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Ampullary hamartoma: Endoscopic diagnosis and treatment
GASTROENTEROLOGY1991;100:795-798
CASE REPORTS
Ampullary Hamartoma: Endoscopic Diagnosis and Treatment RAMA P. VENU, PETER ROLNY, J. E. GEENEN, W. J. HOGAN, and RICHARD A. KOMOROWSKI Department of Gastroenterology and Pathology, Medical College of Wisconsin, Milwaukee, Wisconsin, and Digestive Disease Center, St. Luke's Hospital, Racine, Wisconsin
This study shows a patient who presented with intermittent biliary tract obstruction caused by ampullary hamartoma. Endoscopic retrograde cholangi o p a n c r e a t o g r a p h y showed a large ulcerated papilla and dilated biliary ducts. Tissue diagnosis was established by a large particle biopsy obtained with a snare. The patient underwent a successful endoscopic sphincterotomy and has remained symptom free for 4 years.
A
wide variety of neoplastic lesions may involve the ampulla of Vater (1,2}. Conventional imaging techniques such as upper gastrointestinal roentgenology, ultrasonography, or computed tomography (CT} scans often do not identify these tumors. Endoscopic
retrograde cholangiopancreatography (ERCP) has become an indispensable tool in the diagnosis of ampullary tumors as i t allows direct visualization of the t u m o r as.well as tissue biopsy. In selective instances: ERCP can also be useful in therapeutic intervention. This s t u d y shows the first case of ampullary hamartoma that was diagnosed by endoscopic biopsy and subsequently managed by endoscopic sphincterotomy.
Case Report A 69-year-old white woman was evaluated with a 3-year history of intermittent epigastric and right upper quadrant abdominal pain and jaundice in April 1984. In February 1982, she had been admitted to another hospital with similar symptoms. Physical examination showed mild jaundice. Laboratory studies showed white blood cell count of 9,600 mm3/L, hemoglobin 13 g/L, bilirubin 3 mg/dL {51.3 p.mol/L} [normal 0.2-1 mg/dL (3.4-17.1 p.mol/L}], alkaline phosphatase 316 U/L {normal 30-115 U/L}, SGOT 60 U/L (normal 5-40 U/L), and SGPT 70 U/L (normal 4---45 U/L}. Abdominal ultrasound examination and CT scan showed dilatation of the bile duct and a normal gallbladder. Upper gastrointestinal contrast study and barium enema x-ray examination were normal.
Despite the normal studies, a cholecystectomy was performed because of strong clinical suspicion of gallbladder disease and no stones were seen. Intraoperative cholangiogram showed dilatation of the bile duct; however, the common bile duct was not explored. Following the operatiori, the patient continued to experience intermittent episodes of severe epigastric and right upper quadrant abdominal pain often precipitated by eating. There.was no history of fever, chills, or pruritus. Our findings were as follows: the patient was noted to be jaundiced and laboratory studies showed a mild cholestatic pattern similar to that of her previous studies. Repeat ultrasound and CT-scan examinations of the abdomen again showed dilated biliary ducts. Endoscopic retrograde cholangiopancreatography examination was performed using an Olympus JIFIT20 lateral-viewing endoscope (Olympus Corporation, Lake Success, NY}. On inspection, the papilla appeared large and covered with granular, nonfriable mucosa. A superficial ulcer was present at its summit (Figure 1}. The common bile duct was cannulated and the cholangiogram showed a dilated common bile duct [15 ram}. The terminal end of the common bile duct was broad, bulbous, and clubbed (Figure 2). Contrast drainage into the duodenum was markedly delayed. Endoscopic biopsies using standard forceps showed papillary tissue with mild inflammation and ulceration. Two days later, a large particle biopsy was performed using a snare cautery following an endoscopic sphincterotomy to establish drainage. Histologically, the ampullary lesion was a sessile polyp representing a hamartoma. It was composed of a mixture of elements normally found in a papilla. The surface was focally villous in nature. The lamina propria contained glands, some lined by mucous-secreting cells and others by absorptive cells interspersed with goblet cells. Many of the glands were cystic. Thick bundles of smooth muscle coursed through the lamina propria in various directions, some perpendicular to the mucosal surface. The vascular supply consisted of Abbreviations used in this paper: CT, computed tomography; ERCP,endoscopic retrogradecholangiopancreatography. © 1991 by the American GastroenterologicalAssociation 0016-5085/91/$3.00
796 VENUET AL.
GASTROENTEROLOGYVol. 100, No. 3
4D Q
.,A
l~igure 1. Endoscopic picture showing enlarged papilla from hamartoma.
some of the common diagnostic and therapeutic problems often associated with ampullary tumors. The clinical presentation of ampullary tumors can be quite variable. Some patients may remain asymptomatic whereas others may have occult gastrointestinal bleeding or more typically upper abdominal pain and jaundice. The gallbladder is often enlarged. In fact, gallbladder disease and ampullary tumors may often coexist. In this situation, attention is usually given to the gallbladder often overlooking the ampullary disease. Thus, it is not surprising that in one series, 9 out of 12 patients with ampullary tumors underwent cholecystectomy 1 to 10 months before a definitive diagnosis of ampullary tumor (4). In our patient, abdominal pain and fluctuating jaundice were interpreted as symptoms of gallbladder disease culminating in cholecystectomy. On the other hand, biliary ductal dilatation in the absence of gallstones should have alerted the surgeon to exclude the possibility of an ampullary tumor. The ERCP examination has greatly enhanced the prospect of accurate diagnosis of ampullary tumors.
thin-walled, telangiectatic vessels. The stroma was infiltrated by lymphocytes (Figure 3). Examination with ERCP performed at 1 year and 4 years following the initial procedure showed marked improvement in drainage and a patent sphincterotomy opening. The patient has remained symptom-free with corresponding normalization of liver function tests (Table 1). Discussion
Ampullary tumors represent a rare clinical entity and many of them may be malignant. However, a variety of benign tumors such as adenoma, adenomyoma, leiomyoma, carcinoid, neurofibroma, granular Cell blastoma, and hemangioma have been reported to involve the ampulla (2,3). Although the malignant tumors have a relatively favorable prognosis with extensive surgical resection, such an approach is unnecessary in patients with benign lesions (4). Therefore, an accurate tissue diagnosis is essential for appropriate management of this category of patients. The widespread use of ERCP has enhanced the diagnosis of ampullary tumors. However, a distinction between benign and malignant tumors remains difficult on the basis of endoscopic appearance, radiographic findings, and in some instances even after endoscopic biopsy. Hamartoma involving the ampulla of Vater is extremely rare and only mentioned in isolated reports in the literature. In this report, the clinical features and endoscopic findings of ampullary hamartoma are elaborated. Additionally, this case is illustrative of
Figure 2. The cholangiogram shows a broad, clubbed, terminal common bile duct (arrow).
March 1991
DIAGNOSIS AND TREATMENT OF AMPULLARY HAMARTOMA
797
Figure 3. High-power photo of papillary lesion showing glands with a variety of lining cells. Some glands are lined by mucus-secreting cells, some by absorptive cells, and others by both absorptive and goblet cells. Bands of smooth muscle fibers are also seen extending to the mucosal surface (H&E; original magnification × 200).
Three interrelated steps are routinely followed during ERCP examination for diagnosing ampullary tumors: (a) inspection of the papilla, (b) biopsy of tissue, and (c) contrast radiography of ductal structures. On endoscopic inspection, the papilla is often enlarged, irregular, and lobulated. Whereas an exophytic ulcerated mass with spontaneous bleeding is indicative of malignancy, other endoscopic findings are rather nonspecific and may be observed in nonneoplastic conditions such as a choledochocele, impacted ampullary stone, or papillitis (3,5,6). The papilla in our patient was large, granular, and ulcerated--rather unusual "findings for a benign tumor. Thus, endoscopic findings are by no means pathognomonic for malignancy and tissue diagnosis is essential for accurate diagnosis. However, tissue samples obtained using a conventional biopsy forceps may have a low diagnostic yield. Large-particle biopsy using an electrocautery snare has been shown to increase the diagnostic accuracy as shown in our patient (7). This technique may occasionally be complicated by significant bleeding. However, we believe that the benefit of a correct tissue diagnosis outweighs this risk (8).
The most common radiographic finding in ampullary tumors is a dilated bile duct and/or pancreatic duct. An irregular filling defect of the distal common bile duct with delayed common duct drainage may also be seen in many patients. Our patient showed a dilated bile duct, delayed drainage, and a clubbed rather than tapered distal common bile duct. Therefore, it is important to obtain spot films of the distal choledochus and pancreatic duct during radiography to correctly identify ampullary tumors. Endoscopic therapy is usually restricted to highrisk patients with ampullary malignancy and patients with benign tumors. The commonly used endoscopic therapies include: (a) endoscopic sphincterotomy, (b) endoprosthesis, and (c) laser ablation (9). In our patient, endoscopic sphincterotomy resulted in complete resolution of symptoms and normalization of liver function tests. Because the malignant potential is negligible, endoscopic sphincterotomy to establish drainage is justifiable in ampullary hamartoma
(10).
References Table 1. Laboratoor Values and Biliary Drainage Time
Bilirubin Alkaline phosphatase SGPT Contrast drainage time
Before sphincterotomy
At 1-year follow-up
3 mg/dL (51.3 p.mol/L) 316 UfL 70 U/L 60 minutes
0.4 mg/dL (6.84 p.moEL) 96 UfL 26 U/L 10 minutes
1. Roe M, Greenough WG. Marked hypervascularity and arteriovenous shunting in acute pancreatitis. Radiology 1974;113:4748. 2. Huibregtse K, Tytgat GNJ. Carcinoma of the ampulla of Vater: the endoscopic approach. Endoscopy 1988;20:223-226. 3. Leese T, Neoptolemos JP, Wert KP, Talbot IC, Cart-Locke DL. Tumors and pseudo tumors of the region of the ampulla of Vater: an endoscopic, clinical and pathological study. Gut 1986;27:1186-1192.
798 VENUET AL.
4. Knox RA, Kingston RD. Carcinoma of ampulla of Vater. Br J Surg 1986;73:72-73. 5. Hayes DH, Bolton JS, Willis GW, Bowen JC. Carcinoma of ampulla of Vater. Ann Surg 1987;206(5):572-577. 6. Venu RP, Geenen JE, Hogan WJ, Dodds WJ, Wilson SW, Stewart ET, Soergel KH. Role of endoscopic retrograde cholangiopancreatography in the diagnosis and treatment of choledochocele. Gastroenterology 1984;87:1144-1149. 7. Safrany L. Duodenoscopy and biopsy. In: Classen M, Geenen J, Kawai K, eds. The papilla Vateri and its diseases. New York: Verlag, Gerhard, Witzstrock. Baden-Baden, Koln, 1979:66-72. 8. Ponchon T, Berger F, Chavaillon A, Bory R, Lambert R:
GASTROENTEROLOGYVol. 100, No. 3
Contribution of endoscopy to diagnosis and treatment of tumors of the ampulla of Vater. Cancer 1989;64(1):161-167. 9. Venu RP, Geenen JE. Diagnosis and treatment of diseases of the papilla. Clin Gastroentero11986;15:439-458. 10. Dekker A, TenKate FJW, Terpstra OT. Cholangiocarcinoma associated with multiple bile duct hamartomas of the liver. Dig Dis Sci 1989;39:952-958.
Received March 5, 1990. Accepted July 26, 1990. Address requests for reprints to: J. E. Geenen, M.D., 1333 College Avenue, Racine, Wisconsin 53403.
CASE REPORTS
Ampullary Hamartoma: Endoscopic Diagnosis and Treatment RAMA P. VENU, PETER ROLNY, J. E. GEENEN, W. J. HOGAN, and RICHARD A. KOMOROWSKI Department of Gastroenterology and Pathology, Medical College of Wisconsin, Milwaukee, Wisconsin, and Digestive Disease Center, St. Luke's Hospital, Racine, Wisconsin
This study shows a patient who presented with intermittent biliary tract obstruction caused by ampullary hamartoma. Endoscopic retrograde cholangi o p a n c r e a t o g r a p h y showed a large ulcerated papilla and dilated biliary ducts. Tissue diagnosis was established by a large particle biopsy obtained with a snare. The patient underwent a successful endoscopic sphincterotomy and has remained symptom free for 4 years.
A
wide variety of neoplastic lesions may involve the ampulla of Vater (1,2}. Conventional imaging techniques such as upper gastrointestinal roentgenology, ultrasonography, or computed tomography (CT} scans often do not identify these tumors. Endoscopic
retrograde cholangiopancreatography (ERCP) has become an indispensable tool in the diagnosis of ampullary tumors as i t allows direct visualization of the t u m o r as.well as tissue biopsy. In selective instances: ERCP can also be useful in therapeutic intervention. This s t u d y shows the first case of ampullary hamartoma that was diagnosed by endoscopic biopsy and subsequently managed by endoscopic sphincterotomy.
Case Report A 69-year-old white woman was evaluated with a 3-year history of intermittent epigastric and right upper quadrant abdominal pain and jaundice in April 1984. In February 1982, she had been admitted to another hospital with similar symptoms. Physical examination showed mild jaundice. Laboratory studies showed white blood cell count of 9,600 mm3/L, hemoglobin 13 g/L, bilirubin 3 mg/dL {51.3 p.mol/L} [normal 0.2-1 mg/dL (3.4-17.1 p.mol/L}], alkaline phosphatase 316 U/L {normal 30-115 U/L}, SGOT 60 U/L (normal 5-40 U/L), and SGPT 70 U/L (normal 4---45 U/L}. Abdominal ultrasound examination and CT scan showed dilatation of the bile duct and a normal gallbladder. Upper gastrointestinal contrast study and barium enema x-ray examination were normal.
Despite the normal studies, a cholecystectomy was performed because of strong clinical suspicion of gallbladder disease and no stones were seen. Intraoperative cholangiogram showed dilatation of the bile duct; however, the common bile duct was not explored. Following the operatiori, the patient continued to experience intermittent episodes of severe epigastric and right upper quadrant abdominal pain often precipitated by eating. There.was no history of fever, chills, or pruritus. Our findings were as follows: the patient was noted to be jaundiced and laboratory studies showed a mild cholestatic pattern similar to that of her previous studies. Repeat ultrasound and CT-scan examinations of the abdomen again showed dilated biliary ducts. Endoscopic retrograde cholangiopancreatography examination was performed using an Olympus JIFIT20 lateral-viewing endoscope (Olympus Corporation, Lake Success, NY}. On inspection, the papilla appeared large and covered with granular, nonfriable mucosa. A superficial ulcer was present at its summit (Figure 1}. The common bile duct was cannulated and the cholangiogram showed a dilated common bile duct [15 ram}. The terminal end of the common bile duct was broad, bulbous, and clubbed (Figure 2). Contrast drainage into the duodenum was markedly delayed. Endoscopic biopsies using standard forceps showed papillary tissue with mild inflammation and ulceration. Two days later, a large particle biopsy was performed using a snare cautery following an endoscopic sphincterotomy to establish drainage. Histologically, the ampullary lesion was a sessile polyp representing a hamartoma. It was composed of a mixture of elements normally found in a papilla. The surface was focally villous in nature. The lamina propria contained glands, some lined by mucous-secreting cells and others by absorptive cells interspersed with goblet cells. Many of the glands were cystic. Thick bundles of smooth muscle coursed through the lamina propria in various directions, some perpendicular to the mucosal surface. The vascular supply consisted of Abbreviations used in this paper: CT, computed tomography; ERCP,endoscopic retrogradecholangiopancreatography. © 1991 by the American GastroenterologicalAssociation 0016-5085/91/$3.00
796 VENUET AL.
GASTROENTEROLOGYVol. 100, No. 3
4D Q
.,A
l~igure 1. Endoscopic picture showing enlarged papilla from hamartoma.
some of the common diagnostic and therapeutic problems often associated with ampullary tumors. The clinical presentation of ampullary tumors can be quite variable. Some patients may remain asymptomatic whereas others may have occult gastrointestinal bleeding or more typically upper abdominal pain and jaundice. The gallbladder is often enlarged. In fact, gallbladder disease and ampullary tumors may often coexist. In this situation, attention is usually given to the gallbladder often overlooking the ampullary disease. Thus, it is not surprising that in one series, 9 out of 12 patients with ampullary tumors underwent cholecystectomy 1 to 10 months before a definitive diagnosis of ampullary tumor (4). In our patient, abdominal pain and fluctuating jaundice were interpreted as symptoms of gallbladder disease culminating in cholecystectomy. On the other hand, biliary ductal dilatation in the absence of gallstones should have alerted the surgeon to exclude the possibility of an ampullary tumor. The ERCP examination has greatly enhanced the prospect of accurate diagnosis of ampullary tumors.
thin-walled, telangiectatic vessels. The stroma was infiltrated by lymphocytes (Figure 3). Examination with ERCP performed at 1 year and 4 years following the initial procedure showed marked improvement in drainage and a patent sphincterotomy opening. The patient has remained symptom-free with corresponding normalization of liver function tests (Table 1). Discussion
Ampullary tumors represent a rare clinical entity and many of them may be malignant. However, a variety of benign tumors such as adenoma, adenomyoma, leiomyoma, carcinoid, neurofibroma, granular Cell blastoma, and hemangioma have been reported to involve the ampulla (2,3). Although the malignant tumors have a relatively favorable prognosis with extensive surgical resection, such an approach is unnecessary in patients with benign lesions (4). Therefore, an accurate tissue diagnosis is essential for appropriate management of this category of patients. The widespread use of ERCP has enhanced the diagnosis of ampullary tumors. However, a distinction between benign and malignant tumors remains difficult on the basis of endoscopic appearance, radiographic findings, and in some instances even after endoscopic biopsy. Hamartoma involving the ampulla of Vater is extremely rare and only mentioned in isolated reports in the literature. In this report, the clinical features and endoscopic findings of ampullary hamartoma are elaborated. Additionally, this case is illustrative of
Figure 2. The cholangiogram shows a broad, clubbed, terminal common bile duct (arrow).
March 1991
DIAGNOSIS AND TREATMENT OF AMPULLARY HAMARTOMA
797
Figure 3. High-power photo of papillary lesion showing glands with a variety of lining cells. Some glands are lined by mucus-secreting cells, some by absorptive cells, and others by both absorptive and goblet cells. Bands of smooth muscle fibers are also seen extending to the mucosal surface (H&E; original magnification × 200).
Three interrelated steps are routinely followed during ERCP examination for diagnosing ampullary tumors: (a) inspection of the papilla, (b) biopsy of tissue, and (c) contrast radiography of ductal structures. On endoscopic inspection, the papilla is often enlarged, irregular, and lobulated. Whereas an exophytic ulcerated mass with spontaneous bleeding is indicative of malignancy, other endoscopic findings are rather nonspecific and may be observed in nonneoplastic conditions such as a choledochocele, impacted ampullary stone, or papillitis (3,5,6). The papilla in our patient was large, granular, and ulcerated--rather unusual "findings for a benign tumor. Thus, endoscopic findings are by no means pathognomonic for malignancy and tissue diagnosis is essential for accurate diagnosis. However, tissue samples obtained using a conventional biopsy forceps may have a low diagnostic yield. Large-particle biopsy using an electrocautery snare has been shown to increase the diagnostic accuracy as shown in our patient (7). This technique may occasionally be complicated by significant bleeding. However, we believe that the benefit of a correct tissue diagnosis outweighs this risk (8).
The most common radiographic finding in ampullary tumors is a dilated bile duct and/or pancreatic duct. An irregular filling defect of the distal common bile duct with delayed common duct drainage may also be seen in many patients. Our patient showed a dilated bile duct, delayed drainage, and a clubbed rather than tapered distal common bile duct. Therefore, it is important to obtain spot films of the distal choledochus and pancreatic duct during radiography to correctly identify ampullary tumors. Endoscopic therapy is usually restricted to highrisk patients with ampullary malignancy and patients with benign tumors. The commonly used endoscopic therapies include: (a) endoscopic sphincterotomy, (b) endoprosthesis, and (c) laser ablation (9). In our patient, endoscopic sphincterotomy resulted in complete resolution of symptoms and normalization of liver function tests. Because the malignant potential is negligible, endoscopic sphincterotomy to establish drainage is justifiable in ampullary hamartoma
(10).
References Table 1. Laboratoor Values and Biliary Drainage Time
Bilirubin Alkaline phosphatase SGPT Contrast drainage time
Before sphincterotomy
At 1-year follow-up
3 mg/dL (51.3 p.mol/L) 316 UfL 70 U/L 60 minutes
0.4 mg/dL (6.84 p.moEL) 96 UfL 26 U/L 10 minutes
1. Roe M, Greenough WG. Marked hypervascularity and arteriovenous shunting in acute pancreatitis. Radiology 1974;113:4748. 2. Huibregtse K, Tytgat GNJ. Carcinoma of the ampulla of Vater: the endoscopic approach. Endoscopy 1988;20:223-226. 3. Leese T, Neoptolemos JP, Wert KP, Talbot IC, Cart-Locke DL. Tumors and pseudo tumors of the region of the ampulla of Vater: an endoscopic, clinical and pathological study. Gut 1986;27:1186-1192.
798 VENUET AL.
4. Knox RA, Kingston RD. Carcinoma of ampulla of Vater. Br J Surg 1986;73:72-73. 5. Hayes DH, Bolton JS, Willis GW, Bowen JC. Carcinoma of ampulla of Vater. Ann Surg 1987;206(5):572-577. 6. Venu RP, Geenen JE, Hogan WJ, Dodds WJ, Wilson SW, Stewart ET, Soergel KH. Role of endoscopic retrograde cholangiopancreatography in the diagnosis and treatment of choledochocele. Gastroenterology 1984;87:1144-1149. 7. Safrany L. Duodenoscopy and biopsy. In: Classen M, Geenen J, Kawai K, eds. The papilla Vateri and its diseases. New York: Verlag, Gerhard, Witzstrock. Baden-Baden, Koln, 1979:66-72. 8. Ponchon T, Berger F, Chavaillon A, Bory R, Lambert R:
GASTROENTEROLOGYVol. 100, No. 3
Contribution of endoscopy to diagnosis and treatment of tumors of the ampulla of Vater. Cancer 1989;64(1):161-167. 9. Venu RP, Geenen JE. Diagnosis and treatment of diseases of the papilla. Clin Gastroentero11986;15:439-458. 10. Dekker A, TenKate FJW, Terpstra OT. Cholangiocarcinoma associated with multiple bile duct hamartomas of the liver. Dig Dis Sci 1989;39:952-958.
Received March 5, 1990. Accepted July 26, 1990. Address requests for reprints to: J. E. Geenen, M.D., 1333 College Avenue, Racine, Wisconsin 53403.