Ampullary tumours are un-common. However, becauseof their strategic location at

the confluence of the common bileduct and pancreatic duct, they areusually symptomatic.1–3 Periampullaryneoplasms are far more common, andin this group carcinoma of the head ofpancreas is most frequently seen.3,4 Be-cause of the difficulties in differentiat-ing ampullary from periampullary tu-mours clinically5 and radiologically3

and the problems in establishing a de-finitive tissue diagnosis based on en-doscopic biopsies,1–3 it is often impos-sible to categorize these tumourspreoperatively.3,6 A high proportion ofthe ampullary tumours are malig-nant,1,3,6,7 so there is a tendency tomanage them by extensive surgical ex-cision.1,3,5–9 We describe the case of a

man who had a sporadic ampullaryhamartoma that was treated by localtransduodenal excision.

CASE REPORT

A 67-year-old man had severe epi-gastric pain and cachexia. He had ahistory of 2 episodes of acute uncom-plicated pancreatitis 4 and 7 years previously. Endoscopic retrogradecholangiopancreatography (ERCP),abdominal ultrasonography and ab-dominal CT done shortly after thesecond episode gave normal results.His serum amylase, bilirubin and liverenzyme levels were normal except formild elevation of the γ-glutamyl trans-ferase level. Over the next 4 years, theepigastric pain worsened. It was “bor-ing” in character, radiated to the back

and was aggravated by eating. He lostapproximately 9 kg over 18 months.On admission to hospital, the serumamylase, bilirubin and liver enzymelevels were again normal except for amildly elevated γ-glutamyl transferaselevel at 67 U/L (normal 10 to 50U/L). A secretin test indicated a di-minished duodenal bicarbonate con-centration of less than 70 mmol/L,and the bentiromide (Chymex) testvalue was 41% (normal 70% to 100%).ERCP demonstrated mild dilatationof the common bile duct. He was con-sidered to have chronic pancreatitisand was treated with pancrelipase.After 6 months of pancrelipase

therapy, the pain was still severe butnow was worse at night and whensupine. He had lost his appetite andcontinued to lose weight. Serum

Case ReportÉtude de cas

SPORADIC AMPULLARY HAMARTOMA SIMULATING CANCER

Terence N. Moyana, MD;* Grant G. Miller, MD;† Roger G. Keith, MD†

From the *Department of Pathology and †Department of Surgery, College of Medicine, University of Saskatchewan, Saskatoon, Sask.

Accepted for publication June 25, 1996

Correspondence and reprint requests to: Dr. Terence Moyana, Department of Pathology, Royal University Hospital, 103 Hospital Dr., Saskatoon SK S7N 0W8

© 1997 Canadian Medical Association (text and abstract/résumé)

Ampullary tumours are uncommon. They may occur with familial polyposis syndromes or neurofibromato-sis. It can be difficult to distinguish them from their periampullary counterparts on clinical, radiologic orhistologic grounds. Because most ampullary and periampullary tumours are malignant, they tend to betreated by radical surgery. A 67-year-old man was seen with a sporadic ampullary hamartoma that simu-lated cancer. It was succesfully treated by local excision through a transverse duodenotomy.

Les tumeurs de l’ampoule sont rares. Elles peuvent accompagner le syndrome de la polypose familiale ou laneurofibromatose. Elles peuvent être difficiles à distinguer des tumeurs périampulaires par des moyens clin-iques, radiologiques ou histologiques. Parce que la plupart des tumeurs ampulaires et périampulaires sontmalignes, on a tendance à les traiter par chirurgie radicale. On a examiné un homme de 67 ans qui avait unhamartome ampulaire sporadique ressemblant à un cancer. On a traité la tumeur avec succès en procédantà une excision locale par duodénotomie transversale.

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bilirubin and amylase levels were nor-mal, the serum aspartate aminotrans-ferase level was 115 U/L (normal 10to 40 U/L), the serum alanine amino-transferase level was 85 U/L (normal0 to 40 U/L), the serum alkalinephosphatase level was 205 U/L (nor-

mal 30 to 110 U/L) and the serum γ-glutamyl transferase level was 298U/L (normal 10 to 50 U/L). Ab-dominal ultrasonography revealedmildly dilated common bile and com-mon hepatic ducts. CT of the ab-domen gave normal results as did he-

patobiliary nuclear imaging. ERCPdemonstrated a dilated common bileduct and pancreatic duct (Fig. 1). Theampulla of Vater was abnormally pro-tuberant and granular, but a biopsyspecimen could not be obtained ow-ing to technical difficulties.It was suspected that this abnormal

ampulla represented the cause of thepatient’s symptoms, and because ofsuspected malignant disease of theampulla, the patient agreed to un-dergo laparotomy. The mass wasfound to be limited to the ampulla,and there was no suspected peri-ampullary involvement. For this rea-son, the ampulla was exploredthrough a transverse duodenotomy.The mass was not unduly firm, andthe mucosa overlying it appeared un-remarkable; a frozen section was notordered. The entire mass was removedby local excision of the ampulla ofVater (Fig. 2) with reanastomosis ofthe pancreatic and common bile ductsto the duodenum. A cholecystectomywas carried out at the same time.Pathological examination of the am-pullary mass showed it was a hamar-toma; it was composed of a haphazardadmixture of cystically dilated glandu-lar elements, associated with a slightdegree of fibromuscular proliferation

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FIG. 1. Endoscopic retrograde cholangiopancreatography showing dilatation of the common bile andpancreatic ducts. Note the irregular filling defect (arrows) suggestive of a neoplasm in the ampullaryregion. R = right.

FIG. 2. Low-power view (left) of part of the hamartoma that is centred around a dilated duct (hematoxylin–eosin, original magnification × 5). High-powerview (right) of the hamartoma showing a disorderly admixture of blood vessels, lymphatics, glandular structures and smooth-muscle elements (hema-toxylin–eosin, original magnification × 115).

and an increased number of ectaticblood vessels and lymphatics (Fig. 2).The gallbladder was devoid of calculiand was essentially normal. The pa-tient recovered from the procedurewithout complications and was dis-charged home pain free on the 14thpostoperative day on the pancreaticenzyme supplement. More than 4years later, he remained pain free andhis weight was stable. At no time pre-or postoperatively did physical exami-nation or upper and lower gastroin-testinal endoscopy disclose any otherhamartomas, nor did the patient haveany family history of this problem.

DISCUSSION

The ampulla of Vater is a vase-shaped structure adjacent to the sec-ond part of the duodenum below thesurface of the papilla into which thecommon bile duct and, in most cases,the main pancreatic duct drain.3,4 Theembryologic development of thepapilla and the variations thereof arecomplex,2–4 which in turn makes theclinicopathologic manifestations of itslesions rather interesting. The am-pullary hamartoma is a case in point;these lesions are uncommon, and onlya small number of cases have been de-scribed in the literature.1,2,9,10 Theyhave also variously been referred to asadenomyosis, adenomyomas, cystade-nomas or fibroadenomas dependingon their histologic constitution.2,9,10

However, the central tenet to all theseappellations is that they represent ahaphazard, non-neoplastic over-growth of tissues normally found atthat site.1,4,9,10

With respect to ampullary growthsas a whole, hamartomas are far out-numbered by real tumours.2,3,5,7 Thecommonest entity in the latter cate-gory is carcinoma,3,5 followed by otherneoplasms such as adenomas, carci-noids, leiomyomas, fibromas, lipomas,

lymphangiomas, hemangiomas andgranular cell tumours.2–4,8–10 However,the clinical presentation of ampullaryhamartomas is generally similar to thatof the real neoplasm, the features be-ing largely due to partial, intermittentor complete common biliary or pan-creatic duct obstruction, or both,1,3,8–12

as was the case in this report.Imaging modalities such as barium

series, CT, routine and endoscopic ul-trasonography and ERCP help consid-erably in defining the topography ofthese lesions.5,6,13,14 Despite this, it maybe difficult to determine whether a tu-mour is primarily ampullary, withspread to the periampullary region, orvice versa.3,5,6 More importantly, it maybe more difficult to detect small am-pullary lesions especially if they do notprotrude into the papilla;1,3 thus, thesymptoms they produce may be erro-neously attributed to chronic relapsingpancreatitis or to biliary lithiasis.1,3,11,12

Even with endoscopic biopsies, takenfor instance at ERCP, there are also po-tential diagnostic pitfalls that relate tothe special nature of biliary tract histol-ogy, particularly in the ampullary re-gion.2,4,6 Hence, normal ampullary tis-sues can be mistaken for carcinoma byvirtue of such features as perineural in-vasion. Alternatively, lesions harbour-ing carcinoma may be labelled as nor-mal or benign especially if the biopsy issuperficial or from a well-differentiatedcarcinoma.2–4

There is an association between am-pullary neoplasms and hamartomas onthe one hand and familial polyposissyndromes such as Gardner syndromeand Peutz–Jeghers syndrome,15–18 orneurofibromatosis,12 on the other.However, ampullary lesions can alsobe isolated and sporadic as in our case.Apart from ampullary neoplasms, het-erotopic pancreatic tissue has to beconsidered in the differential diagno-sis, on embryologic grounds.3,4 How-ever, the predominance of vascular

structures with haphazardly ar rangedelements of smooth muscle and in-testinal epithelium, as well as the ab-sence of both exocrine and endocrinepancreatic tissue in the present case au-gurs against this possibility.

References

1. Venu RP, Rolny P, Geenen JE,Hogan WJ, Komorowski RA. Am-pullary hamartoma: endoscopic diag-nosis and treatment. Gastroenterology1991;100(3):795-8.

2. Albores-Saavedra J, Henson DE. Tu-mors of the gallbladder and extrahep-atic bile ducts. In. Atlas of tumorpathology. 2nd ser, fasc 22. Washing-ton: Armed Forces Institute ofPathology; 1984. p. 153-81.

3. Buck JL, Elsayed AM. Ampullary tu-mors: radiologic-pathologic correlation.Radiographics 1993;13(1):193-212.

4. Cubilla AL, Fitzgerald PJ. Tumors ofthe exocrine pancreas. In. Atlas of tu-mor pathology. 2nd ser, fasc 19. Wash-ington: Armed Forces Institute ofPathology; 1982. p. 53-97.

5. Hayes DH, Bolton JS, Willis GW,Bowen JC. Carcinoma of the ampulla ofVater. Ann Surg 1987;206(5): 572-7.

6. Buice WS, Walker LG Jr. The role ofintra-operative biopsy in the treat-ment of resectable neoplasms of thepancreas and periampullary region.Am Surg 1989;55(5):307-10.

7. Michelassi F, Erroi F, Dawson PJ,Pietrabissa A, Noda S, Handcock M,et al. Experience with 647 consecu-tive tumors of the duodenum, am-pulla, head of pancreas, and distalcommon bile duct [review]. AnnSurg 1989;210(4):544-56.

8. Ricci JL: Carcinoid of the ampulla ofVater. Local resection or pancreatico-duodenectomy [review]. Cancer 1993;71 (3): 686-90.

9. Ulich TR, Kollin M, Simmons GE,Wilczynski SP, Waxman K. Adenomy-oma of the papilla of Vater. ArchPathol Lab Med 1987;111(4):388-90.

10. Bergdahl L, Andersson A: Benign tu-mors of the papilla of Vater. Am Surg1980;46(10):563-6.

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11. Guzzardo G, Kleinman MS, KrackovJH, Schwartz SI. Recurrent acutepancreatitis caused by ampullary vil-lous adenoma [review]. J Clin Gas-troenterol 1990;12(2):200-2.

12. Kahrilas PJ, Hogan WJ, Geenen JE,Stewart ET, Dodds WJ, AndorferRC. Chronic recurrent pancreatitissecondary to a submucosal ampullarytumor in a patient with neurofibro-matosis. Dig Dis Sci 1987;32(1):102-7.

13. Tio TL, Mulder CJJ, Eggink WF.Endosonography in staging early car-

cinoma of the ampulla of Vater. Gas-troenterology 1992;102:1392-5.

14. Rosch T, Braig C, Gain T, FeuerbachS, Siewet JR, Schusdziarra V, et al.Staging of pancreatic and ampullarycarcinoma by endoscopic ultrasonog-raphy. Comparison with conventionalsonography, computed tomography,and angiography. Gastroenterology1992; 102(1):188-9.

15. Jagelman DG, DeCosse JJ, Bussey HJ.Upper gastrointestinal cancer in familial adenomatous polyposis. Lancet1988;1:1149-51.

16. Guyton DP, Schreiber H. Intestinalpolyposis and periampullary carci-noma: changing concepts. J Surg On-col 1985;29:158-9.

17. Pauli RM, Pauli ME, Hall JG. Gard-ner syndrome and periampullary ma-lignancy. Am J Med Genet 1980;6:205-9.

18. Perzin KH, Bridge MF. Adenoma-tous and carcinomatous changes inhamartomatous polyps of the smallintestine (Peutz–Jeghers syndrome):report of a case and review of the lit-erature. Cancer 1982;49:971-83.

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