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1217
Case
Report
..
..:::.‘.
Obstructing Villous of the Bile Ducts
Adenoma
and Papillary
David L. Harshfield,1 Steven K. Teplick,1 Mike Stanton,2 Kishore Tunuguntla,3 Raymond C. Read2
Benign mucosal tumors of the bile ducts are extremely uncommon [1], and the cholangiographic appearance of bile duct villous adenomas and papillary adenomas has not been described
in the
radiologic
who had obstructive
literature.
jaundice
noma in the common diffuse ductal papillary
We
caused
describe
bile duct. In addition, adenomatosis. The
lowed up for 1 4 years established.
before
a patient
by a large villous adethe patient had patient was fol-
a definitive
diagnosis
was
Case Report man originally
on a chest
radiograph
presented
in 1 975 with chest pain. and an upper gastrointestinal series
were normal. The patient had several normal gastrointestinal studies for nonspecific abdominal complaints in 1978 and 1980. In January 1982, he returned with persistent pain in the right upper quadrant. visualization and results of a sonogram were normal. In April 1 982, the patient presented with acute subsequently underwent exploratory laparotomy.
An oral cholecystogram
was
performed,
revealed
presumably
langiogram
showed
poor
because
were due to nonalcoholic
pancreatitis.
the acute
of the gallbladder, abdominal pain and A cholecystectomy abdominal
An intraoperative
symptoms
T-tube cho-
a filling defect of the distal common bile of Vater. This abnormality persisted on follow-up cholangiograms, and 1 month after the cholecystectomy, the patient underwent a common bile duct exploration, which revealed a polypoid lesion in the distal common bile duct (Fig. 1 A). Multiple biopsy samples were obtained, and the histologic diagnosis was duct
C. Diner,1 and
papillary adenoma. A T-tube was reinserted and choledochojejunostomy was performed. The decision to perform a definitive operation such as the Whipple procedure was deferred, because of the presumed benign nature of papillary adenomas in the common bile duct. In 1 989, the patient presented with pain in the right lower quadrant and midepigastnc area. An upper gastrointestinal series showed enlargement of the papilla ofVater(Fig. 1 B). An ERCP was performed, which showed a mass in the common bile duct causing partial obstruction (Fig. 1 C). A CT scan showed enlargement of the head of the pancreas, but was otherwise normal. At surgery, the common bile duct was opened and biopsy samples were obtained, which revealed a benign villous adenoma. An attempt biopsies showed tumor in the resented margins. Finally, a hepaticojejunostomy was performed. In addition to the villous adenoma, the patient had diffuse papillary adenomatosis extending into the right
and left intrahepatic revealed chronic his postoperative
ducts.
Biopsies
of the head of the pancreas
pancreatitis. The patient recovered course has remained uneventful.
uneventfully,
and
Discussion This case is unusual not only because of the rarity of bile duct obstruction from a benign tumor, but also because it is the first reported case documenting the progression of a
single benign bile duct tumor to multiple benign tumors of different histologic types. The initial diagnosis was a papillary adenoma of the distal common bile duct, which, during 7 years,
progressed
to diffuse
papillary
adenomatosis
plus the
20, 1989: accepted after revision January 3. 1990. of Radiology, University of Arkansas for Meaical Sciences, 4301 W. Markham St., Little Rock, AR 72205. Address reprint requests to S. K. Teplick
November
1 Department at Slot 556. 2
either
of the papilla
or spasm
Received
Wilma
was made to take biopsy samples proximal to the tumor, but repeated
A 78-year-old Findings
Adenomatosis
Department
3Department AJR 154:1217-1218,
of Surgery. University of Arkansas for Medical Sciences, 4301 W. Markham St., Little Rock, AR 72205. of Gastroenterology, June
University of Arkansas for Medical Sciences,
1990 0361 -803X/90/1
546-1217
4301 W. Markham St., Little Rock,
© American Roentgen Ray Society
AR 72205.
HARSHFIELD
Downloaded from www.ajronline.org by 117.255.255.205 on 10/15/15 from IP address 117.255.255.205. Copyright ARRS. For personal use only; all rights reserved
1218
ET AL.
AJR:154, June 1990
Fig. 1.-A, cholangiogram reveals a polypoid filling defect in common bile duct just proximal to papilla of Vater (arrows). B, Upper gastrointestinal series shows a mass in region of papilla of Vater (arrows). C, ERCP reveals common bile duct to be diffusely abnormal from papilla to just proximal to level of bifurcation of right and left intrahepatic ducts (arrows), where there is an abrupt transition (arrowhead). In involved area, multiple polypoid filling defects with diffuse mucosal abnormality represent villous adenoma. More proximally located area of diffuse papillary adenomatosis is not shown.
formation of a large villous adenoma, causing obstruction of the common bile duct. In 1 967, Edmondson presented a classification of benign tumors of the bile ducts in which all benign epithelial tumors are classified as adenomas [2]. Distinctions were made according to the gross configuration of the tumors. The tumors were divided into papillary adenomas, pedunculated adenomas, and sessile adenomas; this classification replaced an earlier classification of papillomas, polyps, and adenomas, respectively [3]. Villous adenomas are thus classified as frondlike
sessile
adenomas,
and
papillomas
are classified
as pap-
illary adenomas. Histologically, the most common benign tumors are adenomas [2]. These almost always arise in the common bile duct, with only a single case arising in the intrahepatic fibroma,
ducts lipoma,
[4]. neuroma,
Other
histologic
granuloma,
types melanoma,
reported and
are carci-
noid [5]. The frequency of benign epithelial tumors in the bile ducts is not known, but is much lower than that in the gallbladder (these tumors occur in the gallbladder in about 1 % of the population), even though the gallbladder and bile ducts are lined with the same mucosal epithelium. In a few reported cases, benign bile duct tumors caused symptoms, but most were incidentally discovered during treatment for other biliary problems such as gallstones or cholecystitis. In our patient, the tumors themselves caused intermittent symptoms, which eventually led to definitive surgery. These benign lesions are thought to remain dormant for long periods and, in their early stages of the disease, cause vague and variable symptoms [5]. In other reported cases, the duration of symptoms referable to the gastrointestinal tract or biliary system varied from several weeks to 12 years. Usually, untreated patients eventually present with progressive biliary obstruction. Rarely these tumors cause acute severe biliary colic. Several investigators think that any adenoma in the gastrointestinal tract is premalignant [3, 5]. Although only a few
cases of villous adenomas in the common bile duct have been reported, most either were malignant or resulted in the patient’s death because of their aggressive behavior [6, 7]. The serious prognosis of this tumor warrants aggressive treatment. Unfortunately, partial lobectomy, curettage, or debulking procedures have not had much success [6-8]. Some
success
was reported
chemotherapy
with
in one patient who was given systemic BCNU
urea) late in the course
(1 ,3-bis[2-choroethyl]-1
of the disease
-nitroso-
[7]. This resulted
in
regression of tumor mass, although the patient eventually died. A technique described by Smith [8] indicates that direct instillation of chemotherapeutic agent into the biliary drainage catheter can be even more effective. In our patient, the entire tumor was not excised. There might be residual malignant foci or eventual proliferation of the tumors causing ductal obstruction. However, for a 78patient
without
ation performed procedure.
year-old
was
a diagnosis
considered
of malignancy,
preferable
to
the
oper-
a Whipple
REFERENCES
1 . Saxe J, Lucas C, Ledgerwood AM, Sugawa C. Villous adenoma of the common bile duct. Arch Surg 1988:123:96-97 2. Edmondson HA. Tumours of the gallbladder and extrahepatic bile ducts. Washington. DC: Armed Forces Institute of Pathology, 1967 3. Hulten J, Johannson H, Olding L. Adenomas of the gallbladder and extrahepatic bile ducts. Acta Chir Scand 1970;136:203-207 4. McIntosh AM, Gilles AD. Two tumours of the extrahepatic bile ducts. Med J Aust 1940;1 :268-269 5. Chu PR. Benign neoplasms of the extrahepatic biliary ducts. Arch Pathol Lab Med 1950;50:84-97 6. Neumann AD, LiVolsi VA, Rosenthal NS, Burrell M, Ball TJ. Adenocarcinoma in biliary papillomatosis. Gastroenterology 1976;70:779-782 7. Madden JJ, Smith GW. Multiple biliary papillomatosis. Cancer 1974;34: 131 6-1 320 8. Smith
R. Hepaticojejunostomy
1964:51:186-194
with transhepatic intubation. Br J Surg
1217
Case
Report
..
..:::.‘.
Obstructing Villous of the Bile Ducts
Adenoma
and Papillary
David L. Harshfield,1 Steven K. Teplick,1 Mike Stanton,2 Kishore Tunuguntla,3 Raymond C. Read2
Benign mucosal tumors of the bile ducts are extremely uncommon [1], and the cholangiographic appearance of bile duct villous adenomas and papillary adenomas has not been described
in the
radiologic
who had obstructive
literature.
jaundice
noma in the common diffuse ductal papillary
We
caused
describe
bile duct. In addition, adenomatosis. The
lowed up for 1 4 years established.
before
a patient
by a large villous adethe patient had patient was fol-
a definitive
diagnosis
was
Case Report man originally
on a chest
radiograph
presented
in 1 975 with chest pain. and an upper gastrointestinal series
were normal. The patient had several normal gastrointestinal studies for nonspecific abdominal complaints in 1978 and 1980. In January 1982, he returned with persistent pain in the right upper quadrant. visualization and results of a sonogram were normal. In April 1 982, the patient presented with acute subsequently underwent exploratory laparotomy.
An oral cholecystogram
was
performed,
revealed
presumably
langiogram
showed
poor
because
were due to nonalcoholic
pancreatitis.
the acute
of the gallbladder, abdominal pain and A cholecystectomy abdominal
An intraoperative
symptoms
T-tube cho-
a filling defect of the distal common bile of Vater. This abnormality persisted on follow-up cholangiograms, and 1 month after the cholecystectomy, the patient underwent a common bile duct exploration, which revealed a polypoid lesion in the distal common bile duct (Fig. 1 A). Multiple biopsy samples were obtained, and the histologic diagnosis was duct
C. Diner,1 and
papillary adenoma. A T-tube was reinserted and choledochojejunostomy was performed. The decision to perform a definitive operation such as the Whipple procedure was deferred, because of the presumed benign nature of papillary adenomas in the common bile duct. In 1 989, the patient presented with pain in the right lower quadrant and midepigastnc area. An upper gastrointestinal series showed enlargement of the papilla ofVater(Fig. 1 B). An ERCP was performed, which showed a mass in the common bile duct causing partial obstruction (Fig. 1 C). A CT scan showed enlargement of the head of the pancreas, but was otherwise normal. At surgery, the common bile duct was opened and biopsy samples were obtained, which revealed a benign villous adenoma. An attempt biopsies showed tumor in the resented margins. Finally, a hepaticojejunostomy was performed. In addition to the villous adenoma, the patient had diffuse papillary adenomatosis extending into the right
and left intrahepatic revealed chronic his postoperative
ducts.
Biopsies
of the head of the pancreas
pancreatitis. The patient recovered course has remained uneventful.
uneventfully,
and
Discussion This case is unusual not only because of the rarity of bile duct obstruction from a benign tumor, but also because it is the first reported case documenting the progression of a
single benign bile duct tumor to multiple benign tumors of different histologic types. The initial diagnosis was a papillary adenoma of the distal common bile duct, which, during 7 years,
progressed
to diffuse
papillary
adenomatosis
plus the
20, 1989: accepted after revision January 3. 1990. of Radiology, University of Arkansas for Meaical Sciences, 4301 W. Markham St., Little Rock, AR 72205. Address reprint requests to S. K. Teplick
November
1 Department at Slot 556. 2
either
of the papilla
or spasm
Received
Wilma
was made to take biopsy samples proximal to the tumor, but repeated
A 78-year-old Findings
Adenomatosis
Department
3Department AJR 154:1217-1218,
of Surgery. University of Arkansas for Medical Sciences, 4301 W. Markham St., Little Rock, AR 72205. of Gastroenterology, June
University of Arkansas for Medical Sciences,
1990 0361 -803X/90/1
546-1217
4301 W. Markham St., Little Rock,
© American Roentgen Ray Society
AR 72205.
HARSHFIELD
Downloaded from www.ajronline.org by 117.255.255.205 on 10/15/15 from IP address 117.255.255.205. Copyright ARRS. For personal use only; all rights reserved
1218
ET AL.
AJR:154, June 1990
Fig. 1.-A, cholangiogram reveals a polypoid filling defect in common bile duct just proximal to papilla of Vater (arrows). B, Upper gastrointestinal series shows a mass in region of papilla of Vater (arrows). C, ERCP reveals common bile duct to be diffusely abnormal from papilla to just proximal to level of bifurcation of right and left intrahepatic ducts (arrows), where there is an abrupt transition (arrowhead). In involved area, multiple polypoid filling defects with diffuse mucosal abnormality represent villous adenoma. More proximally located area of diffuse papillary adenomatosis is not shown.
formation of a large villous adenoma, causing obstruction of the common bile duct. In 1 967, Edmondson presented a classification of benign tumors of the bile ducts in which all benign epithelial tumors are classified as adenomas [2]. Distinctions were made according to the gross configuration of the tumors. The tumors were divided into papillary adenomas, pedunculated adenomas, and sessile adenomas; this classification replaced an earlier classification of papillomas, polyps, and adenomas, respectively [3]. Villous adenomas are thus classified as frondlike
sessile
adenomas,
and
papillomas
are classified
as pap-
illary adenomas. Histologically, the most common benign tumors are adenomas [2]. These almost always arise in the common bile duct, with only a single case arising in the intrahepatic fibroma,
ducts lipoma,
[4]. neuroma,
Other
histologic
granuloma,
types melanoma,
reported and
are carci-
noid [5]. The frequency of benign epithelial tumors in the bile ducts is not known, but is much lower than that in the gallbladder (these tumors occur in the gallbladder in about 1 % of the population), even though the gallbladder and bile ducts are lined with the same mucosal epithelium. In a few reported cases, benign bile duct tumors caused symptoms, but most were incidentally discovered during treatment for other biliary problems such as gallstones or cholecystitis. In our patient, the tumors themselves caused intermittent symptoms, which eventually led to definitive surgery. These benign lesions are thought to remain dormant for long periods and, in their early stages of the disease, cause vague and variable symptoms [5]. In other reported cases, the duration of symptoms referable to the gastrointestinal tract or biliary system varied from several weeks to 12 years. Usually, untreated patients eventually present with progressive biliary obstruction. Rarely these tumors cause acute severe biliary colic. Several investigators think that any adenoma in the gastrointestinal tract is premalignant [3, 5]. Although only a few
cases of villous adenomas in the common bile duct have been reported, most either were malignant or resulted in the patient’s death because of their aggressive behavior [6, 7]. The serious prognosis of this tumor warrants aggressive treatment. Unfortunately, partial lobectomy, curettage, or debulking procedures have not had much success [6-8]. Some
success
was reported
chemotherapy
with
in one patient who was given systemic BCNU
urea) late in the course
(1 ,3-bis[2-choroethyl]-1
of the disease
-nitroso-
[7]. This resulted
in
regression of tumor mass, although the patient eventually died. A technique described by Smith [8] indicates that direct instillation of chemotherapeutic agent into the biliary drainage catheter can be even more effective. In our patient, the entire tumor was not excised. There might be residual malignant foci or eventual proliferation of the tumors causing ductal obstruction. However, for a 78patient
without
ation performed procedure.
year-old
was
a diagnosis
considered
of malignancy,
preferable
to
the
oper-
a Whipple
REFERENCES
1 . Saxe J, Lucas C, Ledgerwood AM, Sugawa C. Villous adenoma of the common bile duct. Arch Surg 1988:123:96-97 2. Edmondson HA. Tumours of the gallbladder and extrahepatic bile ducts. Washington. DC: Armed Forces Institute of Pathology, 1967 3. Hulten J, Johannson H, Olding L. Adenomas of the gallbladder and extrahepatic bile ducts. Acta Chir Scand 1970;136:203-207 4. McIntosh AM, Gilles AD. Two tumours of the extrahepatic bile ducts. Med J Aust 1940;1 :268-269 5. Chu PR. Benign neoplasms of the extrahepatic biliary ducts. Arch Pathol Lab Med 1950;50:84-97 6. Neumann AD, LiVolsi VA, Rosenthal NS, Burrell M, Ball TJ. Adenocarcinoma in biliary papillomatosis. Gastroenterology 1976;70:779-782 7. Madden JJ, Smith GW. Multiple biliary papillomatosis. Cancer 1974;34: 131 6-1 320 8. Smith
R. Hepaticojejunostomy
1964:51:186-194
with transhepatic intubation. Br J Surg
