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CASE REPORT Table of Contents   
Year : 2014  |  Volume : 11  |  Issue : 1  |  Page : 79-81
Duodenal adenocarcinoma in a 10-year-old boy


Department of Pediatric Surgery, Habib Thameur Hospital, Tunis, Tunisia

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Date of Web Publication20-Mar-2014
 

   Abstract 

Gastrointestinal malignancies are extremely rare in the paediatric population and duodenal cancers represent an even more unusual entity. It represents 0.3-1% of all gastrointestinal tumours. A case report of a 10-year-old boy with duodenal adenocarcinoma is reported and the difficulties of diagnosing and treating this rare tumour are discussed.

Keywords: Duodenal adenocarcinoma, paediatric population, surgery

How to cite this article:
Mohamed Z, Habib B, Rabia BA, Youssef H, Riath BM, Youssef G, Nejib K. Duodenal adenocarcinoma in a 10-year-old boy. Afr J Paediatr Surg 2014;11:79-81

How to cite this URL:
Mohamed Z, Habib B, Rabia BA, Youssef H, Riath BM, Youssef G, Nejib K. Duodenal adenocarcinoma in a 10-year-old boy. Afr J Paediatr Surg [serial online] 2014 [cited 2019 Nov 19];11:79-81. Available from: http://www.afrjpaedsurg.org/text.asp?2014/11/1/79/129244

   Introduction Top


Duodenal adenocarcinomas are rare in children. They represents 0.3-1% of gastrointestinal cancers and 25-35% of small bowel malignancies. [1],[2] This rarity and the lack of specificity of their symptoms largely explain the delay in diagnosis and especially, their prognosis.


   Case Report Top


A 10-year-old boy presented to our unit in January 2012 with a 1-month history of abdominal pains, repeated vomiting and weight loss. An oesophagogastroduodenoscopy was performed revealing a duodenal ulcer treated medically. The evolution was marked by the persistence of clinical symptoms. A subsequent barium meal showed a bulbar stenosis with a large gastric stasis [Figure 1]. The child was initially operated with the diagnosis of stenosing ulcer of the bulb; he had bilateral vagotomy with pyloroplasty and making a check valve according to Dorr. During the operation, a pyloric lymphadenopathy was found, taken and sent for histological examination.
Figure 1: Bulbar stenosis

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The histopathological findings reported a lymph node metastasis of adenocarcinoma probably of digestive origin.

Post-operatively, there was a regression of pain with less vomiting. The post-operative gastroscopy showed two polypoid and budding masses: The first in the bulb and the second level in the gastric antrum with respectively 20 and 6 mmdiameter. Biopsies were taken and showed aduodenal adenocarcinomawith same morphology as the node. Immunohistochemical evaluation showed strong staining of the neoplastic cells for Cytokeratin (CK7), Vimentin and CD10. These same cells were negative for CK20 and AFP. This profile suggested a kidney cancer. Abdominal MRI revealed a nodule in the liver [Figure 2], the kidneys were intact.
Figure 2: Hepatic nodule

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Patient underwent surgery, during which a 2 cm wide neoplasia was found in the first portion of the duodenum. The tumour extends to D2, the gastric antrum and the pancreas. The liver size, colour and consistency were normal without palpable nodule. The falciform ligament of the liver, which was cut during the first intervention, was retracted exactly in the seat of the nodule visualised by MRI. The essential components of the operation included duodeno-cephalo-pancreatectomy with gastric antrum resection, gastrojejunal end-to-side anastomosis, anastomosis between the jejunum to the common bile duct and pancreatic slice section, lymph node dissection and cholecystectomy.

The histopathological findings reported "well-differentiated adenocarcinoma extending through the duodenal serosa and infiltrating the pancreatic parenchyma with lymph node metastases (5N+/8N); pT4 N2". Post-operative period was uneventful with no complications. Patient resumed oral feeding on the 8 th post-operative day and was discharged on the 15 th post-operative day; four cycles of adjuvant chemotherapy were administered after oncological advice. A 1-year clinical-instrumental follow-up showed no locoregional recurrence of the disease.


   Discussion Top


Gastrointestinal malignancies are extremely rare in the paediatric population and duodenal cancers represent an even more unusual entity. [1] It represents 0.3-1% of all gastrointestinal tumours and 25-35% of malignant tumours of the small intestine. [2],[3] Associations with familial adenomatous polyposis (FAP), Crohn's disease, [4] Peutz-Jeghers syndrome [5] and neurofibromatosis 1 [6] have been reported. Duodenal adenocarcinoma often presents with vague and non-specific symptoms that lead to delayed diagnosis, patients can present with abdominal pain, bleeding, weight loss, obstruction or jaundice and most of the cases are accidentally discovered. [7],[8],[9] Oesophagogastroduodenoscopy with biopsy is the diagnostic gold standard. Nevertheless, computed abdominal tomography may be essential to show the neoplasia in the duodenal wall and determine the tumour staging and define the therapeutic strategy. Duodenal adenocarcinoma is known for its low sensitivity to chemotherapy. The only treatment for duodenal adenocarcinoma, which offers the possibility of a cure is excision by radical surgery. [10] Radical pancreaticoduodenectomy (Whipple's operation) is the classical curative operation and is still the treatment of choice for tumours in the first and second parts of the duodenum. Segmental resection is appropriate for selected patients, especially with lesions of the distal duodenum. [11] For unresectable tumours due to mesenteric vascular invasion or a major extension to surrounding organs or peritoneal carcinomatosis or distant metastases only simple bypass, type of gastro-jejunal-anastomosis or biliary-digestive bypass is advised, with a survival of 6 months. [12],[13],[14]

The 5-year survival rate for curatively resected adenocarcinomas of the duodenum is of the order of 50-60%. [15] Metastasis to lymph nodes, advanced tumour stage and positive resection margins are associated with decreased survival in patients with duodenal adenocarcinoma. [3]

The role of adjuvant chemotherapy is still unclear likely due to the rarity of this malignant neoplasm and the small patients numbers in most published experiences. [3],[9],[16],[17] Some studies show important statistical benefits for medium- and long-term survival. [9],[17] Information in the literature concerning this approach is scarce and needs further evaluation. Radiotherapy is not applicable as the tumour is radio-resistant and the small bowel has poor tolerance to radiation. [18] Hopefully, early diagnosis will correlate with improved long-term survival.

 
   References Top

1.Roy S, Raskin L, Raymond VM, Thibodeau SN, Mody RJ, Gruber SB. Pediatric duodenal cancer and biallelic mismatch repair gene mutations. Pediatr Blood Cancer 2009;53:116-20.  Back to cited text no. 1
    
2.Sohn TA, Lillemoe KD, Cameron JL, Pitt HA, Kaufman HS, Hruban RH, et al. Adenocarcinoma of the duodenum: Factors influencing long-term survival. J Gastrointest Surg 1998;2:79-87.  Back to cited text no. 2
    
3.Bakaeen FG, Murr MM, Sarr MG, Thompson GB, Farnell MB, Nagorney DM, et al. What prognostic factors are important in duodenal adenocarcinoma? Arch Surg 2000;135:635-41.  Back to cited text no. 3
    
4.Mansari OE, Parc Y, Lamy P, Parc R, Tiret E, Beaugerie L. Adenocarcinoma complicating Crohn's disease of the duodenum. Eur J Gastroenterol Hepatol 2001;13:1259-60.  Back to cited text no. 4
    
5.Nakamura T, Suzuki S, Yokoi Y, Kashiwabara H, Maruyama K, Baba S, et al. Duodenal cancer in a patient with Peutz-Jeghers syndrome: Molecular analysis. J Gastroenterol 2002;37:376-80., et al.   Back to cited text no. 5
    
6.Joo YE, Kim HS, Choi SK, Rew JS, Park CS, Kim SJ. Primary duodenal adenocarcinoma associated with neurofibromatosis type 1. J Gastroenterol 2002;37:215-9.  Back to cited text no. 6
    
7.Chung WC, Paik CN, Jung SH, Lee KM, Kim SW, Chang UI, et al. Prognostic factors associated with survival in patients with primary duodenal adenocarcinoma. Korean J Intern Med 2011;26:34-40.  Back to cited text no. 7
    
8.Stock C, Keutgen XM, Pisapia D, Crawford C, Zarnegar R. Heterotopic pancreatic neoplasm presenting as an obstructing mass at the fourth portion of the duodenum. JOP 2011;12:241-3.  Back to cited text no. 8
    
9.Onkendi EO, Boostrom SY, Sarr MG, Farnell MB, Nagorney DM, Donohue JH, et al. Neoadjuvant treatment of duodenal adenocarcinoma: A rescue strategy. J Gastrointest Surg 2012;16:320-4.  Back to cited text no. 9
    
10.Santoro E, Sacchi M, Scutari F, Carboni F, Graziano F. Primary adenocarcinoma of the duodenum: Treatment and survival in 89 patients. Hepatogastroenterology 1997;44:1157-63.  Back to cited text no. 10
    
11.Tocchi A, Mazzoni G, Puma F, Miccini M, Cassini D, Bettelli E, et al. Adenocarcinoma of the third and fourth portions of the duodenum: Results of surgical treatment. Arch Surg 2003;138:80-5.  Back to cited text no. 11
    
12.Lointier P, Beorchia S. Primary duodenal adenocarcinoma. Rev Prat 1995;45:271-3.  Back to cited text no. 12
    
13.Prêtre R, Huber O, Robert J, Soravia C, Egeli RA, Rohner A. Results of surgical palliation for cancer of the head of the pancreas and periampullary region. Br J Surg 1992;79:795-8.  Back to cited text no. 13
    
14.Trede M, Schwall G, Saeger HD. Survival after pancreatoduodenectomy. 118 consecutive resections without an operative mortality. Ann Surg 1990;211:447-58.  Back to cited text no. 14
    
15.Sarela AI, Brennan MF, Karpeh MS, Klimstra D, Conlon KC. Adenocarcinoma of the duodenum: Importance of accurate lymph node staging and similarity in outcome to gastric cancer. Ann Surg Oncol 2004;11:380-6.  Back to cited text no. 15
    
16.Pozzetto B, Guarino G, Tonello C, Liguori G. Treatment of adenocarcinoma of the duodenum: Presentation of 4 clinical cases and review of the literature. Chir Ital 2002;54:195-201.  Back to cited text no. 16
    
17.Czaykowski P, Hui D. Chemotherapy in small bowel adenocarcinoma: 10-year experience of the British Columbia Cancer Agency. Clin Oncol (R Coll Radiol) 2007;19:143-9.  Back to cited text no. 17
    
18.Lee CC, Ng WK, Lin KW, Lai TW, Li SM. Adenocarcinoma of the duodenum. Hong Kong Med J 2008;14:67-9.  Back to cited text no. 18
    

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Correspondence Address:
Zouari Mohamed
Ain Road 3042, Sfax, Tunis
Tunisia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.129244

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