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Primary hepatic leiomyosarcoma in a patient with autosomal dominant polycystic kidney disease

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Abstract

Primary hepatic leiomyosarcoma is an extremely rare tumor. The diagnosis is difficult, and its etiologic factors have not been clarified. A 63-year-old woman with numerous cysts in her kidneys and liver was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). Several members of her family also had ADPKD. She underwent treatment with tolvaptan to inhibit cyst growth and slow the decline in kidney function. Eight months after the start of the therapy, she was hospitalized with fatigue and fever of unknown origin. Diagnostic imaging showed a very large hepatic tumor, and histologic examination of a fine-needle biopsy specimen revealed the tumor to be malignant. Differentiation between carcinoma and sarcoma was difficult based on the histological findings. The tumor was thought to be excisable; therefore, hepatic resection was attempted. At the time of surgery, as the tumor had grown larger than when imaged, complete resection was impossible. However, a part of the tumor was resected. Histopathological and immunohistological examinations of the surgical specimen confirmed a primary hepatic leiomyosarcoma. Whether the tumor was associated with the presence of ADPKD remains unclear, however, this is the first report of the combination of these two diseases in a patient.

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Authors and Affiliations

  1. Department of Nephrology and Dialysis, Moriguchi Keijinkai Hospital, 2-47-12 Yagumohigashi, Moriguchi, Osaka, 570-0021, Japan

    Takashi Iida, Yoshifumi Amari, Takatomi Yurugi & Fumitaka Nakajima

  2. Department of Pathology, Moriguchi Keijinkai Hospital, 2-47-12 Yagumohigashi, Moriguchi, Osaka, 570-0021, Japan

    Tamaki Maeda

  3. Department of Surgical Pathology, Hyogo College of Medicine, Hyougo Medical University, 1-1 Mukogawa, Nishinomiya, Hyougo, 663-8131, Japan

    Yoshitane Tsukamoto

Authors
  1. Takashi Iida
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  2. Tamaki Maeda
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  3. Yoshifumi Amari
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  4. Takatomi Yurugi
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  5. Yoshitane Tsukamoto
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  6. Fumitaka Nakajima
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Correspondence to Takashi Iida.

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All the authors have declared no competing interest.

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This article does not contain any studies with human participants performed by any of the authors.

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Informed consent was obtained from the patient in this report.

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Iida, T., Maeda, T., Amari, Y. et al. Primary hepatic leiomyosarcoma in a patient with autosomal dominant polycystic kidney disease. CEN Case Rep 6, 74–78 (2017). https://doi.org/10.1007/s13730-017-0247-4

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  • DOI: https://doi.org/10.1007/s13730-017-0247-4

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