinal duplication is an uncommon congenital abnormality, and malignant change in a duplication is extremely rare. Differential diagnosis of a cystic mass located in or adjacent to the gastrointestinal tract should include duplication. When serum CEA level is high, and/or calcification within the mass is observed, the possibility of adenocarcinoma arising in a duplication cyst should be considered.

Notes

No potential conflict of interest relevant to this article was reported.

References

1. Orr MM, Edwards AJ. Neoplastic change in duplications of the alimentary tract. Br J Surg. 1975; 62:269–274. PMID: 1131505.
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2. Tamoney HJ Jr, Testa RE. Carcinoma arising in a duplicated colon: case report. Cancer. 1967; 20:478–481. PMID: 6019822.
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3. Heiberg ML, Marshall KG, Himal HS. Carcinoma arising in a duplicated colon: case report and review of literature. Br J Surg. 1973; 60:981–982. PMID: 4764750.
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4. Lee J, Jeon YH, Lee S. Papillary adenocarcinoma arising in a duplication of the cecum. Abdom Imaging. 2008; 33:601–603. PMID: 17912582.
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5. Jung KH, Jang SM, Joo YW, et al. Adenocarcinoma arising in a duplication of the cecum. Korean J Intern Med. 2012; 27:103–106. PMID: 22403508.
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6. Inoue Y, Nakamura H. Adenocarcinoma arising in colonic duplication cysts with calcification: CT findings of two cases. Abdom Imaging. 1998; 23:135–137. PMID: 9516499.
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7. Hattori H. Adenocarcinoma occurring just at the attached site of colonic duplication in an adult man. Dig Dis Sci. 2005; 50:1754PMID: 16133984.
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8. Hsu H, Gueng MK, Tseng YH, Wu CC, Liu PH, Chen CC. Adenocarcinoma arising from colonic duplication cyst with metastasis to omentum: a case report. J Clin Ultrasound. 2011; 39:41–43. PMID: 20812340.
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9. Arkema KK, Calenoff L. Adenocarcinoma in tubular duplication of the sigmoid colon. Gastrointest Radiol. 1977; 2:137–139. PMID: 210081.
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10. Nakayama H, Akikusa B, Kondo Y, Saito N, Sarashina H, Okui K. Mucinous cystadenocarcinoma of the colon: report of a case. Dis Colon Rectum. 1989; 32:243–246. PMID: 2920632.
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Fig. 1
Radiologic findings. (A) A hypoechoic mass with an echogenic dot (arrow) in the right lower quadrant is observed on ultrasonography. (B) A cystic mass with linear calcification in the mesentery adjacent to the ascending colon is observed on computed tomography.
kjpathol-48-62-g001
Fig. 2
Gross and microscopic findings. (A) The cystic mass measured 8.6×6.4×2.9 cm and contained brownish-colored mucoid material. (B) Full-thickness of two smooth muscle layers and adjacent mesenteric soft tissue are invaded by tumor cells. (C) The tumor is moderately differentiated adenocarcinoma. (D) A focal area of atypical columnar epithelium, compatible with dysplasia, is noted on the surface. (E) The dysplastic epithelial cells are positive for p53.
kjpathol-48-62-g002
Table 1.
Clinicopathologic summary of cases with adenocarcinoma arising in a colonic duplication cyst
Case Site Age (yr)/Sex Symptom Serum CEA Size (cm) Calcification Depth of invasion Treatment Prognosis
1 [2] Cecum 61/F Abdominal pain ND 9 ND Muscle layer RHC, omentectomy, bilateral oophorectomy NED
2 [3] Cecum 50/F Lower abdominal pain ND 8 ND Pericystic mesenteric soft tissue RHC ND
3 [4] Cecum 40/F Palpable mass 11 N Muscle layer RHC ND
4 [5] Cecum 41/M Palpable mass Normal 6 Y Pericystic mesenteric soft tissue RHC, CT NED
5 [1] Ascending 41/F RLQ pain ND 4 Y Thin fibrous wall (muscle layer) Excision NED
6 [6] Ascending 38/M Palpable mass ND Y Capsule (muscle layer) RHC ND
7 [6] Ascending 59/F Fever and lumbar pain ND Y Pericystic mesenteric soft tissue and right psoas muscle Excision ND
8 [7] Ascending 59/M Melena due to adenocarcinoma in the ascending colon ND 10 ND Subserosa RHC ND
9 [1] Transverse 57/F RLQ pain ND 11 ND Pericystic mesenteric soft tissue RHC NED
10 [8] Transverse 40/M Palpable mass ND 16 ND Pericystic mesenteric soft tissue and omentum Excision, CT ND
11 [4] Descending 33/F Palpable mass ND 15 N ND Excision ND
12 [9] Sigmoid 69/F Epigastric pain ND 15 Y Pericystic mesenteric soft tissue and retroperitoneum Excision Died 41 mos later
13 [10] Sigmoid 72/F Epigastric pain due to cholelithiasis ND 3 ND Subserosa Sigmoid colectomy ND
Present case Ascending 23/F Palpable mass 8.6 Y Pericystic mesenteric soft tissue Excision, CT NED

CEA, carcinoembryonic antigen; F, female; ND, no data available; RHC, right hemicolectomy; NED, no evidence of disease; N, no; M, male; Y, yes; CT, chemotherapy; RLQ, right lower quadrant.

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