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Table of Contents   
CASE REPORT
Year : 2023  |  Volume : 28  |  Issue : 6  |  Page : 532-536
 

Biliary cystadenoma in a child: A rare entity


1 Department of General Surgery, Darbhanga Medical College, Darbhanga, Bihar; Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
3 Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha; Department of Pediatric Surgery, All India Institute of Medical Sciences, Mangalagiri, Andhra Pradesh, India

Date of Submission06-Mar-2023
Date of Decision04-Aug-2023
Date of Acceptance09-Aug-2023
Date of Web Publication02-Nov-2023

Correspondence Address:
Subrat Kumar Sahoo
Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_52_23

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   Abstract 


Biliary cystadenoma, a rare potentially malignant hepatic cystic lesion, is characterized by multiloculations and septations. It is common in middle-aged females (about 5% of nonparasitic liver cysts); only 12 cases are described in children. We report a rare case of hepatic biliary cystadenoma in a 3-year-old girl, with a gradually increasing lump in the right upper abdomen. Complete excision with a healthy liver margin was done.


Keywords: Biliary cystadenoma, hepatic mass, mucinous cystadenoma


How to cite this article:
Ahmad S, Sahoo SK, Manekar AA, Narahari J, Tripathy BB, Sable M, Mohanty MK. Biliary cystadenoma in a child: A rare entity. J Indian Assoc Pediatr Surg 2023;28:532-6

How to cite this URL:
Ahmad S, Sahoo SK, Manekar AA, Narahari J, Tripathy BB, Sable M, Mohanty MK. Biliary cystadenoma in a child: A rare entity. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Nov 28];28:532-6. Available from: https://www.jiaps.com/text.asp?2023/28/6/532/389327





   Introduction Top


First described by Hueter (1887), biliary cystadenoma is a rare hepatic cystic neoplasm accounting for <5% of nonparasitic hepatic cysts.[1] About 200 cases are reported with a female preponderance (90%).[2] Though often benign, blilary cystadenomas have a malignant potential, with a secondary malignant transformation rate of 20%.[2],[3],[4],[5] Aberrant bile ducts or primitive hepatobiliary stem cells are probable sites of origin.[6],[7] Most have an intrahepatic origin (90%), but few have an extrahepatic origin as well.[2],[8],[9] Both ultrasound and computed tomography (CT) are complimentary for diagnosis; however, radiological differentiation from mesenchymal hamartoma is difficult.[2] Complete excision and thorough histopathological examination are the gold standard for specific diagnosis.[1]


   Case Report Top


A 3-year-old girl presented with a gradually increasing upper abdominal lump for about a year, associated with anorexia, failure to thrive, and occasional vomiting. There was no jaundice, hematemesis, or melena. Her weight was below the 3rd percentile for her age. The general survey was, otherwise, unremarkable. Abdominal examination revealed a 12.5 cm × 10 cm mass in the right hypochondrium and lumbar and umbilical regions. It was nontender, smooth-surfaced, with a well-defined margin, and continuous with the liver. There was no free fluid in the abdomen. Complete blood count, liver and renal function tests, and tumor markers (alpha-fetoprotein, lactate dehydrogenase, and beta-human chorionic gonadotropin) were normal.

Ultrasonography (USG) revealed a 13.6 cm × 6.5 cm cystic mass with echogenic septations and internal echoes without any solid component or calcification. On contrast-enhanced computed tomography (CT), a well-defined multiloculated and lobulated thick-walled exophytic cystic mass was seen arising from the right lobe of the liver (segment VI, 13.1 cm × 7.6 cm × 12.8 cm), with thick enhancing septations and multiple dilated vessels on the surface [Figure 1]. The feeding artery was from the right branch of the hepatic artery and drained into the right branch of the portal vein. There was a significant mass effect on the adjacent viscera. All major intra-abdominal vessels were normal. There were no calcifications, lymphadenopathy, or free fluid.
Figure 1: Contrast-enhanced computed tomography of the abdomen and pelvis, coronal section, showing the large cystic mass arising from segment 6 of the liver. S - thick enhancing septa

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Thinking it to be benign, complete excision was planned. Per-operatively, the 10 cm × 8 cm × 10 cm bosselated mass was found arising from the right hepatic lobe, free from the surrounding structures [Figure 2], and a complete excision with a 1-cm rim of the normal liver was done. Surgery was well-tolerated, oral feeds started on POD2, and the child was discharged on POD5.
Figure 2: Intraoperative photograph of excision of the mass from the under surface of the liver (M - mass, VI - segment VI of the liver, GB - gallbladder, TC - transverse colon)

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On gross pathology, it was a well-circumscribed mass with multiple cysts (6–90 mm) containing thick, yellowish mucinous fluid. No solid areas, calcifications, or papillary excrescences were identified. Microscopy revealed cysts lined by simple columnar epithelium and surrounding fibrocollagenous tissue with mixed inflammatory infiltrate and focal collection of hemosiderin-laden macrophages [Figure 3]a and [Figure 3]b. The epithelial cells showed clear cytoplasm [Figure 3]c. Cords of hepatocytes, biliary ducts, and nerve twigs were seen entrapped in the fibrous capsule peripherally. There was no parasitic structure, granuloma, necrosis, dysplasia, or evidence of malignancy. The final histological diagnosis of benign biliary cystadenoma was rendered.
Figure 3: Microphotograph showing (a) a cystic lesion lined by a single layer of cuboidal to columnar epithelium with underlying fibrocollagenous tissue (H and E, ×200); (b) The cyst wall shows the absence of ovarian stroma (H and E, ×400); (c) At places, the epithelium shows intracytoplasmic mucin (H and E, ×400)

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   Discussion Top


Biliary cystadenoma is a slow-growing benign lesion; thus, presents late. It is common in middle-aged females.[2] Only 12 pediatric cases are reported, the smallest being an 8-month-old girl.[13] However, lately, there is a rise in pediatric cases possibly due to the improvement in imaging.[7],[12] They commonly present as multiloculated cysts, but rarely can be unilocular.[4] The exact pathogenesis is unknown; however, hormonal influence, congenital biliary tract anomalies, and aberrant growth of hepatobiliary progenitors are postulated.[13],[14] Biliary cystadenomas are usually insidious or asymptomatic or present with vague symptoms of adjacent visceral compression.[13] Intrahepatic lesions are more common than biliary lesions; most arise from the right lobe [Table 1].[15],[16]
Table 1: Pediatric biliary atresia cases reported in literature

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USG shows an anechoic lesion with multiloculations and septations (>85%) or a “cyst-within-cyst” appearance (>50%). Less commonly, irregular cyst walls, papillary projections, or calcifications may be seen.[12] CT compliments ultrasound in establishing the nature of the mass; however, exact differentiation between benign and malignant is difficult radiologically.[2] A jaundiced child may require magnetic resonance cholangiopancreatography. Cyst fluid can be clear or mucinous; however, blood-stained fluid indicates malignancy (cystadenocarcinoma).[4] Rarely, it is bile-stained, purulent, proteinaceous, or gelatinous. The septa within the cyst may rarely show calcification.[10]

Histologically, the lining may be cuboidal or nonciliated columnar, with basal nuclei and mucin-containing cytoplasm.[2],[16],[17] The epithelium may contain variable dysplasia. Usually, three histological subtypes are identified; ovarian-like mesenchymal stroma, nonovarian-like stroma with the absence of mesenchyme, and eosinophilic epithelial lining in the absence of mesenchyme.[13] The first variety may have an indolent course; however, the subtypes lacking mesenchymal stroma tend to be more aggressive, higher propensity for malignant transformation, and have poor prognosis.[5],[7],[10],[11]

The gold-standard treatment is a complete excision with clear margins due to the risk of recurrence and later malignant transformation.[2] The liver-sparing procedures have >60% rate of recurrence.[16] The rate of malignant transformation in adults is 20%, although not described in children.[2]

Tumor markers, cancer antigen 19-9 and cancer embryonic antigen, can be elevated in cystic lesions of the liver, being normally expressed in the biliary epithelium and bile ducts.[18] However, their utility for diagnosis in biliary cystadenoma remains controversial.[2] A more specific marker for mucinous hepatic lesion is tumor-associated glycoprotein, measured in cystic fluid before surgery. This is not expressed by normal biliary cells either at the baseline or during inflammation.[19]


   Conclusion Top


Biliary cystadenoma is a rare benign tumor in middle-aged females, even rare in children. Our case is only the 13th case of pediatric biliary cystadenoma as found in the literature. The presentation is often nonspecific; conventional imaging may be inconclusive and misleading. It should always be considered a differential diagnosis of pediatric hepatic mass. Preoperative estimation of cyst fluid tumor-associated glycoprotein level may provide a valuable adjunct to diagnosis; however, its utility has to be further evaluated. Although benign, the lesion bears a risk of recurrence and future malignant transformation. Hence, resection with an adequate margin is the gold-standard treatment. Follow-up with abdominal ultrasound will help identify recurrence in histologically proven cystadenomas.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
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.  Back to cited text no. 1
    
2.
Soares KC, Arnaoutakis DJ, Kamel I, Anders R, Adams RB, Bauer TW, et al. Cystic neoplasms of the liver: Biliary cystadenoma and cystadenocarcinoma. J Am Coll Surg 2014;218:119-28.  Back to cited text no. 2
    
3.
Tsiftsis D, Christodoulakis M, de Bree E, Sanidas E. Primary intrahepatic biliary cystadenomatous tumors. J Surg Oncol 1997;64:341-6.  Back to cited text no. 3
    
4.
Ishak KG, Willis GW, Cummins SD, Bullock AA. Biliary cystadenoma and cystadenocarcinoma: Report of 14 cases and review of the literature. Cancer 1977;39:322-38.  Back to cited text no. 4
    
5.
Devaney K, Goodman ZD, Ishak KG. Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol 1994;18:1078-91.  Back to cited text no. 5
    
6.
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;56:1434-45.  Back to cited text no. 6
    
7.
Vogt DP, Henderson JM, Chmielewski E. Cystadenoma and cystadenocarcinoma of the liver: A single center experience. J Am Coll Surg 2005;200:727-33.  Back to cited text no. 7
    
8.
Thomas KT, Welch D, Trueblood A, Sulur P, Wise P, Gorden DL, et al. Effective treatment of biliary cystadenoma. Ann Surg 2005;241:769-73.  Back to cited text no. 8
    
9.
Manouras A, Markogiannakis H, Lagoudianakis E, Katergiannakis V. Biliary cystadenoma with mesenchymal stroma: Report of a case and review of the literature. World J Gastroenterol 2006;12:6062-9.  Back to cited text no. 9
    
10.
Palacios E, Shannon M, Solomon C, Guzman M. Biliary cystadenoma: Ultrasound, CT, and MRI. Gastrointest Radiol 1990;15:313-6.  Back to cited text no. 10
    
11.
Marcial MA, Hauser SC, Cibas ES, Braver J. Intrahepatic biliary cystadenoma. Clinical, radiological, and pathological findings. Dig Dis Sci 1986;31:884-8.  Back to cited text no. 11
    
12.
Lee CW, Tsai HI, Lin YS, Wu TH, Yu MC, Chen MF. Intrahepatic biliary mucinous cystic neoplasms: Clinicoradiological characteristics and surgical results. BMC Gastroenterol 2015;15:67.  Back to cited text no. 12
    
13.
Tran S, Berman L, Wadhwani NR, Browne M. Hepatobiliary cystadenoma: A rare pediatric tumor. Pediatr Surg Int 2013;29:841-5.  Back to cited text no. 13
    
14.
Cacciaguerra S, Lucenti A, Vasta G, Gangarossa S, Milone P, Rizzo L, et al. Hepatobiliary cystadenoma with hyaline stroma: A pediatric case associated with multiple hemangiomas. Pediatr Surg Int 1996;11:380-3.  Back to cited text no. 14
    
15.
Williamson JM, Rees JR, Pope I, Strickland A. Hepatobiliary cystadenomas. Ann R Coll Surg Engl 2013;95:507-10.  Back to cited text no. 15
    
16.
Yoon MH, Yoon JW, Han BH. Mucinous cystadenoma of the liver with ovarian-like stroma: The need for complete resection. J Korean Surg Soc 2011;81 Suppl 1:S51-4.  Back to cited text no. 16
    
17.
Luo JJ, Baksh FK, Pfeifer JD, Eastman JT, Beyer FC 3rd, Dehner LP. Abdominal mucinous cystic neoplasm in a male child. Pediatr Dev Pathol 2008;11:46-9.  Back to cited text no. 17
    
18.
Kishida N, Shinoda M, Masugi Y, Itano O, Fujii-Nishimura Y, Ueno A, et al. Cystic tumor of the liver without ovarian-like stroma or bile duct communication: Two case reports and a review of the literature. World J Surg Oncol 2014;12:229.  Back to cited text no. 18
    
19.
Fuks D, Voitot H, Paradis V, Belghiti J, Vilgrain V, Farges O. Intracystic concentrations of tumour markers for the diagnosis of cystic liver lesions. Br J Surg 2014;101:408-16.  Back to cited text no. 19
    


    Figures

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    Tables

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