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

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Abstract

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.

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Authors and Affiliations

  1. Department of Pathology, Tachikawa General Hospital, Nagaoka, Japan

    Hiroshi Kobayashi

  2. Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan

    Kenji Notohara

  3. Department of Biochemistry, Hamamatsu University School of Medicine, Hamamatsu, Japan

    Mitsuko Nakashima & Hirotomo Saitsu

  4. Department of Radiology, Tachikawa General Hospital, Nagaoka, Japan

    Masuo Ujita

  5. Department of Radiology, Tsubame Rosai Hospital, Tsubame, Japan

    Toru Takano

  6. Department of Gastroenterology, Tachikawa General Hospital, Nagaoka, Japan

    Shunsuke Tsubata & Souichi Sugitani

  7. Histopathology Core Facility, Faculty of Medicine, Niigata University, Niigata, Japan

    Riuko Ohashi

  8. Division of Molecular and Diagnostic Pathology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan

    Riuko Ohashi

  9. Department of Gastroenterology, Murakami General Hospital, Murakami, Japan

    Souichi Sugitani

Authors
  1. Hiroshi Kobayashi
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  2. Kenji Notohara
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  3. Mitsuko Nakashima
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  4. Masuo Ujita
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  5. Toru Takano
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  6. Shunsuke Tsubata
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  7. Riuko Ohashi
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  8. Hirotomo Saitsu
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  9. Souichi Sugitani
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Contributions

Hiroshi Kobayashi, Kenji Notohara, and Souichi Sugitani have designed and drafted the manuscript. Hiroshi Kobayashi, Kenji Notohara, and Riuko Ohashi made the histological diagnosis with the case. Shunsuke Tsubata and Souichi Sugitani played an important role in the management of the patient to compile the clinical data. Masao Ujita and Toru Takano made the imaging diagnosis to select representative figures of the CT imaging. Mitsuko Nakamura and Hirotomo Saitsu performed the genetic study of the patient to interpret the result. All the authors have read and approved the final manuscript.

Corresponding author

Correspondence to Hiroshi Kobayashi.

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This study has been approved by the institutional review boards of Tachikawa General Hospital and the Hamamatsu Medical School.

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The authors declare no competing interests.

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Kobayashi, H., Notohara, K., Nakashima, M. et al. Multiple hemangiomas (hepatic small vessel neoplasia) in the liver with Budd-Chiari syndrome. Virchows Arch 482, 1085–1089 (2023). https://doi.org/10.1007/s00428-023-03505-w

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  • DOI: https://doi.org/10.1007/s00428-023-03505-w

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