Gastrointest Radiol 15:53-57 (1990)
Gastrointestinal
Radiology 9 Springer-VerlagNewYorkInc. 1990
Lymphoma in the Wall of the Bile Ducts: Radiologic Imaging V. Marie T a r t a r a n d Dennis M. Balfe Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, Missouri, USA
Abstract. E x t r a n o d a l l y m p h o m a is n o t u n c o m m o n ; however, l y m p h o m a t o u s i n v o l v e m e n t o f the wall o f the bile duct is rare, with only a few case reports available. Three cases were i m a g e d with c o m p u t e d t o m o g r a p h y (CT) a n d direct cholangio g r a p h y at o u r institution. In one, H o d g k i n disease recurred in the duct wall, p r o d u c i n g a r a d i o g r a p h i c p a t t e r n indistinguishable f r o m sclerosing c h o l a n gitis. In another, central sclerosis on c h o l a n g i o g r a p h y was associated with a separate liver mass identified by CT. This presentation o f n o n - H o d g k i n l y m p h o m a m i m i c k e d c h o l a n g i o c a r c i n o m a . The third patient h a d multifocal, diffuse histiocytic l y m p h o m a arising in the gallbladder a n d cystic duct, as well as in the kidneys a n d pancreas. Alt h o u g h the c o n d i t i o n is unusual, the diagnosis o f l y m p h o m a in the bile duct wall should be considered, particularly when the c h o l a n g i o g r a p h i c picture o f diffuse central sclerosis is associated with little or n o observable mass o n CT.
tion o f the biliary tract, which f o r m the basis o f this report.
Materials and Methods Review of the pathology files at Barnes Hospital revealed 3 pathologically documented cases of lymphoma originating in or directly invading the wall of the gallbladder or bile ducts. All 3 had had radiologic imaging procedures consisting of computed tomography (CT) and transhepatic cholangiography (PTC). Computed tomographic scans were performed on a Siemens DR-H unit (Siemens Medical Systems, Inc., Iselin, N J). In 2 patients high-volume intravenous contrast material combined with rapid table incrementation was employed. In both of these, 150 ml of 60% meglumine diatrozoate was administered in 2 min using a mechanical injector. In the third patient, renal failure precluded our use of intravenous contrast material. Transhepatic cholangiography was accomplished in the standard fashion in all cases using a 22 gauge Chiba needle.
Results
Key words: L y m p h o m a , e x t r a n o d a l - Bile duct ob-
The clinical presentations, radiologic observations, a n d p a t h o l o g i c findings are s u m m a r i z e d below.
struction - C h o l a n g i o c a r c i n o m a , differential diagnosis.
Case 1
Patients with l y m p h o m a m a y develop jaundice for a variety o f reasons [1, 2]; diffuse l y m p h o m a t o u s liver involvement, cholestasis due to n o d a l enlargem e n t in the p o r t a hepatis, a n d hepatocellular disease related to the toxic effects o f c h e m o t h e r a p y are the m o s t c o m m o n causes. Rarely, l y m p h o m a m a y directly infiltrate the wall o f the biliary tree, p r o d u c i n g biliary obstruction. W e e n c o u n t e r e d 3 such cases o f e x t r a n o d a l l y m p h o m a t o u s infiltraAddress reprint requests to: Dennis M. Balfe, M.D., Mallinck-
rodt Institute of Radiology, Washington University School of Medicine, 510 South Kingshighway Boulevard, St. Louis, MO 63110, USA
A 35-year-old white male, RM, had a 15-year history of stage IV nodular sclerosing Hodgkin disease (pulmonary and cervical nodes and bone marrow involvement). His current admission was precipitated by a six-week history of increasing jaundice. A CT scan demonstrated intrahepatic bile duct dilatation. No mass was identified at the site of duct termination, and no portal adenopathy was detected (Fig. 1A). Percutaneous transhepatic cholangiography showed strictures of the right and left hepatic ducts at their confluence (Fig. 1B). Duct contour was irregular, but no masses were detected. Exploratory laparotomy revealed a 1-cm nodule in the right lobe of the liver, which was biopsied and found to be nodular sclerosing Hodgkin disease. The common bile duct was firm and irregular without extrinsic mass. Sections of the duct wall also revealed nodular sclerosing Hodgkin disease. A T-tube was implanted, and radiation therapy was subsequently performed. Multiple cholangiograms performed during the next 18 months documented relentless progression of the diffuse bili-
54
V.M. Tartar and D.M. Balfe: Lymphoma in the Bile Duct Wall
Fig. 1. Case 1. A 35-year-old white male with stage IV nodular sclerosing Hodgkin disease for 15 years, now with obstructive jaundice. A Upper abdominal CT scan at the level of the porta shows intrahepatic biliary ductal dilatation. The region of the common hepatic duct (arrow) demonstrates no adenopathy. B A cholangiogram, performed perioperatively via a T-tube, demonstrates extensive stricturing of the common hepatic duct and adjacent segments of the right and left hepatic ducts (arrowheads).
ary strictures, with concomitant hepatic decompensation. The patient died 18 months after icterus was first noted.
mission, and he remains clinically disease-free 14 months after his initial presentation.
Case 2
Case 3
A previously healthy 48-year-old black male, AC, presented for evaluation of painless jaundice. Laboratory evaluation revealed elevation of bilirubin and liver enzymes. A sonogram demonstrated biliary ductal dilatation with a solid mass within the common hepatic duct. An intravenous contrast-enhanced abdominal CT demonstrated bile duct dilatation with multiple low-attenuation regions within the left lobe of the liver, including the region of the porta hepatis (Fig. 2A-C). The findings were thought to represent eholangiocarcinoma. A transhepatic cholangiogram was performed to further evaluate the anatomy of the biliary tree and to assess the possibility of performing curative surgery. This showed a long irregular segmental narrowing of the entire common hepatic duct with extension into the right and left hepatic ducts (Fig. 2 D). Surgical exploration revealed a firm tumor in the hepatic hilus involving the common hepatic ducts, proximal common bile duct, and the surface of the left lobe of the liver. A left hepatic lobectomy and cholecystectomy was performed, and a Roux-en-Y choledochojejunostomy created. Pathologic examination of the resected specimen revealed malignant nonHodgkin lymphoma (Fig. 2 E and F). A bone marrow biopsy performed in the postoperative period showed no evidence of lymphomatous involvement. Six months after diagnosis, CT evaluation of the chest, abdomen, and pelvis demonstrated evidence of widespread systemic lymphoma, with extensive mediastinal, retroperitoneal, and mesenteric lymphadenopathy. Systemic chemotherapy induced a re-
A 69-year-old white female, GC, was transferred from an outside hospital for evaluation of jaundice, nausea, vomiting, and acute renal insufficiency. Initial evaluation one month prior to transfer revealed an elevated bilirubin and a positive Hepatitis A antibody. Three weeks later, she developed increasing icterus and had laboratory evidence of renal failure. A C T scan performed on the day of transfer demonstrated biliary ductal dilatation and a 3 cm mass in the region of the pancreatic head (Fig. 3A and B). Also noted were diffusely enlarged kidneys, ascites, and a right pleural effusion. A PTC demonstrated biliary ductal dilatation with an abrupt narrowing of the common bile duct in the region of the pancreas and another mass obstructing the origin of the cystic duct (Fig. 3 C). The patient became progressively hypotensive after placement of a drainage catheter, and emergency laparotomy was performed. At laparotomy, 5-7 units of blood and ascites were evacuated. The bleeding site was identified as a hepatic puncture site above the catheter location, presumably from the fine needle diagnostic puncture. Three days later, the patient was re-explored for recurrent bleeding. At that time, the gallbladder was noted to be distended, and a 2-3 cm mass was identified near the cystic duct. A frozen section of a biopsy of the gallbladder mass revealed a small cell neoplasm, consistent with lymphoma, and cholecystectomy was performed (Fig. 3 D). A mass in the head of the pancreas was noted, but no biopsy was performed.
Fig. 2. Case 2. A previously healthy black male with painless jaundice. A The CT scan through the liver demonstrates intrahepatic biliary ductal dilatation and irregularly marginated lowattenuation masses in the left lobe of the liver (arrows). B A CT scan 3 cm caudal shows a lower portion of the mass (arrow) and a separate mass (arrowheads) replacing the common hepatic duct. C A C T scan through the liver (performed 15 min after initial scans) shows persistent contrast enhancement of the previously low-attenuation left hepatic lobe mass (arrows). D Percutaneous transhepatic cholangiogram demonstrates intrahe-
patic biliary ductal dilatation proximal to strictures (arrowheads) narrowing the common hepatic duct and adjacent portions of the right and left intrahepatic ducts. E Histologic section through the common hepatic duct. Lymphomatous mass compresses adjacent liver and involves a large portal tract (center). Uninvolved portal tracts show features of obstruction (H & E stain; x40). F Microscopic section of the common bile duct wall, which is greatly expanded by lymphoid infiltrate. The ductal epithelium (upper left) is partially sloughed, but in most areas was well preserved (H & E stain; x 40).
56
Fig. 3. Case 3. A 69-year-old white female with acute jaundice and renal failure. A A C T image at the level of the gallbladder (GB), which contains high-attenuation material, demonstrates intra- and extrahepatic biliary ductal dilatation, with a soft tissue density mass (arrow) in the region of the cystic duct. The kidneys are diffusely enlarged. (RK, right kidney; LK, left kidney; CBD, common bile duct.) B A C T scan 4 c m caudal at the level of the uncinate process of the pancreas (P) shows a second soft tissue density mass (arrow) between the contrast-filled duodenum (D) and the pancreas (P). (GB, gallbladder; cbd, common bile duct; RK, right kidney;
V.M. Tartar and D.M. Balfe: Lymphoma in the Bile Duct Wall
LK, left kidney.) C Cholangiogram obtained during placement of a biliary drainage catheter shows marked biliary ductal dilatation proximal to a mass (arrowheads) at the distal common bile duct, with effacement of the descending-portion of the duodenum (thick arrows). The cystic duct is also abruptly truncated (thin arrow). A lamellated gallstone is seen (curved arrow). (D, duodenum) D Microscopic section of the gallbladder wall shows full thickness replacement of the wall by malignant large cell lymphoma. The mucosal villi are blunted, but the epithelium is intact (H & E stain; x 100).
V.M. Tartar and D.M. Balfe: Lymphoma in the Bile Duct Wall The patient's clinical condition deteriorated markedly in the postoperative period, and she died on the ninth postoperative day.
Discussion
Lymphoma, or its treatment, can produce jaundice in a variety of ways [1, 2], including compression of the common duct by involved nodes in the hepatoduodenal ligament or pancreatoduodenal chain, lymphomatous infiltration of the liver causing diffuse hepatocellular disease, or toxic side effects of the chemotherapeutic regimen producing hepatic failure. Direct infiltration of the wall of a biliary structure is quite uncommon; the literature contains only isolated case reports [3-7]. There are three clinical situations in which lymphomatous infiltration of the biliary system may occur: (a) as a solitary site of extranodal lymphoma, (b) as one of many sites in a systemic lymphoma, and (c) as a site of recurrence after therapy directed to systemic lymphoma. Our case material illustrates each of the three mechanisms mentioned above. Primary extranodal non-Hodgkin lymphoma arising in the bile duct is extremely rare, having been reported in only one other previous case, in which a patient presenting with acute obstructive jaundice was found to have diffuse lymphohistiocytic infiltration of the extrahepatic bile ducts [7]. Radiographically and clinically, this case and Case 2 have striking parallels. Cholangiograms in both cases showed a smooth, tapered stricture involving the common hepatic duct, suggesting sclerosing cholangitis or cholangiocarcinoma. Preoperative imaging and surgical exploration showed no extrahepatic foci of lymphoma, but in both cases, widespread systemic disease occurred within months after initial exploration. In our case, an intrahepatic lymphomatous nodule was also present. Its discovery on preoperative CT suggested direct liver invasion by cholangiocarcinoma; there were no CT features suggestive of lymphoma. There was a puzzling CT finding in this case: the hepatic tumor was a low-attenuation mass during the bolus phase of examination, but in the equilibrium phase, it exhibited persistent contrast enhancement compared with the normal liver (Fig. 2C). We have no explanation for this observation. Infiltration of the gallbladder by a systemic
57
lymphoma is uncommon, but it has been described in the clinical literature both in the setting of known disease [4] and as a previously unsuspected pathologic diagnosis made from a cholecystectomy specimen [5]. In our patient, the additional finding of diffuse enlargement and nodularity of the kidneys, with concomitant renal failure, allowed a correct preoperative diagnosis of lymphoma. Patients with a long-standing history of Hodgkin lymphoma often develop jaundice due to compression of biliary structures by portal lymph nodes. Infiltration of the duct wall is quite uncommon in the absence of nodal disease. Preoperative imaging in Case 1 suggested that nodal obstruction was n o t the cause, since high-resolution CT scans obtained through the hepatoduodenal ligament demonstrated no evidence of nodal masses. Cholangiography showed diffuse stricturing of intrahepatic and extrahepatic ducts, in a pattern that mimicked sclerosing cholangitis. The diagnosis of lymphomatous involvement of the biliary system is difficult by any nonoperative means. In a patient with known lymphoma, cholangiographic findings of smooth, tapered strictures combined with the absence of a portal mass on CT should suggest the diagnosis. In patients with no previous history, the radiographic differential diagnosis includes cholangiocarcinoma, and only histologic material can successfully differentiate the two. References 1. Bouroncle BA, Old JW Jr, Vasques AG: Pathogenesis of jaundice in Hodgkin's disease. Arch Int Med 110:872-883, 1962 2. Levitan R, Diamond HD, Craver LF: Jaundice in Hodgkin's disease. Am J Med 30: 99-111, 1961 3. Botha JB, Kahn LB : Primary lymphoma of the gallbladder: case report and review of the literature. S Afr Med J 48:1345-1348, 1974 4. Van Slyck EJ, Schuman BM: Lymphocytic lymphosarcoma of the gallbladder. Cancer 30: 810-816, 1972 5. Gillespie J J, Ayala AG, MacKay B, Silliman YE: Diagnosis of lymphoma from a choleeystectomy specimen: case report and review of the literature. South Med J 70: 353-354, 1977 6. Ochoa JI, Keene WR: Obstructive jaundice in Hodgkin's disease due to infiltration of the biliary ducts. J Fla Med Assoc 57:21-23, 1970 7. Nguyen GK: Primary extranodal non-Hodgkin's lymphoma of the extrahepatic bile ducts. Cancer 50: 2218-2222, 1982 Received: February 16, 1989; accepted: March 14, 1989
Gastrointestinal
Radiology 9 Springer-VerlagNewYorkInc. 1990
Lymphoma in the Wall of the Bile Ducts: Radiologic Imaging V. Marie T a r t a r a n d Dennis M. Balfe Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, Missouri, USA
Abstract. E x t r a n o d a l l y m p h o m a is n o t u n c o m m o n ; however, l y m p h o m a t o u s i n v o l v e m e n t o f the wall o f the bile duct is rare, with only a few case reports available. Three cases were i m a g e d with c o m p u t e d t o m o g r a p h y (CT) a n d direct cholangio g r a p h y at o u r institution. In one, H o d g k i n disease recurred in the duct wall, p r o d u c i n g a r a d i o g r a p h i c p a t t e r n indistinguishable f r o m sclerosing c h o l a n gitis. In another, central sclerosis on c h o l a n g i o g r a p h y was associated with a separate liver mass identified by CT. This presentation o f n o n - H o d g k i n l y m p h o m a m i m i c k e d c h o l a n g i o c a r c i n o m a . The third patient h a d multifocal, diffuse histiocytic l y m p h o m a arising in the gallbladder a n d cystic duct, as well as in the kidneys a n d pancreas. Alt h o u g h the c o n d i t i o n is unusual, the diagnosis o f l y m p h o m a in the bile duct wall should be considered, particularly when the c h o l a n g i o g r a p h i c picture o f diffuse central sclerosis is associated with little or n o observable mass o n CT.
tion o f the biliary tract, which f o r m the basis o f this report.
Materials and Methods Review of the pathology files at Barnes Hospital revealed 3 pathologically documented cases of lymphoma originating in or directly invading the wall of the gallbladder or bile ducts. All 3 had had radiologic imaging procedures consisting of computed tomography (CT) and transhepatic cholangiography (PTC). Computed tomographic scans were performed on a Siemens DR-H unit (Siemens Medical Systems, Inc., Iselin, N J). In 2 patients high-volume intravenous contrast material combined with rapid table incrementation was employed. In both of these, 150 ml of 60% meglumine diatrozoate was administered in 2 min using a mechanical injector. In the third patient, renal failure precluded our use of intravenous contrast material. Transhepatic cholangiography was accomplished in the standard fashion in all cases using a 22 gauge Chiba needle.
Results
Key words: L y m p h o m a , e x t r a n o d a l - Bile duct ob-
The clinical presentations, radiologic observations, a n d p a t h o l o g i c findings are s u m m a r i z e d below.
struction - C h o l a n g i o c a r c i n o m a , differential diagnosis.
Case 1
Patients with l y m p h o m a m a y develop jaundice for a variety o f reasons [1, 2]; diffuse l y m p h o m a t o u s liver involvement, cholestasis due to n o d a l enlargem e n t in the p o r t a hepatis, a n d hepatocellular disease related to the toxic effects o f c h e m o t h e r a p y are the m o s t c o m m o n causes. Rarely, l y m p h o m a m a y directly infiltrate the wall o f the biliary tree, p r o d u c i n g biliary obstruction. W e e n c o u n t e r e d 3 such cases o f e x t r a n o d a l l y m p h o m a t o u s infiltraAddress reprint requests to: Dennis M. Balfe, M.D., Mallinck-
rodt Institute of Radiology, Washington University School of Medicine, 510 South Kingshighway Boulevard, St. Louis, MO 63110, USA
A 35-year-old white male, RM, had a 15-year history of stage IV nodular sclerosing Hodgkin disease (pulmonary and cervical nodes and bone marrow involvement). His current admission was precipitated by a six-week history of increasing jaundice. A CT scan demonstrated intrahepatic bile duct dilatation. No mass was identified at the site of duct termination, and no portal adenopathy was detected (Fig. 1A). Percutaneous transhepatic cholangiography showed strictures of the right and left hepatic ducts at their confluence (Fig. 1B). Duct contour was irregular, but no masses were detected. Exploratory laparotomy revealed a 1-cm nodule in the right lobe of the liver, which was biopsied and found to be nodular sclerosing Hodgkin disease. The common bile duct was firm and irregular without extrinsic mass. Sections of the duct wall also revealed nodular sclerosing Hodgkin disease. A T-tube was implanted, and radiation therapy was subsequently performed. Multiple cholangiograms performed during the next 18 months documented relentless progression of the diffuse bili-
54
V.M. Tartar and D.M. Balfe: Lymphoma in the Bile Duct Wall
Fig. 1. Case 1. A 35-year-old white male with stage IV nodular sclerosing Hodgkin disease for 15 years, now with obstructive jaundice. A Upper abdominal CT scan at the level of the porta shows intrahepatic biliary ductal dilatation. The region of the common hepatic duct (arrow) demonstrates no adenopathy. B A cholangiogram, performed perioperatively via a T-tube, demonstrates extensive stricturing of the common hepatic duct and adjacent segments of the right and left hepatic ducts (arrowheads).
ary strictures, with concomitant hepatic decompensation. The patient died 18 months after icterus was first noted.
mission, and he remains clinically disease-free 14 months after his initial presentation.
Case 2
Case 3
A previously healthy 48-year-old black male, AC, presented for evaluation of painless jaundice. Laboratory evaluation revealed elevation of bilirubin and liver enzymes. A sonogram demonstrated biliary ductal dilatation with a solid mass within the common hepatic duct. An intravenous contrast-enhanced abdominal CT demonstrated bile duct dilatation with multiple low-attenuation regions within the left lobe of the liver, including the region of the porta hepatis (Fig. 2A-C). The findings were thought to represent eholangiocarcinoma. A transhepatic cholangiogram was performed to further evaluate the anatomy of the biliary tree and to assess the possibility of performing curative surgery. This showed a long irregular segmental narrowing of the entire common hepatic duct with extension into the right and left hepatic ducts (Fig. 2 D). Surgical exploration revealed a firm tumor in the hepatic hilus involving the common hepatic ducts, proximal common bile duct, and the surface of the left lobe of the liver. A left hepatic lobectomy and cholecystectomy was performed, and a Roux-en-Y choledochojejunostomy created. Pathologic examination of the resected specimen revealed malignant nonHodgkin lymphoma (Fig. 2 E and F). A bone marrow biopsy performed in the postoperative period showed no evidence of lymphomatous involvement. Six months after diagnosis, CT evaluation of the chest, abdomen, and pelvis demonstrated evidence of widespread systemic lymphoma, with extensive mediastinal, retroperitoneal, and mesenteric lymphadenopathy. Systemic chemotherapy induced a re-
A 69-year-old white female, GC, was transferred from an outside hospital for evaluation of jaundice, nausea, vomiting, and acute renal insufficiency. Initial evaluation one month prior to transfer revealed an elevated bilirubin and a positive Hepatitis A antibody. Three weeks later, she developed increasing icterus and had laboratory evidence of renal failure. A C T scan performed on the day of transfer demonstrated biliary ductal dilatation and a 3 cm mass in the region of the pancreatic head (Fig. 3A and B). Also noted were diffusely enlarged kidneys, ascites, and a right pleural effusion. A PTC demonstrated biliary ductal dilatation with an abrupt narrowing of the common bile duct in the region of the pancreas and another mass obstructing the origin of the cystic duct (Fig. 3 C). The patient became progressively hypotensive after placement of a drainage catheter, and emergency laparotomy was performed. At laparotomy, 5-7 units of blood and ascites were evacuated. The bleeding site was identified as a hepatic puncture site above the catheter location, presumably from the fine needle diagnostic puncture. Three days later, the patient was re-explored for recurrent bleeding. At that time, the gallbladder was noted to be distended, and a 2-3 cm mass was identified near the cystic duct. A frozen section of a biopsy of the gallbladder mass revealed a small cell neoplasm, consistent with lymphoma, and cholecystectomy was performed (Fig. 3 D). A mass in the head of the pancreas was noted, but no biopsy was performed.
Fig. 2. Case 2. A previously healthy black male with painless jaundice. A The CT scan through the liver demonstrates intrahepatic biliary ductal dilatation and irregularly marginated lowattenuation masses in the left lobe of the liver (arrows). B A CT scan 3 cm caudal shows a lower portion of the mass (arrow) and a separate mass (arrowheads) replacing the common hepatic duct. C A C T scan through the liver (performed 15 min after initial scans) shows persistent contrast enhancement of the previously low-attenuation left hepatic lobe mass (arrows). D Percutaneous transhepatic cholangiogram demonstrates intrahe-
patic biliary ductal dilatation proximal to strictures (arrowheads) narrowing the common hepatic duct and adjacent portions of the right and left intrahepatic ducts. E Histologic section through the common hepatic duct. Lymphomatous mass compresses adjacent liver and involves a large portal tract (center). Uninvolved portal tracts show features of obstruction (H & E stain; x40). F Microscopic section of the common bile duct wall, which is greatly expanded by lymphoid infiltrate. The ductal epithelium (upper left) is partially sloughed, but in most areas was well preserved (H & E stain; x 40).
56
Fig. 3. Case 3. A 69-year-old white female with acute jaundice and renal failure. A A C T image at the level of the gallbladder (GB), which contains high-attenuation material, demonstrates intra- and extrahepatic biliary ductal dilatation, with a soft tissue density mass (arrow) in the region of the cystic duct. The kidneys are diffusely enlarged. (RK, right kidney; LK, left kidney; CBD, common bile duct.) B A C T scan 4 c m caudal at the level of the uncinate process of the pancreas (P) shows a second soft tissue density mass (arrow) between the contrast-filled duodenum (D) and the pancreas (P). (GB, gallbladder; cbd, common bile duct; RK, right kidney;
V.M. Tartar and D.M. Balfe: Lymphoma in the Bile Duct Wall
LK, left kidney.) C Cholangiogram obtained during placement of a biliary drainage catheter shows marked biliary ductal dilatation proximal to a mass (arrowheads) at the distal common bile duct, with effacement of the descending-portion of the duodenum (thick arrows). The cystic duct is also abruptly truncated (thin arrow). A lamellated gallstone is seen (curved arrow). (D, duodenum) D Microscopic section of the gallbladder wall shows full thickness replacement of the wall by malignant large cell lymphoma. The mucosal villi are blunted, but the epithelium is intact (H & E stain; x 100).
V.M. Tartar and D.M. Balfe: Lymphoma in the Bile Duct Wall The patient's clinical condition deteriorated markedly in the postoperative period, and she died on the ninth postoperative day.
Discussion
Lymphoma, or its treatment, can produce jaundice in a variety of ways [1, 2], including compression of the common duct by involved nodes in the hepatoduodenal ligament or pancreatoduodenal chain, lymphomatous infiltration of the liver causing diffuse hepatocellular disease, or toxic side effects of the chemotherapeutic regimen producing hepatic failure. Direct infiltration of the wall of a biliary structure is quite uncommon; the literature contains only isolated case reports [3-7]. There are three clinical situations in which lymphomatous infiltration of the biliary system may occur: (a) as a solitary site of extranodal lymphoma, (b) as one of many sites in a systemic lymphoma, and (c) as a site of recurrence after therapy directed to systemic lymphoma. Our case material illustrates each of the three mechanisms mentioned above. Primary extranodal non-Hodgkin lymphoma arising in the bile duct is extremely rare, having been reported in only one other previous case, in which a patient presenting with acute obstructive jaundice was found to have diffuse lymphohistiocytic infiltration of the extrahepatic bile ducts [7]. Radiographically and clinically, this case and Case 2 have striking parallels. Cholangiograms in both cases showed a smooth, tapered stricture involving the common hepatic duct, suggesting sclerosing cholangitis or cholangiocarcinoma. Preoperative imaging and surgical exploration showed no extrahepatic foci of lymphoma, but in both cases, widespread systemic disease occurred within months after initial exploration. In our case, an intrahepatic lymphomatous nodule was also present. Its discovery on preoperative CT suggested direct liver invasion by cholangiocarcinoma; there were no CT features suggestive of lymphoma. There was a puzzling CT finding in this case: the hepatic tumor was a low-attenuation mass during the bolus phase of examination, but in the equilibrium phase, it exhibited persistent contrast enhancement compared with the normal liver (Fig. 2C). We have no explanation for this observation. Infiltration of the gallbladder by a systemic
57
lymphoma is uncommon, but it has been described in the clinical literature both in the setting of known disease [4] and as a previously unsuspected pathologic diagnosis made from a cholecystectomy specimen [5]. In our patient, the additional finding of diffuse enlargement and nodularity of the kidneys, with concomitant renal failure, allowed a correct preoperative diagnosis of lymphoma. Patients with a long-standing history of Hodgkin lymphoma often develop jaundice due to compression of biliary structures by portal lymph nodes. Infiltration of the duct wall is quite uncommon in the absence of nodal disease. Preoperative imaging in Case 1 suggested that nodal obstruction was n o t the cause, since high-resolution CT scans obtained through the hepatoduodenal ligament demonstrated no evidence of nodal masses. Cholangiography showed diffuse stricturing of intrahepatic and extrahepatic ducts, in a pattern that mimicked sclerosing cholangitis. The diagnosis of lymphomatous involvement of the biliary system is difficult by any nonoperative means. In a patient with known lymphoma, cholangiographic findings of smooth, tapered strictures combined with the absence of a portal mass on CT should suggest the diagnosis. In patients with no previous history, the radiographic differential diagnosis includes cholangiocarcinoma, and only histologic material can successfully differentiate the two. References 1. Bouroncle BA, Old JW Jr, Vasques AG: Pathogenesis of jaundice in Hodgkin's disease. Arch Int Med 110:872-883, 1962 2. Levitan R, Diamond HD, Craver LF: Jaundice in Hodgkin's disease. Am J Med 30: 99-111, 1961 3. Botha JB, Kahn LB : Primary lymphoma of the gallbladder: case report and review of the literature. S Afr Med J 48:1345-1348, 1974 4. Van Slyck EJ, Schuman BM: Lymphocytic lymphosarcoma of the gallbladder. Cancer 30: 810-816, 1972 5. Gillespie J J, Ayala AG, MacKay B, Silliman YE: Diagnosis of lymphoma from a choleeystectomy specimen: case report and review of the literature. South Med J 70: 353-354, 1977 6. Ochoa JI, Keene WR: Obstructive jaundice in Hodgkin's disease due to infiltration of the biliary ducts. J Fla Med Assoc 57:21-23, 1970 7. Nguyen GK: Primary extranodal non-Hodgkin's lymphoma of the extrahepatic bile ducts. Cancer 50: 2218-2222, 1982 Received: February 16, 1989; accepted: March 14, 1989
