Adenocarcinoma

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Definition

Uncommon malignant epithelial tumor of the small intestine, commonly seen in the region of the ampulla of Vater.

Clinical Features

40–60 times less common than adenocarcinoma in large bowel
Usually elderly
No gender predilection1,2
Can be associated with:
- hereditary nonpolyposis colorectal carcinoma syndrome 3
- Peutz–Jeghers syndrome
- Recklinghausen's disease4
- bowel duplication5
- Crohn's disease
- ileostomy sites6,7
- surgically bypassed duodenum 8
- jejunal limb of a Roux-en-Y esophagojejunostomy9
- ileal segment of a defunctionalized ileocystoplasty10
Occasionally:
- multiple11
- primary malignant neoplasms in other sites12,13
At diagnosis:
- most have extended deeply into wall
- may have metastasized to regional lymph nodes

Gross Pathology

Any segment
More common in upper portions 14–16
≈40–50% in duodenum:17,18
- tends to have papillary configuration:
  - amenable to brush cytologic diagnosis 19
More distal lesions:
- usually napkin-ring appearance
- partial intestinal obstruction with marked dilatation of the proximal bowel
- ≈20% predominantly polypoid or fungating:
  - perhaps due to origin in adenomatous polyp or villous adenoma14

Histopathology

Usually moderately well differentiated adenocarcinoma
Ultrastructurally:
- may be prominent microvilli20

Special Stains and Immunohistochemistry

Usually:
- mucin production
- CEA reactivity21
May be immunoreactivity for lysozyme:
- suggesting focal differentiation toward Paneth cells22
Often:
- with pattern similar to that of large bowel adenocarcinoma23 express:
  - COX-2
  - sPLA2
  - cPLA2:
- scattering of endocrine cells:
  - particularly in ileal tumors
  - positive for:
    - chromogranin
    - 5-HT
  - may be immunoreactive for peptide hormones, including:
    - somatostatin
    - YY peptide
    - neurotensin
    - glucagon
    - glycentin24

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Adenocarcinoma

Prognosis

Depends on regional lymph node involvement:
- mortality rate in one study:14
  - 88% if positive nodes
  - 45% if negative nodes

References

1 Adler SN, Lyon DT, Sullivan PD. Adenocarcinoma of the small bowel. Clinical features, similarity to regional enteritis, and analysis of 338 documented cases. Am J Gastroenterol. 1982;77:326–330.

2 Arai T, Murata T, Sawabe M, Takubo K, Esaki Y. Primary adenocarcinoma of the duodenum in the elderly: clinicopathological and immunohistochemical study of 17 cases. Pathol Int. 1999;49:23–29.

3 Rodriguez-Bigas MA, Vasen HF, Lynch HT, Watson P, Myrhoj T, Jarvinen HJ, et al. Characteristics of small bowel carcinoma in hereditary nonpolyposis colorectal carcinoma. Cancer. 1998;83:240–244.

4 Benharroch D, Sion-Vardi N, Goldstein J. Neurofibromatosis involving the small bowel associated with adenocarcinoma of the ileum with a neuroendocrine component. Pathol Res Pract. 1992;188:959–963.

5 Adair HM, Trowell JE. Squamous cell carcinoma arising in a duplication of the small bowel. J Pathol. 1981;133:25–31.

6 Baciewicz F, Sparberg M, Lawrence JB, Potichá SM. Adenocarcinoma of an ileostomy site with skin invasion. A case report. Gastroenterology. 1983;84:168–170.

7 Cuesta MA, Donner R. Adenocarcinoma arising at an ileostomy site. Report of a case. Cancer. 1976;37:949–952.

8 Lipper S, Graves GV. Villous adenocarcinoma arising in the bypassed duodenum 18 years after a Billroth II subtotal gastrectomy. Report of a case and review of the literature. Am J Gastroenterol. 1985;80:174–176.

9 Guadagni S, Catarci M, Ventura T, Leocata P, Carboni M. Primary adenocarcinoma arising in the jejunal limb of a Roux-en-Y esophagojejunostomy. A case report. Jpn J Clin Oncol. 1993;23:59–63.

10 Takahashi A, Tsukamoto TD, Kumamoto Y, Sato Y, Shibuya A, Sato M. Adenocarcinoma arising in the ileal segment of a defunctionalized ileocystoplasty. Hinyokika Kiyo. 1993;39:753–755.

11 Wagner KM, Thompson J, Herlinger H, Caroline D. Thirteen primary adenocarcinomas of the ileum and appendix. A case report. Cancer. 1982;49:797–801.

12 Barclay THC, Schapira DV. Malignant tumors of the small intestine. Cancer. 1983;51:878–881.

13 Stemmermann GN, Goodman MT, Nomura AM. Adenocarcinoma of the proximal small intestine. A marker for familial and multicentric cancer? Cancer. 1992;70:2766–2771.

14 Bridge MF, Perzin KH. Primary adenocarcinoma of the jejunum and ileum. A clinicopathologic study. Cancer. 1975;36:1876–1887.

15 Howe JR, Karnell LH, Menck HR, Scott-Conner C. The American College of Surgeons Commission on Cancer and the American Cancer Society. Adenocarcinoma of the small bowel: review of the National Cancer Data Base, 1985-1995. Cancer. 1999;86:2693–2706.

16 Lien G-S, Mori M, Enjoji M. Primary carcinoma of the small intestine. A clinicopathologic and immunohistochemical study. Cancer. 1988;61:316–323.

17 Rudan N, Nola P, Popovic S. Primary adenocarcinoma of the duodenum. Report of two cases. Cancer. 1984;54:1105–1109.

18 Spira IA, Ghazi A, Wolff WI. Primary adenocarcinoma of the duodenum. Cancer. 1977;39:1721–1726.

19 Bardales RH, Stanley MW, Simpson DD, Baker SJ, Steele CT, Schaefer RF, et al. Diagnostic value of brush cytology in the diagnosis of duodenal, biliary, and ampullary neoplasms. Am J Clin Pathol. 1998;109:540–548.

20 Yamashina M. Primary adenocarcinoma of the small intestine with emphasis on microvillous differentiation. Acta Pathol Jpn. 1987;37:1061–1070.

21 Blackman E, Nash SV. Diagnosis of duodenal and ampullary epithelial neoplasms by endoscopic biopsy. A clinicopathologic and immunohistochemical study. Hum Pathol. 1985;16:901–910.

22 Lundqvist M, Wilander E. Exocrine and endocrine cell differentiation in small intestinal adenocarcinomas. Acta Pathol Microbiol Immunol Scand A. 1983;91:469–474.

23 Wendum D, Svrcek M, Rigau V, Boëlle P-Y, Sebbagh N, Parc R, et al. COX-2, inflammatory secreted PLA2, and cytoplasmic PLA2 protein expression in small bowel adenocarcinomas compared with colorectal adenocarcinomas. Mod Pathol. 2003;16:130–136.

24 Iwafuchi M, Watanabe H, Ishihara N, Enjoji M, Iwashita A, Yanaihara N, et al. Neoplastic endocrine cells in carcinomas of the small intestine. Histochemical and immunohistochemical studies of 24 tumors. Hum Pathol. 1987;18:185–194.

Last updated: 18 Mar 2006