G Chir Vol. 32 - n. 5 - pp. 255-258

May 2011

 

 

Ectopic liver nodules: a rare finding during cholecystectomy

 

M. Catani, R. De Milito, F. Romagnoli, P. Mingazzini, V. Silvestri,

V. Usai, V. Romeo, C. Modini

 

 

“Sapienza” University of Rome, Italy

Emergency Department (DEA)

(Chief: Prof. C. Modini)

 

 

Summary: Ectopic liver nodules: a rare finding during cholecystectomy.

 

M. Catani, R. De Milito, F. Romagnoli, P. Mingazzini,

V. Silvestri, V. Usai, V. Romeo, C. Modini

 

The ectopic liver (or choristoma) is a rare condition found during autopsy or abdominal exploration for various indications.

The authors report two cases of ectopic liver found during laparoscopic cholecystectomy for acute cholestytis.

The ectopic liver tissue has been reported to develope in several sites as thoracic cavity, gastrohepatic ligament, adrenal glands, pancreas, esophagus and, above all, gallbladder. The Authors review the literature and report their experience as a contribution to the knowledge of this rare pathological entity.

Riassunto: Fegato ectopico: un raro reperto in corso di colecistectomia laparoscopica.

 

M. Catani, R. De Milito, F. Romagnoli, P. Mingazzini,

V. Silvestri, V. Usai, V. Romeo, C. Modini

 

Il fegato ectopico (o coristoma) è una rara condizione che  si può rilevare in corso di autopsia o durante l’esplorazione chirurgica dell’addome per varie indicazioni.

Gli Autori riportano due casi di fegato ectopico riscontrati durante colecistectomia laparoscopica per colecistite acuta.

Il tessuto epatico ectopico si può sviluppare in molteplici siti, come la cavità toracica, il legamento epato-gastrico, i surreni, il pancreas, l’esofago e soprattutto la colecisti. Gli Autori riportano la loro esperienza e fanno una ressegna della letteratura  per contribuire alla conoscenza di questa rara entità patologica.

 

Key Words:  Ectopic liver - Laparoscopic cholecystectomy.

                Fegato ectopico – Colecistectomia per via laparoscopica.

 

 

Introduction

 

The presence of hepatic tissue, located in an other position than the orthotopic one, is named choristoma (term established in 1904 by Albert) or, more commonly, ectopic or heterotopic liver.

It is a rare entity and documented cases of ectopic liver are less than 100 (4 during the autopsy and 72 during the surgical exploration or radiologic study). The ectopic liver is a tissue histologically normal, but it can be subjected to the same histopathological changes like  the arthropic liver. It’s usually asymptomatic, even if it can be associated to abdominal pain, portal hypertension and respiratory failure. The radiological diagnosis, before surgery or autopsy, is hard.

The Authors report their experience and a briefly review the literature. The aim of the study is to give a contribution to knowledge of this rare entity.

 

 

Case reports

 

Case 1

A 83 year old woman was admitted in elective setting at our tertiary level hospital for “gallstones”.

The blood test analysis revealed slight rise of cholestasis indices; HBV and HCV tests were negative and the inflammatory markers were within normal range. A pre-operative hepatic ultrasonography showed only distended gallbladder with 3 small stones. The patient had  no history of drinking, cirrhosis or other liver diseases.

An elective laparoscopic cholecystectomy was performed. At surgery, a subserosal ectopic liver nodule (1.4cm) on the left lateral side of gallbladder was found (Figs. 1, 2). The nodule seemed drizzled by an arterial branch that ran along the front part of the gallbladder. A standard laparoscopic cholecystectomy (1) including ectopic liver nodule was performed.

Postoperative course was uneventful and the patient was discharged on the third post-operative day.

Histology showed “hepatic tissue with evidence of portal spaces and sublobular veins associated to moderate distortion of trabecular architecture”; no island of malignant degeneration was found at pathology.

 

Case 2

A 72 year old woman was admitted in emergency setting at our tertiary level hospital for “acute cholecystitis”.

The blood test analysis revealed only neutrophil leukocytosis (WBC 14610 and  NEU 85.8%) and high level of LDH 234 IU/L. A pre-operative hepatic sonography showed only distended gallbladder with thicken walls and biliary sludge. The patient had history of hypercholesterolemia and 4 coronary stents.

For this reason an elective low pressure laparoscopic cholecystectomy (5-6 mmHg of pneumoperitoneum) was performed. At surgery, an ectopic liver nodule (approximately 1.5 cm) on the fundus of the gallbladder was found. The nodule had its vascular peduncle. A standard laparoscopic cholecystectomy, including ectopic liver nodule, was performed.

Postoperative course was uneventful and the patient was discharged on the fifth post-operative day. The histological examination showed “hepatic tissue with evidence of portal spaces and sublobular veins” without islands of malignant degeneration.

 

 

Discussion and conclusion

 

The ectopic liver is a rare condition. The first case dates back to 1922 (2): it was an ectopic liver lobe attached to the wall of gallbladder; another similar case was reported  by Cullen in 1925 (3).

Documented cases of ectopic liver are 73 (excepted ours): 4 were found during autopsy and 69  during surgical exploration or radiologic studies (Table 1). The incidence ectopic liver during autopsy is 5.4% .

There is a simple classification (4)  of this anatomical abnormality:

1.         ectopic liver, which is not connected to the “mother” liver and usually attached to the gallbladder or intrabdominal ligaments (as in our cases);

2.         microscopic ectopic liver found occasionally in the gallbladder wall;

3.         a large accessory liver lobe attached to the “mother” liver by a stalk;

4.         a small accessory liver lobe attached to the “mother” liver.

Finding out an ectopic liver by imaging studies before surgery or during autopsy is rare because it often has a small size and/or the radiologists may not recognize this unusual entity. The ectopic liver usually is asymptomatic; rarely it can be associated with abdominal pain due to torsion of the stalk, bowel obstruction, portal vein thrombosis with portal hypertension, or respiratory failure when the ectopic mass is in the chest cavity.

The ectopic tissue have the same anatomic and structural characteristics of native liver and as well as the “mother” liver it is subject to the same  histopathological changes such as steatosis, cirrhosis and hepatocellular carcinoma (HCC). Recent literature discusses the potential for increased risk of HCC in ectopic liver: several studies show as the hepatic heterotopic tissue has a greater tendency to malignant degeneration than mother liver (5).

The reports showed that, in absence of hepatic disease, the big liver ectopic nodules have a normal histological picture, differently in the small ectopic liver nodules (<2 cm) always coexist, even if aspecific, cellular and structural alterations as in our case.

For these reasons, we stress the need to remove and analyze always the operative specimen, to exclude areas of malignant degeneration.

 

 

References

 

1. Catani M, De Milito R, Romagnoli F, Luciani G, Simonelli L, Carocci V, Usai V, Silvestri V, Modini C. Emergency laparoscopic cholecistectomy in the treatment of acute cholecystis: when and how?. Chir Ital 2009; 61(4):435-447.

2. Corsy F. Lobe sunumeraire due foie implnate sur la face interieure de la vescicule billaire. C R Soc Biol 1922; 86: 695-697.

3. Cullen T. Accessory lobes of the liver. Arch Surg 1925; 11:718–721.

4. Collan Y, Hakkiluoto A, Hastbacka J. Ectopic  liver. Ann Chir Gynaecol 1978; 67:27-29.

5. Arakawa M, Kimura Y, Sakata K, Kubo Y, Fukushima T, Okuda K. Propensity of ectopic liver to hepatocarcinogenesis: case reports and a review of the literature. Hepatology 1999; 29(1):57-61.

6. Houriuchi S, Kitamura T, Okuda S, Tateishi R, Wada A, Omori K. A case of hepatoma residing in the retroperitonium separate from the liver. Acta Hepatol Jpn 1969; 10:259-262.

7. Lipp W. Die entwicklung der parenchymarchitektur der leber. Ver Ana Ges 1952; 50:241–249.

8. Fellbaum C, Beham A, Schmid C. Isolierte nebenleber (hepar succenturiatum) am gallenblasenhals. Fallbericht mit Literaturubersicht. Wien Klin Wochenschr 1987; 99:825–827.

9. Iacconi P, Masoni T. Accessory liver, report of 2 cases. Acta Chir Belg 1990; 90:228–230.

10. Hamdani SD, Baron RL. Ectopic liver simulating a mass in the gallbladder wall: imaging findings. Am J Radiol 1994; 162:647–648.

11. Svane S, Knudtzon J. Ectopic liver in the gallbladder and cholestasis. Tidsskr Nor Laegeforen 1991; 111:2643–2644.

12. Angquist KA, Boquist L, Domellof L. Ectopic liver lobule with portal cirrhosis. Acta Chir Scand 1975; 141:238–241.

13. Ashby EC. Accessory liver lobe attached to the gallbladder. J Surg 1969; 56:311–312.

14. Beltran MA, Barria C, Pujado B, Barrera R, Mendez P, Pozo C. Hepatic heterototopic tissue on the gallbladder wall: an incidental finding with pathological potential. J Gastrointestin Liver Dis 2007; 16(3):347-9

15. Koh CE, Hunt R. Ectopic liver encountered during laparoscopic cholecystectomy. Asian J Surg 2007; 30(3):227-9.

16. Wang Y, Liu FJ. Ectopic liver tissue in the gallbladder serosa: a case report.  Zhonghua Gan Zang Bing Za Zhi 2006; 14(5):369.

17. Ikeda H, Suzuki M, Takahashi H, Kobayashi M, Okuse N, Moriya H, Koike J, Maeyama S, Yotsuyanagi H, Itoh F. Hepatocellular carcinoma with silent and cirrhotic non-alcoholic steatohepatitis, accompanying ectopic liver tissue attached to gallbladder. Pathol Int 2006 Jan; 56(1):40-5.

18. Lundy J, Johnson E, Edwards K, Rivera D. Laparoscopic management of gallbladder-associated ectopic liver. JSLS 2005; 9(4):485-7.

19. Acar T, Taçyildiz R, Karakayali S. Ectopic liver tissue attached to the gallbladder. Acta Chir Belg 2002 Jun; 102(3):210-1.

20. Sakarya A, Erhan Y, Aydede H, Kara E, Ilkgül O, Ciftdoğan C. Ectopic liver (choristoma) associated with the gallbladder encountered during laparoscopic cholecystectomy: a case report. Surg Endosc 2002; 16(7):1106.

21. Dieter RA Jr, Rebchook A, Heymann M. Laparoscopic resection of an accessory liver and gallbladder. Surg Endosc 2000; 14(7):680.

22. Kodama T, Yokoyama T. Heterotopic tissues in the gallbladder, heterotopic gastric mucosa, heterotopic intestinal epithelium, heterotopic pancreatic tissue and ectopic liver in the gallbladder. Ryoikibetsu Shokogun Shirizu 1996; (9):360-2.

23. Castro Viera GA, Sanuy ME, Cámara HA, Córdoba OC. Accessory liver in gallbladder. apropos of a case report. Rev Esp Enferm Dig 1990; 77(4):295-6.

24. Tejada E, Danielson C. Ectopic or heterotopic liver (choristoma) associated with the gallbladder. Arch Pathol Lab Med 1989; 113(8):950-2.

25. Festen C, Severijnen RS, vd Staak FH. Gallbladder embedded in an accessory liver lobe in umbilical cord hernia. J Pediatr Surg 1988; 23(10):978-9.

26. Natori T, Hawkin S, Aizawa M, Asai T, Kameda Y, Ikuyohashi K. Intra-cholecystic ectopic liver. Acta Pathol Jpn 1986; 36(8):1213-6.

27. Torchio B, Maconi G. Nodule of ectopic hepatic parenchyma in the wall of the gallbladder. Minerva Chir1978; 33(1-2):39-44.

28. Watanabe M, Matsura T, Takatori Y, Ueki K, Kobatake T, Hidaka M, Hirakawa H, Fukukmoto S, Shimada Y. Five cases of ectopic liver and a case of accessory lobe of the liver. Endoscopy 1989; 21(1):39-42.

29. Heid GJ, von Haan E: Hepatic heterotype in the splenic capsule. ArchPathol 1948; 46:377.

30. Kim KA, Park CM, Kim CH, Choi SY, Park SW, Hong SJ, Seol HY, Cha IH. Hepatocellular carcinoma in an ectopic liver: CT findings. Eur Radiol 2003; 13 Suppl 4:L45-7.

31. Liu KL, Ho MC, Chen PJ. Ectopic liver with hepatocellular carcinoma in the peritoneum. Am J Roentgenol 2007; 188(2):W206-7.

32. Leone N, Saettone S, De Paolis P, Carucci P, Brunello F, De Angelis C, Menozzi G, Rizzetto M. Ectopic livers and related pathology: report of three cases of benign lesions. Dig Dis and Sci 2005; 50(10):1818-22.

33. Cardona D, Grobmyer S, Crawford JM, Liu C. Hepatocellular carcinoma arising from ectopic liver tissue in the pancreas. Virchows Arch 2007; 450(2):225

34. Kubota K, Kita J, Rokkaku K, Iwasaki Y, Sawada T, Imura J, Fujimori T. Ectopic hepatocellular carcinoma arising from pancreas: a case report and review of the literature. World J Gastroenterol 2007; 13(31):4270-3.

35. Pesce C, Colacino R, Tobia F. Heterotopic liver tissue with micronodular cirrhosis. A case report. Ann  Pathol 1984; 4:389–390.

36. Davies JNP. Accessory liver in an african. Br Med J 1946; 2:736–737.

37. Honoré LH. Intra-adrenal hepatic heterotopia. J Urol 1985; 133(4):652-4

38. Matley PJ, Rode H, Cywes S. Portal vein obstruction by ectopic liver tissue. J Pediatr Surg 1989;  24:1163–1164.

39. Huang TW, Chan DC, Lee HS, Yao NS, Lee SC, Cheng YL. Ectopic hepatocellular carcinoma of the diaphragm. Dig Dis Sci 2007; 52(4):1118-20.

40. Luoma R, Raboei E: Supradiaphragmatic accessory liver: a rare cause of respiratory distress in a neonate. J Pediatr Surg 2003; 38(9):1413-4.

41. Takayasu K, Itabashi M, Moriyama N: Case report: ectopic hepatocellular carcinoma arising from the left diaphragm. Clin Radiol 1994;49(8) :579-81.

42. Bedii Salman A: Left-sided congenital diaphragmatic hernia associated with intrathoracic ectopic liver lobule.  Eur J Cardiothorac Surg 2002; 21:558–560.

43. Ferro F, Lais A, Boldrini R, De Peppo F, Federici G, Bosman C: Hepatogonadal fusion. J Pediatr Surg1996; 31:435–436.

44. Park WH, Choi SO, Lee SS, Randolph JG: Ectopic umbilical liver in conjunction with biliary atresia: uncommon association. J Pediatr Surg 1991; 26:219–222.

45. Tsushimi T, Enoki T, Harada E, Orita M, Noshima S, Masuda M, Hamano K: Ectopic hepatocellular carcinoma arising in the bile duct. J Hepatobiliary Pancreat Surg 2005; 12(3):266-8.

46. Elsayes HB, Elzein MA, Razik AM, Olude IO: Torsion of an ectopic liver in a young child. J Pediatr Surg 2005; 40(3):E55-8.

47. Choi SU, Kim HK, Kim J: Heterotopic supradiaphragmatic liver combined with intralobar pulmonary sequestration. Ann Thorac Surg 2008; 85(5):1809-10.

48. Slone T, Emil S, Meissner N, Behjatnia B, Fairbanks T, Romansky S: Sternal cleft, Morgagni hernia, and ectopic liver: a unique chest wall anomaly. J Pediatr Surg 2007; 42(12):2132-5.

49. Gehér P, Füredi A, Kecskés LI: Videothoracoscopic removal of ectopic liver. Magy Seb 2007; 60(6):310-1.

50. Babu R, Van der Avoirt A: Ectopic intrathoracic liver. Pediatr Surg Int 2001; 17(5-6):461-2.

51. Asselah T, Condat B, Cazals-Hatem D, Hassani Z, Bernuau J, Groussard O, Mussot S, Lesèche G, Marcellin P, Erlinger S, Valla D: Ectopic hepatocellular carcinoma arising in the left chest wall: a long-term follow-up. Eur J Gastroenterol Hepatol 2001; 13(7):873-5.

52. Moriwaki Y, Higashino K, Taketa K, Hada T, Tamura S, Yamaguchi K: Immunohistochemical and affinity electrophoretic studies of alpha-fetoprotein of a patient with hepatocellular carcinoma in the intrathoracic space. Am J Gastroenterol 1987; 82(11):1207-11.

53. Rendina EA, Venuta F, Pescarmona EO, Martelli M, Ricci C: Intrathoracic lobe of the liver. Case report and review of the literature. Eur J Cardiothorac Surg 1989; 3(1):75-8.

54. Djuricić S, Zlatković M, Stanković I, Plamenac P: Heterotopic liver tissue in the fundus of the gallbladder. Srp Arh Celok Lek 1999; 127(11-12):412-5.

55. Iber T, Rintala R: Intrapulmonary ectopic liver. J Pediatr Surg 1999; 34(9):1425-6.

56. Wax JR, Pinette MG, Cartin A, Blackstone J: Ectopic liver: a unique prenatally diagnosed solid umbilical cord mass. J Ultrasound Med 2007; 26(3):377-9.

57. Preminger A, Udassin R, Pappo O, Arad I: Ectopic liver tissue within the umbilical cord. J Pediatr Surg 2001; 36(7):1085-6.

58. Shigemori M, Kondo M, Azechi H, Inoue F, Tamura J, Kobayashi H, Saiga T: A case of ectopic hepatocellular carcinoma in the jejunum. J Gastroenterol 2006; 41(9):913-8.

59. Magid MS, Godwin TA, Zheng W. Hepatic heterotopias in the jejunum: a case study over time showing progressive degenerative changes. Pediatr Pathol Lab Med 1997; 17(4):663-70.

60. Patel Y, McNally J, Ramani P. Left congenital diaphragmatic hernia, absent pericardium, and liver heterotopia: a case report and review. J Pediatr Surg 2007; 42(5):E29-31.

61. Brustmann H. Heterotopic liver in the right cardiac auricle. Ann Diagn Pathol 2002; 6(4):248-9.

62. Kawahara E, Kitamura T, Ueda H, Ogino T, Mai M, Ooi A, Nakanishi I. Hepatocellular carcinoma arising in the abdominal cavity. An autopsy case of ectopic liver origin. Acta Pathol Jpn 1988; 38(12):1575-81.

63.Yamashita M, Nagamine Y, Ozaki K, Ueshima S, Takahashi H, Inoue H. An autopsy case of a cirrhotic ectopic liver with a review of the literature. Acta Hepatol Jpn 1985; 26:510–514.

64. Nakashima T, Okuda K, Kojiro M, Jimi A, Yamaguchi R, Sakamoto K, Ikari T. Pathology of hepatocellular carcinoma in Japan. 232 Consecutive cases autopsied in ten years. Cancer 1983; 51(5):863-77.

65.  Caygill CP, Gatenby PA. Ectopic liver and hepatocarcinogenesis. European Journal of Gastroenterology and Hepatology 2004; 16(8):727-9.

66. Seo UH, Lee HJ, Ryu WS, Kwak JM, Shin BK, Kim WB, Lee SI, Park SS, Choi JW, Kim SH, Choi SY, Mok YJ. Laparoscopic resection of a hepatocellular carcinoma arising from an ectopic liver. Surg Laparosc Endosc Percutan Tech 2008; 18(5):508-10.

67. Leone N, De Paolis P, Carrera M, Carucci P, Musso A, David E, Brunello F, Fronda GR, Rizzetto M. Ectopic liver and hepatocarcinogenesis: report of three cases with four years' follow-up. Eur J Gastroenterol Hepatol 2004; 16(8):731-5.

68. Hofmann HS, Spillner J, Hammer A, Diez C. A solitary chest wall metastasis from unknown primary hepatocellular carcinoma. Eur J Gastroenterol Hepatol 2003; 15(5):557-9.