Adenocarcinoma of the fourth portion of duodenum presenting as intussusception: an unusual manifestation of rare pathology

Abstract

Primary adenocarcinoma of the fourth portion of the duodenum (D-IV) is reported infrequently than other parts of the duodenum. Its diagnosis is often late because of non-specific symptoms and signs. We encountered a 48-year-old male patient who was diagnosed as duodeno-duodenal intussusception, underwent segmental duodenal resection with duodenojejunal anastomosis and confirmed as adenocarcinoma of D-IV. He received adjuvant chemotherapy and is doing well at 1 year of follow-up. This report describes about the rare case of isolated adenocarcinoma of the D-IV presented as intussusception which is never reported before and successfully treated by segmental resection of the duodenum and jejunum.

Background

Duodenal adenocarcinoma (DAC) is a rare malignancy and presents unique task in terms of preoperative diagnosis, treatment decisions and postoperative management. Primary adenocarcinoma of the fourth portion of the duodenum (D-IV) is reported less frequently and it is described either in conjunction with third part of duodenum (D-III) or duodenojejunal flexure (DJF).^1–3 It is done because the presentation, investigation and treatment modality are similar for these locations. However, tumour restricted to D-IV may pose some unique challenges in terms of extent of disease or treatment offered.^{4 5} Here, we describe a case of primary adenocarcinoma of D-IV which presented with features of intussusception, managed by segmental resection with adjuvant chemotherapy and its associated review of literature.

Case presentation

A 48-year-old man, without any known comorbidities and addictions, presented with complaints of recurrent episodes of vomiting for the past 2 months, which occurred ~1–2 hours after food intake, non-projectile and bilious in nature. It was associated with pain in upper abdomen which was intermittent and colicky in nature. He also had significant anorexia and weight loss (non-documented) in the past 1 month. There was no history of haematemesis, melena, altered bowel habits or constipation.

Investigations

An esophagogastroduodenoscopy (EGD) was performed which showed narrowing of the lumen of D-IV with infolding of mucosa, but biopsy could not be taken. Contrast-enhanced computed tomography (CECT) of abdomen showed enhancing circumferential wall thickening involving D-IV (anterior and to the left of aorta) for a length of ~2.5 cm causing significant luminal narrowing. There was telescoping of this thickened portion into the third part of duodenum secondary to the lesion acting as lead point suggestive of duodeno-duodenal intussusception (figure 1A–B). It was associated with perilesional fat stranding and lesion was abutting distal portion of superior mesenteric artery (SMA) and inferior mesenteric vein (IMV), however intervening fat planes were well preserved.

Figure 1.

Contrast-enhanced CT of the abdomen. (A) Axial contrast-enhanced CT image showing moderately enhancing circumferential wall thickening at D4 (arrows). (B) Coronal reformatted images showing telescoping of this mass into the D3 resulting into intussusception (dashed arrow).

Treatment

He underwent exploratory laparotomy and mass like thickening was found at the D-IV till DJF region (figure 2A–B). The third and fourth part of the duodenum along with ~10 cm of proximal jejunum was resected with adequate margins and mesentery. Duodeno-jejunal (second part of duodenum and jejunum) side to side hand sewn anastomosis was performed. Patient did well in the postoperative period and was allowed orally on second postoperative day (POD) which was well tolerated. He was discharged on fifth POD.

Figure 2.

Intraoperative images. (A–B) Tumour in the fourth part of duodenum reaching upto duodenojejunal flexure.

Outcome and follow-up

Gross examination of tumour corroborated with the CECT findings. A circumferential grey white firm growth was seen in the D-IV and part of jejunal segment was seen telescoping into the adjacent part with growth acting as the lead point. Histopathology from the growth revealed tumour cells arranged in glandular and papillary pattern with moderate pleomorphism, hyperchromatic nuclei, coarse chromatin and moderate amount of cytoplasm. Tumour cells were reaching up to subserosal tissue. Perineural invasion was present and 7 out of 11 resected lymph nodes (LNs) were infiltrated by tumour, however all resection margins were free (figure 3A–D). Hence, the final diagnosis was well-differentiated adenocarcinoma of duodenum at D-IV (pT3N2, AJCC eighth edition).

Figure 3.

Histopathology images. (A) Duodenum with tumour cells infiltrating up to subserosal tissue (H&E 4×). (B) Tumour cells arranged in glandular pattern showing hyperchromatic nucleus, coarse chromatin and moderate amount of cytoplasm (H&E 40×). (C) Tumour cells arranged in papillary pattern (H&E 10×). (D) Lymph node infiltrated by tumour cells (H&E 4×).

He received adjuvant chemotherapy for 6 months in the form of capecitabine and oxaliplatin (CAPEOX) and was doing well with no recurrence at 1-year follow-up.

Discussion

The small intestine comprises nearly 90% of the surface area of the gastrointestinal (GI) tract but make up only 3% of all GI malignancies.^{1 3} Though the duodenum is involved in more than half of the cases of small bowel adenocarcinoma, it constitutes only 0.3%–0.5% of all the digestive tract tumours.^{3 4} This low incidence of DAC is attributed to the less transit time of liquid food material, thus decreasing the exposure, and a low bacterial count in the proximal bowel that does not allow bile acids to convert into potential carcinogens. Apart from this, the incidence pattern of DAC usually follows the relative length of each part of the duodenum.^{1 2 4} D-IV is the last and shortest part of duodenum in the retroperitoneum (~2.5 cm), lies left of the aorta and ends at DJF. The isolated incidence of D-IV adenocarcinoma is very rare and reported rate is nearly 5%–12%.^{4 6 7}

The risk factors for the development of DAC include life style factors, inflammatory bowel disease and certain familial syndromes like Lynch syndrome, familial adenomatous polyposis, and so on.^{1 8 9} The malignancy in the intestine can act as a lead point and may lead to intussusception. It is commonly encountered in ileocolic or colocolic region, but not reported at DJF before, as in our case.

The main challenge in the diagnosis of such tumours is their insidious onset and vague presenting symptoms. DAC at D-IV usually presents as recurrent bilious vomiting and crampy abdominal pain suggestive of obstructive nature of disease, as in the present case. The occult GI bleeding, anorexia and weight loss are other frequently encountered presentations. An EGD has a limited and challenging role for the diagnosis of D-IV adenocarcinoma. The tumour location is frequently inaccessible as tumour involves the ascending part of duodenum or DJF and study is usually terminated before reaching the actual site. The assessment of such pathology may need a double balloon enteroscopy or capsule endoscopy, but its usage has many limitations.¹⁰ An abdominal CECT is the investigation of choice as it provides information about local extent as well as rules out distant metastasis. CT scan offers the exact location of the tumour, relation with adjacent structures like pancreas and vessels (SMA, IMV, left renal vein) and its suitability for resection.

Surgical resection with R0 status is the only curative option for DAC. The intervention lies between radical surgery, like pancreatoduodenectomy (PD), to only segmental resection of duodenum.¹ PD is usually considered for DAC especially for tumour arising in the second portion of duodenum or invading into ampulla or pancreas. Segmental duodenal resection is feasible for tumour involving third or fourth part of duodenum and small lesion in its first part. It is recommended to retrieve at least eight LNs as they have important prognostic value, and studies also showed better outcome with more number of LNs assessed during surgery.¹¹ Overall, the 5-year survival rate among DAC ranges from 21%–27% with median survival of 34–37.5 months, which improves with less LNs positivity.^{1 3 12}

Further, the role of adjuvant therapy for DAC is equivocal based on the retrospective studies. However, it has been recommended that 6 months of adjuvant treatment with either leucovorin, 5-fluorouracil (5-FU) and oxaliplatin (FOLFOX), CAPEOX or 5-FU with leucovorin alone for locally advanced disease, LN or margin positivity, less LNs examined and tumour perforation.^{11 13}

In conclusion, D-IV adenocarcinoma is a rare tumour with vague and variable symptomatology. The surgical resection with R0 margin along with LN retrieval is the treatment of choice. Due to its low incidence and poor prognosis, it is necessary to share the current experience-based information to predict its future course.

Learning points.

• Duodenum is the the most common organ among small bowel adenocarcinoma, however involvement of fourth portion of the duodenum (D-IV) is rarest.

• Often presents with vague abdominal pain with features of gastric outlet obstruction.

• Endoscopy is less fruitful and contrast-enhanced computed tomography abdomen is the investigation of choice for D-IV adenocarcinoma.

• Surgery with R0 resection margin is the best curative option with role of adjuvant chemotherapy in selective cases.

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