Biliary cystadenoma –an enigmatic diagnosis still?

Original Article

Biliary cystadenoma –an enigmatic diagnosis still?

*Corresponding author:Amarjothi J M V ,500, Tower block II, Rajiv Gandhi Government General Hospital, Chennai, Tamilnadu, India; E-mail: drmosesvikramamarjothi@hotmail.com

Abstract

Aim: Biliary cystadenomas are rare cystic lesions involving the liver. Due to their rarity and plethora of presentations, a primary diagnosis of biliary cystadenoma is not common place and suboptimal procedures like fenestration or cyst aspiration are done leading to early recurrences. The aim of the article is to show how biliary cystadenomas are misdiagnosed as other lesions, primarily as simple cysts leading to a difference in management. An accurate diagnosis of biliary cystadenoma is a must in order to tailor effective treatment.

Methods: This is a retrospective study of    cases of histologically proven biliary cystadenoma between 2010 and 2017, its clinical presentation, previous treatment and surgical treatment offered in our institution. The patients were also followed up rigorously to detect earlyrecurrence.

Results: A series of eight cases of biliary cystadenoma were operated in our institution between 2010 and 2017. Out of the eight, seven were female (87.5%) with the mean age of presentation was 47 years (range—28—61 yrs). All the patients were symptomatic   with the most common presentation being   abdominal pain and discomfort. (n=7, )87.5% were found to have had history of previous intervention/surgery before referral. Most common procedures included fluid aspiration(n=7,87.5%), laparoscopic fenestration(n=3,37.5%) and previous open excision (n=1, 12.5%). Most of the above procedures were done with a mistaken diagnosis of simple cyst(n=6) Seven patients(n=7, 87.5%)  had  enhancing  cystic lesion involving the left lobe with  a mean size of 12.75 cm (range-8-20 cm)  and clear  fluid with raised CA 19-9 levels  more than 1000 IU/ml in  (n=7,87.5%).The procedures offered included left hepatectomy (n=2,25%) and  complete  excision (n=6,75%). Out of the sixpatients undergoing complete excision, (n=3,50%) developed bile leak which was managed conservatively. In the two patients undergoing hepatectomy, one(n=1,50%) developed bile leak which was managed conservatively.  All the patients are on follow up (12-28 months) with no recurrence.

Introduction

Biliary cystadenoma are rare cystic neoplasms (<5%) of the liver [1]. They arise from the biliary epithelium and are most commonlymultiloculated, cystic lesions lined by cuboidal or columnar epithelium with or without ovarian stroma. [2]and are seen in the intrahepatic location (85%).

The exact etiology is incompletely understood. As the lesions are more common among middle aged female patients, a hormonal etiology involving estrogen is plausible [3,4]. Clinical features may be variable and may range   from   asymptomatic   to abdominal pain, distension or rarely jaundice. The differential diagnosis for intrahepatic   biliary cystadenomas includes a myriad of liver cysts like simple cysts, hydatid cysts, atypical liver abscess and degeneration of malignancy [1].

Investigations

Radiological investigations are not specific to diagnose biliary cystadenomas or cystadenocarcinomas     due to lack of specific features and are commonly misdiagnosed as liver cysts (simple or hydatid). However, features like internal septations, internal echoes, wall enhancement, hypovascular wall, thickened wall, mural nodules andcalcifications mayoccur [1]. Thickened wall, mural nodules and calcifications may also be seen in case of cystadenocarcinomas and are not definite markers of malignancy. The incidence of malignant transformation is around 30% [5] and there are no reliable features to diagnose biliary cystadenocarcinomas from biliary cystadenomas.

Ultrasound is highly sensitive to diagnose the fine septae but is highly dependent on accessible location and operator expertise [6]. CT done can detect calcifications   and   the portal venous phase is needed to diagnose the septate accurately. MRI in the T2 [6] is best to diagnose the fine septae   and intracystic bleeding. Cysts with a high mucin content can be diagnosed on T1. DWI (Diffusion weighted imaging) is helpful to diagnose malignant transformation.

In a retrospective study of 20 cases of preoperative diagnosis of    biliary cystadenomas, [ 1] 6 were histologically proven after surgery with a diagnostic accuracy of only 30%. 50% of the preoperatively misdiagnosed   biliary cystadenoma(n=5) were actually livercysts (simple-3, hemorragic-1, infected -1).Two large cysts in the study   which were thought to be simple cysts were persistently recurrent to various modalities like alcohol sclerotherapy and laparoscopic fenestration and ultimately required complete removal of the cyst whichwere histologically positive for biliary cystadenoma reflecting on the need for complete cyst wall excision to adequately treatmisdiagnosed biliary cystadenoma. Another study by Thomas et al [7] gave a very high diagnostic accuracy of 95% of biliary cystadenomas but the exact diagnostic features employed are unknown.s.CA19-9 levels may be elevated [8] but is not specific. FNAC and frozen section are not accurate due to high false negative rates butmay reveal clear fluid with high ca 19-9 levels and mildly elevated CEA levels [9]. The raise in fluid CA 19-9 levels is not diagnostic of biliary cystadenoma as it is seen with malignant transformation also [1]. It is not known whether the levels of CA19-9 correlate with malignancy and no cut off levels have been describedso far.Histology of the excised specimen is diagnostic for biliary cystadenoma with some showing ovarian stroma. It is to be noted that those associated with ovarian stroma are associated with better prognosis though having higher predisposition for malignant transformation [10].

Treatment

Treatment for biliary cystadenoma stresses on complete excision of cyst wall to prevent recurrence [11]. Enucleation of the cyst wall may be preferable to liver resection as it is associated with lower morbidity [12]. For lesions not amenable to complete excision, partial excision with adequate treatment of the remnant cyst wall must be carried out earnestly to prevent recurrence [6]. Liver transplantation may be last and only resort for large unresectable biliary cystadenomas [13,14].

Figure 1: Cect showing minimally enhancing cystic lesion which turned out to be cystadenoma

 

Figure 2: MRI showing hyperintense lesion which turned to be cystadenoma 

Prognosis  

The prognosis for completely excised biliary cystadenomas is excellent emphasizing that nothing but complete removal of the cyst wall is necessary to prevent recurrence and malignant transformation.

Conclusion

It is to be noted that a high index of diagnostic suspicion for biliary cystadenoma must be entertained in all preoperative diagnosis of simple liver cysts especially those with high fluid ca 19-9 levels. Treatment of such cysts should be tailored such that those which are undergoing surgical excision (enucleation or liver resection) should undergo complete excision of their wall in order to prevent recurrence.

Financial disclosures-Nil

Conflict of interest-Nil

References

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