A rare encounter - Unveiling the mysteries of biliary hamartoma: A case report.

INTRODUCTION: Biliary hamartomas are rare congenital development anomaly of bile ducts, which are detected incidentally. They often present as multiple lesions on liver surface which resembles metastatic lesions. We report a case of acute calculous cholecystitis ultimately diagnosed to have asymptomatic multiple biliary hamartomas. CASE DESCRIPTION: A 42-year-old male with no co-morbidities presented with pain in upper abdomen associated with fever and vomiting. Contrast enhanced computed tomography (CECT) of abdomen showed acute calculous cholecystitis, hepatomegaly with fatty changes in liver. On laparoscopy the liver was found to have grey-white nodular lesions of about 0.5 cm in diameter scattered on the surface of both the lobes. One of the lesion was biopsied along with cholecystectomy. DISCUSSION: Biliary hamartoma commonly referred to as "von Meyenburg complexes" are uncommon lesions found in the liver which are usually asymptomatic. In this case the patient presented with symptoms of acute cholecystitis but the biopsy report from liver lesions proved to be benign biliary hamartoma which on initial impression looked like multiple liver secondaries. CONCLUSION: We have described a case of an adult with multiple biliary hamartoma which was an incidental finding. Biliary hamartoma is a rare entity which can sometime mimic metastasis in the liver. Thus, histopathological confirmation is essential before planning any further treatment.

Intrahepatic Cholangiocarcinoma Arising from Biliary Hamartomas in Patients with Recurrent Acute Cholangitis: A Case Report and Literature Review.

Biliary hamartomas are tumor-like malformations of the liver. Biliary hamartomas are a type of fibrocystic disorder originating from ductal plate malformation and are typically considered benign, but with the risk of malignant transformation. In this case report, we present a rare occurrence of intrahepatic cholangiocarcinoma (ICC) that developed from biliary hamartomas, along with a literature review. A 76-year-old man with a diagnosis of biliary hamartomas had a history of recurrent cholangitis for 12 years, necessitating cholecystectomy, ERCP, and repeated antibiotic treatments. During his last episode, imaging studies revealed a hypervascular infiltrative mass in the right posterior liver segment. A liver biopsy confirmed adenocarcinoma and subsequent surgical pathology revealed ICC originating from biliary hamartomas. Chronic inflammation in the bile duct associated with biliary hamartomas may serve as a potential trigger for malignant transformation, as observed in this case. Therefore, close surveillance is essential for patients with biliary hamartomas presenting with infectious complications.

RECURRENT SEPSIS IN A PATIENT WITH BILIARY HAMARTOMAS.

Biliary hamartomas or von Meyenburg complexes (VMCs) are hepatic tumour-like lesions related to congenital malformation of the ductal plate, and are part of the ciliopathy spectrum of disorders. The exact pathogenesis of VMCs is unclear and it remains controversial whether they have the potential for malignant transformation. Patients are often asymptomatic and VMCs are usually encountered as an incidental finding on imaging. We report a case of recurrent sepsis with an unidentified focus. It was later confirmed that biliary hamartomas were acting as a sanctuary for the persistent pathogenic agent. The authors hope to draw attention to the existence of this unusual focus of recurrent sepsis. LEARNING POINTS: Hepatobiliary sepsis is an unusual clinical presentation of biliary hamartomas.Clinicians should be aware of the infectious complications of these diffuse structural biliary ductal abnormalities.Early recognition of this atypical life-threatening clinical presentation is important for the prognosis.

A Case of Multicystic Biliary Hamartoma with a Marked Peribiliary Gland Component Successfully Treated by Purely Laparoscopic Anatomical Liver Resection.

BACKGROUND: Multicystic biliary hamartoma (MCBH) is an extremely rare benign liver lesion characterized by a gross well-circumscribed multicystic honeycomb appearance. This report presents a MCBH case with a marked peribiliary gland component which showed unusual histology. CASE PRESENTATION: A 63-year-old Japanese male was admitted to our hospital for a detailed examination of a hepatic cystic lesion, which was originally detected 14 years ago and had slowly enlarged. A preoperative imaging study revealed a well-demarcated multicystic lesion without communication to the biliary tracts. The possible clinical diagnoses were mucinous cystic neoplasm (MCN) or MCBH. The lesion was successfully resected by purely laparoscopic right anterior sectionectomy. The cut surfaces of resected specimens grossly exhibited a well-circumscribed multicystic lesion with a thick septum. Histologically, the cyst wall was covered by cuboidal epithelial cells resembling epithelium of the bile duct while abundant small ducts, which morphologically resembled peribiliary glands, were observed among the fibrous stroma of the thick septum. Although possible pathological diagnosis varied, including intrahepatic cholangiocarcinoma, intraductal papillary neoplasm of the bile duct, biliary adenofibroma, MCN and MCBH, the lesion was finally diagnosed as MCBH with a marked peribiliary gland component. CONCLUSIONS: MCBH can contain abundant peribiliary glands in the fibrous stroma. A pathologist should be careful not to diagnose such peribiliary glands in MCBH as neoplastic glands.

"Starry liver" - Von Meyenburg complex clinical case presentation and differential diagnosis discussion: A case report.

BACKGROUND: Von Meyenburg complex (VMC) (i.e., biliary hamartoma) is a rare congenital disorder characterized by multiple dilated cystic bile ducts, without clear trends in sex or age predominance. Due to the low number of published cases and the lack of recognized guidelines, the management of such patients remains a clinical challenge. CASE SUMMARY: We present a case of symptomatic VMC that was diagnosed after imaging and histopathological examinations. Considering the patient's condition, a conservative treatment strategy was chosen. Instrumental, laboratory, and clinical follow-up demonstrated the stable condition of the patient receiving conservative treatment. CONCLUSION: VMC is a potentially non-life threatening condition, but its recognition is crucial for the management of patients.

Intrahepatic multicystic biliary hamartoma: A case report.

BACKGROUND: Multicystic biliary hamartoma (MCBH) is a rare hamartomatous nodule of the liver, which has recently been described as a new category of hepatic nodular cystic lesion. Most of them are benign. The imaging findings are similar to those of many other hepatic cystic lesions, but MCBH also has some notable features, such as large cysts, smooth cyst walls, and lack of communication with the hepatic duct. Due to the non-specific radiology, preoperative diagnosis is difficult, and is usually diagnosed by postoperative pathology. Complete resection is the best treatment option, and the postoperative prognosis is good. CASE SUMMARY: When the patients have MCBH, the symptoms may not very typical, and they require a combination of imaging and pathology for diagnosis. Under normal circumstances, the prognosis of MCBH is good. However, in patients with MCBH, more cases need to be observed for verification. CONCLUSION: When the patients have MCBH, the symptoms may not very typical, and they require a combination of imaging and pathology for diagnosis. Under normal circumstances, the prognosis of MCBH is good. However, in patients with MCBH, more cases need to be observed for verification.

Biliary hamartoma: a report of two cases.

Biliary hamartoma, also known as biliary micro hamartoma or Von Meyenburg complex, is a rare benign liver lesion, thought to be a ductal plate malformation rather than a true neoplasm. It is often seen incidentally on imagery or surgery as multiple small subcapsular nodules, scattered throughout the liver, making it likely to be mistaken for metastatic nodules. The histological presentation can also be deceptive, leading to the misdiagnosis of an adenocarcinoma of hepato-biliary differentiation or a metastasis. We hereby present two cases of biliary hamartoma, found incidentally on imagery and surgery, the first one in a 94-year-old woman, and the second in a 48-year-old man, which was initially misdiagnosed as an adenocarcinoma, along with a discussion of key clinical and pathological findings to help avoid this diagnostic pitfall.

Characteristics of multicystic biliary hamartoma: A case report.

Introduction: Multicystic biliary hamartoma (MCBH) is a very rare hepatic benign neoplasm that manifests as a localized cystic-solid mass. Only 17 cases have been described in the literature to date. MCBH diagnosis is currently dependent on imaging and pathology following surgical resection and no precise standards are in place. Case Presentation: This case study involves a middle-aged male patient with a history of drinking but no other liver diseases. A routine ultrasound examination showed a 6.0 × 5.5 cm inhomogeneous echo mass in the right lobe of the liver. The patient experienced no discomfort or other symptoms, and blood tests were normal. Imaging revealed a localized cystic-solid neoplasm in segment 6 of the liver that did not have the features of a malignant tumor. Surgical resection was performed. Based on imaging, macroscopic examination, and histological results, a final diagnosis of MCBH was made. Conclusion: The imaging and pathological features of MCBH were summarized based on the published case reports to date. As a non-invasive examination, the imaging features will aid in the diagnosis of MCBH. Furthermore, these features, along with tumor size and patient symptoms, will facilitate clinicians in selecting surgical resection or follow-up for individual patients.

Case Report: Incidentally Discovered a Rare Cystic Lesion of Liver: Multicystic Biliary Hamartoma.

Multicystic biliary hamartoma (MCBH) is an extremely rare cystic lesion of the liver. A 37-year old male patient was admitted to our hospital for incidentally discovered hepatic cystic lesions on abdominal ultrasonography. Abdominal contrast-enhanced computed tomography (CT) showed a multilocular cystic lesion in the segment VI, with mild enhancement in the septae and peripheral wall within the lesion. Only alanine transaminase (ALT) and carbohydrate antigen 19-9 (CA19-9) increased slightly above normal value. Preoperative tests suggested possibility of a benign mucinous cystic neoplasm (MCN) or intraductal papillary neoplasm of the bile duct (IPNB). Laparoscopic complete resection of the lesion was performed. Histopathological examination showed numerous variably sized ductal structures surrounded by periductal glands and fibrous connective tissues containing small blood vessels and smooth muscle bundles. Immunohistochemical staining (IHC) revealed that dilated ducts were positive for cytokeratin CK19, characteristic for biliary tract. Histopathological findings confirmed diagnosis of multicystic biliary hamartoma (MCBH). No recurrence occurred during 6 months follow-up. In conclusion, MCBH should be differentiating from hepatic cystic lesion and could be resected laparoscopically safely.

Multicystic biliary hamartoma with extremely elevated CA19-9: a case report.

Multicystic biliary hamartoma (MCBH) is a rare cystic disease of the liver. We herein report a case of MCBH associated with extremely elevated levels of serum carbohydrate antigen (CA) 19-9. A 53-year-old man was referred to our hospital because of extremely elevated CA19-9 levels (more than 12,000 U/mL). Enhanced abdominal computed tomography and magnetic resonance imaging (MRI) revealed a multicystic tumor with a calcified wall in the left lobe of the liver, although no apparent intracystic nodule was detected. Because of the possibility of a malignant tumor, such as intraductal papillary neoplasm of the bile duct or cystadenocarcinoma, the patient underwent left hepatectomy. Based on the postoperative pathological findings, the lesion was diagnosed as MCBH. The serum CA19-9 level drastically decreased after surgery. We encountered a rare case of MCBH with extremely elevated CA19-9 levels.

Proliferative index facilitates distinction between benign biliary lesions and intrahepatic cholangiocarcinoma.

Differentiation between benign and malignant lesions of the hepatic biliary tree may pose a diagnostic problem because well-differentiated intrahepatic cholangiocarcinoma may mimic biliary hamartoma, bile duct adenoma, or parenchymal extinction. We evaluated Ki-67 proliferative index and p53 status by immunohistochemical staining to aid in exclusion of cholangiocarcinoma. Fourteen biliary hamartomas, 21 bile duct adenomas, and 11 livers with parenchymal extinction were compared with 26 intrahepatic cholangiocarcinomas (16 well-differentiated and 10 moderately or poorly differentiated tumors). We found an increased proliferative index in intrahepatic cholangiocarcinomas compared with benign biliary lesions (average 23.0% in cholangiocarcinoma versus 1.4% in all benign biliary lesions, n = 26 versus n = 46, P < .001). No difference in average proliferative index was observed between well-differentiated and moderately/poorly differentiated cholangiocarcinomas (average 22.7% versus 23.3%, n = 16 versus n = 10, P = .92). Average proliferation indices of benign biliary lesions were uniformly low (biliary hamartoma, 1.2%; bile duct adenoma, 2%; parenchymal extinction, 0.5%). Most cholangiocarcinomas (23/26; 88.5%), but none of the benign lesions (0/46; 0%), had proliferative indices greater than 10%. Strong nuclear p53 immunohistochemical staining was only seen in cholangiocarcinomas (9/26; 34.6%) and not in benign biliary lesions (0/46; 0%), although many of the benign lesions showed weak to moderate staining. Immunohistochemical staining for Ki-67 facilitates distinction between benign and malignant lesions of the intrahepatic biliary tree, whereas p53 immunohistochemical staining is less helpful.

Neoplasias hepáticas benignas: variantes histológicas, problemas diagnósticos y diagnóstico diferencial / Diagnosis and Differential Diagnosis Problems with Histological Variants of Benign Liver Neoplasms

Un principio básico de la patología es que las neoplasias se diferencian según sus células de origen y en el hígado semejan sus constituyentes, sean las células hepáticas, del epitelio biliar, endoteliales, mesenquimales o una combinación de estas. Es importante recordar aquí que son las metástasis el tumor maligno más frecuente del hígado, con una relación de 30:1 en pacientes sin enfermedad hepática crónica o cirrosis subyacente; es rara la presencia de las mismas en hígados cirróticos. Las neoplasias gastrointestinales del colon, páncreas, vía biliar extrahepática, estómago, tumores neuroendocrinos y GIST, o extraintestinales del pulmón, mama, melanoma o tumores de cabeza y cuello, son las más frecuentes (1). En este artículo solo revisaremos las más frecuentes. Iniciaremos con las neoplasias benignas y las lesiones pseudotumorales haciendo especial énfasis en aquellas con dificultades diagnósticas, en la utilidad de estudios especiales de inmunohistoquímica o moleculares para su adecuada clasificación y diagnóstico diferencial.

A basic principle of pathology is that neoplasms differ according to their cells of origin. Neoplasms of the liver resemble its constituent liver, biliary, epithelial, endothelial, mesenchymal cells or some combination of these different types of cells. It is important to remember here that metastases are the most frequent malignant liver tumor occurring at ratio of 30: 1 in patients without underlying chronic liver disease or cirrhosis. Metastases are rare in cirrhotic livers. The most common primary sites are the colon, pancreas, common bile duct, stomach, neuroendocrine tumors and GIST, or extraintestinal tumors from the lung, breast, head, neck and skin (1). In this article we review only the most frequent benign neoplasms and tumor-like lesions with particular emphasis on diagnostic difficulties, special studies, and the usefulness of immunohistochemical or molecular studies for proper classification and differential diagnosis.

Appearances of von meyenburg complex on cross sectional imaging.

The von Meyenburg complex (VMC) is an uncommon congenital malformation and is characterized by benign bile duct hamartomas. These are usually discovered incidentally and may represent a diagnostic dilemma when liver metastases are suspected. MRI of VMC shows distinct imaging characteristics, but reporting of lesional contrast enhancement has been inconsistent, whilst microbubble contrast enhanced ultrasound provides 'real-time' evaluation of soft tissue vascularity. Given the diagnostic uncertainty over imaging in VMC, biopsy is often recommended as the definitive diagnosis. We report a biopsy proven case of VMC on a background of primary colonic malignancy investigated with ultrasound, contrast enhanced ultrasound, computed tomography CT, and magnetic resonance imaging MRI, and review the key imaging features.

Biliary Hamartoma.

Biliary hamartoma or von Mayenburg complex (VMCs) is a rare benign congenital malformation of the biliary duct. Patients are usually asymptomatic. Hepatic lesions are incidentally discovered on ultrasonography (US) and subsequent radiological methods are necessary for confirmation. A correct diagnosis is established when typical imaging findings are present, otherwise histological confirmation might be needed.

Liver transplantation from a donor with multiple biliary hamartomata.

Biliary hamartomata are rare benign lesions. Herein, we report on a 48-year-old man with a history of end-stage liver disease secondary to alcoholic liver disease. The patient received an orthotropic liver transplant from a brain-death woman. At the time of recovery, there were multiple lesions in the transplanted liver measuring 7-10 mm. Pathology revealed multiple biliary hamartomata. The postoperative course of the recipient was uncomplicated and he was discharged home 10 days after the transplantation.

von Meyenburg's complex in a patient with primary malignancy: role of MRI.

von Meyenburg's complexes are benign liver malformations histologically constituting cystic dilatations of the bile ducts, surrounded by abundant fibrous stroma. Appearance of these lesions on MRI is characteristic and a fine needle aspiration or biopsy can be avoided and judicious management planned. We report a case of esophageal carcinoma who had suspicious lesions in the liver on CT scan, which after MRI were confirmed as biliary hamartomas.