Biliyer Kistadenomlar
Özet
Biliyer kistadenomlar, karaciğerin safra epitelinden köken alan, multiloküler yapıda ve nadir görülen iyi huylu tümörlerdir. Genellikle 40-50 yaş arası orta yaşlı kadınlarda, erkeklere kıyasla 4:1 oranında daha sık saptanırlar ve çoğunlukla sağ lobda yerleşirler. Embriyonik safra kanallarının ektopik kalıntılarından köken aldığı düşünülen bu lezyonlar, mukus salgılayan küboidal veya kolumnar epitel ve estrojen/progesteron reseptörleri içeren ovaryen tip stroma ile karakterizedir. Klinik bulguları sıklıkla spesifik olmamakla birlikte; karın ağrısı, şişkinlik ve bulantı gibi semptomlar tümör büyüdükçe ortaya çıkabilir. Tanıda ultrason, BT ve MRG birbirini tamamlayıcı ve kritik öneme sahip görüntüleme yöntemleridir. Tümörün %20'ye varan oranlarda biliyer kistadenokarsinoma dönüşme riski (malign transformasyon) ve yüksek nüks eğilimi bulunduğundan, perkütan aspirasyon veya unroofing gibi yetersiz girişimlerden kaçınılmalıdır. Lokal nüksü ve kötü huylu dönüşümü tamamen önlemek amacıyla tümörün cerrahi olarak tam rezeksiyonu (enükleasyon veya lobektomi) zorunludur. Başarılı cerrahi tedavi sonrası nüks oranları son derece düşük (%5-10) ve uzun dönem sağkalım %90'ın üzerindedir; ancak hastaların postoperatif dönemde ilk yıl 6 aylık, ardından yıllık görüntüleme yöntemleriyle yakın takibi gereklidir.
Biliary cystadenomas are rare, benign, multilocular cystic tumors originating from the biliary epithelium of the liver. They are predominantly observed in middle-aged women (40-50 years old) with a 4:1 female-to-male ratio, mostly located in the right hepatic lobe. Hypothesized to arise from ectopic remnants of embryonic bile ducts, these lesions are lined with mucus-secreting cuboidal or columnar epithelium and characterized by an ovarian-like stroma expressing estrogen and progesterone receptors. Clinical presentation is highly variable and non-specific; symptoms such as abdominal pain, bloating, and nausea typically surface only when the tumor reaches a significant size causing compressive effects. Ultrasound, CT, and MRI serve as complementary and crucial imaging modalities for preoperative diagnosis. Due to a high malignant transformation risk of up to 20% into biliary cystadenocarcinoma and a strong propensity for recurrence, incomplete interventions like percutaneous aspiration or unroofing are inappropriate. Complete surgical resection via enucleation or lobectomy is mandatory to prevent local recurrence and malignant evolution. Following radical resection, recurrence rates are remarkably low (5-10%) with long-term survival exceeding 90%; nevertheless, close postoperative follow-up with imaging every 6 months for the first year and annually thereafter remains imperative.
Referanslar
Miguel A Hernandez Bartolome, Sagrario Fuerte Ruiz, Israel Manzanedo. Biliary cystadenoma. World Journal of Gastroeneterology. 2009 Jul28;15(28):3573-3575. Published online Jul 28.
Ho Gak Kim. Biliary cystic neoplasm: biliary cystadenoma and biliary cystadenocarcinoma. Review. Korean J Gastroenterol. 2006 Jan;47(1):5-14
Davies W, Chow M, Nagorney D. Extrahepatic biliary cystadenomas and cystadenocarcinoma. Report of seven cases and review of the literature. Ann Surg. 1995; 222:619–625.
Short WF, Nedwich A, Levy HA, Howard JM. Biliary cystadenoma. Report of a case and review of the literature. Arch Surg. 1971; 102:78–80.
Lewis WD, Jenkins RL, Rossi RL, et al. Surgical treatment of biliary cystadenoma. A report of 15 cases. Arch Surg. 1988; 123:563–568
Vogt DP, Henderson JM, Chmielewski E. Cystadenoma and cystadenocarcinoma of the liver: a single center experience. J Am Coll Surg. 2005; 200:727–733.
Devaney K, Goodman ZD, Ishak KG. Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol. 1994; 18:1078–1091.
Mortele KJ, Ros PR. Benign liver neoplasms. Clin Liver Dis. 2002; 6:119–145.
Williams DM, Vitellas KM, Sheafor D. Biliary cystadenocarcinoma: seven year follow-up and the role of MRI and MRCP. Magn Reson Imaging. 2001; 19:1203–1208.
Wheeler DA, Edmondson HA. Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. A clinicopathologic study of 17 cases, 4 with malignant change. Cancer. 1985; 56:1434–1445.
Akwari OE, Tucker A, Seigler HF, Itani KM. Hepatobiliary cystadenoma with mesenchymal stroma. Ann Surg. 1990;2 11:18
Ratti F, Paganelli M, Cipriani F. Biliary cistadenoma: short and long-term outcome after radical hepatic resection. Updates Surg. 2012 Mar. 64(1):13-8.
Ishak KG, Willis GW, Cummins SD, Bullock AA. Biliary cystadenoma and cystadenocarcinoma: report of 14 cases and review of the literature. Cancer. 1977; 39:322–338.
Choi BI, Lim JH, Han MC, et al. Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. Radiology. 1989; 171:57–
Cheung YK, Chan FL, Leong LL, et al. Biliary cystadenoma and cystadenocarcinoma: some unusual features. Clin Radiol. 199143:183–185.
Zen Y, Pedica F, Patcha VR, et al. Mucinous cystic neoplasms of the liver: a clinicopathological study and comparison with intraductal papillary neoplasms of the bile duct. Mod Pathol. 2011; 24:1079–1089.
Ferrell L. Benign and malignant tumors of the liver. In: Odze JG RD, Crawford JM, editors. Surgical Pathology of the GI tract, Liver, Biliary Tract and Pancreas. Saunders; Philadelphia: 2004. pp. 1015–1016.
Abdul-Al HM, Makhlouf HR, Goodman ZD. Expression of estrogen and progesterone receptors and inhibin-alpha in hepatobiliary cystadenoma: an immunohistochemical study. Virchows Arch. 2007; 450:691–697.
Daniels JA, Coad JE, Payne WD, et al. Biliary cystadenomas: hormone receptor expression and clinical management. Dig Dis Sci. 2006; 51:623–628.
Ahanatha Pillai S, Velayutham V, Perumal S, et al. Biliary cystadenomas: a case for complete resection. HPB Surg. 2012; 2012:501705.
Suyama Y, Horie Y, Suou T, et al. Oral contraceptives and intrahepatic biliary cystadenoma having an increased level of estrogen receptor. Hepatogastroenterology. 1988; 35:171–174.
Soares KC, Arnaoutakis DJ, Kamel I, Anders R, Adams RB, Bauer TW, Pawlik TM. Cystic neoplasms of the liver: biliary cystadenoma and cystadenocarcinoma. J Am Coll Surg. 2014 Jan;218(1):119-28.
Hai S, Hirohashi K, Uenishi T, et al. Surgical management of cystic hepatic neoplasms. J Gastroenterol. 2003; 38:759–764.
Sudo Y, Harada K, Tsuneyama K, et al. Oncocytic biliary cystadenocarcinoma is a form of intraductal oncocytic papillary neoplasm of the liver. Mod Pathol. 2001; 14:1304–1309.
Erdogan D, Busch OR, Rauws EA, van Delden OM, Gouma DJ, van-Gulik TM. Obstructive jaundice due to hepatobiliary cystadenoma or cystadenocarcinoma. World J Gastroenterol. 2006 Sep 21;12(35):5735-8.
Xu M, Shi X, Wan T, Wang H, He L, Chen M, Liang Y, Dong J. [Comparison of clinicopathological characteristics and prognosis of intrahepatic biliary cystic tumor between male and female patients]. Zhonghua Yi Xue Za Zhi. 2015 Oct 13;95(38):3110-4.
Fukunaga N, Ishikawa M, Ishikura H, et al. Hepatobiliary cystadenoma exhibiting morphologic changes from simple hepatic cyst shown by 11-year follow up imagings. World J Surg Oncol. 2008 Dec 11. 6:129
Choi HK, Lee JK, Lee KH, et al. Differential diagnosis for intrahepatic biliary cystadenoma and hepatic simple cyst: significance of cystic fluid analysis and radiologic findings. J Clin Gastroenterol. 2010 Apr. 44(4):289-93.
Zhang YL, Yuan L, Shen F, Wang Y. Hemorrhagic hepatic cysts mimicking biliary cystadenoma. World J Gastroenterol. 2009 Sep 28. 15(36):4601-3.
Mantke R, Ridwelski K, Rocken C, Pross M, Schulz HU, Lippert H. [Hepatobiliary cystadenoma] Chirurg. 2001; 72:277–280.
Terada T, Nakanuma Y, Ohta T, et al. Mucin-histochemical and immunohistochemical profiles of epithelial cells of several types of hepatic cysts. Virchows Arch A Pathol Anat Histopathol. 1991; 419:499–504.
Fuks D, Voitot H, Paradis V, et al. Intracystic concentrations of tumour markers for the diagnosis of cystic liver lesions. Br J Surg. 2014 Mar. 101(4):408-16.
Kovacs MD, Sheafor DH, Burchett PF, Picard MM, Hardie AD. Differentiating biliary cystadenomas from benign hepatic cysts: Preliminary analysis of new predictive imaging features. Clin Imaging. 2018 May- Jun. 49:44-7.
Ahmad Z, Uddin N, Memon W, Abdul-Ghafar J, Ahmed A. Intrahepatic biliary cystadenoma mimicking hydatid cyst of liver: a clinicopathologic study of six cases. J Med Case Rep. 2017 Nov 10. 11(1):317
Xu HX, Lu MD, Liu LN, et al. Imaging features of intrahepatic biliary cystadenoma and cystadenocarcinoma on B-mode and contrast-enhanced ultrasound. Ultraschall Med. 2012 Dec. 33(7): E241-9.
Ariff A, Hassan H, John G. Biliary cystadenoma - computed tomography findings. Malays J Med Sci. 2002 Jan; 9(1):49-51.
Filippi de la Palavesa MM, Vasilescu C, Habersetzer F, Roy C. Biliary cystadenocarcinoma: sonographic and cytologic findings. J Clin Ultrasound. 1999 May; 27(4):210-2.
Korobkin M, Stephens DH, Lee JK, Stanley RJ, Fishman EK, Francis IR, Alpern MB, Rynties M. Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. AJR Am J Roentgenol. 1989 Sep; 153(3):507-11.
Thomas KT, Welch D, Trueblood A, Sulur P, Wise P, Gorden DL, Chari RS, Wright JK Jr, Washington K, Pinson CW. Effective treatment of biliary cystadenoma. Ann Surg. 2005 May; 241(5):769-73; discussion 773-5.
Lewin M, Mourra N, Honigman I, Fléjou JF, Parc R, Arrivé L, Tubiana JM. Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma.. Eur Radiol. 2006 Feb; 16(2):407-13.
Delis SG, Touloumis Z, Bakoyiannis A, et al. Intrahepatic biliary cystadenoma: a need for radical resection. Eur J Gastroenterol Hepatol. 2008; 20:10–14.
Lewin M, Mourra N, Honigman I, et al. Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma. Eur Radiol. 2006; 16:407–413.
Billington PD, Prescott RJ, Lapsia S. Diagnosis of a biliary cystadenoma demonstrating communication with the biliary system by MRI using a hepatocyte-specific contrast agent. Br J Radiol. 2012; 85: e35–36.
Lewin M, Mourra N, Honigman I, et al. Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma. Eur Radiol. 2006; 16:407–413.
Debenes B, Pauwels A, Levy VG Kyste solitaire et polikystose hepatique de l'adulte cystadenome hepatique. Ed Tecinniques Encyc1 Med-Chir Paris France Hepatologie. 1992. p. 18.
Vyas S, Markar S, Ezzat T, et al. Hepato-biliary cystadenoma with intraductal extension: unusual cause of obstructive jaundice. J Gastrointest Cancer. 2011
Sutton CD, White SA, Berry DP, Dennison AR. Intrahepatic biliary cystadenoma causing luminal common bile duct obstruction. Dig Surg. 2000; 17:297–299.
Erdogan D, Kloek J, Lamers WH, et al. Mucinous cystadenomas in liver: management and origin. Dig Surg. 2010; 27:19–23.
Delis SG, Touloumis Z, Bakoyiannis A, et al. Intrahepatic biliary cystadenoma: a need for radical resection. Eur J Gastroenterol Hepatol. 2008; 20:10–14.
Teoh AY, Ng SS, Lee KF, Lai PB. Biliary cystadenoma and other complicated cystic lesions of the liver: diagnostic and therapeutic challenges. World J Surg. 2006; 30:1560–1566.
Emre A, Serin KR, Ozden I, et al. Intrahepatic biliary cystic neoplasms: Surgical results of 9 patients and literature review. World J Gastroenterol.
Koffron A, Rao S, Ferrario M, Abecassis M. Intrahepatic biliary cystadenoma: role of cyst fluid analysis and surgical management in the laparoscopic era. Surgery. 2004; 136:926–936
Manouras A, Lagoudianakis E, Alevizos L, et al. Laparoscopic fenestration of multiple giant biliary mucinous cystadenomas of the liver. World J Gastroenterol. 2008; 14:4257–4259.
Romagnoli R, Patrono D, Paraluppi G, et al. Liver transplantation for symptomatic centrohepatic biliary cystadenoma. Clin Res Hepatol Gastroenterol. 2011; 35:408–413.
Gao J, Zheng J, Cai J, et al. Differentiation and management of hepatobiliary mucinous cystic neoplasms: a single centre experience for 8 years. BMC Surg. 2021 Mar 20. 21(1):146.
Veroux M, Fiamingo P, Cillo U, Tedeschi U, Brolese A, Veroux P, Basso S, Buffone A, D’Amico DF. Cystadenoma and laparoscopic surgery for hepatic cystic disease: a need for laparotomy? Surg Endosc. 2005; 19:1077–1081
Fiamingo P, Veroux M, Cillo U, Basso S, Buffone A, D’Amico DF. Incidental cystadenoma after laparoscopic treatment of hepatic cysts: which strategy? Surg Laparosc Endosc Percutan Tech. 2004; 14:282–284.
Gamblin TC, Holloway SE, Heckman JT, Geller DA. Laparoscopic resection of benign hepatic cysts: a new standard. J Am Coll Surg. 2008 Nov. 207(5):731-6.
Abu Hilal M, Di Fabio F, Teng MJ, Godfrey DA, Primrose JN, Pearce NW. Surgical management of benign and indeterminate hepatic lesions in the era of laparoscopic liver surgery. Dig Surg. 2011. 28(3):232-6.
Lauffer JM, Baer HU, Maurer CA, et al. Biliary cystadenocarcinoma of the liver: the need for complete resection. Eur J Cancer. 1998; 34:1845–1851.[
Ratti F, Ferla F, Paganelli M, Cipriani F, Aldrighetti L, Ferla G. Biliary cystadenoma: short- and long-term outcome after radical hepatic resection. Updates Surg. 2012 Mar. 64(1):13-8.
