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Fulltext Exploring the molecular pathogenesis, diagnosis and treatment of fibrolamellar hepatocellular carcinoma: A state of art review of the current literature

Alshareefy Y, Shen CY, Prekash RJ

Pathol Res Pract, 2023 Aug;248:154655.
PMID: 37429175 DOI: 10.1016/j.prp.2023.154655

This paper aims to present a detailed overview of fibrolamellar carcinoma (FLC), a variant of hepatocellular carcinoma (HCC) that accounts for approximately 1-9% of all cases a. according to the SEER database. Despite ongoing research, the aetiology of FLC tumours remains unclear. Nevertheless, FLC is believed to have a better overall prognosis than other primary liver tumours, such as hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma. This study aims to present a comprehensive overview of fibrolamellar carcinoma (FLC), with a focus on its epidemiology, pathogenesis, diagnosis, treatment, and prognosis. FLC frequently incorporate features of stomach pain, weight loss, and malaise in their clinical signs and symptoms, which are generally nonspecific Ultimately, the most common physical finding is an abdominal mass or hepatomegaly. With this said, several unusual presentations have been documented such as Budd Chiari syndrome, severe anaemia, non-bacterial thrombotic endocarditis and many more. In regards to this tumour's genetic analysis, it is characterised by a 400 kb deletion on chromosome 19 leading to a functional DNAJB1-PRKACA chimeric transcript in addition to tetraploidy in 50% of cases. FLC is chromosomally stable as compared to typical HCC. mTOR pathway activation has also been found to play a critical role in 47% of these tumours and EFGR over-expression is also evident. Fibrolamellar carcinomas (FLCs) exhibit a distinctive gross appearance, characterized by a yellow to pale tan colour, with a consistency that can vary from soft to firm and hard. In addition, a central scar is observed in 60-70% of FLC cases. The central scar is typically white or grey in colour and has a fibrous appearance, which is often surrounded by nodular, tumour-like tissue. Its histologic appearance is characterized by large polygonal cells with abundant eosinophilic cytoplasm, large vesiculated nuclei, large nucleoli, and arranged in lamellar bands of collagen fibres. Lamellar bands of fibrosis, consisting of collagen type I, III and IV, have also been identified as a distinctive histologic feature that is observed under low power magnification. Ultrasound, CT and MRI along with image guided biopsy are the primary modalities in diagnosis. Current management options include systemic therapy which has thus far been unremarkable with platinum-based therapies as well combination therapy with interferon alpha-2b being the most successful options. Surgical resection remains the primary treatment modality and there have been no advances in targeted therapies. Although the prognosis for FLC is favourable as compared to other hepatic cancer subtypes such as intrahepatic cholangiocarcinoma, there is a high rate of recurrence ranging from 33% to 100% with a median recurrence-free survival of 20-48 months. As a result of this there is a low overall cure rate associated with this tumour type and much more research is required to gain an in-depth understanding of the molecular mechanisms occurring in order to provide more adequate treatment to patients who suffer from this condition.

Matched MeSH terms: Bile Ducts, Intrahepatic/pathology
Fulltext Alteration of amino acid and biogenic amine metabolism in hepatobiliary cancers: Findings from a prospective cohort study

Stepien M, Duarte-Salles T, Fedirko V, Floegel A, Barupal DK, Rinaldi S, et al.

Int J Cancer, 2016 Jan 15;138(2):348-60.
PMID: 26238458 DOI: 10.1002/ijc.29718

Perturbations in levels of amino acids (AA) and their derivatives are observed in hepatocellular carcinoma (HCC). Yet, it is unclear whether these alterations precede or are a consequence of the disease, nor whether they pertain to anatomically related cancers of the intrahepatic bile duct (IHBC), and gallbladder and extrahepatic biliary tract (GBTC). Circulating standard AA, biogenic amines and hexoses were measured (Biocrates AbsoluteIDQ-p180Kit) in a case-control study nested within a large prospective cohort (147 HCC, 43 IHBC and 134 GBTC cases). Liver function and hepatitis status biomarkers were determined separately. Multivariable conditional logistic regression was used to calculate odds ratios and 95% confidence intervals (OR; 95%CI) for log-transformed standardised (mean = 0, SD = 1) serum metabolite levels and relevant ratios in relation to HCC, IHBC or GBTC risk. Fourteen metabolites were significantly associated with HCC risk, of which seven metabolites and four ratios were the strongest predictors in continuous models. Leucine, lysine, glutamine and the ratio of branched chain to aromatic AA (Fischer's ratio) were inversely, while phenylalanine, tyrosine and their ratio, glutamate, glutamate/glutamine ratio, kynurenine and its ratio to tryptophan were positively associated with HCC risk. Confounding by hepatitis status and liver enzyme levels was observed. For the other cancers no significant associations were observed. In conclusion, imbalances of specific AA and biogenic amines may be involved in HCC development.

Matched MeSH terms: Bile Ducts, Intrahepatic/metabolism; Bile Ducts, Intrahepatic/pathology; Bile Ducts, Extrahepatic/metabolism; Bile Ducts, Extrahepatic/pathology
Bile duct injury

Strong RW

Med J Malaysia, 2005 Jul;60 Suppl B:141-3.
PMID: 16108198
Matched MeSH terms: Bile Ducts/injuries*
Letter: Liver fluke in a Malaysian Orang Asli (Aborigine)

Prathap K

Trans R Soc Trop Med Hyg, 1973;67(6):881-2.
PMID: 4788767
Matched MeSH terms: Bile Ducts/pathology
Factors influencing outcome after hepatic portoenterostomy among extrahepatic bile duct atresia patients in Hospital Sultanah Bahiyah, Alor Setar

Namasemayam D, Nallusamy M

Med J Malaysia, 2017 12;72(6):329-332.
PMID: 29308768 MyJurnal

INTRODUCTION: Biliary atresia implies high risk of mortality if not diagnosed and treated early. We undertook this study to assess the prognostic factors affecting the outcome of Kasai surgery.
METHODS: A retrospective clinical study was conducted among 58 patients from Hospital Sultanah Bahiyah Alor Setar. Data were analysed with logistic regression analysis using SPSS. Factors studied included age during surgery, bile duct diameter at the porta hepatic, race of patient, preoperative total bilirubin level and cholangitis. These factors were analysed to determine its significance as a prognostic factor affecting the outcome of Kasai surgery.
RESULTS: Older age group above 56 days of life, bile duct diameter at porta hepatis measuring <50μm, pre-operative total bilirubin >10mg% and cholangitis were found to be poor prognostic factors from univariate regression analysis. Variables having p value<0.025 were analysed using the multivariable regression analysis. Only age of patient and diameter of bile duct at the porta hepatis were eligible for this analysis. The final analysis showed that age 57 days of life and above (adjusted odd's ratio (aOR) = 9.412, p value = 0.042, 95% confidence interval (95%CI) = 1.079 to 82.104) and bile duct diameter <50μm (aOR = 13.812, p value = 0.016, 95%CI = 1.616 to 118.042) were significant factors affecting the outcome.
CONCLUSION: In conclusion, age of patient 56 days of life and younger and diameter of bile duct at porta hepatis ≥50μm gave a significantly better outcome after Kasai surgery.

Matched MeSH terms: Bile Ducts, Extrahepatic/surgery*
Intrahepatic biliary lithiasis

Noda A

Trop Gastroenterol, 1991 Jan-Mar;12(1):3-14.
PMID: 2058008

It has been known that intrahepatic biliary lithiasis (IHBL) is prevalent in East Asia including Japan, South Korea, Taiwan, Malaysia, Hong Kong, and Singapore. In contrast, the entity has drawn little attention in Europe and the United States where only scattered reports appear. IHBL can be placed in the category of the benign disease. Its distinctive clinical picture is an intractable course necessitating multiple surgical interventions because recurrence is usual, rather than exceptional. This is in distinct contrast to ordinal stones which originate in the gallbladder. Patients with IHBL do not rarely die of progressive hepatic damage resulting from longstanding obstructive jaundice, cholangitis, liver abscess, septicemia, and so forth.

Matched MeSH terms: Bile Ducts, Intrahepatic/radiography; Bile Ducts, Intrahepatic/surgery
Placement of removable metal biliary stent in post-orthotopic liver transplantation anastomotic stricture

Tee HP, James MW, Kaffes AJ

World J Gastroenterol, 2010 Jul 28;16(28):3597-600.
PMID: 20653071

Postoperative biliary strictures are the most common cause of benign biliary stricture in Western countries, secondary to either operative injury or bile duct anastomotic stricture following orthotopic liver transplantation (OLT). Surgery or endoscopic interventions are the mainstay of treatment for benign biliary strictures. We aim to report the outcome of 2 patients with refractory anastomotic biliary stricture post-OLT, who had successful temporary placement of a prototype removable covered self-expandable metal stent (RCSEMS). These 2 patients (both men, aged 44 and 53 years) were given temporary placement of a prototype RCSEMS (8.5 Fr gauge delivery system, 8 mm x 40 mm stent dimensions) in the common bile duct across the biliary stricture. There was no morbidity associated with stent placement and removal in these 2 cases. Clinical parameters improved after the RCSEMS placement. Long-term biliary patency was achieved in both the patients. No further biliary intervention was required within 14 and 18 mo follow-up after stent removal.

Matched MeSH terms: Bile Ducts/pathology; Bile Ducts/surgery*
Determination of chemical composition of gallbladder stones and their association with induction of cholangiocarcinoma

Hussain SM, Al-Jashamy KA

Asian Pac J Cancer Prev, 2013;14(11):6257-60.
PMID: 24377514

Gallstone disease is a major surgical problem in many populations; it is probably related to diet, especially excessive consumption of meat. The objective of this study was to determine the chemical composition of gallstones and their association with neoplastic changes including cholangiocarcinomas in cholecystectomised patients. The chemical composition of gallstones from 40 patients (8 males and 32 females) was analyzed. This is a prospective study performed in Baquba teaching hospital in the period from 1/10/2012 to 1/1/2013 in which we collected the gallstones for the patients who underwent cholecystectomy, whether open or laparoscopic. The stones were classified according to their chemical composition as a mixed stones (MS), and examined using a stone analysis set (chemical qualitative method) for calcium, magnesium, phosphate, uric acid and oxalate which was used reagent for qualitative determination of main individual components of stones. The results of this study showed the highest incidence of gallstones in the age group 40-49 was 13 cases followed by 11, 8 and 4 cases for age groups 30-39, 50-59, 20-29 and 60 and above, respectively. The chemical analysis showed the majority of gallstones were mixed, 38 containing calcium followed by 37 cases with uric acid, 28 with magnesium, and 25 and 22 stones with oxalate and phosphate, respectively. Microscopically, we confirmed neoplastic changes (17.5%) as cholangiocarcinomas (CCCs) (7.55%) and dysplastic cells of carcinoma in situ in 4 (10%), 31 (77.5%) cases were chronic cholecystitis and 2 (5%) cases were acute cholecystitis with empyema out of bile duct disorders patients. In conclusion, majority of cases had mixed gallstones that involved five and four of inorganic chemicals of calcium, magnesium and phosphate, the highest incidence of gallstones in age group 40-49 years old was 13 cases, and neoplastic changes were confirmed (17.5%) including CCCs, (7.5%) and dysplastic cells of carcinoma in situ (10%), while 31 (77.5%) cases were chronic cholecystitis.

Matched MeSH terms: Bile Ducts, Intrahepatic/metabolism; Bile Ducts, Intrahepatic/pathology*
Undifferentiated (embryonal) sarcoma of the liver in a six-year old: a case report

Rahman Jamal, Sharifah, N.A., Zulfiqar, A., Zakaria, Z.

Malaysian Journal of Child Health, 1997;9(2):199-202.
MyJurnal

We report a rare case of undifferentiated (embryonal) sarcoma of the liver in a six-year-old girl who at presentation, had fever, right hypochondrium pain and hepatomegaly. The diagnosis was clinched by fine needle aspiration cytology and was subsequently reconfirmed by histopathological examination of the resected tumour. Pre-operative chemotherapy was given because primary resection was deemed not possible. The patient underwent a successful extensive hepatectomy followed by continuation chemotherapy

Matched MeSH terms: Bile Ducts, Intrahepatic
Fulltext Right posterior segmental hepatic duct: role of intraoperative cholangiogram to prevent extrahepatic biliary duct injuries

Ikhwan SM, Zamri Z, Hairol O, Razman J, Affirul C

International Medical Journal Malaysia, 2015;14(2):63-66.
MyJurnal

Extrahepatic bile ducts constitute a significant anatomic site for surgeons when performing hepatobiliary
operations from minor procedure such as cholecystectomy to major operation such as hepatectomy. The
challenges for surgeon in performing such cases increase when congenital variance of biliary tree occurs which
may lead to unintentional bile duct injuries. We reported a case of a lady who presented with obstructive
jaundice secondary to huge distal common bile duct stone. The presence of right posterior segmental hepatic
duct, which rarely occurs, was discovered during intraoperative cholangiogram before common bile duct
exploration. The operation was successful without incidence of biliary duct injury.

Matched MeSH terms: Bile Ducts, Extrahepatic
Fulltext Cholangiocarcinoma: a 10-year experience of a single tertiary centre in the multi ethnicity-Malaysia

Yusoff AR, Siti ZM, Muzammil ARM, Yoong BK, Vijeyasingam R

Med J Malaysia, 2012 Feb;67(1):45-51.
PMID: 22582548 MyJurnal

Cholangiocarcinoma (CCA) is a rare biliary tract epithelial malignancy. We described the clinical features of CCA patients in our institution. A 10-year retrospective study of patients with cholangiocarcinoma in University Malaya Medical Centre was conducted. Clinical data and outcomes in relation to the three anatomical groups of CCA were collected and analysed. Of the 69 patients that were included 55% were male. Mean age was 61 years. Twelve (17%) had intrahepatic, 38 (55%) perihilar and 19 (28%) distal tumour. Mean age (p=0.043), median duration of symptoms (p=0.011), jaundice (p<0.001), total bilirubin level (p=0.003), INR (p=0.005) and mean tumour size (p=0.048) were significantly related to the site of tumour. Only 12 patients had curative resection with seven R0 resections. Cholangiocarcinoma is increasingly diagnosed in our population. Despite that, the diagnosis is still often late. Age, jaundice and tumour size may predict anatomical location of CCA.

Matched MeSH terms: Bile Ducts, Intrahepatic*
Acute cholecystitis caused by Clonorchis sinensis

Rohela M, Johari S, Jamaiah I, Init I, Lee SH

Southeast Asian J. Trop. Med. Public Health, 2006 Jul;37(4):648-51.
PMID: 17121288

We are reporting a case of a 43-year-old Chinese male from Hong Kong, who came to see a doctor complaining of acute onset of severe upper abdominal pain. A diagnosis of acute cholecystitis was made and an emergency cholecystectomy was carried out. On opening the common bile duct, lancet-shaped worms were seen emerging from it. About 45 adult worms were collected and sent to the Department of Parasitology University of Malaya for identification. The worms were identified as Clonorchis sinensis. After the operation the patient was treated with praziquantel and he had an uneventful recovery.

Matched MeSH terms: Bile Ducts/parasitology
Recurrent pyogenic cholangitis: 'sump syndrome' following choledochoduodenostomy

Khan TF, Sherazi ZA, Muniandy S, Mumtaz M

Trop Doct, 1997 Jan;27(1):51-2.
PMID: 9030026

An uncommon and late complication of side-to-side choledochoduodenostomy (CDD), the 'sump syndrome', developed in a patient 4 years after surgery. Recurrent right upper abdominal pain, fever with chills and rigors and latterly, mild jaundice made her seek repeated hospital admissions which were treated successfully with antibiotics. During the last admission, ultrasonography, endoscopic retrograde cholangiography (ERC), computerized scanning (CT) and hepatic iminodiacetic acid (HIDA) scan using Tc99m confirmed multiple intrahepatic calculi with proximal dilatation, debris in the distal blind segment and delayed excretion through the CDD. At surgery, the choledochoduodenostomy was taken down and a Rouxen-Y hepaticojejunostomy (RHJ) was fashioned after ductal clearance. The closed end of the Roux loop was placed subcutaneously for subsequent percutaneous access for cholangiography and removal of calculi. She is asymptomatic and well 28 months after surgery.

Matched MeSH terms: Bile Ducts, Intrahepatic/surgery
Type IVa choledochal cysts: surgical management and literature review

Thambi Dorai CR, Visvanathan R, McAll GL

Aust N Z J Surg, 1991 Jul;61(7):505-10.
PMID: 1859310

Type IVa choledochal cysts with cylindrical dilatation of the intrahepatic ducts constitute a relatively less recognized variety of choledochal cysts, and differ from cystic dilatation of intrahepatic ducts in their clinical manifestations and response to treatment. Five patients with type IVa choledochal cysts and cylindrical dilatation of major intrahepatic ducts who underwent cyst excision and Roux-en-Y hepaticojejunostomy are reported. The duration of symptoms was less than 1 year in all patients. Palpable abdominal mass and abdominal pain were present in 3 patients. The traid of jaundice, abdominal pain and mass was present in only 1 patient. The intrahepatic dilatation regressed after excision of the extrahepatic cyst just below the hilum of the liver. The surgical technique is described and the need for excision of the cyst is emphasized.

Matched MeSH terms: Bile Ducts/pathology*
Intrahepatic cholangiocarcinoma and portal hypertension developing in a patient with multicystic biliary microhamartomas

Sugawara T, Shindoh J, Hoshi D, Hashimoto M

Malays J Pathol, 2018 Dec;40(3):331-335.
PMID: 30580365

INTRODUCTION: We report a case of intrahepatic cholangiocarcinoma and portal hypertension developing in a liver with biliary microhamartomas (von Meyenburg's complex).
CASE REPORT: The patient was a 55-year-old man who had a past medical history of diffuse multiple liver abscesses. During follow-up examination, a hypovascular nodule measuring 2.1 cm in diameter was incidentally found in segment 8 of the liver. Surgical resection was performed based on a suspected diagnosis of hepatocellular carcinoma. A gastrofiberscopy examination detected characteristic findings of portal hypertensive gastropathy. During the laparotomy, multiple tiny cystic lesions were observed in a diffuse pattern across the liver surface. The liver parenchyma was slightly fibrotic and haemorrhagic. A histopathological examination revealed intrahepatic cholangiocarcinoma with vascular invasions in von Meyenburg's complex. Multiple biliary adenomas were also observed among the biliary microhamartomas adjacent to the main tumour, suggesting that the malignant transformation of the biliary adenomas might have been responsible for the development of the intrahepatic cholangiocarcinoma. The histopathologic examination also revealed sinusoidal dilation and abnormal spacing of the portal tracts and central veins as evidence of portal hypertension.

Matched MeSH terms: Bile Ducts, Intrahepatic/pathology
Endoscopic intervention for hepatolithiasis associated with sharp angulation of right intrahepatic ducts

Mahadeva S, Prabakharan R, Goh KL

Gastrointest Endosc, 2003 Aug;58(2):279-82.
PMID: 12872105

Hepatolithiasis (intrahepatic stones) is common in Asian patients. Hepatolithiasis with intrahepatic strictures and sharp ductal angulation poses a particularly difficult management problem.

Matched MeSH terms: Bile Ducts, Intrahepatic/pathology*
Fulltext Caroli's disease: functional diagnosis using 99mTc diethyl-iminodiacetic acid (IDA) hepatobiliary scintigraphy

Lai FM, Paramsothy M, Goh KL, Boey C

Singapore Med J, 1997 Apr;38(4):164-5.
PMID: 9269396

A case of Caroli's disease is presented and the findings of various imaging modalities are discussed with emphasis on the role of Technetium 99mTc diethyl-iminodiacetic acid scan in reaching the final diagnosis. The 'beaded' appearance of the dilated intrahepatic ducts seen in this scan is believed to be pathognomonic for this condition.

Matched MeSH terms: Bile Ducts, Intrahepatic/radionuclide imaging*
Fulltext Biliary ascariasis and extrahepatic cholangiocarcinoma: a report of two cases

Lim KG, Sellaiah SP

Singapore Med J, 1994 Aug;35(4):400-2.
PMID: 7899902

We report two cases of rural Malay women in Perak, Malaysia, with extrahepatic cholangiocarcinoma and coexistent biliary ascariasis. In both cases, the narrowed bile duct may have resulted in inability of the migratory nematode to return to the gastrointestinal tract. It may be reasonable, also, to postulate that chronic biliary tract infestation by Ascaris lumbricoides may have contributed to the development of cholangiocarcinoma; a situation similar to that observed in liver fluke infestation.

Matched MeSH terms: Bile Ducts, Extrahepatic/pathology*
Fulltext The utility of hepatocyte paraffin 1 antibody in the immunohistological distinction of hepatocellular carcinoma from cholangiocarcinoma and metastatic carcinoma

Shiran MS, Isa MR, Mohd Sidik S, Rampal L, Hairuszah I, Sabariah AR

Malays J Pathol, 2006 Dec;28(2):87-92.
PMID: 18376797 MyJurnal

Hepatocellular carcinoma (HCC) is the most common primary liver cancer and its diagnosis on routine stains is usually straightforward, except in some cases where there may be difficulty in distinguishing HCCs from metastatic carcinomas (MC) and cholangiocarcinomas (CC). Hepatocyte Paraffin 1 antibody (Hep Par 1) is a new monoclonal antibody which reacts with normal and neoplastic hepatocytes, and this study aims to determine its specificity and sensitivity in distinguishing hepatocellular carcinoma (HCC) from cholangiocarcinoma (CC) and metastatic carcinomas (MC). Hep Par 1 antibody was applied to 28 cases of HCC, 22 cases of MC from varying sites and 8 CCs, and produced a strong, diffuse, granular, cytoplasmic staining of all benign hepatocytes. 23 out of 28 cases of HCC showed heterogeneously positive staining for Hep Par 1 irrespective of their degree of differentiation, while 2 out of 8 cases of cholangiocarcinoma were positive for Hep Par 1, and all 22 cases of metastatic carcinoma were negative. The sensitivity and specificity of Hep Par 1 for HCC was 82.1% and 93.3% respectively; whereby the antibody was noted to show occasional false positivity in cases of cholangiocarcinoma and non-neoplastic bowel mucosa, while its variable staining in HCC produced false negative results in some small biopsies. Thus, Hep Par 1 should be used in a panel with other antibodies to obtain useful information in distinguishing HCC from CC and MC.

Matched MeSH terms: Bile Ducts, Intrahepatic/pathology*; Bile Ducts, Intrahepatic/chemistry
Fulltext A puzzling case of biliary stricture with calcified liver nodules

Leow, V.M., Faizah, M.S., Yang, K.F., Hasnan, M.N., Manisekar, S.S.

International Medical Journal Malaysia, 2013;12(2):79-82.
MyJurnal

Tuberculous (TB) biliary stricture with calcified liver nodules rendering obstructive jaundice is a rare clinical phenomenon. Recently, we encountered a young patient with obstructive jaundice who was investigated in a general hospital. He was sent to our hospital for subsequent management after undergoing a series of investigations and biliary stenting. The radiological imagings performed revealed multiple calcified lesions in the liver with proximal bile duct strictures. Blood investigations, tumor markers and tuberculous work up were not remarkable. Subsequently, he underwent laparotomy and biliary reconstruction. Postoperatively, he was discharged well. Histopathological examination of the hepatoduodenal nodes showed chronic infections and granulomatous lymphadenitis, suspicious of a mycobacterium infection.

Matched MeSH terms: Bile Ducts

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