Malignant tumors of the duodenum

Incidence

Primary malignant tumors of the duodenum represent 0.3% of all gastro-intestinal tract tumors but up to 50% of small bowel malignancies. Primary malignant tumors of the duodenum must be differentiated from malignant tumors of the ampulla, pancreas and common bile duct. The most frequent tumor of the duodenum is adenocarcinoma (1, 2). Other primary tumors are lymphomas, leiomyosarcomas, carcinoid tumors, gastrinomas, stromal tumors. Adenocarcinoma of the duodenum may arise from duodenal polyps observed in familial polyposis or Gardener's syndrome, or be associated with celiac disease (3, 4). The tumor can be located in any part of the duodenum but the most frequent location is the second part.

Clinical presentation

Malignant tumors of the duodenum are observed with the same frequency in men and women? The peak of frequency is the sixth decade, although the disease may develop in younger patients. Signs and symptoms are specific. The main symptoms are: abdominal pain (15 to 60% of patients), weight loss (30 to 59%), nausea and vomiting (25 to 30%), jaundice (20 to 30%), hemorrhage (10 to 38%). A palpable abdominal mass is found in less than 5% of the patients (5).

Diagnosis

Barium studies of the upper intestinal tract have been replaced by fiberoptic endoscopy. Barium examination show in most cases an irregular stricture of the duodenum, but can be normal or lead to the diagnostic of benign stricture. Fiberoptic endoscopy allows a precise location of the tumor and endoscopic biopsies which confirm the diagnosis.

Preoperative staging

No study has evaluated the best method of preoperative staging of malignant lesions of the duodenum. Some authors use ultrasonography for the diagnosis of liver metastases; the accuracy of CT Scan, IRM and angiography have not been studied. These investigations are not performed routinely, most of the patients being operated on if only for a palliative procedure.

Endoscopic ultrasonography has been reported to be useful for the preoperative staging of ampullary and pancreatic carcinomas. No study reports its accuracy in the preoperative evaluation of malignant duodenal tumors. Five to 40% of the patients have distant metastases or peritoneal seeding at the time of diagnosis (6).

Treatment

Due to the low incidence of the disease there is no randomized study comparing different types of treatment. Complete surgical resection is the only hope for cure. Two types of surgical resection are available: pancreatoduodenectomy associated with various types of lymphadenectomies or segmental resections (7, 8). Pancreatoduodenectomy has been advocated as the surgical procedure of choice because it offers the possibility of regional lymph node resection. Nonetheless good long-term results have been observed with segmental resection, particularly for tumors of the distal part of the duodenum (9). When local extension or metastatic disease preclude curative resection, palliative procedures such as gastrojejunal anastomosis can be performed. Laser photocoagulation has been proposed for patients unfit for surgery with good palliation on hemorrhage and obstructive symptoms.

Radiotherapy and chemotherapy have been used in few cases most often as an adjuvant postoperative treatment with no improvement in outcome. Only one study has shown a complete response in 4 patients treated preoperatively by radio-chemotherapy. The treatment was completed by duodenopancreatectomy and all patients are alive 12 to 90 months after treatment (10). Nonetheless, no sound conclusions on the efficacy of these treatments can be established.

Prognostic assessment

The 5 year-survival rate varies widely according to the series published, but is generally reported to be > 40% in case of curative resection. The presence of lymph node metastases is not a factor of bad prognosis in most series and must not preclude an attempt at curative resection (7, 8, 9).

References

1.

Lillemoe K, Inbembo A L. Malignant neoplasms of the duodenum. Surg Gynecol Obstet. (1980);150:822–826. [PubMed: 7376043]

2.

Cunningham J D, Aleali R, Aleali M, Brower S T, Aufses A H. Malignant bowel neoplasms. Histopathologic determinants of recurrence and survival. Ann Surg. (1997);225:300–306. [PMC free article: PMC1190681] [PubMed: 9060587]

3.

Burt R W, Berenson M M, Lee R G, Tolman J W. et al. Upper gastrointestinal polyps in Gardner's syndrome. Gastroenterology. (1984);86:295–301. [PubMed: 6690356]

4.

Homes G K T, Dunn G I, Cockel R, Brookes V S. Adenocarcinoma of the upper small bowel complicating coeliac disease. Gut. (1980);21:1010–1016. [PMC free article: PMC1419282] [PubMed: 7450557]

5.

Spira I A, Ghazi A, Wolff W I. Primary adenocarcinoma of the duodenum. Cancer. (1997);39:1721–1726. [PubMed: 322840]

6.

Rose D M, Hochwald S N, Klimastra D S, Brennan M F. Primary duodenal adenocarcinoma: a ten-year experience with 79 patients. J Am Coll Surg. (1996);183:89–96. [PubMed: 8696551]

7.

Rotman N, Pezet D, Fagniez PL, Cherqui D, Celicout B, Lointier P (1994) Br J Surg 81: 83–85 . [PubMed: 7508805]

8.

Ohigashi H, Ishikawa O, Tamura S, Imaoka S. et al. Pancreatic invasion as the prognostic indicator of duodenal adenocarcinoma treated by pancreatoduodenectomy plus extended lymphadenectomy. Surgery. (1998);124:510–515. [PubMed: 9736903]

9.

Lowel J A, Rossi R L, Munson L, Braash J W. Primary adenocarcinoma of the third and fourth portions of duodenum. Favorable prognosis after resection. Arch Surg. (1992);127:557–560. [PubMed: 1349472]

10.

Coia L, Hoffman J, Scher R, Weese J. et al. Preoperative chemoradiation for adenocarcinoma of the pancreas and duodenum. Int J Radiat Biol Phys. (1994);13:161–167. [PubMed: 8083109]