User menu

Accès à distance ? S'identifier sur le proxy UCLouvain

Mucin-producing hepatic cystic neoplasms: an uncommon but challenging disease often misdiagnosed and mismanaged.

Primary tabs

download
  • Open access
  • PDF
  • 970.40 K
download
  • Open access
  • PDF
  • 1.31 M
Frezin, Julie [UCL] Komuta, Mina [UCL] Zech, Francis [UCL] Annet, Laurence [UCL] Horsmans, Yves [UCL] Gigot, Jean-François [UCL] Mourin, Anne [UCL] Hubert, Catherine [UCL]

Mucin-producing hepatic cystic neoplasms (MHCN) are uncommon and potentially malignant. Nine MHCN were encountered in our centre for over 32 years. Patients' clinical, biological, radiological and pathological features were reviewed. Lesions were classified into Mucinous Cystic Neoplasms (MCN) and Intraductal Papillary Neoplasms of the Bile duct (IPNB) (WHO 2010 classification). Five MCN and 4 IPNB were reviewed. Serum and intracystic tumour markers were insufficient to diagnose malignancy. Complications were encountered in five out of nine patients (56%), mean symptom duration was 26 months (range: 1-132). Three patients were mismanaged pre-referral. Radiological features enabled preoperative diagnosis in eight out of nine patients (89%). Greater tumour size, unilocular lesion and mural nodularity indicated malignancy. Radical tumour excision was achieved in eight patients. One IPNB patient was misdiagnosed and underwent unroofing. For 103 months median follow-up, five out of six patients with benign tumours were alive and disease-free, whereas the misdiagnosed IPNB recurred with fatal malignant transformation seven years later. Among the three patients with malignancies (median follow-up: 77 months), two IPNB died, one from cancer recurrence and one from unrelated causes, whereas the malignant MCN was alive and disease-free. Appropriate MHCN diagnosis is crucial, yet it is often misdiagnosed and mismanaged. The prognosis after complete excision is favourable.

  1. Wittekind C, WHO classification of tumours. Pathology and genetics of tumours of the digestive system, 2, 182 (2000)
  2. Craig JR, Tumors of the liver and intrahepatic bile ducts (1989)
  3. Tsui WMS AN, Crawford JM, Ruban R, et al. Mucinous cystic neoplasm of the liver. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. World Health Organization classification of tumours. Pathology and genetics of tumours of the digestive system. 4th ed. Lyon: IARC Press; 2010. p. 236–238.
  4. Erdogan Deha, Obstructive jaundice due to hepatobiliary cystadenoma or cystadenocarcinoma, 10.3748/wjg.v12.i35.5735
  5. Poggio Paolo Del, Buonocore Marco, Cystic tumors of the liver: A practical approach, 10.3748/wjg.14.3616
  6. Arnaoutakis Dean J., Kim Yuhree, Pulitano Carlo, Zaydfudim Victor, Squires Malcolm H., Kooby David, Groeschl Ryan, Alexandrescu Sorin, Bauer Todd W., Bloomston Mark, Soares Kevin, Marques Hugo, Gamblin T. Clark, Popescu Irinel, Adams Reid, Nagorney David, Barroso Eduardo, Maithel Shishir K., Crawford Michael, Sandroussi Charbel, Marsh Wallis, Pawlik Timothy M., Management of Biliary Cystic Tumors : A Multi-institutional Analysis of a Rare Liver Tumor, 10.1097/sla.0000000000000543
  7. Lee Chao-Wei, Tsai Hsin-I, Lin Yann-Sheng, Wu Tsung-Han, Yu Ming-Chin, Chen Miin-Fu, Intrahepatic biliary mucinous cystic neoplasms: clinicoradiological characteristics and surgical results, 10.1186/s12876-015-0293-3
  8. Teoh Anthony Y.B., Ng Simon S.M., Lee K.F., Lai Paul B.S., Biliary Cystadenoma and Other Complicated Cystic Lesions of The Liver: Diagnostic and Therapeutic Challenges, 10.1007/s00268-005-0461-7
  9. Läuffer J.M, Baer H.U, Maurer C.A, Stoupis Ch, Zimmerman A, Büchler M.W, Biliary cystadenocarcinoma of the liver: the need for complete resection, 10.1016/s0959-8049(98)00166-x
  10. Wang Chu, Miao Ruoyu, Liu Huilin, Du Xiaojing, Liu Liguo, Lu Xin, Zhao Haitao, Intrahepatic biliary cystadenoma and cystadenocarcinoma: An experience of 30 cases, 10.1016/j.dld.2011.11.007
  11. Schoonbroodt D., Horsmans Y., Gigot J. F., Rahier J., Geubel A. P., Biliary cystadenoma of the liver with elevated CA 19-9, 10.1111/j.1600-0676.1994.tb00096.x
  12. Piessevaux Hubert, Beers Bernard Van, Gigot Jean-François, Geubel André-Pierre, Horsmans Yves, Misleading Radiological Presentation of a Simple Hepatic Cyst : , 10.1097/00004836-199506000-00029
  13. Horsmans Yves, Laka Andoni, Gigot Jean-François, Geubel André P., Serum and cystic fluid CA 19-9 determinations as a diagnostic help in liver cysts of uncertain nature, 10.1111/j.1600-0676.1996.tb00738.x
  14. Horsmans Yves, Laka Andoni, Beers Bernard E. Van, Descamps Charles, Gigot Jean-Francois, Geubel Andre P., 10.1023/a:1018842021677
  15. Korobkin M, Stephens DH, Lee JK, Stanley RJ, Fishman EK, Francis IR, Alpern MB, Rynties M, Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings, 10.2214/ajr.153.3.507
  16. Lewin Maïté, Mourra Najat, Honigman Isabelle, Fléjou Jean-François, Parc Rolland, Arrivé Lionel, Tubiana Jean-Michel, Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma, 10.1007/s00330-005-2822-x
  17. Pojchamarnwiputh S, Singapore Med J, 49, 392 (2008)
  18. Couinaud C, Le foie; Études anatomiques et chirurgicales (1957)
  19. Dindo Daniel, Demartines Nicolas, Clavien Pierre-Alain, Classification of Surgical Complications : A New Proposal With Evaluation in a Cohort of 6336 Patients and Results of a Survey, 10.1097/01.sla.0000133083.54934.ae
  20. Nurminen M, Scand J Stat, 14, 67 (1987)
  21. Zen Yoh, Pedica Federica, Patcha Venkata R, Capelli Paola, Zamboni Giuseppe, Casaril Andrea, Quaglia Alberto, Nakanuma Yasuni, Heaton Nigel, Portmann Bernard, Mucinous cystic neoplasms of the liver: a clinicopathological study and comparison with intraductal papillary neoplasms of the bile duct, 10.1038/modpathol.2011.71
  22. Peeters Karen, Delvaux Peter, Huysentruyt Frederik, Intraductal papillary neoplasm of the bile duct: a case report, 10.1080/00015458.2016.1258785
  23. Carrim Z.I., Murchison J.T., The Prevalence of Simple Renal and Hepatic Cysts Detected by Spiral Computed Tomography, 10.1016/s0009-9260(03)00165-x
  24. Gigot Jean‐François, Hubert Catherine, Banice Radu, Kendrick Michael L., Laparoscopic management of benign liver diseases: where are we?, 10.1080/13651820410023950
  25. Gigot J. -F., Metairie S., Etienne J., Horsmans Y., van Beers B. E., Sempoux C., Deprez P., Materne R., Geubel A., Glineur D., Gianello P., The surgical management of congenital liver cysts : The need for a tailored approach with appropriate patient selection and proper surgical technique, 10.1007/s004640090027
  26. Thomas K Tyson, Welch Derek, Trueblood Andrew, Sulur Paulgun, Wise Paul, Gorden D Lee, Chari Ravi S., Wright J Kelly, Washington Kay, Pinson C Wright, Effective Treatment of Biliary Cystadenoma : , 10.1097/01.sla.0000161982.57360.1b
  27. Lewis W. David, Surgical Treatment of Biliary Cystadenoma : A Report of 15 Cases, 10.1001/archsurg.1988.01400290045007
  28. Vogt David P., Henderson J. Michael, Chmielewski Elaine, Cystadenoma and Cystadenocarcinoma of the Liver: A Single Center Experience, 10.1016/j.jamcollsurg.2005.01.005
  29. Simo Kerri A., Mckillop Iain H., Ahrens William A., Martinie John B., Iannitti David A., Sindram David, Invasive biliary mucinous cystic neoplasm: a review, 10.1111/j.1477-2574.2012.00532.x
  30. Heintz A, Zentralblatt Fur Chirurgie, 123, 136 (1998)
  31. Manouras A, Lagoudianakis E, Alevizos L, Markogiannakis H, Kafiri G, Bramis C, Filis K, Toutouzas K, Laparoscopic fenestration of multiple giant biliary mucinous cystadenomas of the liver, 10.3748/wjg.14.4257
  32. Sang Xinting, Sun Yongliang, Mao Yilei, Yang Zhiying, Lu Xin, Yang Huayu, Xu Haifeng, Zhong Shouxian, Huang Jiefu, Hepatobiliary cystadenomas and cystadenocarcinomas: a report of 33 cases : Hepatobiliary cystadenomas and cystadenocarcinomas, 10.1111/j.1478-3231.2011.02560.x
  33. Zacherl J., Scheuba C., Imhof M., Jakesz R., Függer R., Long-term results after laparoscopic unroofing of solitary symptomatic congenital liver cysts, 10.1007/s004649900012
  34. Hansman Matthew F, Ryan John A, Holmes James H, Hogan Stephen, Lee Faye T, Kramer Donna, Biehl Thomas, Management and long-term follow-up of hepatic cysts, 10.1016/s0002-9610(01)00611-0
  35. Borhani Amir A., Wiant Amanda, Heller Matthew T., Cystic Hepatic Lesions: A Review and an Algorithmic Approach, 10.2214/ajr.13.12386
  36. Frick MP, Feinberg SB, Biliary cystadenoma, 10.2214/ajr.139.2.393
  37. Sutton C.D., White S.A., Berry D.P., Dennison A.R., Intrahepatic Biliary Cystadenoma Causing Luminal Common Bile Duct Obstruction, 10.1159/000018857
  38. Baudin Guillaume, Novellas Sébastien, Buratti Massimo Senni, Saint-Paul Marie Christine, Chevallier Patrick, Gugenheim Jean, Bruneton Jean Noël, Atypical MRI features of a biliary cystadenoma revealed by jaundice, 10.1016/j.clinimag.2006.04.003
  39. Yi Bin, Cheng Qing-Bao, Jiang Xiao-Qing, Liu Chen, Luo Xiang-Ji, Dong Hui, Zhang Bai-He, Wu Meng-Chao, A special growth manner of intrahepatic biliarycystadenoma, 10.3748/wjg.15.6134
  40. Takano Yuichi, Nagahama Masatsugu, Yamamura Eiichi, Maruoka Naotaka, Mizukami Hiroki, Tanaka Jun-ichi, Ohike Nobuyuki, Takahashi Hiroshi, Prolapse into the bile duct and expansive growth is characteristic behavior of mucinous cystic neoplasm of the liver: report of two cases and review of the literature, 10.1007/s12328-015-0569-8
  41. Yanai Hidekatsu, Tada Norio, A simple hepatic cyst with elevated serum and cyst fluid CA19-9 levels: a case report, 10.1186/1752-1947-2-329
  42. Yoshida H, Hepatogastroenterology, 49, 1095 (2002)
  43. Dixon E, Can J Surg, 44, 371 (2001)
  44. Koffron Alan, Rao Sambasiva, Ferrario Mario, Abecassis Michael, Intrahepatic biliary cystadenoma: Role of cyst fluid analysis and surgical management in the laparoscopic era, 10.1016/j.surg.2004.06.031
  45. Choi Hyo Kyung, Lee Jong Kyun, Lee Kwang Hyuck, Lee Kyu Taek, Rhee Jong Chul, Kim Kap Hyun, Jang Kee-Taek, Kim Seong Hyun, Park Yulri, Differential Diagnosis for Intrahepatic Biliary Cystadenoma and Hepatic Simple Cyst : Significance of Cystic Fluid Analysis and Radiologic Findings, 10.1097/mcg.0b013e3181b5c789
  46. Yoshida H, Hepatogastroenterology, 50, 507 (2003)
  47. Waanders Esmé, van Keimpema Loes, Brouwer Johannes T., van Oijen Martijn G. H., Aerts Raymond, Sweep Fred C. G. J., Nevens Frederik, Drenth Joost P. H., Carbohydrate antigen 19-9 is extremely elevated in polycystic liver disease, 10.1111/j.1478-3231.2009.02055.x
  48. Fuks D., Voitot H., Paradis V., Belghiti J., Vilgrain V., Farges O., Intracystic concentrations of tumour markers for the diagnosis of cystic liver lesions : Tumour markers for diagnosing cystic liver lesions, 10.1002/bjs.9414
  49. Shimada Mitsuo, Takenaka Kenji, Gion Tomonobu, Fujiwara Yuh, Taguchi Kenichi, Kajiyama Kiyoshi, Shirabe Ken, Sugimachi Keizo, Treatment Strategy for Patients With Cystic Lesions Mimicking a Liver Tumor : A Recent 10-Year Surgical Experience in Japan, 10.1001/archsurg.133.6.643
  50. Regev A, Large cystic lesions of the liver in adults: a 15-year experience in a tertiary center, 10.1016/s1072-7515(01)00865-1
  51. Nakajima Tohru, Sugano Isamu, Matsuzaki Osamu, Nagao Koichi, Kondo Yoichiro, Miyazaki Masaru, Wada Katsunori, Biliary cystadenocarcinoma of the liver. A clinicopathologic and histochemical evaluation of nine cases, 10.1002/1097-0142(19920515)69:10<2426::aid-cncr2820691007>3.0.co;2-3
  52. Zhang You-Lei, Yuan Lei, Shen Feng, Wang Yi, Hemorrhagic hepatic cysts mimicking biliary cystadenoma, 10.3748/wjg.15.4601
  53. Tan Y. M., Ooi London Lucien, Soo K. C., Mack P.O. P., Does laparoscopic fenestration provide long-term alleviation for symptomatic cystic disease of the liver? : Laparoscopic treatment of liver cysts, 10.1046/j.1445-2197.2002.02527.x
  54. Ishak Kamal G., Willis G. W., Cummins S. D., Bullock A. A., Biliary cystadenoma and cystadenocarcinoma.Report of 14 cases and review of the literature, 10.1002/1097-0142(197701)39:1<322::aid-cncr2820390149>3.0.co;2-p
  55. Williams David M., Vitellas Kenneth M., Sheafor Douglas, Biliary cystadenocarcinoma: seven year follow-up and the role of MRI and MRCP, 10.1016/s0730-725x(01)00453-2
  56. Soares Kevin C., Arnaoutakis Dean J., Kamel Ihab, Anders Robert, Adams Reid B., Bauer Todd W., Pawlik Timothy M., Cystic Neoplasms of the Liver: Biliary Cystadenoma and Cystadenocarcinoma, 10.1016/j.jamcollsurg.2013.08.014
  57. Buetow P C, Buck J L, Pantongrag-Brown L, Ros P R, Devaney K, Goodman Z D, Cruess D F, Biliary cystadenoma and cystadenocarcinoma: clinical-imaging-pathologic correlations with emphasis on the importance of ovarian stroma., 10.1148/radiology.196.3.7644647
  58. Dhamija Ekta, Role of contrast enhanced ultrasound in hepatic imaging, 10.7869/tg.201
  59. Dong Yi, Wang Wen-Ping, Mao Feng, Fan Mei, Ignee Andre, Serra Carla, Sparchez Zeno, Sporea Ioan, Braden Barbara, Dietrich Christoph F., Contrast enhanced ultrasound features of hepatic cystadenoma and hepatic cystadenocarcinoma, 10.1080/00365521.2016.1259652
  60. Wheeler David A., Edmondson Hugh A., Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. A clinicopathologic study of 17 cases, 4 with malignant change, 10.1002/1097-0142(19850915)56:6<1434::aid-cncr2820560635>3.0.co;2-f
  61. Davies Ward, Chow Matthew, Nagorney David, Extrahepatic Biliary Cystadenomas and Cystadenocarcinoma : Report of Seven Cases and Review of the Literature, 10.1097/00000658-199511000-00003
  62. Boytchev I, Gastroenterol Clin Biol, 23, 981 (1999)
  63. Devaney Kenneth, Goodman Zachary D., Ishak Kamal G., Hepatobiliary Cystadenoma and Cystadenocarcinoma : A Light Microscopic and Immunohistochemical Study of 70 Patients, 10.1097/00000478-199411000-00002
  64. Woods Gail L., Biliary cystadenocarcinoma: Case report of hepatic malignancy originating in benign cystadenoma, 10.1002/1097-0142(19810615)47:12<2936::aid-cncr2820471234>3.0.co;2-4
  65. Sun Yongliang, Lu Xin, Xu Yiyao, Mao Yilei, Yang Zhiying, Sang Xinting, Zhong Shouxian, Huang Jiefu, Spontaneous rupture of a giant hepatobiliary serous cystadenoma: report of a case and literature review, 10.1007/s12072-010-9221-3
  66. Yu J, Hepatogastroenterology, 57, 4515 (2010)
Bibliographic reference Frezin, Julie ; Komuta, Mina ; Zech, Francis ; Annet, Laurence ; Horsmans, Yves ; et. al. Mucin-producing hepatic cystic neoplasms: an uncommon but challenging disease often misdiagnosed and mismanaged.. In: Acta Chirurgica Belgica, Vol. 120, no. 1, p. 6-15 (2020)
Permanent URL http://hdl.handle.net/2078.1/212955