EUROPEAN JOURNAL OF RADIOLOGY European Journal of Radiology 19 (1994) 32-33 Sarcoidosis of the pancreas mimicking pancreatic cancer: CT features Philippe Soyer*a, Lori Gottliebb, David A. Bluemke”, Elliot Fishman” “Department of Radiology, The Johns Hopkins Hospital, 600 N. Wolfe St., Baltimore. MD 21205, USA bDepartmenr of Surgery, The Johns Hopkins Hospital, 600 N. Wolfe St., Baltimore, MD 21205, USA Received 11 July 1994; accepted after revision 27 July 1994 Keywor& Pancreas, CT; Pancreas, abnormalities; Computed tomography, pancreas; Sarcoidosis, pancreas 1. Introdoetion Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granul- omas, that predominantly affects the lungs and lymph nodes. Although rarely involved, the liver and the stom- ach are the two most commonly involved organs in the gastrointestinal tract [ 1). Pancreatic involvement by sar- coidosis is exceedingly rare, and in cases previously published, the patients had known sarcoidosis at the time pancreatic involvement was discovered [2,3], or systemic sarcoidosis was subsequently demonstrated [4-81. We report a case of surgically proven pancreatic sarcoidosis mimicking a pancreatic neoplasm with bi- liary obstruction, in the absence of systemic sarcoidosis. 2. casf! report A 5 l-year-old black female was seen at the emergency room of another hospital in July 1993 because she developed severe right upper quadrant abdominal pain. The diagnosis of acute cholecystitis was suspected and cholecystectomy was performed. Although the patient’s right upper quadrant pain decreased, the overall condi- tion of the patient continued to worsen, with weight loss, flank pain, nausea and vomiting. In September 1993, she was noted to have an elevated bilirubin (4.5 x normal values), alkaline phosphatase and liver enzymes. The patient underwent endoscopic retrograde * Corresponding author, Department of Radiology, HBpital Foch, 40 rue Worth, BP 36, 92151 Suresnes cedex, France. cholangiopancreatogphy (ERCP). The pancreato- gram was normal but the endoscopist was unable to can- nulate the bile duct due a stricture. Percutaneous tran- shepatic cholangiography showed a high mid ductal stricture (Fig. la). A percutaneous biliary stent was placed at the same time. Contrast-enhanced CT scan showed a hypoattenuating pancreatic mass associated with periportal, periceliac, and retroperitoneal nodes, which suggested the diagnosis of pancreatic adenocar- cinema or pancreatic lymphoma (Fig. lb,c). A surgical biopsy of the pancreatic head mass and peripancreatic nodes was performed. Pathologic examination of the tis- sue block showed hyalinized fibrous tissue with multiple noncaseating granulomata with areas of tibrinoid necrosis. No tumorous cells were present. Stains for acid-fat organisms and fungi were negative. The diagno- sis of pancreatic sarcoidosis was considered. Choledo- chojejunostomy and gastrojejunostomy were perform- ed. After surgery, the patient underwent steroid therapy and her overall condition improved, with a 22 kg weight gain. Extensive evaluation, including skin biopsy, discloses no features suggestive for systemic sarcoidosis. In May 1994, the patient had a repeat contrast-enhanced CT scan, showing slight shrinkage of pancreatic mass and abdominal lymph nodes. 3. DkXusaion Pancreatic involvement by sarcoidosis is rare. Au- topsy series have shown a frequency ranging from 1 to 6% [9, lo]. In the cases previously reported, pancreatic involvement by sarcoidosis coexisted with systemic sar- 0720-048X/94/SO7.00 0 1994 Elsevier Science Ireland Ltd. All rights reserved SSDI 0720-048X(94)00561-P
Transcript
EUROPEAN JOURNAL OF RADIOLOGY
European Journal of Radiology 19 (1994) 32-33
Sarcoidosis of the pancreas mimicking pancreatic cancer: CT features
Philippe Soyer*a, Lori Gottliebb, David A. Bluemke”, Elliot Fishman” “Department of Radiology, The Johns Hopkins Hospital, 600 N. Wolfe St., Baltimore. MD 21205, USA bDepartmenr of Surgery, The Johns Hopkins Hospital, 600 N. Wolfe St., Baltimore, MD 21205, USA
Received 11 July 1994; accepted after revision 27 July 1994
Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granul- omas, that predominantly affects the lungs and lymph nodes. Although rarely involved, the liver and the stom- ach are the two most commonly involved organs in the gastrointestinal tract [ 1). Pancreatic involvement by sar- coidosis is exceedingly rare, and in cases previously published, the patients had known sarcoidosis at the time pancreatic involvement was discovered [2,3], or systemic sarcoidosis was subsequently demonstrated [4-81. We report a case of surgically proven pancreatic sarcoidosis mimicking a pancreatic neoplasm with bi- liary obstruction, in the absence of systemic sarcoidosis.
2. casf! report
A 5 l-year-old black female was seen at the emergency room of another hospital in July 1993 because she developed severe right upper quadrant abdominal pain. The diagnosis of acute cholecystitis was suspected and cholecystectomy was performed. Although the patient’s right upper quadrant pain decreased, the overall condi- tion of the patient continued to worsen, with weight loss, flank pain, nausea and vomiting. In September 1993, she was noted to have an elevated bilirubin (4.5 x normal values), alkaline phosphatase and liver enzymes. The patient underwent endoscopic retrograde
* Corresponding author, Department of Radiology, HBpital Foch, 40 rue Worth, BP 36, 92151 Suresnes cedex, France.
cholangiopancreatogphy (ERCP). The pancreato- gram was normal but the endoscopist was unable to can- nulate the bile duct due a stricture. Percutaneous tran- shepatic cholangiography showed a high mid ductal stricture (Fig. la). A percutaneous biliary stent was placed at the same time. Contrast-enhanced CT scan showed a hypoattenuating pancreatic mass associated with periportal, periceliac, and retroperitoneal nodes, which suggested the diagnosis of pancreatic adenocar- cinema or pancreatic lymphoma (Fig. lb,c). A surgical biopsy of the pancreatic head mass and peripancreatic nodes was performed. Pathologic examination of the tis- sue block showed hyalinized fibrous tissue with multiple noncaseating granulomata with areas of tibrinoid necrosis. No tumorous cells were present. Stains for acid-fat organisms and fungi were negative. The diagno- sis of pancreatic sarcoidosis was considered. Choledo- chojejunostomy and gastrojejunostomy were perform- ed. After surgery, the patient underwent steroid therapy and her overall condition improved, with a 22 kg weight gain. Extensive evaluation, including skin biopsy, discloses no features suggestive for systemic sarcoidosis. In May 1994, the patient had a repeat contrast-enhanced CT scan, showing slight shrinkage of pancreatic mass and abdominal lymph nodes.
3. DkXusaion
Pancreatic involvement by sarcoidosis is rare. Au- topsy series have shown a frequency ranging from 1 to 6% [9, lo]. In the cases previously reported, pancreatic involvement by sarcoidosis coexisted with systemic sar-
0720-048X/94/SO7.00 0 1994 Elsevier Science Ireland Ltd. All rights reserved SSDI 0720-048X(94)00561-P
P. Sayer et al. /Eur. J. Radiol. 19 (1994) 32-33 33
a C
Fig. 1. Fifty-one-year-old female with pathologically proved pancreatic sarcoidosis. (a) Transhepatic percutaneous cholangiography performed before stent placement shows stenosis (arrow) of the main bile duct. Surgical clips placed during cholecystectomy are seen (arrowhead). (b) Contrast- enhanced CT scan shows homogeneous lobulated enlarged pancreatic head (arrow). Enlarged retroperitoneal lymph nodes are present (ar- rowheads). (c) At a upper level, CT scan shows encasement of celiac axis (arrows) by hypoattenuating lymph nodes.
coidosis. In our case, the disease was limited to the pan- creas and peripancreatic lymph nodes. In the study by Britt et al. [I 11, among 16 patients with biopsy-proven sarcoidosis, enlarged lymph nodes at the celiac trunk axis and superior mesenteric artery axis were found in nine and five patients, respectively. However, no pancre- atic involvement was detected on CT in this study.
Pancreatic sarcoidosis usually presents with abdom- inal pain, anorexia, nausea, vomiting and weight loss. Pancreatitis is less frequent [7]. In our case, pancreatic sarcoidosis lead to intra- and extra-hepatic biliary ob- struction. This is an unusual presentation 181. Our pa- tient’s clinical presentation was very similar to that seen in pancreatic adenocarcinoma. However, the diagnosis of sarcoidosis was supported by pathologic features (in- cluding presence of noncaseating granulomas), negative stains for acid-fat organisms and fungi, and a favorable response to steroid therapy.
Changes involving the fat around the celiac trunk and superior mesenteric artery, as seen on CT scan, have been described in pancreatic adenocarcinoma, pan- creatitis, and pancreatic lymphoma [12,13]. This case shows that periarterial changes may also be seen on CT in pancreatic sarcoidosis.
This case shows that sarcoidosis of the pancreas may be difficult to distinguish from metastatic or primary pancreatic neoplasm, as well as pancreatic lymphoma. Although rare, the diagnosis of sarcoidosis should be raised when evaluating bile duct dilatation with an associated pancreatic or peripancreatic mass.
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