The radiologic and pathologic spectrum of biliary hamartomas.

Lev-Toaff, Anna S.; Bach, Ariadne M.; Wechsler, Richard J.; Hilpert, P.; Gatalica, Zoran; Rubin, Rene

doi:10.2214/ajr.165.2.7618546

Cited by 103 publications

(55 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…1,4 Representam uma entidade clínico-patológica rara, tipicamente assintomática e sem alterações nos parâmetros hepáticos, podendo no entanto surgir sob a forma de dor abdominal ou colangite. 5 Apesar de raramente encontrado na prática clínica, a sua incidência em autópsias é estimada em cerca 5,6 % em adultos e 0,9% em crianças.…”

Section: Discussionunclassified

Complexo de Von Meyenburg ou Metástases Hepáticas? Caso Clínico e Revisão da Literatura

et al. 2014

View full text Add to dashboard Cite

(2):271-273 RESUMOAs lesões hepáticas são achados comuns na prática clínica. Os hamartomas dos ductos biliares, vulgarmente conhecidos por complexo de Von Meyenburg, são malformações hepáticas benignas que se apresentam histologicamente como pequenas dilatações quísticas dos ductos biliares, revestidas por tecido fibroso. Representam uma entidade clinico-patológica rara, tipicamente assintomática e sem alterações nos parâmetros hepáticos. O aspeto imagiológico é variável, podendo apresentar-se como múltiplas pequenas lesões dispersas pelo fígado, ou mais raramente como nódulo isolado. Os achados imagiológicos podem mimetizar lesões secundárias e, apesar da aparente benignidade da patologia, estão descritos casos de progressão para colangiocarcinoma. É, por isso, importante incluir este complexo no diagnóstico diferencial das lesões hepáticas focais, sendo fundamental uma investigação minuciosa para a sua diferenciação. Os autores pretendem com este caso dar a conhecer uma apresentação atípica de uma entidade rara e pouco ponderada na prática clínica. Palavras-chave: Hamartoma; Doenças das Vias Biliares. ABSTRACTHepatic lesions represent a common finding in clinical practice. Bile ducts hamartomas, also known as Von Meyenburg complex, are benign hepatic malformations composed of small dilated cystic bile ducts lined by fibrous stroma. They represent a rare and asymptomatic clinic-pathological entity. Imagiological findings are variable, and may present as multiple small scattered lesions, or rarely as a single nodule. These findings may resemble secondary lesions and, although benign, there are cases of progression to colangiocarcinoma. Thus it is important to include this complex in the differential diagnoses of focal hepatic lesions, being necessary a detailed investigation for their differentiation. This case represents an atypical presentation of a rare and not frequently considered entity in clinical practice.

show abstract

Section: Discussionunclassified

Complexo de Von Meyenburg ou Metástases Hepáticas? Caso Clínico e Revisão da Literatura

et al. 2014

View full text Add to dashboard Cite

show abstract

“…However, subsequent liver biopsy showed the presence of a unique group of cystic and dilated bile ducts surrounded by a dense fibrocollagenous stroma, which was revealed to be biliary hamartoma. Biliary hamartomas, also referred to as VMC, are benign liver malformations that histopathologically include a cystic dilated bile duct, typically 0.1-0.3 cm in diameter, embedded in abundant fibrous stroma [26] . As a result of its asymptomatic nature, this relatively rare entity is usually detected incidentally in 5.6% of reported autopsies [27] .…”

Section: Discussionmentioning

confidence: 99%

A variant form of autoimmune pancreatitis successfully treated by steroid therapy, accompanied by von Meyenburg complex

Miura¹,

Kitamura²,

Yamada³

2009

WJG

View full text Add to dashboard Cite

Diagnostic criteria for autoimmune pancreatitis (AIP) have been proposed and used clinically because, despite its unique clinicopathological features, AIP does not have disease-specific serological tests for confirmation. However, diagnosis of a patient with pancreatic lesions mimicking cancer who deviates from these diagnostic criteria is still difficult. We present herein a patient with a variant form of AIP successfully diagnosed by fine-needle biopsy, whose response to steroid therapy was excellent. A 55-year-old Japanese man was admitted to hospital because of jaundice and pancreatic head mass. AIP was considered as one of the differential diagnoses; however, as the patient showed neither pancreatic duct narrowing nor immunological abnormalities, he did not meet the Japanese diagnostic criteria for AIP. Histopathology of the pancreatic mass demonstrated abundant infiltration by lymphocytes and interstitial fibrosis, which suggested AIP. Immunoreaction to IgG4, which is supposed to be specific to AIP, was not observed; however, response to subsequent prednisolone therapy was good, with dramatic pancreatic head mass regression. Aside from the pancreatic head mass, diffusely spreading small lesions were observed throughout the liver. The likelihood of a potential association with extrapancreatic lesions of AIP was considered and led us to carry out a liver biopsy, which revealed biliary hamartoma, also called von Meyenburg complex (VMC). As IgG4-positive plasma cell infiltration was not demonstrated in the hamartomatous regions, the hepatic condition was thought to have occurred incidentally; however, to the best of our knowledge, this is the first report in which the association between AIP and VMC was investigated and discussed.

show abstract

“…20 The prevalence on autopsy ranges from 0.6 to 2.8%. 20 In clinical practice, the majority are discovered incidentally, but may present a diagnostic dilemma when the patient is undergoing staging imaging for known primary malignancy. 21 Sonographic appearances are varied, being described as multiple hypoechoic lesions, hyperechoic lesions and also a mixture of both hypo-and hyperechoic lesions.…”

Section: Bile Duct Hamartoma / Von Meyenberg Complexmentioning

confidence: 99%

“…21 Sonographic appearances are varied, being described as multiple hypoechoic lesions, hyperechoic lesions and also a mixture of both hypo-and hyperechoic lesions. 21 Other features include the presence of a comet tail artefact demonstrated on ultrasound 20 and there is a documented association with liver cysts. Berry et al 22 reported a case of multiple biliary hamartomas that did not demonstrate ....................................................................................................................... contrast enhancement in all phases of contrast ultrasound ( Figure 5).…”

Section: Bile Duct Hamartoma / Von Meyenberg Complexmentioning

confidence: 99%

Benign liver lesions: grey-scale and contrast-enhanced ultrasound appearances

Obaro

Ryan

2015

Ultrasound

View full text Add to dashboard Cite

Ultrasound is often the first point of detection of liver lesions, with up to 75% of liver lesions detected at ultrasound having benign histology. In 2012, NICE issued recommendations that ultrasound contrast be used for the evaluation of incidentally discovered liver lesions. This has been demonstrated to provide a rapid and cost-effective evaluation for incidental liver lesions, in many cases precluding the need for further CT or MRI scans. The aim of this review is to demonstrate the ultrasound features of benign liver lesions, and to demonstrate their further characterisation with contrast ultrasound.

show abstract

The radiologic and pathologic spectrum of biliary hamartomas.

Cited by 103 publications

References 9 publications

Complexo de Von Meyenburg ou Metástases Hepáticas? Caso Clínico e Revisão da Literatura

Complexo de Von Meyenburg ou Metástases Hepáticas? Caso Clínico e Revisão da Literatura

A variant form of autoimmune pancreatitis successfully treated by steroid therapy, accompanied by von Meyenburg complex

Benign liver lesions: grey-scale and contrast-enhanced ultrasound appearances

Contact Info

Product

Resources

About