Giant ectopic liver, hepatocellular carcinoma and pachydermia-a rare genetic syndrome?

Dettmer, Matthias; Itin, Peter; Miny, Peter; Gandhi, Manoj; Cathomas, Gieri; Willi, Niels (2011). Giant ectopic liver, hepatocellular carcinoma and pachydermia-a rare genetic syndrome? Diagnostic pathology, 6(75), p. 75. BioMed Central 10.1186/1746-1596-6-75

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Ectopic liver is a very uncommon developmental anomaly that predisposes to the development of hepatocellular carcinoma. We describe the second documented case of a hepatocellular carcinoma developing in the primary liver of a patient with a rare and uncharacterized genetic symptom complex. Also present was the largest ectopic liver ever reported, measuring 12 cm in diameter which contained a solitary focus of metastatic hepatocellular carcinoma. The primary hepatocellular carcinoma is believed to have arisen in the native liver from a hepatic adenoma that was diagnosed 15 years earlier. The patient's uncharacterised condition featured prominent thick, yellow skin over the dorsum of the fingers, and was associated with follicular hyperkeratosis, abnormal plantar creases, digital clubbing, misshaped ears, a lingua plicata and an angioleiomyolipoma of the right kidney. This unique case of hepatocellular carcinoma arising from liver cell adenoma in a patient with an uncharacterised condition featuring a large ectopic liver invites discussion of the role of local factors in carcinogenesis in the parent liver but not the ectopic liver. It also underlines the imperative ongoing need for clinical autopsies.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Service Sector > Institute of Pathology
04 Faculty of Medicine > Service Sector > Institute of Pathology > Autopsy
04 Faculty of Medicine > Service Sector > Institute of Pathology > Clinical Pathology
04 Faculty of Medicine > Service Sector > Institute of Pathology > Tumour Pathology

UniBE Contributor:

Dettmer, Matthias

Subjects:

500 Science > 570 Life sciences; biology
600 Technology > 610 Medicine & health

ISSN:

1746-1596

Publisher:

BioMed Central

Language:

English

Submitter:

Matthias Dettmer

Date Deposited:

04 Dec 2019 11:09

Last Modified:

28 Nov 2020 02:30

Publisher DOI:

10.1186/1746-1596-6-75

PubMed ID:

21831298

BORIS DOI:

10.7892/boris.136132

URI:

https://boris.unibe.ch/id/eprint/136132

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