Games & Puzzles

Liver transplantation in a patient with primary sclerosing cholangitis suffering from lipomatous pseudohypertrophy of the pancreas

Liver transplantation in a patient with primary sclerosing cholangitis suffering from lipomatous pseudohypertrophy of the pancreas
of 3
All materials on our website are shared by users. If you have any questions about copyright issues, please report us to resolve them. We are always happy to assist you.
Related Documents
  LETTER TO THE EDITOR Liver transplantation in a patient with primary sclerosingcholangitis suffering from lipomatous pseudohypertrophyof the pancreas doi:10.1111/j.1432-2277.2007.00564.x Although the pathogenesis of lipomatous pseudohypertro-phy (LiPH) of the pancreas is not clear, findings of previ-ous 10 cases support the hypothesis that hepatic disordersare closely linked to this condition [1–3]. The first casedates back to 1953 and was published by Robson  et al. [4]. They described the lesion as an abundance of matureadipose tissue with widely separated fibrous septae invest-ing remnants of pancreas parenchyma. The pathogenesisof this disorder remained unknown; however, over thefollowing decades more and more evidences were col-lected that LiPH may be caused by liver disorders [5].Sasaki reported a patient with chronic hepatitis B andsuch a lesion, and Kuroda  et al.  [6,7] confirmed thesefindings. In both cases, there was no evidence for obesity but both patients suffered form liver cirrhosis. Most pub-lished cases come from Japan. Bralet  et al. , on the otherhand, found that LiPH was associated with a squamouscancer of the pancreas in a patient without liver diseaseand the disorder has been reported in association withchronic pulmonary disease by Siegler  et al.  [8,9] Inanother case, the disease caused obstruction of the bileduct and required surgical treatment by a hepaticojejun-ostomy. LiPH also has been reported in the submandibu-lar gland [10]. A case from Russia suggested a congenitaldisorder associated with this obscure lesion [11]. LiPHcan grow to gigantic masses; histology of the pancreasreveals almost complete replacement of the acinar cells by adipocytes with preservation of the islets. Insulin-depen-dent diabetes mellitus is not a typical feature of thisobscure disorder. Although closely linked to liver cirrho-sis, thus far not a single case of LiPH associated with pri-mary sclerosing cholangitis (PSC) or liver transplantation(LT) has been reported.Our patient was a 32-year-old male who was diag-nosed with PSC 2001. His presenting symptoms includedweight loss, watery diarrhea, pruritus, and scleral icterus.His family history included a father diagnosed with PSCwho underwent LT. Liver biopsy in 2001 showed chronichepatitis with bridging fibrosis and early cirrhosis andtypical features of PSC. Endoscopic retrograde cholangio-pancreatography (ERCP) findings for the patient at thetime of diagnosis revealed diffuse areas of narrowing anddilation of the biliary tree. During the following years,multiple biliary stents were placed. He had at least onedocumented episode of cholangitis with sepsis. He devel-oped significant ascites, which was treated with routineparacentesis and continued to have abdominal pain. InFebruary 2007, the patient underwent routine ERCP withremoval of his biliary stents. New stents could not beplaced because of a tight ductal stricture. A few daysafter his procedure, he was admitted for increasedabdominal pain and multiple loose, light-colored stools.Upon admission, laboratory values included mildly elevated liver enzymes (AST 61 IU/ml, ALT 108 IU/ml),a total serum bilirubin of 8.1 mg/dl and alkalinephosphatase of 357 IU/ml. Also serum amylase wasmildly elevated with 141 mg/dl. Magnetic resonancecholangiopancreatography (MRCP) and Magneticresonance imaging (MRI) of the abdomen wereperformed revealing typical features of the PSC and inaddition an intramesenteric lipomatous mass emanatingfrom the pancreatic head (Fig. 1a and b). This mass hadbeen present on MRI studies 5 years prior with sizeunchanged, measuring 18 cm at the greatest diameter.Despite the mass effect that the tumor exerted on thehead and distal body of the pancreas, there was no pan-creatic ductal dilation noted. The patient was evaluatedand listed for LT with a model for end stage liverdisease (MELD) score of 17; biopsy of the mass wasplanned to be performed during LT. Few days laterwhile still hospitalized, a cadaveric graft became avail-able. On exploration of the abdominal cavity, the intra-mesenteric lipomatous mass was found and a wedgeresection was performed prior to hepatectomy. Histology showed mature adipose tissue with admixed with benignpancreatic elements (Fig. 2a and b). The final pathology was LiPH of the pancreas. Resection of the mass did notoccur during the operation because its location srcinat-ing from the pancreatic head would have required aWhipple procedure. Following the confirmation that the ª  2007 The AuthorsJournal compilation  ª  2007 European Society for Organ Transplantation  21  (2008) 89–91  89  lesion was most likely benign, the liver was mobilizedand the hilum was also dissected. Because of large cau-date and retroperitoneal varices, a caval replacementtransplant was attempted. Caval, portal venous and arte-rial anastomoses were performed in the usual fashionand reperfusion was tolerated without complications andLT was completed by biliary reconstruction with Roux en Y hepaticojejunostomy. The procedure was performedwithout complications, and the patient was extubated onpostoperative day 1. The patient quickly recovered fromthe operation and his hospital course was uneventfulexcept for some additional abdominal complaints includ-ing loss of appetite and mild abdominal pain in the epi-gastrium. However, amylase and lipase remained normal,and none of his symptoms indicated pancreatic ductobstruction, leakage or pancreatitis. He was dischargedon day 7 post-LT. After 1 month transplant, he experi-enced an episode of acute cellular rejection requiringmethylprednisone followed by antithymocyte globulintherapy. The rejection was reversed and no further com-plications occurred. The patient is alive with a well-func-tioning graft 3 months post-LT.Liver transplantation has become a standard treatmentof end-stage liver disease, and long-term survival can now be achieved in most cases [12]. Because of the improvedresults, more patients with comorbid conditions can now be offered this challenging surgical procedure. Organshortage has demanded thorough evaluation and there-fore all patients undergo intensive testing prior to livertransplant. This includes, per protocol, computed tomog-raphy scans to exclude pathology such as liver-basedextrahepatic malignancies. In our patient, MRI revealed apancreatic mass; however, as it had not increased in sizeduring a 5-year period, the patient was put on the waitinglist. Frozen section specimens that were taken intra-oper-atively from the rim of the lesion showed a well-differen-tiated lipomatous neoplasm. Because of the fact that asarcoma could not be verified on histology, and in lightof the benign clinical course, LT was carried out. Also, itwas decided not to perform resection of the lesion as aWhipple procedure would have been required and therewas no evidence that the patient may benefit from suchan extensive procedure. There were some technical diffi-culties because of the significant size of the tumor; how- Figure 1  Magnetic resonance imaging(MRI): gigantic intra-abdominal massarising from the head of the pancreas;note typical features of liver cirrhosis. Figure 2  Histology: hematoxylin/eosin-stain ( · 20,  · 40); massive lipomatousinfiltration of the pancreatic tissue, lossof acini but preservation of islets. Letter to the editor ª  2007 The Authors 90  Journal compilation  ª  2007 European Society for Organ Transplantation  21  (2008) 89–91  ever, the intra-operative course was without major com-plications. In addition no postoperative complicationsrelated to LiPH were observed. The patient is well andalive 3 months post-LT with no evidence of pathology associated with this rare lesion. To summarize, we presentthe first case of pancreatic LiPH in a liver recipient suffer-ing from PSC.Tanya Flohr, 1 Hugo Bonatti, 1 Nathan Shumaker, 2 Francis Diaz, 3 Carl Berg, 4 Hilary Sanfey, 1 Timothy Pruett, 1 Robert Sawyer 1 and Timothy Schmitt 1 Departments of 1 Surgery, 2 Pathology,3 Radiology and 4 Gastroenterology,University of Virginia Health system, VA, USA References 1. Yoshimura N, Hayashi S, Fukushima Y. Diffuse Mallory bodies in the liver, diffuse Lewy bodies in the brain anddiffuse fat replacement (lipomatous pseudohypertrophy)of the pancreas in a patient with juvenile Parkinson’sdisease.  Acta Pathol Jpn  1992;  42 : 826.2. Henkinbrant A, Khalek W, Farchakh E, Henry M. Chole-static jaundice complicating lipomatous pseudo-hypertro-phy of the pancreas.  Acta Gastroenterol Belg   1990;  53 : 315.3. Nakamura M, Katada N, Sakakibara A,  et al.  Huge lipoma-tous pseudohypertrophy of the pancreas.  Am J Gastroenterol  1979;  72 : 171.4. Robson HN, Scott GB. Lipomatous pseudohypertrophy of the pancreas.  Gastroenterology   1953;  23 : 74.5. Beresford OD, Owen TK. Lipomatous pseudohypertrophy of the pancreas.  J Clin Pathol   1957;  10 : 63.6. Sasaki M, Nakanuma Y, Ando H. Lipomatous pseudohy-pertrophy of the pancreas in a patient with cirrhosis due tochronic hepatitis B.  Pathol Int   1998;  48 : 566.7. Kuroda N, Okada M, Toi M, Hiroi M, Enzan H. Lipoma-tous pseudohypertrophy of the pancreas: further evidenceof advanced hepatic lesion as the pathogenesis.  Pathol Int  2003;  53 : 98.8. Bralet MP, Terris B, Bregeaud L,  et al.  Squamous cell carci-noma and lipomatous pseudohypertrophy of the pancreas. Virchows Arch  1999;  434 : 569.9. Siegler DI. Lipomatous pseudohypertrophy of the pancreasassociated with chronic pulmonary suppuration in an adult. Postgrad Med J   1974;  50 : 53.10. Melita P, Azzolina P. On lipomatous pseudohypertrophy of the submaxillary gland.  G Ital Chir   1964;  20 : 323.11. Bazan OI. Congenital polycystosis and lipomatous pseudo-hypertrophy of pancreas.  Arkh Patol   1957;  19 : 58.12. Shiffman ML, Saab S, Feng S,  et al.  Liver and intestinetransplantation in the United States, 1995-2004.  Am J Transplant   2006;  6 : 1170. Letter to the editor ª  2007 The AuthorsJournal compilation  ª  2007 European Society for Organ Transplantation  21  (2008) 89–91  91
Similar documents
View more...
Related Search
We Need Your Support
Thank you for visiting our website and your interest in our free products and services. We are nonprofit website to share and download documents. To the running of this website, we need your help to support us.

Thanks to everyone for your continued support.

No, Thanks

We need your sign to support Project to invent "SMART AND CONTROLLABLE REFLECTIVE BALLOONS" to cover the Sun and Save Our Earth.

More details...

Sign Now!

We are very appreciated for your Prompt Action!