Journal of Pancreatic Cancer Journal of

Volume 4.1, 2018

DOI: 10.1089/pancan.2018.0005 Pancreatic Cancer

Open Access

Duodenal Adenocarcinoma in a Patient

with Partial Intestinal Malrotation
William T. Li, Sonia Sethi, Adrienne N. Christopher, Deepika Koganti,* and Charles J. Yeo

Abstract
Background: Small bowel cancers, specifically duodenal cancer, occur at very low rates but require aggressive
surgical resection when diagnosed. An even rarer finding is the presence of intestinal malrotation.
Case Presentation: We present the unique case of a patient with both duodenal cancer and partial intestinal
malrotation undergoing pancreaticoduodenectomy. We discuss the challenges faced and techniques used to
successfully perform a surgical resection in this circumstance.
Conclusion: Understanding of intestinal malrotation and review of the imaging is crucial in preparing for a
resection of a duodenal tumor in a patient with this condition.
Keywords: pancreaticoduodenectomy; intestinal malrotation; congenital anomaly

Background esophagogastroduodenoscopy (EGD) and colonoscopy.

Small bowel neoplasms are a rare form of cancer in the His colonoscopy revealed no worrisome lesions in the
United States, with *10,470 estimated new cases and colon. EGD revealed a large malignant appearing mass
1450 estimated deaths per year.1 Although the small 4–5 cm in size just adjacent to the ampulla, which was biop-
bowel comprises 91% of the total surface area of the al- sied. Pathology analysis returned as invasive moderately
imentary tract, small bowel cancer comprises only 3.3% differentiated adenocarcinoma. He had a computed to-
of estimated new cases of alimentary tract cancers per mography to further delineate the mass and to assist with
year.2 Another rare finding in adults is intestinal mal- operative planning, which showed thickening of the duode-
rotation (IM). A review of cases at the Massachusetts num as well as a partial malrotation at the third and fourth
General Hospital found only 82 confirmed diagnoses portions of the duodenum such that the ligament of Treitz
of IM in adults for a 17-year period.3 In adults, IM is failed to cross the midline (Fig. 1). Laboratories including
often asymptomatic and found incidentally. We pres- serum tumor markers, carcinoembryonic antigen (CEA)
ent a unique case of a 76-year-old male who presented and carbohydrate antigen 19-9 (CA 19-9), were within
with both duodenal cancer and incidental small bowel normal limits. Owing to his biopsy proven duodenal ade-
malrotation with associated radiological studies, pa- nocarcinoma, its proximity to the pancreatic head, and the
thology report, and a review of literature. apparent structural anomalies, the patient was recommen-
ded to undergo surgical resection through a pancreatico-
Presentation of Case duodenectomy. His imaging was carefully reviewed
The patient is a 76-year-old Caucasian male who initially preoperatively, and the partial malrotation was noted.
presented with melanotic stools and anemia requiring mul- The patient was prepared for his Whipple resection.
tiple blood transfusions. He had a medical history of hyper-
tension, hyperlipidemia, chronic kidney disease, and a Surgical findings
myocardial infarction that resulted in a cardiac stent place- The patient was taken to the operating room for an
ment. During his workup for anemia, he underwent an open pancreaticoduodenectomy. Upon exploration, the
Department of Surgery, Jefferson Pancreas, Biliary, and Related Cancer Center, Thomas Jefferson University, Philadelphia, Pennsylvania.

*Address correspondence to: Deepika Koganti, MD, Department of Surgery, Jefferson Pancreas, Biliary, and Related Cancer Center, Thomas Jefferson University, 1015
Walnut Street, Suite 620, Philadelphia, PA 19107, E-mail: koganti.deepi@gmail.com

ª William T. Li et al. 2018; Published by Mary Ann Liebert, Inc. This Open Access article is distributed under the terms of the Creative Commons
License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided
the original work is properly cited.

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Li, et al.; Journal of Pancreatic Cancer 2018, 4.1 31
http://online.liebertpub.com/doi/10.1089/pancan.2018.0005

FIG. 1. A CT scan without IV contrast showed generalized esophageal wall thickening and mild duodenal
(marked by ‘‘D’’) wall thickening (b). The duodenal sweep does not cross the midline (a–c), consistent with a
malrotation. There is no obstruction identified. The transverse colon evaluation was limited due to motion blur. The
SMV (marked by ‘‘V’’) is anterior and to the right of the SMA (marked by ‘‘A’’), as would be found normally. EUS
performed showed a duodenal mass in the second portion of the duodenum that measured 3–4 cm. CT, computed
tomography; EUS, endoscopic ultrasound; SMA, superior mesenteric artery; SMV, superior mesenteric vein.

duodenum and head of the pancreas revealed a palpable Postoperative care

mass at the level of the second part of the duodenum and Postoperatively, the patient experienced transient delir-
extending close to the first part of the duodenum and fur- ium and developed an International Study Group for
ther caudal. It was necessary to proceed with a classic Pancreatic Fistula (ISGPF) grade B pancreatic leak
Whipple procedure since a pylorus-preserving pancreati- that was drained percutaneously. He was discharged
coduodenectomy would have compromised the duodenal to a rehabilitation facility on postoperative day 13.
margin. There was no evidence of carcinomatosis, malig-
Pathological findings
nant lesions in the liver, or omental implants. On explora-
The pancreaticoduodenectomy specimen contained a
tion, various structures were abnormal. The duodenal C
3.2 · 2.5 · 2 cm exophytic tan pink to red firm granular
loop was vertical, and there was an ectopically located lig-
mass within the duodenum, *13 cm from the proxi-
ament of Treitz; it was found on the right side and inferior
mal gastric resection margin. The mass was adjacent
to its typical location. In addition, the presence of malrota-
to a normal ampulla of Vater. Upon sectioning, the
tion in the setting of significant mesenteric adipose tissue
mass grossly appeared to extend into the muscularis
warranted that a longer portion of his jejunum be resected
propria but did not involve the underlying pancreatic
to yield sufficient mobility of the retained proximal jeju-
parenchyma. There was no dilation of the pancreatic
num to allow for a tension-free anastomosis of the
duct. The mass was a moderately differentiated adeno-
jejunum to the pancreas remnant and biliary tree. The pa-
carcinoma of the duodenum with focal invasion of the
tient had normal positioning of the mesenteric arteries,
subserosal fatty tissues. A microscopic neuroendocrine
which facilitated normal dissection of the pancreas around
tumor was also identified within the duodenal wall. It
the superior mesenteric vein (SMV) and superior mesen-
was well differentiated, low grade, and 2 mm in greatest
teric artery (SMA). However, the presence of considerable
dimension. All 12 regional lymph nodes recovered
mesenteric fat and bowel malrotation provided signifi-
were negative for metastatic disease. All resection mar-
cant, although overcomable, challenges to our operation.
gins were also negative for neoplasm. The final staging
The pancreaticojejunostomy, hepaticojejunostomy,
of the duodenal adenocarcinoma was T3N0M0.
and gastrojejunostomy anastomoses were completed
without incident. The specimen was sent off to pathol- Molecular testing
ogy analysis and the frozen section analysis revealed a A Pan-Cancer 42-Gene mutation panel of the specimen
duodenal adenocarcinoma, with all resection margins was performed. Analysis showed a G12V mutation of
being negative for malignancy. the KRAS gene, as well as a T>G nucleotide change in
Li, et al.; Journal of Pancreatic Cancer 2018, 4.1 32
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codon 560-3 of the TP53 gene. Both of these changes portant landmark for identification of the mesenteric vein
were classified as pathogenic. No further mutations during pancreaticoduodenectomy, absence or ectopic
were identified in the other 40 genes in the panel. placement of this structure carries with it the possibility
of intraoperative injury or postoperative complications.
Discussion and Literature Review In our case, the ligament of Treitz was displaced from
Cancer of the small bowel is a rare entity and accounts the normal location and somewhat inferior. We worked
for only about 3.3% of gastrointestinal tract neoplasms. carefully along the plane between the uncinate process
IM is a congenital anomaly that occurs in 1 in 500 live of the pancreas and the transverse mesocolon to identify
births and very rarely presents in adults.4,5 Small bowel and dissect the SMV.
cancer occurring in the setting of IM is a rare combina-
tion and can pose unique surgical challenges as the ana- Conclusion
tomical variations can often be located in the pancreatic We present a rare case of a patient with adenocarcinoma
region. Rittenhouse et al. performed a retrospective re- of the duodenum and IM treated with pancreaticoduo-
view of the incidence of gastrointestinal malrotation dis- denectomy. Close review of imaging and understanding
covered incidentally on preoperative imaging before of IM helped us prepare for the challenging but ulti-
hepatobiliary surgery. They found an incidence of 3 mately successful resection of the tumor.
out of 1220 cases, or 0.2%.6 In planning for these cases, Author Disclosure Statement
the surgeon must be prepared to deviate from the classic No competing financial interests exist.
surgical techniques utilized to ensure a safe resection.
For example, in two cases reported in Rittenhouse References
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trunk in three patients with IM undergoing pancreati-
coduodenectomy.7 These vascular structures deliver Cite this article as: Li WT, Sethi S, Christopher AN, Koganti D, Yeo CJ
most of the blood supply to the pancreas and midgut (2018) Duodenal adenocarcinoma in a patient with partial intestinal
malrotation, Journal of Pancreatic Cancer 4:1, 30–32, DOI: 10.1089/
and are the centerpieces for dissection during pancrea- pancan.2018.0005.
ticoduodenectomy. The presence of abnormalities in
these critical vessels must be closely scrutinized on pre-
operative imaging preoperatively to prevent uninten- Abbreviations Used
tional injury or ligation of the vessels. EGD ¼ esophagogastroduodenoscopy
Finally, the ligament of Treitz can often be found in IM ¼ intestinal malrotation
SMA ¼ superior mesenteric artery
an abnormal position or may be missing altogether in SMV ¼ superior mesenteric vein
patients with IM.9–11 As the ligament of Treitz is an im-