Survival from primary abdominal lymphoma presenting with a mass and obstructive jaundice

Extrahepatic biliary ductal obstruction with lymphoma is a rare cause of jaundice and an even rarer manifestation of lymphoma (1). Primary lymphoma arising in the abdomen may remain localized in the hepatic hilar region leading to an abdominal mass and biliary ductal obstruction; this was recently described for the first time in the English literature in a patient from Hong Kong (2). In that patient, however, the clinical follow-up after biliary tract stenting, surgery and chemotherapy was limited to just over one year. The patient described here presented with clinical and laboratory features of painless jaundice due to an extrahepatic biliary ductal obstruction, which was initially believed to be due to pancreatic carcinoma. Subsequent studies, however, revealed the unusual finding of a localized B cell lymphoma. Now, after completion of chemotherapy and radiation treatment almost 14 years ago, she remains in clinical remission with no evidence of recurrent disease. BRIEF COMMUNICATION


CASE PRESENTATION
A 68-year-old female was admitted to hospital in October 1981 after three to four weeks of anorexia and fatigue followed by the onset of intense pruritus, dark urine, pale stools and jaundice.Examination revealed scleral icterus, linear dermal excoriations on her extremities and a rounded, smooth and nontender mass in the right upper quadrant, typical of a distended gallbladder.There was no lymphadenopathy.
Abdominal ultrasound revealed a distended gallbladder (Figure 1) as well as dilated intrahepatic and extrahepatic bile ducts with a 5 cm mass near the pancreas (Figure 2).No hepatic metastatic lesions were seen.Aspiration needle bi-opsy of the mass revealed atypical lymphoid cells consistent with a poorly differentiated lymphocytic lymphoma (Figure 3).Bone marrow aspiration and trephine biopsy revealed no lymphoma.A transhepatic cholangiogram showed dilation of the intrahepatic bile ducts and the proximal common hepatic duct.
An enlarged gallbladder with a firm mass in the region of the porta hepatis displacing normal pancreas was revealed during laparotomy.Frozen sections of the mass confirmed the presence of malignant lymphoma.An operative cholangiogram (Figure 4) showed dilated intrahepatic and extrahepatic ducts with marked narrowing of the common hepatic duct over a 4 cm segment, approximately 2 cm distal to a patent cystic duct; this narrowed portion of bile duct was smooth and regular.The distal common bile duct was normal.A biopsy from the left lobe of the liver revealed features of large duct obstruction only (Figure 5), while a biopsy of the mass revealed fibrous tissue infiltrated with neoplastic lymphoid and histiocytic cells (Figure 6).Electron microscopy showed tumour cells typical of lymphoma.Cholecystojejunostomy and jejunojejunostomy were done, from which the patient recovered without complication.After discharge, she was treated with three cycles of CHOP chemotherapy (cyclophosphamide, doxorubicin hydrochloride, vincristine and prednisone) and local upper abdominal radiation (30 Gy).A computerized tomographic abdominal scan in January 1982 and April 1982 revealed no residual disease.
The patient was reviewed each year, and in November 1988, a further abdominal computerized tomographic scan was normal.At her last formal evaluation in November 1994 after completion of chemotherapy and radiation treatment, she remained in clinical remission with no evidence of recurrent disease.Blood tests, including liver chemistry tests, and repeat abdominal computerized tomographic scan were normal.

DISCUSSION
The patient's clinical presentation suggested a malignant biliary obstruction, possibly in the pancreas.Indeed, the clinical finding of a palpable and nontender gallbladder in the presence of jaundice (Courvoisier's sign) due to an extrahepatic biliary ductal obstruction has traditionally been regarded as a typical clinical sign of an obstructing pancreatic carcinoma.However, the importance of definitive histological diagnosis was underlined by the detection of lymphoma and this patient's later clinical course, as well as by her subsequent response to treatment with chemotherapy and radiation with no evidence of recurrent or systemic disease.
The patient's response to treatment sharply contrasts with the poor prognosis in disseminated lymphoma complicated by biliary ductal obstruction, despite various treatment regi-mens (1,3,4).In addition, biliary ductal obstruction is usually considered a poor prognostic indicator for lymphoma (4).In recent years, however, some reports have described therapeutic successes in patients with primary pancreatic lymphoma (3,5).Thus, even in the absence of lymphadenopathy, an intra-abdominal mass could be due to a localized lymphoma; in this setting, a precise diagnosis may permit aggressive management and a positive patient outcome.
The causes of jaundice in patients with Hodgkin's and non-Hodgkin's lymphoma have been previously enumerated (6).Often, infiltration of the liver occurs (7).Other causes that have been described include intrahepatic cholestasis, hepatotoxicity due to chemotherapeutic agents used for treatment, superimposed hepatic infections including viral hepatitis or tuberculosis, sclerosing cholangitis, vanishing bile duct syndrome and amyloidosis (1,(8)(9)(10)(11)(12).Jaundice due to obstruction from more generalized involvement of lymph nodes in lymphoma is very uncommon (1,3,4), and even more rare are descriptions of lymphoma in the liver, bile ducts, pancreas and gastrointestinal tract causing jaundice (3,5,(13)(14)(15)(16)(17)(18)(19)(20).Although localized lymphoma in the porta hepatis region has been described following liver transplantation (21), there is only one recent report of de novo primary lymphoma causing obstructive jaundice (2).In some patients, a definite cause for jaundice may not be defined; in these, a paraneoplastic phenomenon may play a role in some of the patients (22).

CONCLUSIONS
The present report describes a rare presentation of lymphoma localized to the porta hepatis.The tumour mass caused obstructive jaundice and was initially thought to be a pancreatic carcinoma.A precise tissue diagnosis of lymphoma, however, led to chemotherapy and radiation treatment with resultant long term survival, for almost 14 years at the last evaluation.

Figure 2 )Figure 1 ) 3 )Figure 4 )
Figure 2) Abdominal ultrasound showing mass lesion in the region of the porta hepatis

Obstructive jaundice and lymphomaFigure 6 )Figure 5 )
Figure 6) Operative biopsy of mass lesion at the porta hepatis fibrous tissue infiltrated with a malignant lymphoma