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Adenocarcinoma arising in a cystic duplication of the small bowel: case report and review of literature.
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MedLine Citation:
PMID:  22490125     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Enteric duplications are rare, but can occur anywhere along the digestive tract. Most of the patients become symptomatic in early childhood and only a few cases of adult patients have been reported in literature. Here we report a unique case of an adenocarcinoma arising in a coincidentally found cystic duplication of the small bowel.
Authors:
Gregor Blank; Alfred Königsrainer; Bence Sipos; Ruth Ladurner
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2012-04-10
Journal Detail:
Title:  World journal of surgical oncology     Volume:  10     ISSN:  1477-7819     ISO Abbreviation:  World J Surg Oncol     Publication Date:  2012  
Date Detail:
Created Date:  2012-05-16     Completed Date:  2012-08-21     Revised Date:  2013-06-26    
Medline Journal Info:
Nlm Unique ID:  101170544     Medline TA:  World J Surg Oncol     Country:  England    
Other Details:
Languages:  eng     Pagination:  55     Citation Subset:  IM    
Affiliation:
Department of General, Visceral and Transplant Surgery, University of Tübingen, Hoppe-Seyler-Strasse 3, D-72076 Tübingen, Germany. gregor.blank@med.uni-tuebingen.de
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MeSH Terms
Descriptor/Qualifier:
Adenocarcinoma / pathology*,  surgery
Cysts / pathology*,  surgery
Humans
Ileal Neoplasms / diagnosis,  pathology*,  surgery
Intestine, Small / abnormalities,  pathology*,  surgery
Jejunal Neoplasms / diagnosis,  pathology*,  surgery
Male
Middle Aged
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Journal ID (nlm-ta): World J Surg Oncol
Journal ID (iso-abbrev): World J Surg Oncol
ISSN: 1477-7819
Publisher: BioMed Central
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Copyright ©2012 Blank et al; licensee BioMed Central Ltd.
open-access:
Received Day: 5 Month: 12 Year: 2011
Accepted Day: 10 Month: 4 Year: 2012
collection publication date: Year: 2012
Electronic publication date: Day: 10 Month: 4 Year: 2012
Volume: 10First Page: 55 Last Page: 55
ID: 3352259
Publisher Id: 1477-7819-10-55
PubMed Id: 22490125
DOI: 10.1186/1477-7819-10-55

Adenocarcinoma arising in a cystic duplication of the small bowel: case report and review of literature
Gregor Blank1 Email: gregor.blank@med.uni-tuebingen.de
Alfred Königsrainer1 Email: alfred.koenigsrainer@med.uni-tuebingen.de
Bence Sipos2 Email: bence.sipos@med.uni-tuebingen.de
Ruth Ladurner1 Email: ruth.ladurner@med.uni-tuebingen.de
1Department of General, Visceral and Transplant Surgery, University of Tübingen, Hoppe-Seyler-Straße 3, D-72076 Tübingen, Germany
2Institute of Pathology, University of Tübingen, Liebermeisterstraße 8, D-72076 Tübingen, Germany

Background

The term 'alimentary tract duplication' was characterized by W.E. Ladd to describe those congenital malformations that involve the mesenteric side of the associated alimentary tract and share a common blood supply with the native bowel [1]. Enteric duplications are unusual, but can occur anywhere along the digestive tract [2-7], most frequently found around the ileocoecal region [2-7]. Most patients become symptomatic within the first year of life [2-5]. Reports of enteric duplications in adulthood are extremely scarce in English language literature [8]. Although rare, malignant change can occur within the intestinal duplication [9]. In this report we present a case of an adenocarcinoma arising in a coincidentally found cystic duplication of the small bowel.


Case presentation

A 51-year-old man with no abdominal symptoms was admitted to our hospital with an external computed tomography (CT) scan showing a cystic mass in the mid-abdomen (Figure 1). The cystic mass was low-density and had enhanced margins. The size of the structure was measured at 4 × 10 cm and it was located in the ileal mesenterium. The differential diagnosis contained a mesenteric cyst, a Meckel's diverticulum, and an enteric duplication.

Physical examination was unremarkable but laboratory tumor marker levels were slightly elevated: carcinoembryonic antigen (CEA) 13.2 μg/l (standard value < 5 μg/l) and CA19-9 55 kU/l (standard value < 37 kU/l).

During explorative laparotomy a cystic mass was found in the mesenterium which looked similar to the small bowel but had no connection to the alimentary tract (Figure 2). The surface was smooth and without deposits. An en-bloc resection of the cystic mass could be performed without the necessity of a small bowel resection. At the end of the operation the specimen was opened (Figure 3). It contained an odorless, cloudy liquid. The luminal surface showed partly brownish deposits, the surface was irregular but smooth and the walls were uniformly 3 mm thick.

Histological examination revealed a duplication of the small bowel in the mesenterium with nearby physiological architecture. The inner lining mucosa showed indicated villi and crypts and numerous mucous cells. The epithelium showed partly dysplastic areas. At one point it contained a high-grade intraepithelial neoplasia with transition into a poorly differentiated invasive adenocarcinoma infiltrating the muscularis propria (Figure 4).

Immunohistochemistry revealed a high expression of CK20 and spot-like CK7. Analogue to the TNM-classification of the small bowel, the tumor was classified as pT2, pN0 (0/25), L0, V0, G2.

Postoperative recovery was unremarkable and the patient was discharged six days after surgery. Regular oncologic follow-up in an outpatient setting for one year after surgery showed no hints of tumor recurrence with inconspicuous physical examination and normal tumor marker levels, ultrasound, and CT findings.


Discussion

Enteric duplications (EDs) are rare but can occur anywhere along the digestive tract from the oral cavity to the rectum [2-7,10]. The majority of ED occurs intra-abdominally and over half of them are ileal duplications [4-7]. According to W.E. Ladd, those congenital malformations involve the mesenteric side of the associated alimentary tract and share a common blood supply with the native bowel [1]. The etiology of ED still remains unknown. Several theories have been postulated such as an abnormal recanalization after the solid epithelial stage of embryonic bowel development [11]. Other theories consider persisting embryologic diverticula or 'aborted Gemini' [12]. The most accepted theory, however, is the 'intrauterine vascular accident theory' [13,14], but no single theory can explain all the known duplications [7].

EDs usually become symptomatic within the first year of life [2-7]. Reports of ED in adulthood are extremely scarce in English language literature [8]. Most frequently, the patients present vague symptoms mimicking other more common pathologies such as volvulus, appendicitis, intussusception, pelvic abscess, diverticulitis, achalasia, and Hirschsprung's disease [4,6,7,15-17].

EDs are most commonly diagnosed when complications like bowel obstruction, perforation, or bleeding occur. Prior to surgery it is difficult to diagnose EDs because of the non-specificity of symptoms and presentation. However, ultrasound, CT scan, and magnetic resonance imaging (MRI) have been useful. Ultrasound can depict the characteristic location adjacent to the bowel and the two-layered wall of EDs [15,18-20]. Bowel duplication cysts present with heterogeneous signal intensity on T1- and homogeneous signal intensity on T2-weighted images on MRI [21,22]. Latter modalities can even assist in prenatal diagnosis [22]. Where duplication is tubular, barium examination may be diagnostic if not contraindicated [7]. Technetium scanning can also be used to diagnose EDs [3]. The majority of EDs are isolated and cystic in structure. Reports of tubular duplications are less common. However, both could be associated with other malformations like intestinal malrotation and genitourinary or spinal malformations [23-25].

Heterotopic mucosa of gastric or pancreatic origin is a common finding in histological examination of ED [7]. In the current case the specimen had a similar physiological architecture to the small bowel with indicated villi, crypts, and a two-layered muscular wall. The epithelium contained many mucous cells. Gastric or pancreatic origin was not confirmed.

Carcinomas arising in duplication cysts are extremely rare complications and only few cases have been reported in literature including carcinoid tumors, squamous cell carcinomas, and common adenocarcinomas [26-50].

Malignant change in small bowel duplications is described most frequently [14,20,22,26-35], followed by colonic [36-43] and rectal [44-46] duplications. There are also reports about carcinomas arising in duplications of the duodenum [47,48] and the stomach [49,50]. Due to the rare presentation with unspecific symptoms the tumors are commonly diagnosed at advanced tumor stage with metastatic disease [26-28,35]. If malignant change is found in small bowel duplications, the high rate of lymph node metastases should be considered [26]. The mode of metastasis is similar to that of primary small bowel cancer [26,27]. Curative resections could hardly be performed [26,28]. Thus, the prognosis is generally poor once malignant change has occurred. Fortunately the suspicion of ED was a coincidental finding in an abdominal CT scan in the present case. This led to a timely operative exploration and malignant change was diagnosed at an early stage. A curative en-bloc resection of the duplication including the tumor could be performed and all of the resected 25 lymph nodes were free of metastasis.

Histological examination depicted dysplastic areas in the epithelium with an area of a high-grade intraepithelial neoplasia and transition into a poorly differentiated invasive adenocarcinoma. This indicates a tendency to undergo malignant change, which was also reported by Orr and Edwards [9]. Moreover, all cases of malignant change in duplication cysts that have been reported have occurred in adults aged 26 to 88 years. This is in contrast to the presentation of benign cysts that are diagnosed in childhood [2,4-7].


Conclusion

The experience of this case and other reports about malignant transformation shows that whenever intestinal duplication is suspected, an immediate operative resection should be performed.


Consent

Written consent was obtained from the patient for the use and publication of this case report and the accompanying images. A copy of the written consent is available for review from the Editor-in-Chief of this journal.


Competing interests

The authors declare that they have no competing interests.


Authors' contributions

GB collected the information, researched the literature, and wrote the article. AK helped with literature research and in preparing the manuscript. BS performed the histological examination and helped prepare the manuscript. RL helped in literature research and edited the final version of the manuscript. All authors read and approved the final version of the manuscript.


References
Ladd WE,Gross RE,Surgical treatment of duplications of the alimentary tractSurg Gynecol ObstetYear: 194070295307
Bower RJ,Sieber WK,Kiesewetter WB,Alimentary tract duplications in childrenAnn SurgYear: 197818866967410.1097/00000658-197811000-00015718292
Hocking M,Young DG,Duplications of the alimentary tractBr J SurgYear: 198168929610.1002/bjs.18006802107459629
Schalamon J,Schleef J,Höllwarth ME,Experience with gastro-intestinal duplications in childhoodLangenbecks Arch SurgYear: 200038540240510.1007/s00423000017011127525
Karnak I,Ocal T,Senocak ME,Tanyel FC,Büyükpamukcu N,Alimentary duplications in children: report of 26 years' experienceTurk J PediatrYear: 20004211812510936977
Kuo HC,Lee HC,Shin CH,Sheu JC,Chang PY,Wang NL,Clinical spectrum of alimentary tract duplication in childrenActa Paediatr TaiwanYear: 200445858815335117
Olajide ARL,Yisau A,Abdulraseed NA,Kashim IO,Olaniyi AJ,Morohunfade AO,Gastrointestinal duplications: experience in seven children and a review of the literatureSaudi J GastroenterolYear: 20101610510910.4103/1319-3767.6123720339180
Johnson JA,Poole GV,Ileal duplications in adults. Presentation and treatmentArch SurgYear: 199412965966110.1001/archsurg.1994.014203001030188204043
Orr MM,Edwards AJ,Neoplastic change in duplications of the alimentary tractBr J SurgYear: 19756226927410.1002/bjs.18006204051131505
Chen MK,Gross E,Lobe TE,Perinatal management of enteric duplication cysts of the tongueAm J PerinatolYear: 19971416116310.1055/s-2007-9941199259920
Bremer JL,Diverticula and duplications of the intestinal tractArch PatholYear: 194438132140
Letelier AM,Barría CM,Beltran MA,Moreno CCH,Duplicación intestinal: Diagnóstico y tratamiento de una condición inusualRev Chil CirYear: 200961171175
Favara BE,Franciosi RA,Akers DR,Enteric duplications. Thirty-seven cases: a vascular theory of pathogenesisAm J Dis ChildYear: 1971122501506
Beltrán MA,Barría C,Contreras MA,Wison CS,Cruces KS,Adenocarcinoma and intestinal duplication of the ileum. Report of one caseRev Méd ChileYear: 200913713411345
Chou YH,Tiu CM,Pan HB,Yeh CJ,Wei CF,Chang TE,Ultrasonographic demonstration of duplication cyst of the ileumZhonghua Yi Xue Za Zhi (Taipei)Year: 1990462372392178058
Otter MI,Marks CG,Cook MG,An unusual presentation of intestinal duplication with a literature reviewDig Dis SciYear: 19964162762910.1007/BF022823538617148
Ameh EA,Jimoh AO,Rafindadi AH,Shehu SM,Sublingual gastric duplication cyst causing respiratory obstruction: case reportEast Afr Med JYear: 20007739439512862161
Kangarloo H,Sample WF,Hansen G,Robinson JS,Sarti D,Ultrasonic evaluation of abdominal gastrointestinal tract duplication in childrenRadiologyYear: 1979131191194424583
Rice CA,Anderson TM,Sepahdari S,Computed tomography and ultrasonography of carcinoma in duplication cystJ Comput Assist TomogrYear: 19861023323510.1097/00004728-198603000-000123512638
Tew K,Soans BK,Millar EA,Adenocarcinoma in an ileal duplication cyst: ultrasound and computed tomography findingsAustralas RadiolYear: 20004422823110.1046/j.1440-1673.2000.00791.x10849993
Berrocal T,Lamas M,Gutieèrez J,Torres I,Prieto C,del Hoyo ML,Congenital anomalies of the small intestine, colon and rectumRadiographicsYear: 1999191219123610489177
Radich GA,Altinook D,Adsay NV,Soulen RL,Papilarry adenocarcinoma in a small-bowel duplication in a pregnant womanAJR Am J RoentgenolYear: 200618689589710.2214/AJR.04.148816498127
Somuncu S,Cakmak M,Caglayan E,Unal B,Intestinal duplication cyst associated with intestinal malrotation anomaly: report of a caseActa Chir BelgYear: 200610661161217168282
Shah KR,Joshi A,Complete genitourinary and colonic duplication: a rare presentation in an adult patientJ Ultrasound MedYear: 20062540741116495506
Chaiyasate K,Bruch S,Colonic duplication associated with anterior spinal bar and left-sided inferior vena cavaSurgeryYear: 200714182382510.1016/j.surg.2006.06.02417560260
Kusunoki N,Shimada Y,Fukumoto S,Iwatani Y,Ohshima T,Arahi E,Miyazaki N,Maeda S,Adenocarcinoma arising in a tubular duplication of the jejunumJ GastroenterolYear: 20033878178510.1007/s00535-002-1146-814505134
Kim TH,Kim JK,Jang EH,Lee JH,Kim YB,Papillary adenocarcinoma arising in a tubular duplication of the jejunumBr J RadiolYear: 201083e61e6410.1259/bjr/6826982620197430
Devos B,Schreurs L,Duponselle E,Hendrix T,Van Dijck H,Van Vuchelen J,Adenocarcinoma optredend in een cystische duplicatie van het ileumActa Chir BelgYear: 1987872352383661003
De Tullio D,Rinaldi R,Pellegrini D,Stano R,Messina F,Cavazzini L,Azzena G,Occhionorelli S,Adenocarcinoma arising in an elderly patient's large ileal duplicationInt J Surg PatholYear: 20081968168418611942
Babu MS,Raza M,Adenocarcinoma in an ileal duplicationJ Assoc Physicians IndiaYear: 20085611912018472514
Micolonghi T,Meissner GF,Gastric-type carinoma arising in duplication of the small intestineAnn SurgYear: 195814712412710.1097/00000658-195801000-0002113498632
Adair HM,Trowell JE,Squamous cell carcinoma arising in duplication of the small bowelJ PatholYear: 1981133253110.1002/path.17113301047205440
Smith JHF,Hope PG,Carcinoid tumor arising in a cystic duplication of the small bowelArch Pathol Lab MedYear: 198510995963838244
Ribaux C,Meyer P,Adenocarcinome dans une duplication intestinale greleAnn PatholYear: 1995154434458720841
Fletcher DJ,Goodfellow PB,Bardsley D,Metastatic adenocarcinoma arising from a small bowel duplication cystEur J Surg OncolYear: 200228939410.1053/ejso.2001.115411869024
Lee J,Jeon YH,Lee S,Papillary adenocarcinoma arising in a duplication of the coecumAbdom ImagingYear: 20083360160310.1007/s00261-007-9330-117912582
Heiberg ML,Marshall KG,Himal HS,Adenocarcinoma arising in a duplicated colon. Case report and review of literatureBr J SurgYear: 19736098198210.1002/bjs.18006012184764750
Arkema KK,Calendoff L,Adenocarcinoma in tubular duplication of the sigmoid colonGastrointest RadiolYear: 1977213713910.1007/BF02256486210081
Hickey WF,Corson JM,Squamous cell carcinoma arising in a duplication of the colon: case report and literature review of squamous cell carcinoma of the colon and of malignancy complicating colonic duplicationCancerYear: 19814760260910.1002/1097-0142(19810201)47:3<602::AID-CNCR2820470330>3.0.CO;2-87226009
Neal JW,Zuk RJ,Baithun SI,Squamous cell carcinoma in a duplicate large intestine. A case reportVirchows Arch A Pathol Anat HistopatholYear: 198941538338510.1007/BF007186412505449
Delladetsima J,Papachristodoulou A,Zografos G,Carcinoma arising in duplicated colonAm SurgYear: 1992587827831456608
Inoue Y,Nakamura H,Adenocarcinoma arising in colonic duplication cysts with calcification: CT findings of two casesAbdom ImagingYear: 19982313513710.1007/s0026199003059516499
Hattori H,Adenocarcinoma occurring just at the attached site of colonic duplication in an adult manDig Dis SciYear: 200550175410.1007/s10620-005-2930-016133984
Weitzel RA,Breed JR,Carcinoma arising in a rectal duplication (enterocystoma)Ann SurgYear: 196315747648010.1097/00000658-196303000-0002113999793
Downing R,Thompson H,Alexander-Williams J,Adenocarcinoma arising in a duplication of the rectumBr J SurgYear: 19786557257410.1002/bjs.1800650813678772
Gibson TC,Edwards JM,Shafig S,Carcinoma arising in a rectal duplication cystBr J SurgYear: 19867337710.1002/bjs.18007305203708289
Falk GL,Young CJ,Parer J,Adenocarcinoma arising in a duodenal duplication cyst: a case reportAust NZ J SurgYear: 19916155155310.1111/j.1445-2197.1991.tb00289.x
Hata H,Hiraoka N,Ojima H,Shimada K,Kosuge T,Shimoda T,Carcinoid tumor arising in a duplication cyst of the duodenumPathol IntYear: 20065627227810.1111/j.1440-1827.2006.01957.x16669876
Coit DG,Mies C,Adenocarcinoma arising within a gastric duplication cystJ Surg OncolYear: 19925027427710.1002/jso.29305004171640716
Kuraoka K,Nakayama H,Kagawa T,Ichikawa T,Yasui W,Adenocarcinoma arising from a gastric duplication cyst with invasion to the stomach: a case report with literature reviewJ Clin PatholYear: 20045742843110.1136/jcp.2003.01394615047751

Figures

[Figure ID: F1]
Figure 1 

Computed tomography scan showing a low-density cystic mass (4 × 10 cm) with an enhanced margin in the mid-abdomen.



[Figure ID: F2]
Figure 2 

Intraoperative photograph showing the cystic mass in the mesenterium without a connection to the surrounding small bowel. The cystic mass bears great similarity to the small bowel.



[Figure ID: F3]
Figure 3 

Macroscopic picture of the opened specimen. The luminal surface shows partly brownish deposits, the surface is irregular but smooth. The thickness of the walls is uniformly 3 mm.



[Figure ID: F4]
Figure 4 

Microscopic picture (hematoxylin and eosin stain) showing a poorly differentiated invasive adenocarcinoma (intestinal type) infiltrating the lamina muscularis propria of the enteric duplication.



Article Categories:
  • Case Report

Keywords: Enteric duplication, Duplication cyst, Malignant change, Small bowel cancer, Adenocarcinoma.

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