INTRODUCTION

R osai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy(SHML), is a rare idiopathic disorder of histio-

cytic proliferation. According to a review of the entity

by Foucar et al (1), the typical patient profile is a 20-year-old white or black male who presents with pain-less bilateral cervical lymphadenopathy. The clinicalcourse is variable ranging from self-limited and indo-lent with spontaneous regression to, less commonly,aggressive and fulminant. A large number of patients

PRACTICAL GASTROENTEROLOGY • DECEMBER 200226

Treated Rosai-Dorfman Disease: An Unusual Cause of Biliary Obstruction

A CASE TO REMEMBER

Zina J. Ricci, M.D., Department of Radiology; Alla M. Rozenblit, M.D., Department of Radiology; Morris Edelman,M.D., Department of Pathology; Daniel Berkowitz, M.D., Department of Internal Medicine; Ronald Kaleya, M.D.,Department of Surgery; all at Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York.

Zina J. Ricci Alla M. Rozenblit, Morris Edelman, Daniel Berkowitz, Ronald Kaleya

We report a case of a 50-year old woman with Rosai-Dorfman disease who presentedfive years prior to admission with vague abdominal pain secondary to bulky peripan-creatic lymphadenopathy mimicking lymphoma on CT scan. She was treated success-fully with chemotherapy. Five years later she presented with right upper quadrantpain, fevers and progressive jaundice. Imaging demonstrated intrahepatic and extra-hepatic biliary ductal dilatation with an appearance of primary sclerosing cholangitis,cholelithiasis, and recurrent small upper abdominal lymph nodes. Abdominal MRIrevealed discrete abnormal peripancreatic signal consistent with fibrosis which webelieve reflected healed Rosai-Dorfman disease leading to intractable biliary obstruc-tion. There are no cases to our knowledge of biliary obstruction related to this uncom-mon disease.

have immunologic abnormalities. The disease hasbeen reported to involve almost every organ of thebody. Although it commonly involves cervical lymphnodes and not infrequently other nodal groups, it isuncommon in the abdomen. Biliary obstruction due tothis disease has not been described. We encountered apatient who developed biliary obstruction five yearsafter successful chemotherapy of extensive peripan-creatic SHML.

CASE REPORTA 50 year-old black woman was admitted to our hos-pital with right upper quadrant pain, vomiting, chills,and night sweats increasing over a two week periodand progressive jaundice. Her past medical historyincluded rheumatic mitral stenosis, atrial fibrillation,congestive heart failure and type II diabetes mellitus.She had a history of vague abdominal pain five yearsprior to admission, at which time bulky peripancreatic(Fig. 1A) and lesser omental lymphadenopathy wasdocumented by CT scan. A laparoscopic biopsy speci-men of the lesion at that time was diagnostic of Rosai-Dorfman disease. The patient improved after treatmentwith a combination of Cyclophosphamide, Vincristineand Prednisone, and later with Methotrexate and Pred-nisone. Seven years prior to admission, she had exci-

sion of a large painless right submandibular mass thatwas initially diagnosed as an inflammatory pseudotu-mor. Later review of the pathology confirmed the diag-nosis of Rosai-Dorfman disease.

On physical exam, the patient had jaundice, low-grade fever, right upper quadrant tenderness, and aMurphy’s sign. Pertinent laboratory data included:white blood cell count of 1600, hemoglobin of 9.4,hematocrit of 27, total bilirubin of 15.4, direct biliru-

PRACTICAL GASTROENTEROLOGY • DECEMBER 2002 27

A CASE TO REMEMBER

Treated Rosai-Dorfman Disease: An Unusual Cause of Biliary Obstruction

Figure 1C. MRI of the abdomen in coronal plane demon-strates markedly hypointense peripancreatic tissue (whitearrows) on SSFSE sequence. Axial images (not shown)demonstrate normal signal in the pancreas proper.

(continued on page 31)

Figure 1A. Contrast enhanced CT scan of the abdomen fiveyears prior to admission shows a dominant lobulated peri-pancreatic soft tissue mass (white arrows) and otherenlarged peripancreatic lymph nodes. Duodenum isstretched laterally around the mass.

Figure 1B. Abdominal sonogram reveals an enlarged com-mon bile duct measuring 1 cm in caliber (white arrow).

PRACTICAL GASTROENTEROLOGY • DECEMBER 2002 31

A CASE TO REMEMBER

Treated Rosai-Dorfman Disease: An Unusual Cause of Biliary Obstruction

bin of 10.0, alkaline phosphatase of 649, AST of 66,and ALT of 120. Sequential serum multi-analysis-7was normal.

Abdominal sonogram revealed intrahepatic andextrahepatic ductal dilatation (Fig. 1B) and cholelithi-asis. Visualization of the pancreas was limited. CTscan of the chest, abdomen and pelvis demonstrated aminimal residual peripancreatic soft tissue density,much smaller than on previous CT scan five years ear-lier. There was, however, recurrent periportal andlesser omental lymphadenopathy and new mediastinallymphadenopathy. MRI of the abdomen (Fig. 1C)demonstrated small peripancreatic tissue, which wasisointense to pancreas on T1, markedly hypointense onT2 and nonenhancing with Gadolinium DTPA. Theappearance was consistent with fibrosis. MR cholan-giogram (Fig. 1D) revealed narrowing of the most dis-tal common bile duct and proximal pancreatic duct, adouble duct appearance. There was mild intrahepaticductal dilatation with a beaded appearance due to mul-tiple strictures. A dominant stricture was present in thecommon hepatic duct at the level of the cystic duct.ERCP (Fig.1E) revealed multiple small stones (2–3mm in size) in the common bile duct which were notdiscerned on MRCP, probably due to their small size.The stones were extracted and a biliary stent placed.Otherwise, the ERCP demonstrated similar findings as

(continued from page 27)

Figure 1D. MRCP shows focal narrowing (white arrows) ofthe distal common bile duct and proximal pancreatic duct(double duct appearance). Areas of stricturing and focalwidening are present in the common hepatic duct and intra-hepatic biliary tree.

Figure 1E. ERCP shows similar findings as MRCP exceptthat multiple small stones are visualized in the common bileduct (white arrows). The proximal pancreatic duct is nar-rowed and the remainder dilated.

PRACTICAL GASTROENTEROLOGY • DECEMBER 200232

A CASE TO REMEMBER

Treated Rosai-Dorfman Disease: An Unusual Cause of Biliary Obstruction

the MRCP. After biliary stent placement, the patienthad some initial improvement with decrease in rightupper quadrant pain and bilirubin levels. Three daysafter biliary stent placement laboratories were: totalbilirubin of 8.2; direct bilirubin of 4.5; alkaline phos-phatase of 446; AST of 43; and ALT of 43. However,the patient’s symptoms were not completely resolvedand she suffered from continued abdominal pain whichled to surgical therapy. She ultimately underwent chole-c y s t e c t o m y, choledochoduodenostomy and intraopera-tive periportal lymph node biopsy. Six days afters u rgery laboratories were: total bilirubin of 2.6; directbilirubin of 1.4; alkaline phosphatase of 101; AST of12; and ALT of 18. Pathology demonstrated changes ofSHML in excised lymph nodes from the porta- hepaticspace and peripancreatic region (Fig. 1F and G). Theresidual small soft tissue contiguous with the pancreatichead was not sampled. There was evidence of chroniccholecystitis and cholelithiasis with mild acute andchronic inflammatory changes of the common bile ductwall. There was no pathologic evidence of SHML inthe distal common bile duct wall.

DISCUSSIONAccording to a review of a registry of 423 patients withSHML by Foucar et al (1), the disease can involve

almost any organ, however, the majority of cases(85.3%) present with cervical lymphadenopathy. Ye t ,other nodal groups, including axillary, inguinal, medi-astinal and miscellaneous areas are affected in a signif-icant number of patients. Forty-three percent of patientsare reported to have at least one site of extranodalinvolvement. The most common extranodal sitesinclude skin, nasal cavity or paranasal sinuses, soft tis-sues, eyelids and orbit, bone, salivary gland and centralnervous system. Occasional cases of abdominalinvolvement, specifically liver, intestine, adrenal, kid-ney and pancreas are reported. The majority of patientswith extranodal disease have one to three extranodalsites involved. The presence of immunologic abnor-malities, involvement of multiple extranodal organ sys-tems and involvement of a larger number of nodegroups tend to correlate with an unfavorable prognosis.

We could find no references in the literature of biliaryobstruction related to Rosai-Dorfman disease. Althoughthere are many etiologies of biliary obstruction, includingp o s t i n f l a m m a t o r y, postsurgical, sclerosing cholangitis,ascending cholangitis, extrinsic processes such asmetastatic disease, lymphoma, choloangiocarcinoma,ampullary/periampullary processes and pancreaticmasses (2), this case appears unique. There are only a fewcase reports of SHML involving the peripancreatic region(1,3,4) and only one case involving the pancreas proper(5). Esquivel et al (5), illustrate abdominal CT scan find-ings of a solid mass in the body of the pancreas of a 48year old black woman. Other reports in the literature(1,3,4) describe retroperitoneal disease without detaileddescription of imaging findings.

Our case is therefore unique in that our patient pre-sented initially with a CT appearance mimicking lym-phoma characterized by a dominant bulky lymph nodemass adjacent to the pancreatic head with associatedmediastinal, hilar, axillary, lesser omental and peripan-creatic lymphadenopathy. Biliary obstruction was notpresent. The patient’s clinical course, although charac-terized by recurrent vague abdominal pain, was some-what indolent. The dominant peripancreatic mass andupper abdominal lymphadenopathy present five yearsprior to this admission had regressed after chemother-apy. Our patient then presented with new obstructivejaundice which we believe was due to chronic extrin-

(continued on page 34)

Figure 1F. Cystic duct lymph node contains dilated sinuseswith histiocytosis. Black arrow marks a histiocyte nucleus(light stained) with numerous intracytoplasmic lymphocytes(dark stained nuclei), a process known as emperipolesis. (Hand E; x200)

PRACTICAL GASTROENTEROLOGY • DECEMBER 200234

A CASE TO REMEMBER

Treated Rosai-Dorfman Disease: An Unusual Cause of Biliary Obstruction

sic mass effect on the distal common bile duct and notdue to choledocholithiasis alone. This longstandingprocess probably caused stasis leading to stone forma-tion and intrahepatic and extrahepatic biliary stric-tures, accounting for the appearance of sclerosingcholangitis seen on ERCP and MRCP.

MRI uncovered a distinct region of peripancreatictissue which was markedly decreased in signal on T2weighted images. This tissue caused the distal com-mon duct and proximal pancreatic duct narrowing(double duct appearance) seen on both ERCP andMRCP. It probably represents healed mass replaced byfibrosis after chemotherapy. The underlying contribu-tor to biliary obstruction in this patient probably lies inthe focal peripancreatic soft tissue that remained aftersuccessful therapy for the earlier peripancreatic dis-ease. Recall that our patient initially presented fiveyears prior to admission with extensive peripancreaticdisease but no biliary obstruction. We propose thathealed SHML accounts for the abnormal dark signalperipancreatic soft tissue and led to extrinsic masseffect on the common bile duct and pancreatic duct,

mimicking a double duct sign. It is documented in theliterature that as SHML regresses, fibrotic tissuereplaces the affected lymph nodes (6). Therefore, eventhough there was no evidence of SHML involving thecommon duct wall pathologically, replacement of peri-pancreatic tissue by fibrosis probably accounted forthe intractable biliary obstruction in this patient. ■

References1. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive

lymphadenopat hy (Rosai-Dorfman Disease): Review of theentity. Semin Diagn Pathol, 1990; 7(1): 19-73.

2. Halpert RD, Goodman P. Gastrointestinal Radiology: the requi -sites, 1st ed. St. Louis, Missouri: Mosby, 1993: 166-170.

3. Wright DH, Richards DB. Sinus histiocytosis with massive lym-phadenopathy (Rosai-Dorfman Disease): report of a case withwidespread nodal and extranodal dissemination. Histopathology,1981; 5: 697-709.

4. Gregor RT, Ninnin D. Rosai-Dorfman disease of the paranasalsinuses. J Laryngol Otol, 1994; 108: 152-155.

5. Esquivel J, Krighnan J, Jundi M, et al. Rosai-Dorfman Disease(Sinus histiocytosis with massive lymphadenopathy) of the pan-creas: first case report. Hepato-gastroenterology, 1999; 46: 1202-1205.

6. McAlister WH, Herman T, Dehner LP. Sinus histiocytosis withmassive lymphadenopathy (Rosai-Dorfman disease). Ped Radiol,1990; 20: 425-32.

PRA CT I CA L GA ST RO ENT EROLO GY

(continued from page 32)

Practical Gastroenterology invites its readers to share theirPEARLS OF GASTROENTEROLOGY

Submissions should be brief (about 200 words maximum). Those accepted for publication may be edited for space and style. An honorarium of $25 will be paid upon publication.

Mail your “Pearls of Gastroenterology” to Practical Gastroenterology9 9 B Main Street, Westhampton Beach, NY 11978 or fax them to us at (631) 288-4435.

Please include your name, address, affiliations, and telephone and fax numbers.

PRA CT I CA L GA ST RO ENT EROLO GY