|Year : 2017 | Volume
| Issue : 4 | Page : 487-500
|Spectrum of hepatobiliary cystic lesions: A 7-year experience at a tertiary care referral center in North India and review of literature
Prasenjit Das1, Pragya Sharma1, Tripti Nakra1, Shouriyo Ghosh1, Rajni Yadav1, Brijnandan Gupta1, Gaurav Khanna1, KS Madhusudhan2, Rajesh Panwar3, MK Anoop2, Ragini Kilambi3, Anand N Singh3, Nihar R Dash3, Sujoy Pal3, Siddhartha Datta Gupta1
1 Department of Pathology, AIIMS, New Delhi, India
2 Department of Radiology, AIIMS, New Delhi, India
3 Department of GI Surgery, AIIMS, New Delhi, India
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|Date of Web Publication||12-Jan-2018|
| Abstract|| |
Context: Cysts arising from the hepatobiliary tree are a group of heterogeneous lesions with regard to pathogenesis, clinical presentation, and radiological finding. They can be intrahepatic or extrahepatic, developmental, secondary to infective/inflammatory etiologies, as well as neoplastic. This study was conducted to determine the spectrum of hepatobiliary cysts in surgically intervened cases, with regard to their prevalence, histological spectrum, and clinicoradiological correlation, wherever possible. Methods: In this retrospective observational study, hematoxylin and eosin stained slides of all cases of hepatobiliary cystic lesions, operated between 2009 and 2016 were reviewed. Special stains as reticulin, Masson's trichrome, and periodic acid Schiff were done wherever necessary. Overall prevalence, age-sex distribution, clinical presentation and histopathological patterns were studied. Relevant imaging findings were correlated wherever possible. Results: A total of 312 cases of hepatobiliary cysts were identified, the majority in females. Choledochal cysts (CCs) were the most common type (n = 198,63.5%), followed by hydatid cysts (n = 73,23.3%), simple hepatic cysts (n = 10,3.2%), congenital hepatic fibrosis (n = 10,3.2%), biliary cystadenomas (n = 4,1.2%) hepatic mesenchymal hamartomas (n = 7,2.2%), and cavernous hemangiomas (n = 3,0.9%). Fibropolycystic liver disease (n = 2,0.6%), Caroli's disease (n = 1, 0.3%), liver abscess (n = 2, 0.6%), infantile hemangioendothelioma (n = 1,0.3%), and biliary cystadenocarcinomas (n = 1,0.3%) were rare. Lesions noted mostly in 1st decade of life were: CCs, fibrocystic liver disease, Caroli's syndrome, cystic mesenchymal hamartoma, and infantile hemangioendotheliomas. Conclusion: In our cohort of surgically intervened cases of hepatobiliary cystic lesions from a tertiary care hospital in North India, the CCs, followed by hydatid cyst were the most common lesions. Histology can play vital role in characterization, as often clinical findings and radiology can overlap.
Keywords: Biliary cyst, choledocal cyst, hepatic cyst, hepatobiliary cystic lesions, histological spectrum, hydatid cyst
|How to cite this article:|
Das P, Sharma P, Nakra T, Ghosh S, Yadav R, Gupta B, Khanna G, Madhusudhan K S, Panwar R, Anoop M K, Kilambi R, Singh AN, Dash NR, Pal S, Gupta SD. Spectrum of hepatobiliary cystic lesions: A 7-year experience at a tertiary care referral center in North India and review of literature. Indian J Pathol Microbiol 2017;60:487-500
|How to cite this URL:|
Das P, Sharma P, Nakra T, Ghosh S, Yadav R, Gupta B, Khanna G, Madhusudhan K S, Panwar R, Anoop M K, Kilambi R, Singh AN, Dash NR, Pal S, Gupta SD. Spectrum of hepatobiliary cystic lesions: A 7-year experience at a tertiary care referral center in North India and review of literature. Indian J Pathol Microbiol [serial online] 2017 [cited 2022 Aug 11];60:487-500. Available from: https://www.ijpmonline.org/text.asp?2017/60/4/487/222985
| Introduction|| |
Cysts of the hepatobiliary tree are a group of heterogeneous lesions with regard to the pathogenesis, clinical presentation, diagnostic findings, and therapeutic management. Most of them are asymptomatic and incidentally detected on abdominal imaging such as ultrasonography (USG), computed tomography (CT), and magnetic resonance imaging (MRI). A few of them, however, may be symptomatic, and rarely associated with serious morbidity and mortality.,, The latter, are the larger cysts, which cause complications such as spontaneous hemorrhage, rupture into the peritoneal cavity or bile duct, infection, and compression of adjacent biliary tree. Rarely, the ruptured cyst content can cause further complications, as anaphylactic shock. The spectrum of the hepatobiliary cystic lesions might vary in different geographical regions, due to differences in etiological factors in different climatic conditions. Since only a sparse data are available regarding the prevalence of hepatobiliary cystic lesions, our study was aimed at studying their spectrum, based on surgically intervened referred cases at our tertiary care center in North India.
| Methods|| |
We performed a retrospective analysis of 312 cases of hepatobiliary cystic lesions diagnosed in the department of Pathology, over a period of the past 7 years (January 2009–July 2016). All cases with evidence of cystic hepatobiliary diseases were included in this study. Relevant clinical and imaging findings were reviewed wherever available. All the cases were reviewed by two histopathologists individually, and a consensus diagnosis was made. Along with hematoxylin and eosin stained slides, silver reticulin, Masson's trichrome, periodic acid Schiff (PAS), and Voehrhoff Van Gieson stains were performed and analyzed as per applicability. Although an attempt was made to correlate the histological findings to radiological features for all, in many cases the same was not possible, as some of them were identified incidentally in the outpatient department or the radiology was not done primarily in our referral hospital.
| Results|| |
Out of the total 312 cases of hepatobiliary cystic lesions identified over a period of the past 7 years, 200 (64.1%) were females; age ranging from new born to 65 years; and 112 (35.8%) were males; age ranging from 19 day old to 92 years (M: F-1:1.8). The distribution of extrahepatic, nonbiliary hepatic, and biliary intrahepatic cysts were 198 (63.5%), 79 (25.3%), and 35 (11.2%), respectively. Overall, the choledochal cysts (CCs) comprised the majority (63.5%) of these surgically intervened cystic lesions, followed by the hydatid cysts [Graph 1]. Other cystic lesions such as simple hepatic cysts, cystic mesenchymal hamartomas (cMH), congenital hepatic fibrosis (CHF) with Caroli's syndrome, cavernous hemangiomas, and biliary cystadenomas were uncommon; whereas the fibropolycystic liver diseases of small and intermediate bile ducts, Carolis disease of larger bile ducts, hemangioendothelioma, and biliary cystadenocarcinoma were further rare [Graph 1]. The CCs, cMH, and CHF with Caroli's syndrome were mostly noted in the 1st and 2nd decades of life (69.8% of them in first two decades vs. 30.2% in adults) [Graph 2].
The choledocal cysts, which comprised the major cystic lesions in this study (n-198; 63.4%), were noted in patients aged just 19 days to 92 years of age, though a mean age intervention was 17 years. Overall, 52.5% of these lesions were noted in the first decade itself, with a striking female preponderance (M:F 1:2.6) [Table 1]. Macroscopically, the cysts ranged in size from 0.5 to 22 cm, with wall thickness of 0.1–0.4 cm. No inspissated bile or calculi were identified within the cysts when examined by the attending pathologist. Based on Todani classification of bile duct cysts, they are further classified into five types, based on type of cystic dilatation and position of the cysts [Figure 1]a,[Figure 1]b,[Figure 1]c and [Figure 2]a. Microscopically, the cysts were variably lined by discontinuous columnar, cuboidal, or flattened epithelium. Squamous metaplasia was observed in two cases. Focal epithelial denudation and ulceration were observed in the majority. Wall of most these cysts showed thickening with fibrocollagenous connective tissue, chronic mononuclear cell infiltrate, and an occasional lymphoid cell aggregate. Interlacing strands of smooth muscles were noted more in the children and young adults. On the other hand, density of inflammation, fibrosis, and hyalinization were gradually more marked in the older age group. In addition, peribiliary glands with mucus retention (in most) and a few showing pyloric metaplasia (7.6%), congested blood vessels, and hypertrophic nerve bundles were also observed in the cyst wall. Pancreatic tissue in cyst wall was present in 5.5% of index cases [Figure 2]e.
|Figure 1: (a) Choledocal cyst type 1. Magnetic resonance cholangiopancreaticography image showing fusiform dilatation of common bile duct (arrow) with normal caliber of intrahepatic biliary ducts. (b) Choledochal cyst type 4A. Coronal image of contrast-enhanced computed tomography scan showing globular dilatation of the common bile duct with fusiform dilatation of right and left hepatic ducts. (c) Choledochal cyst type 4A. Axial contrast-enhanced computed tomography image showing fusiform dilatation of central biliary ducts with normal peripheral bile ducts. The CBD was also dilated. (d) Hydatid cyst. Axial contrast-enhanced computed tomography image showing mildly thick and smooth walled cystic lesion in left lobe of liver (arrow) with multiple thin internal septations. (e) Alveolar echinococcosis. Axial contrast-enhanced computed tomography scan showing a large thin-walled cystic lesion with thick internal septation in the left lobe of liver. (f) Simple cyst. Axial contrast-enhanced computed tomography scan showing a large thin-walled cyst in the right lobe of liver without any internal septation. (g) Mesenchymal hamartoma. Axial contrast-enhanced computed tomography scan showing a thick walled predominantly cystic lesion in the right lobe of liver with thick internal septations and enhancing solid areas in the periphery. (h) Caroli's disease. Axial contrast-enhanced computed tomography scan showing fusiform dilatation of multiple segments of intrahepatic biliary ducts. (i) Biliary cystadenoma. Axial T1-weighted MR image showing a lobulated cystic lesion with internal septations (arrow) in the right lobe of liver. (j) Polycystic liver disease. Axial contrast-enhanced computed tomography scan showing multiple variable sized thin walled cysts in both lobes of liver|
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|Figure 2: (a) Gross photograph of an excised type II choledochal cyst. (b) Gross photograph of partial hepatectomy for alveolar Echinococcosis, showing heterogenous mass, with varying size cysts (arrows), mimicking a tumor. (c) Cut surface of a mesenchymal hamartoma, showing spongy and mucoid appearance. (d) Excised biliary cystadenoma, showing shooth inner cyst wall. (e) Photomicrograph of an excised choledochal cyst showing ectopic bening pancreatic parenchyma (arrow) in the cyst wall (H and E, ×40). (f) Hepatic Echinococcal cyst showing laminated membrane and protoscolices (arrow) (H and E, ×100). (g) Pericyst of hepatic hydatid cyst, showing palisaded histiocytes layer (arrow) (H and E, ×200). (h) Hepatic simple cyst, showing fibrous wall, without epithelial cell lining, and benign bile ducts in its wall (arrow) (H and E, ×100). (i) Cystic mesenchymal hamartoma show multiple cystic spaces, amidst myxoid loose stroma (H and E, ×100). (j). Biliary cystadenoma showing cuboidal cyst lining cells, with rare ciliary metaplasia (arrow) (H and E, ×200). (k) Hepatic cavernous hemangioma, showing varying sized thrombosed large vascular spaces (H and E, ×40). (l) Infantile hemangioendothelioma, showing multiple small, nonconnected vascular spaces, lined by plemp endothelial cells. A few bile ducts are seen in adjacent area (H and E, ×100). (m) Sections showing polycystic liver disease (H and E x 100). (n) Autopsy performed in the same case showing presence of polycystic kidney disease (arrows dilated tubules) (H and E x100). (o) Geographical bland fibrosis noted in a case of congenital hepatic fibrosis (H and E x 40). (p) A case of congenital hepatic fibrosis with Caroli's disease (arrow) (H and H x40)|
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About, 159 specimens of CCs, out of the total 198 included in this study (80.3%) were received along with attached gallbladder, which showed features of chronic cholecystitis with thickening, fibrosis, and muscle hypertrophy in 82.4%, atretic thin gallbladder in 1.8%, and adenomyomatous hyperplasia in one (0.6%); while remaining were within normal histological limits. Additional liver biopsies were performed in a total 44 CCs (22.2%). Among the liver biopsies, 36.3% showed portoportal bridging fibrosis, along with septal bile ductular proliferation, intrahepatic, canalicular as well as ductal cholestasis and complete nodule formation, suggestive of extrahepatic biliary obstruction by the choledocal cyst, with secondary biliary cirrhosis. In addition, 13.6% biopsies showed only features of obstructive cholangiopathy, in the form of ductal branching, ascending cholangitis, and periductal concentric fibrosis in septal portal tracts. The remaining biopsies were within normal limits.
The hydatic cysts were identified in a total 73 (23.3%) cases, 57.5% cases of which were diagnosed in 3rd and 4th decades, with equal sex distribution. The majority were single, unilocular, pearly white cysts, and measured 1–13 cms in diameter with wall thickness of 0.1–0.6 cm. On USG, mildly thick-walled anechoic lesions with or without internal septations, or daughter cysts were seen. On CT scan, they appeared as hypodense lesions with internal septations. Calcification was seen in the wall and in septae [Figure 1]d. On MRI, the cysts were hyperintense on T2-weighted images with hypointense fibrous wall. Daughter cysts were better seen as cysts within the cyst. Microscopically, both pericyst and ectocysts were identified in 56.2% cases; while in 16.4% cases only pericyst was seen, comprising of fibrocollagenous cyst wall, lined by palisaded histiocytes, foci of calcification, and dense chronic inflammatory cell infiltrate, including a few eosinophils [Figure 2]f and [Figure 2]g. Chronic inflammatory granulation tissue and a few foreign body type giant cells were also noted at the interface of the pericysts and adjacent hepatic parenchyma. Calcification and hyalinization were noted in 2.7% and 4.1%, respectively. In another 27.4% cases, only ectocyst with lamellated membranes was noted. Scolices, daughter cysts, hooklets, brood capsules, and germinal layers were variably identified in 13.7% [Figure 2]f. Alveolar Ecchinococcosis was identified in two of our cases [Figure 1]e, which radiologically and on gross-examination resembled hepatic tumor mass, with multiple varying size cavities and areas of hemorrhage [Figure 2]b. Extensive pericyst reactions were noted in the intercystic regions.
Simple benign hepatic cysts or the solitary nonparasitic cysts were identified in 10 of our cases (3.2%), all in 5th to 7th decades of life, with equal sex distribution. These cysts were mostly single and unilocular, ranging 0.5–19 cm in size [Figure 1]f. Histologically, they were lined by a single layer of cuboidal to columnar cells, resembling the biliary epithelium, with frequent attenuation, and denudation. Thin wall of mature fibrous tissue, containing bile ducts, and large blood vessels, with an outer zone of compressed hepatic parenchyma was seen around the cysts [Figure 2]h. No bile was identified within these cysts.
Cystic hepatic mesenchymal hamartoma was identified in 7 of our cases (2.2%), all in children ≤2 years of age, in right lobe of liver, with a male preponderance [Graph 3]. These were solitary multicystic masses varying in size from 9.5 to 20 cm; while the cyst size varied from 0.5 to 11.5 cm [Figure 1]g. Predominantly solid areas were seen in two cases. Cyst content and cut surface were gelatinous [Figure 2]c. All of these children presented with progressive abdominal enlargement and lump. Pain was identified in two of them. Microscopically, the lesions consisted of myxomatous connective tissue containing scattered bland spindle or stellate mesenchymal cells with cysts spaces, devoid of any lining epithelium [Figure 2]i. Many branching bile ducts lined by cuboidal to columnar epithelium were noted, collared by loose mesenchyme containing mild scattered chronic inflammatory cell infiltrate. Periductual polymorphs were observed in a few. Cords of mature hepatocytes with proliferating bile ducts were seen at the periphery of the lesions. Extramedullary hematopoesis was observed in one case.
CHF was identified in 10 cases (3.2%), including one case of associated Caroli's Syndrome (autosomal recessive fibropolycystic disease of liver and kidney) and Caroli's disease each [Figure 1]h. Most of these (80%) were identified in first decade of life, while the remaining were noted in late childhood and adults, with a male preponderance (M:F 1:0.4). Most common symptoms were abdominal distension, right upper quadrant pain, and hepatomegaly. Two had associated splenomegaly and jaundice and five patients had fever. Radiologically, intrahepatic biliary radicles were dilated [Figure 1]h. Bilateral medullary nephrocalcinosis was radiologically observed in one case. Majority of the cases where diagnosis of CHF was given, were tru-cut liver biopsies. In two cases, partial hepatectomy was also received, measuring 12 and 15 cm each, in their maximum dimension. The liver biopsies showed distorted lobular architecture with thick interconnecting map like fibrous bands, containing irregular bile ducts with normal cuboidal epithelium, and dysmorphic thick arteries [Figure 2]o. Mild chronic septal inflammation with periseptal interphase hepatitis were also observed. Caroli's syndrome was reported in an 8-year-old boy, whose liver biopsy showed distorted architecture with concentric fibrosis around the markedly dilated bile ducts, containing inspissated bile [Figure 2]p. Caroli's disease was diagnosed in a 29-year-old male. The partial hepatectomy specimens showed nodularity, fibrosis, and dilated biliary channels filled with inspissated bile. Microscopically, irregular dilatation of the larger bile duct, with inspissated bile, periductal fibrosis, and foreign body giant cell reactions, chronic inflammation and a few obliterated portal veins were also seen [Figure 2]o.
Biliary cystadenomas were identified in four (1.2%) cases, all in women aged 32–50 years. Two of them were located superficially in the left lobe of liver, one of them involving the liver segments II and IV [Figure 1]i attached by a fibrotic pedicle. The cysts were already cut open when received and measured between 5 and 6 cm, with wall thickness of 0.2–0.4 cm. The inner surface was smooth, and no solid areas or papillary projections could be identified [Figure 2]d. Microscopically, the cysts were lined by focally denuded uniform cuboidal to columnar epithelium resting on a basement membrane with basally arranged small, round to oval nuclei and apical mucin. No stratification, nuclear atypia or mitosis was present. Out of the four, in three cases, the supporting stroma immediately subjacent to the epithelium was very compact and cellular, resembling ovarian stroma. Minimal chronic inflammatory cell infiltrate was noted in all. In one of these cases, dense subepithelial fibrosis with hyalinization and foci of calcification were noted. Normal compressed hepatic parenchyma was identified at the periphery in all. One, out of these 4 cases, in a 45-year-old female, in addition, exhibited ciliary metaplasia in the lining epithelium [Figure 2]j. USG as well as CT scan revealed a multiloculated cystic lesion within the right lobe of liver involving the segments V and VI [Figure 1]j. Macroscopically, this cyst was filled with clear serous fluid. The inner surface was smooth, and no solid areas or papillary projections could be identified. Due to the presence of ciliated lining epithelium, possibility of a ciliated foregut cyst was also considered, however, the lesion lacked smooth muscle in its wall. The lining epithelium also showed positivity for CK19, while the carcinoembryonic antigen (CEA) was negative, unlike that of ciliated hepatic foregut cysts.
Biliary cystadenocarcinoma was identified in only one of these 312 cases (0.32%), in a 40-year-old female, appeared as a multiloculated cystic lesion in left hepatic lobe, measuring 12 cm in maximum size. The cyst wall thickness was 0.5–2.5 cm and the lumen was filled with gelatinous material. Microscopically, a tumor with papillary and tubulopapillary architecture projecting into cyst cavity with atypical cuboidal to columnar cell lining was seen. There were nuclear stratification, loss of polarity, pleomorphism, prominent nucleoli, and mitotic activities. Invasion into the underlying cellular compact ovarian-like stroma was also identified. The tumor cells showed immunopositivity for CK7 and CK 20.
Cavernous hemangioma was noted in 3 cases (0.96%), all in adults [Graph 1]. Two of them were noted in males, aged 64 and 30 years and the other in female aged 35 years. Two lesions involved the right hepatic lobe and measured 7.7 and 19 cm, respectively. Macroscopically, lesions were superficial, solitary and showed honey combing with large compressible areas. In addition, fibrosis, necrosis, and focal calcification were present in one. In the third case, there was diffuse involvement of liver, which weighed 6 kg. Microscopically, tumor contained variably sized blood-filled spaces lined by single layer of flat endothelial cells. Organized thrombi were observed in one [Figure 2]k. The intervening stroma was fibrous with focal myxoid change. Dense chronic inflammatory cell infiltrate, necrosis, and focal calcification were noted in one case.
Two cases of liver abscesses were also identified in a 32-year-old female and 73-year-old male, respectively (0.6% out of all). The abscesses were solitary and microscopically showed acute inflammatory exudate, admixed with a few mononuclear cells, foamy histiocytes, foreign body type giant cells and inflammatory granulation tissue in surrounding areas. At the periphery of the lesion, fibrosis and palisading histiocytes were also observed. No fungal profile or amoebae was identified in either of them.
Hemangioendothelioma with cystic change was identified only in a 2-year-old male child (0.32% of all). The lesion was solitary, cystic, measuring 8 cm in maximum diameter with areas of necrosis and calcification. Microscopically, the tumor comprised of small cystic spaces lined by single layer of spindled to plump endothelial cells with round to oval nuclei, dispersed chromatin and single nucleoli. Bile ducts were identified within the intervening fibromyxoid stroma [Figure 2]l. Areas of focal hyalinization and calcification were also seen.
Fibropolycystic liver disease was noted in two cases (0.64%), one in a 52-year-old female and the other in a female preterm baby, born at 30 weeks of gestation, died thereafter at 4th day of life. Grossly, the neonatal liver was congested and weighed 50 g, while the adult liver biopsy showed variably sized dilatation of the small as well as medium-sized biliary radicals. These cysts were mostly unilocular and not connected to each other. On microscopic examination cuboidal to flattened epithelium-lined irregular cysts were noted with peri-cystic fibrous stroma. Outpouching of these cystic spaces were noted inside the hepatocyte lobules [Figure 2]m. At places, the expanded compressed bile ducts were seen to surround the portal vascular channels, suggesting ductal plate malformation. Extramedullary hematopoesis was observed in the neonatal liver, while variable fibrosis and chronic inflammation were noted in adult liver. In addition, the neonatal kidney showed multiple corticomedullary cysts measuring 0.1 cm in size, lined by cuboidal epithelium with intervening normal renal parenchyma [Figure 2]n. Caroli's diases was identified in one, where the cystic change was noted mainly of the subcapsular larger biliary channels.
| Discussion|| |
Cystic lesions of the hepatobiliary tree represent a broad spectrum of entities, ranging from benign developmental cysts (simple cyst, hamartomas) to potentially malignant (cystadenoma) or overtly malignant lesions (cystadenocarcinoma). The wide range of pathologic processes that may result in cystic liver lesions can present a difficult diagnostic conundrum. Depending on their location, these lesions can be classified as extrahepatic and intrahepatic. In the index study, we have identified different spectrum of cystic diseases of hepatobiliary tract; however, we did not find any case of operated amoebic liver abscess or example of cystic hepatic metastasis in this cohort.
The most common lesion identified was the CC (63.6%), followed by the hydatid cysts (23.3%) and simple hepatic cysts (3.2%). Our data is based on the surgically intervened cases of hepatobiliary cysts, over a period of 7 years. Most of these cases were referred, mostly from the North, middle, and some parts of Eastern India. Hence, though, this data may not truly reflect the prevalence of hepatobiliary cysts in general Indian population, it may reflect the prevalence of such lesions who needed medical intervention.
Choledoch, a word that refers to the common bile duct, comes from the Greek words chole (bile) and dechomai (to receive). The word cyst, comes from the Greek kystis (sac, bladder), and is defined as a closed cavity or sac lined by epithelium. CCs are characterized by congenital cystic dilatation of the intrahepatic or extrahepatic biliary tree and was first described in 1723, by Vater and Elzer. While CCs are rare in Western countries (1 in 100,000–1500,000), in Asia they are more common (1 in 1000), mostly noted in females of <15 years of age. However, around 20% are diagnosed in elderly patients.,,, Based on the site and type of dilatation of the biliary tree, these cysts are divided into five types. Many pathogenesis have been proposed: While Yotsuyanagi (1935) suggested unequal proliferation of CBD epithelium as a cause; Babbitt proposed anomaly of the pancreaticobiliary junction as the possible etiology. Normally, the CBD joins the pancreatic duct at a distance of 2.5–3 cm from the sphincter of Oddi, however, a more proximal union, leads to reflux of pancreatic juice from high-pressure pancreatic ducts into the CBD, leading to dilatation (>5 mm diameter), epithelial damage, epithelial hyperplasia, inflammation, fibrosis, or even dysplasia. High amylase level has also been demonstrated in CCs. Matsumoto et al. proposed that abnormal fusion of CBD to the ventral pancreatic duct leads to development of these cysts. Microscopically, a CC is differentiated from the gallbladder, by its flattened or denuded epithelium, with subepithelial fibrosis and inflammation. During normal embryonic fusion, the left anlage of pancreas fuses with the right anlage or dorsal pancreas, side-by-side. CC may develop when the left ventral anlage persists resulting in delayed recanalization of bile ducts and therefore leading to intra- and extra-hepatic bile ducts dilatation. Redundant pancreatic tissue may be present in the wall of CCs as seen in eleven cases in our study.
The classical triad of pain abdomen, jaundice, and palpable abdominal mass is seen in < 20% of patients aged < 1 year; while in adults biliary or pancreatic symptoms and abdominal pain are seen. Gall stones (45%–70%), acute cholecystitis, or both can attribute to biliary stasis. Most patients with CC show varying degree of liver involvement, as fibrosis, inflammation, increased bile duct profiles, ductal cholestasis, or even secondary biliary cirrhosis. Pancreatitis, spontaneous perforation, cholelithiasis, cholangitis, portal hypertension and cholangiocarcinoma can be seen. The incidence of malignant transformation varies from 2.5% to 17.5%; while the risk being 0.7% in the 1st decade and 14.3% after 20 years of age. Such risk is more common in type I, IV or V CCs (Caroli's Disease), and when is associated with intracystic lithiasis. While, USG has a sensitivity of 71%–97%, to evaluate the intrahepatic biliary system and pancreatic ducts, CT is important. It also identifies cyst wall thickening due to malignancy. CT and MRI can identify stones, cirrhosis, portal hypertension and varices. CT cholangiography and magnetic resonance cholangiopancreaticography (MRCP) have almost comparable efficacy in diagnosis. On the other hand, endoscopic retrograde cholangiopancreatography (ERCP) is regarded as the most sensitive radiodiagnostic modality and is the choice because of additional provision of therapeutic sphincterotomy. However, all these can cause recurrent inflammation, radiation exposure and pancreatitis. Hence, MRCP, being nonradiation-based modality, is regarded as the “gold standard” for the diagnosis of CCs, with sensitivity up to 90%–100%. Complete excision of the extrahepatic system and Roux-en-Y hepaticojejunostomy is the treatment of choice in type I (supra-duodenal fusiform CBD dilatation) and most of type IV CCs (Supra-duodenal CBD and intra-hepatic BDs dilated). Type II cysts (Supra-duodenal CBD, eccentric dilatation) are managed with simple excision, while choledochoceles are managed by endoscopic sphincterotomy. Localized intrahepatic type IVA CCs and Caroli's disease (type V, marked dilatation of large intrahepatic bile ducts) with complications are considered for hepatic resection whereas diffuse intrahepatic disease with complications in type IVA and Caroli's disease are offered liver transplantation.
Next most common type of HB cystic disease identified was the hydatid cyst, caused by E. granulosus, characterized by a typical large, single, round, or ovoid well defined cyst (single in 75% cases), filled with clear to whitish fluid. However, we also identified alveolar Echinococcosis in two of our cases, in patients, who were referred from Kashmir and Jammu districts, respectively, close to the Northern forest belt. Alveolar Echinococcosis is prevalent in Alaska, Canada, Central Western Europe, Siberia, and Japan. The irregular, small, fluid-filled spongy mass, caused by Echinococcus multilocularis often radiologically and peroperatively resembles an invasive malignancy [Figure 2]b., Overall incidence of cystic echinococcosis of liver varies from 1 to 200/105 people, which is more clustered in Andhra Pradesh and Tamil Nadu.,, Most of these cases, in our study was seen in 3rd and 4th decades, however, can be seen in any age group, with equal incidence in males and females. Studies by Papadimitriou et al., Mehta et al. and Rao et al. have also reported similar age presentations. However, Rao et al. reported higher incidence in females. Rubin and Farber reviewed 157 patients with hydatid disease of the liver and found that, hepatomegaly was the most common sign. Other common presentations were pain, nausea, dyspnea, dysphagia, and jaundice. The cysts can be small and usually become symptomatic when they enlarge up to 10–20 cm in size. In cystic echinococcosis, the cyst wall comprises of three layers. The inner germinal layer which is 10–25 μ in thickness contains nuclei, which give rise to brood capsules attached with short stalks and daughter cysts. Protoscolices with double row of refractile, birefringent acid-fast hooklets of 22–40 μ, and four round suckers may be seen [Figure 2]f. These, along with daughter cysts and calcospherules form the hydatid sand. Laminated membrane (ectocyst) outside the germinal layer is around 1 mm thick avascular, eosinophilic, refractile, chitinous, strongly PAS and GM positive. The outermost adventitial layer, the pericyst is a layer of host tissue, comprising of fibrovascular structures, chronic inflammatory cells, eosinophils and variable calcification. Pericyst, however, is seen in older cysts and sometimes take long time (up to 5 years) to develop [Figure 2]g. In alveolar echinococcosis, on histological examination mostly the laminated membrane is identified, whereas brood capsule is seen only in 10% of cases. Germinal layer and protoscolices are further rarer. A consistent histological finding is the presence of pericystic layer of palisaded histiocytes, where the histiocytes nuclei are arranged perpendicularly over the laminated membrane and giant cells [Figure 2]g. However, similar features can also be seen in hepatic cat scratch disease and atleast a Warthin Starry stain should be done when identified. USG is also the method of choice for the detection of both hepatic and extrahepatic echinococcal cysts, with sensitivity up to 93%–98%. CT scan can confirm the diagnosis by detecting the presence of daughter cysts and calcification in the cyst wall with a sensitivity of 90%–97% [Figure 1]d and [Figure 1]e. The MRI also delineates all the features accurately.
Solitary nonparasitic cysts of the liver, or the simple cysts, are seen in 5% of population. Most in women and only 10%–15% of them are symptomatic., Asuquo et al. observed a F:M of 1.5:1 in asymptomatic and 9:1 in symptomatic or complicated simple cysts, indicating role of female hormones. Similar findings were also noticed by Cowles and Mulholland, and Caremani et al., However, in the index study an equal M: F was noted, mostly in 6th to 7th decades, followed by 5th decade. These cysts are lined by benign biliary epithelium, and are believed to arise due to aberrant bile duct development, secondary to defects in chromosome 16. The latter forms microhamartoma, seperates out from the draining biliary tree and forms a cyst in later life, due to accumulation of clear yellow fluid, but the content can be mucoid, bloody, or purulent [Figure 2]h. Within the cysts bile, amylase and white blood cells are not present. The smaller cysts enlarge very slowly; hence, only an occasional become symptomatic due to compression of adjacent structures or stretching of capsules and becomes palpable.,,, The largest cyst in this study measured 19 cm in diameter. The USG and CT scan are highly sensitive in picking up these cysts, while MRI, NMR, and hepatic angiography have limited role in diagnosis. Simple cysts are thin-walled, and show a homogenous low-density interior [Figure 1]f. The largest reported simple cyst in literature contained 17 litres of fluid. Histologically, they are lined by a single layer of cuboidal to columnar epithelium that resembles biliary epithelium [Figure 2]h. The epithelial cells contain small quantities of mucin and demonstrate weak immunostaining for epithelial membrane antigen and CEA. The epithelium may be attenuated, denuded, or exhibit squamous metaplasia. Surrounding the epithelial layer a thin wall of mature fibrous tissue is seen, which may contain bile ducts and large blood vessels, with a peripheral zone of compressed hepatic parenchyma. Common differential diagnoses are: Cysts in a polycystic liver disease (PCLD), parasitic cysts, Caroli's disease, CHF, and liver abscesses. However, the former is usually not connected with the primary biliary tree. Complications as rupture, torsion, intracystic hemorrhage and bacterial permeation may be seen. Rarely, an adenocarcinoma may develop in simple cysts, although squamous carcinoma and adenosquamous carcinoma have also been reported.,,,
Cystic mesenchymal hamartoma
cHMH is a rare, benign tumor representing about 18%–29% of all primary hepatic tumors. It is a hamartomatous growth of mesenchymal tissue around the portal tracts and appears a congenital, localized, abnormality of the ductal plate development, due to interstitial deletion near chromosome 19q13.4.,, Characteristically, this mesenchymal lesion undergoes cystic degeneration and due to accumulation of cyst fluid, the size enlarges., Eighty percent of them are seen in children younger than 2 years, though can be seen up to the age of 5. In the index study, all HMH were identified in children, who were <2 years old with a male preponderance (M:F 2.5:1), as was also reported by Stocker and Ishak. Association with PCKD, CHF, and biliary hamartoma have been reported. While the smaller cysts are nontender and asymptomatic, larger masses can produce ascites, jaundice, compression of adjacent structures, and even cause congestive heart failure. USG demonstrates either a multicystic or solid mass, while CT and MRI, are usualy not required for diagnosis, though may help in planning further management. Even intrauterine, HMHs have been documented in several reports.
Size of these lesions can vary, from a few centimeters up to 30 cm (Avg. 4–7 cm) and may bulge from the surface of the liver or can be pedunculated. In the index study, the largest lesion measured 20 cm, while the sizes of others varied from 0.5 to 11.5 cm in diameter. Two-third of such lesions occur on the right side of liver, as was also noted in the index study. Content of HMH can be clear to yellow or gelatinous. Histomorphology is exactly same as we described in our cases. The cysts are devoid of epithelial lining and the content appears to be walled by the mesenchyme layer itself. Extramedullary hematopoiesis is noted in approximately 85% of cases, we however, observed it in only one case. Although rare, malignant transformation to embryonal sarcoma has been reported. The differential diagnoses include: bile duct adenoma, cystadenoma, bile duct hamartoma, infantile hemangioma, infantile hemangioendothelioma, embryonal sarcoma, and mixed epithelial-mesenchymal hepatoblastoma. Treatment consists of surgical resection.
Congenital hepatic fibrosis
CHF is characterized by hepatic fibrosis, portal hypertension, and cystic renal disease. It is one of the fibropolycystic diseases, which also includes Caroli disease, autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD)., Although exact incidence and prevalence are not known; only a few hundred patients have been reported. The disease appears in both sporadic and familial patterns. Isolated CHF is rare. The total prevalence of syndromes that include CHF as a feature is estimated to be 1 in 10,000–20,000 live births. It represents a malformation of the ductal plate, the embryological precursor of the biliary system with periportal fibrosis., The fibrotic process also obliterates the portal vein, resulting in portal hypertension. The ductal plate is the compressed layer of biliary channel that surrounds a branch of the portal vein. Both interlobular and intralobular bile ductules develop from the ductal plate, starting at 12 weeks' gestation. The lack of remodeling of the ductal plate results in persistence of these embryonic structures, resulting in this malformation, which may also be variably dilated., Although classically the ARPKD is associated with CHF; ADPKD can also be identified in adults.
Although the age of presentation and severity of symptoms vary, in our study, 80% presented in the 1st decade, the youngest being 3 months old and the oldest was in 4th decade. Cases even may be diagnosed late in the 5th decades. Although no gender predilection has been reported in literature, we found a slight male preponderance (M:F 1:0.4). Four clinical forms have been defined: (1) Portal hypertensive: Is the most common, followed by (2) Cholangitic: Cholestasis with recurrent cholangitis (3) mixed and (4) Latent presentation at a late age. Most patients are asymptomatic, while some may complain of mild right upper quadrant pain and hepatomegaly with predominant involvement of the left lobe, splenomegaly and nephromegaly. Marked elevations in alkaline phosphatase (ALP), γ-glutamyl transpeptidase (GGT), and bilirubin are seen in patients with a predominantly cholangitic clinical picture. Leukopenia and thrombocytopenia may be seen secondary to hypersplenism. Abnormal renal functions tests are associated with extensive cystic renal disease.
The combination of conventional and high-resolution USG with MRCP reveals the extent of liver and renal disease without exposure to ionizing radiation and contrast agents., Color Doppler should be performed to assess the portal venous system. CT scan can demonstrate the abnormal shape and size of the liver and can demonstrate periportal thickening, varices, and splenomegaly, but has limited role in patients with associated renal disease. In the index cases interconnected thick bands of map-like fibrosis were noted. These septa contained many ectatic and irregularly shaped proliferating bile ducts, lined by normal cuboidal epithelium. Cholestasis may be observed in cholangitic variant. Cystic dilatation of small bile ducts and hypoplasia of the portal vein branches in association with super numerous, thick hepatic artery branches may be observed in some cases. In addition, congenital absence of the portal vein has been reported in a pediatric patient with CHF. However, unlike cirrhosis, the hepatic lobules are usually normal. CHF needs to be differentiated from Caroli disease, hepatic cysts, and noncirrhotic portal hypertension. Complications include variceal bleeding, hypersplenism, cholangitis, and to a lesser extent, biliary stones, cholangiocarcinoma, and hepatocellular carcinoma.
Fibropolycystic disease liver
PCLD comprise a group of rare (prevalence 1:100,000 population) genetic diseases, which may be sporadic autosomal dominant or associated with ADPKD, affecting the small to medium size bile ducts. Mutation of PRKCSH and SEC63 genes are associated with defects in the function of the proteins hepatocystin and Sec63, respectively, and are responsible for 33%–50% of these cases. However, exact mechanism is not yet known. Almost, 80% are seen in women and older age. However, we did not identify female predominance in our cases, and most of our cases were identified in 1st decade. While the majority are asymptomatic, 3% patients can have abdominal symptoms. Massive hepatomegaly can cause abdominal distension, pain, early satiety, gastroesophageal reflux, and extreme malnutrition. Inferior vena cava or portal vein compression and Budd–Chiari syndrome can rarely occure due to compression. Moreover, cysts may get infection or undergo torsion, rupture, or hemorrhage. The screening of these conditions is not recommended unless there is a clinical suspicion or indication for transplantation.,
Although USG and CT scan can detect these cysts, MRI is most sensitive., The diagnosis is considered when >20 isolated cysts are seen in the liver. Laboratory tests are nonspecific and hepatic transaminases, γ GGT and ALP usually have normal values. Tumor marker, CA 19.9 is elevated in 45% of cases due to secretion by biliary epithelium. Macroscopically, an enlarged liver shows multiple unilocular variably sized cysts. Histologically, multiple diffuse cystic lesions resembling solitary cysts, lined by cuboidal to flat biliary epithelium are seen. Von Mayenberg complexes are present in up to 40% cases. Liver transplantation is the only curative treatment.
Caroli's disease and Caroli's syndrome
Unlike, PCLD, in Caroli's disease the large intrahepatic bile ducts show cystic dilatation. However, in Caroli syndrome malformations of small bile ducts with associated CHF can be seen. Arrest in ductal plate remodeling is the main defect in its pathogenesis. This process can be either diffuse or segmental and may be limited to one lobe of the liver, commonly the left lobe. Unlike PCLD, these ducts are connected to the bilary tree, hence, are predisposed to bile stasis, bacterial cholangitis, intrahepatic pigment stone formation, and increased risk of cholangiocarcinoma. Caroli's disease can be sporadic, with a prevalence is 1 in 1,000,000 population, less common than Caroli's syndrome, which is inherited as autosomal recessive or dominant disease. We had one case of Caroli's disease in a 29-year-old male and one case of Caroli's syndrome in an 8-year-old male. These patients can present with cholangitis, hepatolithiasis, and stones. Clinical presentation of Caroli's syndrome is highly variable. The laboratory findings are nonspecific. Transaminase levels may be slightly elevated. Macroscopically, liver shows multiple cystic dilatations of the intrahepatic bile ducts. Histologically, in Caroli's disease, the dilated ducts are lined by the biliary epithelium, which may be hyperplastic and ulcerated. In Caroli's syndrome, complex dilated ectatic bile ducts with marked periductal fibrosis are seen. In presence of associated cholangitis, an acute and chronic inflammatory cell infiltrate is noted around the dilated bile ducts. Demonstration of communication between sacculi and bile ducts is important in Caroli's disease by either USG, CT, MRCP, or ERCP.,, Other cystic liver diseases, as CHF, PCLD, cholelithiasis, and primary sclerosing cholangitis can be clinical differential diagnosis.
Biliary Cystadenomas account for <5% of the cystic neoplasms of liver., Approximately 85% of them are intrahepatic. Commonly, the right lobe is involved (55%), followed by left lobe (29%) and rarely they are bilobar (16%). In this study, all such lesions are found in females with a mean age of 44 years. Role of female hormones in middle-aged women have been implicated. They though are mostly asymptomatic and detected incidentally. Clinically, they can present with vague abdominal discomfort, fullness, nausea, pain in upper abdomen, and rarely with obstructive biliary symptoms such as jaundice or cholangitis.,
Although its origin is unclear, possible origin from embryonal hepatobiliary stem cells or congenital aberrant bile ducts have been suggested., Raised serum as well as intracystic fluid level of CEA and CA19-9 have been reported. Any solitary cystic lesion described till now can be a differential diagnosis, as: Hydatid cyst, simple cysts, liver abscesses, intraductal papillary mucinous tumor, and biliary cystadenocarcinoma.
Recurrence and malignant transformation have been reported when incompletely excised.,, Hypoechoic or anechoic cystic lesion with internal septations can be picked up on USG. Hyperechoic shadows are suggestive of focal wall fibrosis, intracystic hemorrhage, or papillary areas., CT scan can also reveal well-demarcated multiloculated cystic lesion filled with mucin or blood with internal septations. However, histological analysis is mandatory, as maliganacy cannot be ruled out completely on radiology., Macroscopically, it presents as large multiloculated cyst with smooth external and internal surfaces, filled with clear serous or mucinous fluid. Rarely, hemorrhagic or purulent content may be seen, if infected. Microscopically, the cysts are lined by flat cuboidal to columnar nonciliated mucin-secreting epithelium resting on the basement membrane and supported by hyalinized acellular stroma or more commonly, dense fibroblastic mesenchymal stroma, described as ovarian stroma, seen in 85% of these cases. The latter, can also show expression of progesterone receptor stain. Other markers demonstrated on immunohistochemistry are CA19-9, CEA, and cytokeratin.
Biliary cystadenocarcinoma rarely can arise in a biliary cystadenoma. Like the index study, this malignany is usually found in elderly women, in their late fifties. Size of these can vary from 3 to 26 cm in largest diameter.
Microscopically, tubulopapillary structures, with mucin secreting tall columnar cells are seen. These glands often show back-to-back complex arrangement, with no intervening stroma. Invasion into surrounding stroma with dense desmoplastic reaction can be noted. At areas solid or adenosquamous differentiation can be seen. Marked nuclear pleomorphism, loss of polarity, multilayering of epithelium, and mitotic figures suggest malignant transformation. Increased tumor markers, as CEA and CA 19-9, in serum or cystic fluid have been reported. However, level of these tumor markers or immunohistochemistry cannot distinguish cystadenocarcinoma from a cystadenoma. Stromal invasion or complex back-to-back tumor arrangement hence must be demonstrated.
CH is the most common benign liver tumors and commonly affects the right lobe., They occur in 0.4%–20% of the general population as reported by Karhunen in an autopsy series. CH comprises multiple, large vascular channels lined by a single layer of endothelial cells, and supported by collagenous walls. Women are more often affected, and studies have shown increase in the tumor size in women exposed to exogenous estrogen, progesterone, contraceptives, or hormone replacement therapies.,, Although mostly asymptomatic, the larger ones may present with right hypochondrial pain, hepatomegaly, and perceptible arterial bruit. It poses diagnostic challenge because of its resemblance to other hypervascular liver tumors, as focal nodular hyperplasia, hepatic adenoma, hepatic cysts, hemangioendothelioma, hepatic angiosarcoma, hepatic metastasis, and primary HCC. USG, though is sensitive (70%–80% diagnosis rate), MRI is the most accurate (95% sensitive, and up to 100% specificity)., Sequestration and destruction of platelets, hypofibrinogenemia may be seen due to intratumoral fibrinolysis, and serum alpha-fetoprotein, CA 19-9, CEA levels are usually normal. CHs are mostly solitary, and size ranges from 2 mm to >20 cm in diameter. Multiple and diffuse hepatic lesions are infrequent. Cut surface shows well demarcated subcapsular bulge or pedunculated lesions, with reddish-blue multicystic lesion. Histologically, an unencapsulated tumor with irregular borders, comprising of variably sized vascular spaces lined by a single layer of endothelial cells, separated by myxoid or fibrous stroma are noted. Thrombosis, calcification, and phleboliths may be seen. Elastin and trichrome stain may reveal vessels in old fibrous lesions. Differential diagnosis includes infantile hemangioendothelioma, hemangiomatosis, hereditary hemorrhagic telangiectasia, and peliosis hepatis. While surgical resection is curative for larger lesions, smaller lesions may automatically involute.
Infantile hepatic hemangioendothelioma
In infants and toddlers, infantile hepatic hemangioendothelioma (IHHE) is the most common vascular liver tumor, accounting for 12% of all childhood hepatic tumors, and 85% of them are diagnosed during the first 6 months of life. A male-to-female ratio of 1.3–2:1 is usually seen., The most common presentation is abdominal mass, hepatomegaly, high-output cardiac failure, skin hemangiomas, thrombocytopenia, hemolytic anemia, and peritoneal bleeding. Kim et al. reported 23 cases of IHHE over a period of 14 years with a female preponderance, with presentation as an abdominal mass with high cardiac output failure and consumptive coagulopathy in more than half of their patients. On USG, discrete, hypoechoic, either solitary or multiple lesions may be seen, while CT scan has an advantage of more precise anatomical localization, tissue enhancement, and characterization.,, Calcifications or shunting may be seen on Doppler evaluation. MRI shows well-defined spherical lesions, hypointense to the liver on T1 sequences and hyperintense on T2, with flow-voids and centripetal enhancement after the administration of gadolinium. Grossly, these tumors are soft spongy, can be solitary or multiple ranging from 0.1 to 15 cm, red to tan in color. Larger nodules may have hemorrhagic, fibrotic, or calcified central areas. Histopathologically, Type I changes are common (80%), with orderly proliferation of small, capillary-like irregular vascular spaces, lined by bland, or plump endothelial cells. The supporting stroma may contain abundant myxohyaline material with interspersed small bile ducts. Extramedullary hematopoiesis in seen in 60% cases, often in vascular lumina. Larger lesions may show thrombosis, fibrosis, myxoid change, and calcification.
Type 2 changes are equivalent to angiosarcoma with irregular branching vascular structures lined by pleomorphic, hyperchromatic endothelial cells, and with frequent mitotic activity. These lesions must be histologically distinguished from cavernous hemangioma, epithelioid hemangioendothelioma, angiosarcoma, and mesenchymal hamartoma. The tumor cells are positive for CD31, CD34, and are negative for CK7, CK19, and Hep Par 1 antigens.,,,,,
The diagnosis of hepatic abscess can be a challenge, as signs and symptoms may vary. The most common type is the pyogenic abscess (incidence: 3 cases/100,000 people/year), followed by amoebic abscess, which accounts for only 3%–9% of all cases of liver abscess in developing countries. In the index study, both liver abscesses identified were pyogenic abscesses. Male are commonly affected (3.3 vs. 1.3 per 100,000).,Escherichia coli, Klebsiella species, and Entamoeba histolytica are the most commonly implicated organisms.
Abscesses can seed to the liver from biliary diseases, inflammatory bowel disease, diverticulitis, and appendicitis through the portal venous system, in patients, especially suffering from other immune-modulatory disease. Rarely, blunt and penetrating trauma can result in hepatic abscess. In a majority of cases, a source cannot identified. These are known as cryptogenic abscesses. The right lobe is more commonly affected due the fact that the right lobe portal laminar blood flow is supplied predominantly by the superior mesenteric vein, whereas the left lobe portal blood flow is supplied by the splenic vein. Symptoms of hepatic abscess include fever, jaundice, abdominal pain, nausea, vomiting, and weight loss. Hepatomegaly may be present. Complications include rupture and spread of infection into the thoracic cavity or even formation of hepatobronchial fistulae.,
Both USG and CT scan are reliable in the diagnosis and follow-up of the liver abscesses. The distinction between pyogenic and amoebic liver abscess is difficult by imaging studies. The two can be differentiated by amoebic serology. Liver function tests and stool microscopy are non-discriminatory, and positivity rate of bacterial culture in aspirate and blood is only 50%., Grossly, pyogenic abscesses present as single or multiple cavities, filled with foul-smelling, creamy yellow, necrotic material which may have a fibrous capsule. Microscopically, necrotic material with numerous polymorphs are seen. Adjacent hepatocytes appear reactive. The liver involvement in amebiasis consists of central necrosis and peripheral histiocytes and inflammation. Fine-needle aspiration cytology (FNAC) from the center of the abscess hence does not yield any trophozoites, and only in 15% cases, FNAC can contribute in diagnosis. The peripheral abscess wall if sampled may yield amoebic trophozoites, as they commonly invade the surrounding hepatocytes. The abscess contains grossly chocolate-colored fluid that resembles anchovy paste and consists predominantly of necrotic hepatocytes. Periportal fibrosis may be present.
In the index study, we also tried to compare the prevalence of hepatobiliary cystic lesions (Institution based) between India and other Asian countries, as well as with the Western data [Table 2]. Extensive review of literature revealed that most of the data are from Japan,,,, where CCs are the most common. However, CCs, are less common in the West (2%–7% overall incidence).,, Asian prevalence of 1:1000 is mostly contributed by data from Japan (incidence: 1:13,000) and Korea.,,, Few cases also have been reported from Middle East., Few studies done in India suggest higher prevalence of extrahepatic biliary cystic lesions, especially the CCs, which is more common in North India than the south., However, overall incidence of all hepatobiliary cystic lesions have never been studied.
| Conclusions|| |
Cystic lesions of the liver and biliary tract encompass a wide spectrum of disorders, predominantly congenital, related to the embryological developmental or ductal plate malformation and a few acquired lesions. Hepatobiliary cysts are more common in females. Although in different geographical regions, type of cysts varies, CC was the most common in our cohort, followed by hydatid cysts, CHF, and simple hepatic cysts. Histological examination is important in diagnosis, as often the clinicoradiological findings are overlapping. Histological examination can also rule out presence of early changes of malignancy.
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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.
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Conflicts of interest
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Dr. Prasenjit Das
Department of Pathology, AIIMS, New Delhi
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]
[Table 1], [Table 2]
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