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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 20  |  Issue : 2  |  Page : 153-156

Case series: Two rare cases of primary jejunal adenocarcinoma with different clinical presentation


Department of Medicine and Medical Oncology, INHS Asvini, Mumbai, Maharashtra, India

Date of Submission17-Jun-2018
Date of Acceptance01-Oct-2018
Date of Web Publication10-Jan-2019

Correspondence Address:
Surg Lt Purvesh Agrawal
Department of Medicine, INHS Asvini, Colaba, Mumbai - 400 005, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmms.jmms_39_18

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  Abstract 

Small bowel tumors are rare, accounting for <5% of gastrointestinal neoplasms, 1%–2% of these being malignant. The most common histological subtype is adenocarcinoma. Small bowel adenocarcinoma (SBA) is challenging to diagnose, often presents at a late stage, and has a poor prognosis. Therefore, a high index of clinical suspicion is required to diagnose such patients, and a better outcome with early-stage detection is expected. We report here two cases of jejunal adenocarcinoma. (a) A 53-year-old male presenting with iron deficiency anemia. (b) An 81-year old-female presenting with subacute intestinal obstruction. Diagnosis of SBA remains a challenge. A physician's suspicion and awareness are crucial in such patients. The early diagnosis will affect overall survival and mortality rate. Because the incidence of SBA is very low, there is a need for further studies to evaluate the possible application of newer investigative agents and strategies to obtain a better outcome.

Keywords: Capsule endoscopy, diagnosis, FOLFOX, small bowel adenocarcinoma, surgery


How to cite this article:
Chowdhary GS, Agrawal P. Case series: Two rare cases of primary jejunal adenocarcinoma with different clinical presentation. J Mar Med Soc 2018;20:153-6

How to cite this URL:
Chowdhary GS, Agrawal P. Case series: Two rare cases of primary jejunal adenocarcinoma with different clinical presentation. J Mar Med Soc [serial online] 2018 [cited 2019 Jul 23];20:153-6. Available from: http://www.marinemedicalsociety.in/text.asp?2018/20/2/153/249761


  Introduction Top


Malignant neoplasm's of the small bowel are among the rarest types of cancer, accounting for only 1%–2% of all gastrointestinal (GI) cancers.[1] The diagnosis of small bowel tumors is often difficult due to the rarity of these lesions and the nonspecific and variable nature of the presenting signs and symptoms. Thus, delay in diagnosis is common, which may result in the discovery of disease at a late stage and hence poor treatment outcomes. The incidence of small bowel adenocarcinomas (SBAs) is on the rise for unknown reasons. The 5-year overall survival is around 30% with median overall survival being 19 months approximately.[2] There is a paucity of published data, especially of randomized controlled data regarding the optimal front-line chemotherapy regimen in advanced stages. Molecular alterations in SBA are closer to those in colorectal cancer than those in gastric cancer, with low levels of human epidermal growth factor receptor 2 (HER 2) overexpression and high frequencies of KRAS mutations.[3] This explains why we use folinic acid, fluorouracil and oxaliplatin (FOLFOX), 5 fluorouracil/leucovorin or folinic acid, 5 fluorouracil and irinotecan as preferred chemotherapy regimens to treat this cancer.

Several tumors can arise within the small bowel, both malignant (adenocarcinoma, carcinoid, lymphoma, and sarcomas) and benign (adenoma, leiomyoma, and lipoma). SBA accounts for around 40% of all cancers of the small bowel.[4],[5],[6] The frequency of small bowel tumor is higher in the duodenum (38%–55.2%) and decreases distally toward the ileum.[4],[5] The diagnosis of such cases is difficult as the upper and lower GI endoscopies are often normal. Capsule endoscopy (CE) or double balloon enteroscopy may show bleeding site and lesion, although biopsy being a challenging issue. Mucosal abnormalities can be picked up by computed tomography (CT) or magnetic resonance enteroclysis and GI bleeding from the mid-gut can be detected by red blood cell scans from the nuclear medicine department if the rate of blood loss is more than 0.5 ml/min.


  Case Reports Top


Case 1

A 53-year-old male who was previously in good health presented to the outpatient department with symptoms of exertional dyspnea and easy fatiguability in August 2017. There was no previous medical or any surgical history. He denied any history of drug intake, worm infestation, or overt bleeding from natural orifices. The laboratory investigations revealed microcytic hypochromic anemia on peripheral blood smear, hemoglobin (Hb) = 8.7 g/dl, mean corpuscular volume (MCV) = 71 fl, serum ferritin = 4.0 ng/ml, stool for occult blood – negative, and chest X-ray (posteroanterior view) – Normal and ultrasound abdomen was also normal. Due to a positive family history for thalassemia, Hb electrophoresis was done which was suggestive of beta-thalassemia trait. Both upper and lower GI tract endoscopy were normal. He was treated on lines of coexisting iron deficiency anaemia and thalassemia trait with ferric carboxymaltose infusions for 3 months. During the next few months, initially, there was improvement (Hb – 9.2 g/dl) on monthly follow-up following which there was again aggravation of anemia Hb = 7.2 g/dl, MCV = 67.6 fl, and progressive asthenia, without evident blood loss or gastrointestinal symptoms. A repeat stool for occult blood done this time was positive; therefore, a CE [Figure 1] was done which revealed active small intestinal bleeding from the jejunum. Contrast-enhanced CT abdomen [Figure 2] revealed exophytic heterogeneously enhancing hypodense mass in proximal jejunum and involving mesenteric border with multiple discrete homogeneous lymph nodes at mesenteric, splenic artery, and aortocaval locations.
Figure 1: Capsule endoscopy showing multiple bluish-red globular lesions in proximal jejunum. Lesions showing bleeding

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Figure 2: Contrast-enhanced computed tomography abdomen showing 43 mm × 42 mm × 50 mm exophytic enhancing mass involving mesenteric border. Multiple discrete retroperitoneal lymph node also visualized

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Surgery was performed with resection of proximal jejunal growth till root of mesentery [Figure 3] with side-to-side small bowel anastomosis, and the histopathology of the removed segment confirmed the moderately differentiated adenocarcinoma [Figure 4] and [Figure 5]. Single mesenteric lymph node dissected close to tumour was involved by the tumor with both the proximal and distal margin being spared by the invasive carcinoma. Whole-body positron emission tomography (PET)–CT study [Figure 6] revealed metastatic mesenteric and retroperitoneal lymph node involvement with peritoneal/omental thickening and deposits noted in prehepatic, pregastric, and precolic regions. He was, therefore, diagnosed as Stage IV SBA and planned for 6 cycles of FOLFOX. The patient has completed six cycles of his total six cycles of palliative chemotherapy and is doing well as of his last follow-up.
Figure 3: Tumour resected en bloc with jejunal loop. 15 cm × 10 cm mass in root of jejunal mesentery with loop of jejunum buried within approximately 20 cm from duodenojejunal flexure. Omentum adherent

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Figure 4: High-power magnification (×400) showing adenocarcinoma

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Figure 5: Microscopic image of the tumor from the pathologic specimen, hematoxylin and eosin staining. Low-power magnification (×100) showing a moderately differentiated adenocarcinoma of the jejunum

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Figure 6: Fluorodeoxyglucose positron emission tomography scan – Multiple fluorodeoxyglucose avid discrete and conglomerate lymph nodes in retroperitoneal level D12 to L2 abdominal stations. Fluorodeoxyglucose avid peritoneal/omental thickening and deposits in prehepatic, pregastric, and precolic regions likely to be due to mitotic activity

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Case 2

An 81-year-old female who presented with a history of nausea, vomiting, and constipation over the last 6 months. She has been not able to eat well secondary to bloating, abdominal discomfort with reported significant weight loss of 8 kgs over the last 6 months. She underwent endoscopy at our institute and was noted to have esophagitis with minimal to no improvement on proton-pump inhibitors. Further evaluation of patient was done at Intermountain Medical Center, Utah, USA. A CT abdomen and pelvis was done in December 2016 which was normal. Considering a negative CT and endoscopy, she was treated on lines of functional dyspepsia. She continued to have persistent symptoms of nausea and vomiting. She presented to emergency care on February 16, 2017, where CT scan with oral and IV contrast showed 100% obstructive proximal jejunal mass. She then underwent small bowel resection. Pathology from the small bowel resection showed invasive moderately differentiated adenocarcinoma of jejunum with focal mucinous features, with lymphovascular and perineural invasion present. One out of 11 lymph nodes were positive for metastatic carcinoma, one mesenteric tumor deposit was identified. She was, therefore, diagnosed as Stage 3 SBA. Postoperative CT chest and abdomen showed no distant metastasis. She recovered well from surgery and was started on single-agent capecitabine as adjuvant therapy in May 2017. As of now, she has completed three cycles of chemotherapy in the United States and due for additional five cycles of chemotherapy at our institute.


  Discussion Top


Small bowel cancer normally occurs in elderly patients. The mean age of diagnosis of small bowel tumors is 60 years of age, with a slight male preponderance. Small bowel represents 75% of the length of the digestive tract and 90% of the absorptive mucosal surface area. Whereas SBAs are only 1%–2% of all GI cancers and jejunal are even rarer.[7] The possible explanation to this includes several features that include rapid transit of food, low bacterial content, presence of enzyme benzopyrene hydroxylase, rapid turnover of epithelial cells, and IgA elaborating lymphoid tissue having protective effect.[8],[9]

SBA and large bowel adenocarcinoma share similar pathologic (development from adenomatous polyps), genetic (mutations of oncogene ras and elevated levels of p53), and epidemiologic features.[10] Familial adenomatous polyposis, hereditary nonpolyposis colorectal cancer, Crohn's disease, and celiac disease and diet (animal fat, red meat, and smoked/salted food) are the strongest risk factors.[1] However, SBAs tend to cluster away from the colon, toward the gastric end of the small intestine. The predominant location of adenocarcinoma is the duodenum and proximal jejunum, with the incidence decreasing distally.[11] The exception to this presentation is seen in Crohn's disease, where most adenocarcinomas occur in the ileum.

The symptoms of SBA are initially nonspecific; diagnosis is delayed and usually in the context of emergency involving an occlusion or bleeding, which is similar to the presentation of both of our patients. Our first case had occult GI bleeding of longstanding duration and the case history illustrates the delay that often takes place in the diagnosis of small-bowel tumors in patients with obscure GI bleeding. The patient's SBA was detected with CE. Aggressive evaluation is needed in cases where initial endoscopic workup is nondiagnostic in the presence of obscure GI bleeding. Repeating a procedure may often be necessary to make the diagnosis. The diagnosis was achieved by the combination of the CE results, CT images, PET/CT images, and carcinoembryonic antigen (CEA) levels. Our second case presented with nonspecific complaints for 6 months duration and was being treated on lines of functional dyspepsia. The diagnosis was only clinched when the patient presented with acute intestinal obstruction and a repeat contrast enhanced CT Abdomen which showed 100% proximal jejunal obstruction. While CT scans can detect the lesions, they cannot provide precise data of the intestinal mucosa and miss some small or flat lesions. The PET-CT technique is being used to differentiate small intestinal malignant tumors from benign ones. The uptake of fluorodeoxyglucose (18F-FDG) is related to tumor size, infiltration, and lymph node metastasis; the higher the uptake of 18F-FDG, the higher the tumor invasiveness.[12] Gastroscopy and enteroscopy can be appropriate if the tumour is located close to the proximal duodenum or far from the terminal ileum. The rest of the small bowel cannot be accessed without the use of CE. CE is suitable for diagnosing scattered, small and multiple lesions, as well as active bleeding; it is convenient, noninvasive, secure, and comfortable. A baseline plasmatic CEA and carbohydrate antigen (CA 19-9) assay is necessary, especially in cases of advanced disease, because CEA and CA 19-9 levels are of prognostic value.[2]

Surgical resection with clear margins and regional lymph node resection remains the treatment of choice in localized SBA; indeed, it is often required even in metastatic SBA due to the high probability of obstruction or severe hemorrhage.[13] To date, there has been no standard chemotherapy regimen against SBA. Radiotherapy does not seem to be effective or benefit survival. It is technically difficult to localize the target field due to the mobile nature of the small intestine mesentery. From limited clinical reports, a combination of fluoropyrimidine and platinum compounds (FOLFOX or CAPOX) has been proposed as the first-line treatment for palliative chemotherapy in metastatic SBA treatment.[14]

The prognosis of small bowel tumors is poor, with most series reporting a 5-year-survival of 15%–35%.[4],[5] After curative surgical resection, this rate increases to 40%–65% in most studies.


  Conclusion Top


We report two rare cases of jejunal adenocarcinoma with different clinical presentation. Diagnosis of SBA remains a challenge. A physician's suspicion and awareness are crucial in such patients. Early diagnosis will affect overall survival and mortality rate. Surgery remains the primary treatment. Because the incidence of SBA is very low, there is a need for further studies to evaluate the possible application of newer investigative agents and strategies to obtain a better outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

We would like to thank the Department of Gastroenterology, INHS Asvini, who provided the data of the imaging technique and endoscopy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Neugut AI, Jacobson JS, Suh S, Mukherjee R, Arber N. The epidemiology of cancer of the small bowel. Cancer Epidemiol Biomarkers Prev 1998;7:243-51.  Back to cited text no. 1
    
2.
Zaanan A, Costes L, Gauthier M, Malka D, Locher C, Mitry E, et al. Chemotherapy of advanced small-bowel adenocarcinoma: A multicenter AGEO study. Ann Oncol 2010;21:1786-93.  Back to cited text no. 2
    
3.
Aparicio T, Svrcek M, Zaanan A, Beohou E, Laforest A, Afchain P, et al. Small bowel adenocarcinoma phenotyping, a clinicobiological prognostic study. Br J Cancer 2013;109:3057-66.  Back to cited text no. 3
    
4.
Bilimoria KY, Bentrem DJ, Wayne JD, Ko CY, Bennett CL, Talamonti MS, et al. Small bowel cancer in the United States: Changes in epidemiology, treatment, and survival over the last 20 years. Ann Surg 2009;249:63-71.  Back to cited text no. 4
    
5.
Lepage C, Bouvier AM, Manfredi S, Dancourt V, Faivre J. Incidence and management of primary malignant small bowel cancers: A well-defined French population study. Am J Gastroenterol 2006;101:2826-32.  Back to cited text no. 5
    
6.
Jemal A, Siegel R, Ward E, Hao Y, Xu J, Murray T, et al. Cancer statistics, 2008. CA Cancer J Clin 2008;58:71-96.  Back to cited text no. 6
    
7.
Dabaja BS, Suki D, Pro B, Bonnen M, Ajani J. Adenocarcinoma of the small bowel: Presentation, prognostic factors, and outcome of 217 patients. Cancer 2004;101:518-26.  Back to cited text no. 7
    
8.
Varghese R, Weedon R. ‘Metachronous’ adenocarcinoma of the small intestine. Int J Clin Pract Suppl 2005;24:106-8.  Back to cited text no. 8
    
9.
Speranza G, Doroshow JH, Kummar S. Adenocarcinoma of the small bowel: Changes in the landscape? Curr Opin Oncol 2010;22:387-93.  Back to cited text no. 9
    
10.
Neugut AI, Marvin MR, Rella VA, Chabot JA. An overview of adenocarcinoma of the small intestine. Oncology (Williston Park) 1997;11:529-36.  Back to cited text no. 10
    
11.
Lowenfels AB, Sonni A. Distribution of small bowel tumors. Cancer Lett 1977;3:83-6.  Back to cited text no. 11
    
12.
Cronin CG, Scott J, Kambadakone A, Catalano OA, Sahani D, Blake MA, et al. Utility of positron emission tomography/CT in the evaluation of small bowel pathology. Br J Radiol 2012;85:1211-21.  Back to cited text no. 12
    
13.
Aparicio T, Zaanan A, Svrcek M, Laurent-Puig P, Carrere N, Manfredi S, et al. Small bowel adenocarcinoma: Epidemiology, risk factors, diagnosis and treatment. Dig Liver Dis 2014;46:97-104.  Back to cited text no. 13
    
14.
Zaaimi Y, Aparicio T, Laurent-Puig P, Taieb J, Zaanan A. Advanced small bowel adenocarcinoma: Molecular characteristics and therapeutic perspectives. Clin Res Hepatol Gastroenterol 2016;40:154-60.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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