Diagnostic and Treatment Errors in Biliary Cystadenomas and Cystadenocarcinomas: Clinical Cases

Background. Biliary cystadenomas and cystadenocarcinomas are rare cystic tumors of the liver. Complicated differential diagnostics for simple cysts often leads to errors in surveillance of patients with these tumors. Cystadenoma and cystadenocarcinoma should be suspected upon detection of single or multilocular cystic neoplasms of the liver with septa and blood flow loci  in the cyst wall, especially in middle-aged women. The localization of the tumor is critical. The most common localization is segment IV of the liver. Urgent intraoperative biopsy is required to determine the extent of surgery.

Case description. Clinical observations with analysis of the examination and treatment data of two female patients aged 38 and 56 were presented. Both clinical observations illustrate the underestimation of the preoperative examination data that served as a ground for diagnosis of liver cysts with inadequate extent of surgery. In the first case, the resection was incomplete, and, as such, the biliary cystadenoma recurred in the resection area, the capsule of the neoplasm was ruptured and an encysted fluid collection was formed. In the second case, lack of histological examination of the excised neoplasm, due to confidence in its morphological verification as a cyst, resulted in cystadenoma recurrence in the resection zone with metastasis to the contralateral lobe of the liver.

Conclusion. Hepatic cystadenomas and cystadenocarcinomas are often misdiagnosed as simple cysts. These tumors should be suspected in central localization of the tumor in the liver, especially in young women. The clinical and instrumental symptomatology and radiological semiotics of the disease require careful evaluation. The recurrence of a cystic lesion in the resection zone in a patient previously operated for a hepatic cyst serves as an additional signal for detecting biliary cystadenoma. Rational strategy for surgical management of cystic liver lesions should include hepatectomy within healthy tissues (both anatomical and atypical) with mandatory intraoperative ultrasound and urgent histological examinations.

1. Bertino G., Ardiri A., Demma S., Giuseppe Calvagno S., Toro A., Basile E., Campagna D., Ferraro G., Frazzetto E., Proiti M., Malaguarnera G., Bertino N., Malaguarnera M., Malaguarnera M., Amaradio M.D., Pricoco G., Di Carlo I. Rare benign tumors of the liver: still rare? J. Gastrointest. Cancer. 2014; 45(2): 202– 217. DOI: 10.1007/s12029-014-9580-4

2. Adam Y.G., Nonas C.J. Hepatobiliary cystadenoma. South Med. J. 1995; 88(11): 1140–1143. DOI: 10.1097/00007611-199511000-00011

3. Reid-Lombardo K.M., Khan S., Sclabas G. Hepatic cysts and liver abscess. Surg. Clin. North Am. 2010; 90(4): 679–697. DOI: 10.1016/j.suc.2010.04.004

4. Lantinga M.A., Gevers T.J., Drenth J.P. Evaluation of hepatic cystic lesions. World J. Gastroenterol. 2013; 19(23): 3543–3554. DOI: 10.3748/wjg.v19.i23.3543

5. Emre A., Serin K.R., Ozden I., Tekant Y., Bilge O., Alper A., Güllüoğlu M., Güven K. Intrahepatic biliary cystic neoplasms: Surgical results of 9 patients and literature review. World J. Gastroenterol. 2011; 17(3): 361–365. DOI: 10.3748/wjg.v17.i3.361

6. Vogt D.P., Henderson J.M., Chmielewski E. Cystadenoma and cystadenocarcinoma of the liver: a single center experience. J. Am. Coll. Surg. 2005; 200(5): 727–733. DOI: 10.1016/j.jamcollsurg.2005.01.005

7. Fragulidis G.P., Vezakis A.I., Konstantinidis C.G., Chondrogiannis K.K., Primetis E.S., Kondi-Pafiti A., Polydorou A.A. Diagnostic and Therapeutic Challenges of Intrahepatic Biliary Cystadenoma and Cystadenocarcinoma: A Report of 10 Cases and Review of the Literature. Int. Surg. 2015; 100(7–8): 1212–1219. DOI: 10.9738/INTSURG-D-15-00025.1

8. van Aerts R.M.M., van de Laarschot L.F.M., Banales J.M., Drenth J.P.H. Clinical management of polycystic liver disease. J. Hepatol. 2018; 68(4): 827–837. DOI: 10.1016/j.jhep.2017.11.024

9. Lantinga M.A., Gevers T.J., Drenth J.P. Evaluation of hepatic cystic lesions. World J. Gastroenterol. 2013; 19(23): 3543–3554. DOI: 10.3748/wjg.v19.i23.3543

10. Nguyen H., Nguyen N.C., Thieu T.T.M., Luong T.H., Tran N.M., Nguyen D.H., Nguyen A.K. Complete resection for a giant recurrent biliary cystadenoma: A surgical case report and review of literature. Ann. Med. Surg. (Lond). 2022; 78: 103785. DOI: 10.1016/j.amsu.2022.103785

11. Soares K.C., Arnaoutakis D.J., Kamel I., Anders R., Adams R.B., Bauer T.W., Pawlik T.M. Cystic neoplasms of the liver: biliary cystadenoma and cystadenocarcinoma. J. Am. Coll. Surg. 2014; 218(1): 119–128. DOI: 10.1016/j.jamcollsurg.2013.08.014

12. Mavilia M.G., Pakala T., Molina M., Wu G.Y. Differentiating Cystic Liver Lesions: A Review of Imaging Modalities, Diagnosis and Management. J. Clin. Transl. Hepatol. 2018; 6(2): 208–216. DOI: 10.14218/JCTH.2017.00069

13. Cnossen W.R., Drenth J.P. Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management. Orphanet. J. Rare. Dis. 2014; 9: 69. DOI: 10.1186/1750-1172-9-69

14. Li X., Zhang J.L., Wang Y.H., Song S.W., Wang F.S., Shi R., Liu Y.F. Hepatobiliary cystadenoma and cystadenocarcinoma: a single center experience. Tumori. 2013; 99(2): 261–265. DOI: 10.1177/030089161309900223

15. Lewin M., Mourra N., Honigman I., Fléjou J.F., Parc R., Arrivé L., Tubiana J.M. Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma. Eur. Radiol. 2006; 16(2): 407–413. DOI: 10.1007/s00330-005-2822-x

16. 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(4): 926–936. DOI: 10.1016/j.surg.2004.06.031

17. Halefoglu A.M., Oz A. Primary undifferentiated embryonal sarcoma of the liver misdiagnosed as hydatid cyst in a child: a case report and review of the literature. JBR-BTR. 2014; 97(4): 248–250. DOI: 10.5334/jbr-btr.91

18. Xu M.Y., Shi X.J., Wan T., Liang Y.R., Wang H.G., Zhang W.Z., He L., Chen M.Y., Lyu S.C., Zhang W.W., Li H.X. Clinicopathological characteristics and prognostic factors of intrahepatic biliary cystadenocarcinoma. Chin. Med. J. (Engl). 2015; 128(9): 1177–1183. DOI: 10.4103/0366-6999.156108

19. Nakajima T., Sugano I., Matsuzaki O., Nagao K., Kondo Y., Miyazaki M., Wada K. Biliary cystadenocarcinoma of the liver. A clinicopathologic and histochemical evaluation of nine cases. Cancer. 1992; 69(10): 2426–2432. DOI: 10.1002/1097-0142(19920515)69:10<2426::aid-cncr2820691007>3.0.co;2-3

20. Vogt D.P., Henderson J.M., Chmielewski E. Cystadenoma and cystadenocarcinoma of the liver: a single center experience. J. Am. Coll. Surg. 2005; 200(5): 727–733. DOI: 10.1016/j.jamcollsurg.2005.01.005

21. Jwa E.K., Hwang S. Clinicopathological features and post-resection outcomes of biliary cystadenoma and cystadenocarcinoma of the liver. Ann. Hepatobiliary Pancreat. Surg. 2017; 21(3): 107–113. DOI: 10.14701/ahbps.2017.21.3.107