Multiple hemangiomas (hepatic small vessel neoplasia) in the liver with Budd-Chiari syndrome

Hepatic small vessel neoplasia (HSVN) is a recently recognized hemangioma of the liver with uncertain malignant potential. Almost all the patients are asymptomatic. Budd-Chiari syndrome (BCS) is a rare disorder characterized by noncardiogenic hepatic venous outflow obstruction. Benign hepatocellular...

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Bibliographic Details
Published in:Virchows Archiv : an international journal of pathology 2023-06, Vol.482 (6), p.1085-1089
Main Authors: Kobayashi, Hiroshi, Notohara, Kenji, Nakashima, Mitsuko, Ujita, Masuo, Takano, Toru, Tsubata, Shunsuke, Ohashi, Riuko, Saitsu, Hirotomo, Sugitani, Souichi
Format: Article
Language:English
Subjects:
Autopsies
Autopsy
Blood flow
Blood vessels
Brief Report
Budd-Chiari Syndrome - pathology
Carcinoma, Hepatocellular - pathology
Gastroenterology
Hemangioma
Hospitals
Humans
Hyperplasia
Liver
Liver cancer
Liver Neoplasms - pathology
Medical imaging
Medicine
Medicine & Public Health
Metastasis
Nodules
Pathology
Tumors
Veins & arteries
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Summary:Hepatic small vessel neoplasia (HSVN) is a recently recognized hemangioma of the liver with uncertain malignant potential. Almost all the patients are asymptomatic. Budd-Chiari syndrome (BCS) is a rare disorder characterized by noncardiogenic hepatic venous outflow obstruction. Benign hepatocellular nodules have been acknowledged for a long time in the liver with the chronic BCS. However, there has been no case report of BCS associated with HSVN. The patient was diagnosed with BCS 13 years ago. The imaging test initially displayed multiple hepatic nodules that were suspected of benign hepatocellular nodules. They gradually increased in size and number in the course of the disease. At an autopsy, these nodules were confirmed to be multifocal HSVN. The tumor of the present case could not be proved to have GNAQ and GNQ14 mutations. We describe the case focusing on the chronological imaging changes and discuss on the relationship between BCS and HSVN.
ISSN:0945-6317
1432-2307
DOI:10.1007/s00428-023-03505-w