The cancer of duodenum is a rare but aggressive disease and radical surgery to achieve complete tumor excision is the only potentially curative treatment. Due to rarity, these tumors are often grouped either with periampullary or small bowel cancers and therefore available literature for their management is limited.
Traditionally pancreatoduodenectomy or Whipple’s procedure is the recommended surgical procedure for duodenal cancers. Whipple’s procedure is a complex procedure and still carries a mortality of up to 5% and morbidity in the range of 30-50% at well endowed centers. Moreover for non-ampullary duodenal cancers the risks associated with Whipple’s procedure are reported to be higher due to risk factors including non dilated pancreatic duct & soft pancreas.
As per the recent NCCN guidelines while the preferred treatment for duodenal cancers is Whipple’s procedure however in select patients limited segmentectomy with regional lymphadenectomy is an acceptable alternative. We present one such patient recently managed by our team.
A 57 years gentleman with a recent history (May 2019) of coronary artery bypass graft (CABG) and aortic valve replacement (AVR) presented in emergency room with massive upper GI bleed in August 2019. He also had history of recurrent vomiting of 20-25 days duration and also loss of weight of approx 15 kg over 2 months following cardiac surgery. Following initial resuscitation in ICU and discontinuation of anti-platelet & anti-coagulation medications, an urgent upper GI endoscopy was performed. On endoscopy except for bleeding from stricture in fourth part of duodenum, rest of the upper GI tract was normal (Figure 1).
Figure 1. Upper GI endoscopy – bleed from stricture duodenum
Bleeding was controlled with injection sclerotherapy and the biopsy from the lesion was reported as tubulo-villous adenoma with high grade dysplasia. A subsequent CECT whole abdomen was suggestive of circumferential duodenum wall thickening in third part of duodenum (Figure 2).
Figure 2. CECT abdomen displaying circumferential wall thickening in III part of duodenum (white arrows) & additional findings of horse shoe kidney (asterisk )
The tumor markers CEA (1.31) & Ca 19.9 (0.8) were within normal range and colonoscopic examination did not reveal any abnormality. After thorough work up and discontinuation of anticoagulants 12 hours prior he was taken up for surgery.
In our patient because of recent CABG and AVR surgery, early resumption of anticoagulants & anti-platelet medications was warranted. Hence PPDD was an obvious alternative especially because tumor was confined to the wall of duodenum and distally located (III & IV part of duodenum).
At surgery there was stricture in III & IV part of duodenum and enlarged regional lymph nodes. A PPDD with 5 cm proximal and 10 cm distal margin along with regional lymphadenectomy was performed. A side to side duodeno-jejunal anastomosis (second part of duodenum & proximal jejunum) and feeding jejunostomy was fashioned. In post operative period injection Enoxaparin (0.6 mg sc BD) was re-started on post operative day (POD) 1 and subsequently anti-platelet medications were added on POD 4. Except for delayed gastric emptying his postoperative period was uneventful. Histopathology reported well differentiated adenocarcinoma of duodenum and tumour was invading muscularis propria & focally into subserosa (pT3). No lympho-vacular emboli or perineural spread was seen. All 17 lymph nodes and surgical margins (proximal, distal and radial) were free from tumour.
Primary adenocarcinoma of the duodenum is an uncommon neoplasm but the prognosis is better in comparison to cancer of pancreas or distal bile duct.
Because of the soft pancreatic texture and small size pancreatic duct, the rate of pancreatic fistula following Whipple’s procedure for non-ampullary duodenal cancers is reported to be in the range of 28.9 – 32.5% which is almost double in comparison to Whipple’s procedure for all other pathologies.1,2
There are conflicting reports on the extent of surgery for duodenal cancers. Some advocate PD for all duodenal cancers regardless of location while others support segmental resection (PPDD) for distal duodenal cancers if margin negative resection is feasible.
In one of the early study from Johns Hopkins Hospital, significant 5 year overall survival advantage with PD (69%) was reported in comparison to PPDD (0%).3 However several subsequent studies have not supported these results in favour of PD. A study from Mayo Clinic reported comparable overall survival between the groups of patients undergoing PD or PPDD for duodenal cancer. The 5 year survival estimate in PPDD group was 52% and in PD group was 42 % (p=0.50).4 A study of SEER database involving 1611 patients’ also reported that PD does not impact 5 year survival (37.6%) compared with segmental resection (41.3 %) for duodenal cancers (p > 0.05).5 In a recent systematic review of largest collective number of patients (n = 1728) authors concluded that aggressive surgical approach to achieve complete tumor excision should be pursued and PPDD should be considered if tumor invasion is confined to duodenal wall especially for distal duodenal tumors.6
In summary, PPDD is an acceptable alternative to Whipple’s procedure for non ampullary duodenal cancers particularly in high risk cases.
1. Le CHA, Shingler G, Mowbray NG et al. Surgical outcomes for duodenal adenoma and adenocarcinoma: a multicentre study in Australia and the United Kingdom. ANZ J Surg. 2018;88:E157-E161. Doi:10.1111/ans.13873
2. Shamali A, McCrudden R, Bhandari P et al. Pancreaticoduodenectomy for nonampullary duodenal lesions: indications and results. Eur J Gastroenterol Hepatol. 2016;28(12):1388-1393
3. Sohn TA, Lillemoe KD, Cameron JL et al. Adenocarcinoma of the duodenum: factors influencing long-term survival. J Gastrointest Surg. 1998;2(1):79-87
4. Onkendi EO, Boostrom SY, Sarr MG et al. 15-year experience with surgical treatment of duodenal carcinoma: a comparison of periampullary and extra-ampullary duodenal carcinomas. J Gastrointest Surg. 2012;16(4):682-691
5. Cloyd JM, Norton JA, Visser BC et al. Does the extent of resection impact survival for duodenal adenocarcinoma? Analysis of 1,611 cases. Ann Surg Oncol. 2015;22(2):573-580
6. Debang Li, Xiaoying Si, Tao Wan et al. Outcomes of surgical resection for primary duodenal adenocarcinoma: A systematic review. Asian J Surg. 2019;42(1):46-52
Suggested for further reading:
Dr Nitin Vashistha, MS, FIAGES, FACS
Dr Dinesh Singhal, MS, FACS, DNB (Surg Gastro)
Department of Surgical Gastroenterology,
Max Super Speciality Hospital, Saket, New Delhi, India
E mail: firstname.lastname@example.org