Case Report Primary Neuroendocrine Tumor of the Left Hepatic Duct: a Case Report with Review of the Literature

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Case Report Primary Neuroendocrine Tumor of the Left Hepatic Duct: a Case Report with Review of the Literature Hindawi Publishing Corporation Case Reports in Surgery Volume 2012, Article ID 786432, 7 pages doi:10.1155/2012/786432 Case Report Primary Neuroendocrine Tumor of the Left Hepatic Duct: A Case Report with Review of the Literature Ajay H. Bhandarwar, Taher A. Shaikh, Ashok D. Borisa, Jaydeep H. Palep, Arun S. Patil, and Aditya A. Manke Division of GI and HPP Surgery, Department of Surgery, Grant Medical College & Sir JJ Group of Hospitals, Byculla, Mumbai 400008, India Correspondence should be addressed to Ajay H. Bhandarwar, [email protected] Received 29 April 2012; Accepted 29 August 2012 Academic Editors: T. C¸ olak and M. Ganau Copyright © 2012 Ajay H. Bhandarwar et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary Biliary Tract Neuroendocrine tumors (NET) are extremely rare tumors with only 77 cases been reported in the literature till now. We describe a case of a left hepatic duct NET and review the literature for this rare malignancy. To the best of our knowledge the present case is the first reported case of a left hepatic duct NET in the literature. In spite of availability of advanced diagnostic tools like Computerized Tomography (CT) Scan and Endoscopic Retrograde Cholangio Pancreaticography (ERCP) a definitive diagnosis of these tumors is possible only after an accurate histopathologic diagnosis of operative specimens with immunohistochemistry and electron microscopy. Though surgical excision remains the gold standard treatment for such tumors, patients with unresectable tumors have good survival with newer biologic agents like Octreotride. 1. Introduction 2. Case Report NET is derived from the embryonal neural crest cells called A 69-years-old female presented with colicky pain in the Argentaffin or Kulchitsky cells and have a potential for right hypochondrium since 3 years. She had past history of secreting serotonin. This tumor can arise anywhere in the open cholecystectomy done for gall stones 15 years back. distribution of the Argentaffin cell system. In addition to On physical examination the patient was anicteric with the most common sites of occurrence, namely, ileum and soft abdomen. An Ultrasonography (USG) of the abdomen appendix these tumors have reported to occur in bladder, showed a hypoechoic lesion of size 3.5 cm × 4cm in prostate, rectum, stomach, bronchi, pancreas, and biliary segment 4 of the liver. Computerized Tomography (CT) tree. of the abdomen showed a 4.1 × 3.7 cm heterogeneously Primary Biliary Tract NETs are rare and account for enhancing mass lesion in segment IV of liver abutting the 0.2%–2% [1, 2] of all gastrointestinal neuroendocrine left branch of portal vein (Figure 1). A Magnetic Resonance tumors. Imaging (MRI) of upper abdomen with Magnetic Resonance The Literature documents about 77 cases of neuroen- CholagioPancreaticography (MRCP) showed a filling defect docrine tumor arising from the biliary tree which includes in the left hepatic duct with lesion in the adjacent part of common bile duct, common hepatic duct, cystic duct, and liver in segment IV, suggestive of a left hepatic duct tumor hilar confluence. with infiltration in the liver (Figure 2). Tumor marker serum We report a case of Primary Biliary NET arising from the alfa fetoprotein was mildly raised. Patient was worked up left hepatic duct. An extensive search of the literature has for left hepatectomy. Intraopertaively a lesion arising from yielded no reference regarding a NET arising from the left the left hepatic duct involving the left branch of portal Hepatic ducts. The present case is the first reported case of a vein and extending upto the portal confluence was found NET arising in the left hepatic duct. (Figure 3) rendering the tumor unresectable. A biopsy was 2 Case Reports in Surgery Figure 4: Histopathology showing a typical rosette appearance of neuroendocrine tumor. Figure 1: CT scan showing mass lesion in segment 4 abutting the left branch of portal vein. Figure 2: MRCP showing a filling defect in the left hepatic duct. (a) (b) Figure 5: Immunohistochemistry staining positive for Chromo- Figure 3: Intraoperative picture showing tumor arising from the granin (a) and Synaptophysin (b). left hepatic duct. CBD hooked in yellow, hepatic artery in red, and portal vein in blue. taken from the mass and procedure was abandoned in somatostatin receptor expressing lesion in the hepatobiliary view of inoperability. Histopathology showed typical rosette system (Figure 7). appearance of a neuroendocrine tumor (Figure 4)and The patient was started on long-acting Octreotride immunohistochemistry positive for CD56, Chromogranin therapy single dose every month. The patient has received and Synaptophysin (Figure 5). Ultrastructural study of the 12 of such doses and is doing well after a 1-year followup cell with electron microscopy (Figure 6) showed the presence without any complications. A follow up MRI (Figure 8) of multiple neurosecretory granules with muscle tissue. upper abdomen with MRCP at 1 year showed the absence of A whole body Positron Emission Tomography (PET) filling defect in the Left hepatic duct that was seen previously scan and an Octreotride labeled radionulceotide scan showed which showed tumor regression. Case Reports in Surgery 3 x4000 767-10-a 80 kV x30000 123 135 122 214 Figure 6: Electron microscopy picture showing multiple granules of varying sizes. Figure 8: Follow up MRCP at 1 year showing absence of any filling defect in the left hepatic duct. (a) 3. Discussion Primary Biliary-tract NETs are very rare. They account for 0.2–2% of all gastrointestinal NET [1, 2] reason being the paucity of enterochromaffin cells from which NETs arise in this area. Chronic inflammation of the bile duct epithelium is responsible for metaplasia of these enterochromaffincells and formation of NET. Davies [3] in 1959 reported NET of the distal bile duct and pancreatic duct which represented more of a periampullary NET rather than a biliary tract. Pilz [4]in 1961 was credited to report the first case of a Biliary Tract NET. After an extensive search of the Medline only 77 cases of Biliary Tract NET have been reported so far in the literature (b) since 1961 (Table 1). Till now no NET in the literature has been reported in Figure 7: PET Scan (a) and Octreotride labelled (b) scan showing the isolated left hepatic duct possibly making our case the tumor limited to the hepatobiliary system. first reported case of an isolated left hepatic duct NET. 4 Case Reports in Surgery Table 1: Showing study of reported cases of Biliary tract NET. Table 1: Continued. No. Case reference Age Sex Complaint Location No. Case reference Age Sex Complaint Location Hao et al., [33] Incidental (1) Pilz, [4] 1961 55 F Weakness CBD (30) 47 M CBD 1996 finding Little et al., [5] RUQ pain, (2) 41 F Hilar Kopelman et al., 1968 jaundice, (31) 44 M Jaundice CBD [34] 1996 Autopsy Distal (3) Bergdahl, [6] 1976 79 F Belli et al., [35] finding CBD (32) 78 M Jaundice CBD 1996 CBD Judge et al., [7] Jaundice, (4) 19 M Hilar Bembenek et al., 1976 pain (33) 12 F Jaundice Hilar [36] 1998 Gerlock and Nahas et al., [37] (5) Muhletaler [8] 32 M Jaundice CBD (34) 61 F Jaundice Hilar 1979 1998 Vitaux et al., [9] Distal Ross et al., [38] (6) 24 M Jaundice (35) 65 F Jaundice CBD 1981 CBD 1999 Nakamuara et al. Chamberlain and (7) 58 F Jaundice CBD [10] 1981 (36) Blumgart [39] 37 F Itching Hilar 1999 Abe et al., [11] (8) 64 M RUQ pain CBD 1983 Chamberlain and (37) Blumgart [39] 67 F Itching Hilar Goodman et al., (9) 28 F RUQ Pain Cystic duct 1999 [12] 1984 Hermina et al., Jutte et al., [13] (38) 69 M RUQ pain Cystic duct (10) 62 M Back Pain CHD [40] 1999 1986 Perakath et al. [42] Jaundice, Nicolescu and (39) 36 F CHD (11) 50 F RUQ pain CBD 1999 Pain Popescu, [14] 1986 Chan et al [41] Alexander et al., (40) 14 M Jaundice Hilar (12) 64 F Hematemesis CBD 2000 [15] 1986 Maitra et al., [42] Gastinger et al. Jaundice, (41) 53 F Jaundice, CBD (13) 65 F Hilar 2000 [16] 1987 Pain Maitra et al., [42] Jaundice, Reinhardt et al. Jaundice, (42) 61 F Hilar (14) 71 F CBD 2000 itching [17] 1988 fever Juturi et al., [43] Jaundice, Chittal and Ra, (43) 43 M CBD (15) 46 F RUQ pain Cystic duct 2000 itching [18] 1989 Turrion´ et al., [44] Jaundice, Fujita et al., [19] (44) 51 F Hilar (16) 55 F RUQ pain CBD 2002 itching 1989 Pawlik et al., [45] Bickerstaff and (45) 59 M Jaundice Hilar (17) 57 F Jaundice CBD 2003 Ross [20] 1989 Podnos et al., [46] Brown et al., [21] (46) 65 F Cholecystitis CBD (18) 35 F Jaundice Hilar 2003 1990 Podnos et al., [46] Jaundice, Bumin et al., [22] (47) 27 M CBD (19) 38 F Jaundice CBD 2003 itching 1990 Volpe et al., [47] Jaundice, Fellows et al. [23] (48) 19 M CBD (20) 30 M Jaundice CBD 2003 pain 1990 (49) Menezes et al., [48] 30 M Jaundice CHD (21) Besznyak´ et al. [24] 13 F Jaundice Hilar Ligato et al., [49] Irritable Angeles-Angeles et (50) 33 F Hilar (22) 39 F Jaundice CBD 2005 bowel al., [25] 1991 Hubert et al., [50] Barron-Rodriguez Jaundice, (51) NA M Jaundice CBD (23) 36 M CBD 2005 et al., [26] 1991 RUQ pain Hubert et al., [50] Newman et al., (52) NA M Jaundice CBD (24) 15 F N/A CBD 2005 1992 [27] Hubert et al., [50] Dixon et al., 1992 (53) NA F Jaundice CBD (25) 60 F RUQ pain CBD 2005 [28] Nesi et al., [51] Jaundice, Rugge et al., [29] Jaundice, Cysticduct, (54) 30 M CBD (26) 64 F 2006 iarrhoea 1992 RUQ pain CBD Kim et al., [52] Gembala et al., (55) 67 F Jaundice CBD (27) 28 M Jaundice Hilar 2006 [30] 1993 Caglikulekci et al., Mandujano-Vera (56) 40 F Jaundice Hilar (28) 53 F Jaundice CBD [53] 2006 et al., [31] 1995 Honda et al., [54] Jaundice, Sankary et al., [32] (57) 76 M CBD (29) 47 F Jaundice Hilar 2006 pain 1995 Case Reports in Surgery 5 Table 1: Continued.
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