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Pancreatic involvement in Japanese patients with von Hippel-Lindau disease: results of a nationwide survey

  • Original Article—Liver, Pancreas, and Biliary Tract
  • Published:
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Abstract

Background

The frequency and prognosis of pancreatic endocrine tumors (PNET)/pancreatic cystic tumors (PCT) in Japanese patients with von Hippel-Lindau disease (VHL) are still open to question.

Methods

We conducted the first nationwide epidemiological study of VHL disease in Japan to elucidate this question. Data on 377 VHL patients (PNET, 53; PCT, 152) were reported, and then their clinical characteristics were analyzed.

Results

PNET was found in 14.1 % and PCT in 40.3 %; 4.5 % had both. The onset of PNET and PCT mostly occurred at 30–39 years of age (median ages, 34 and 33 years, respectively). Metastasis was observed in 7.5 % of PNET patients at diagnosis, and 64.2 % underwent surgery including enucleation, partial and total pancreatectomy, and bypass surgery. Two patients received non-surgical therapies. No PNET-related deaths were observed. In PCT patients, no metastasis was observed at diagnosis, and 9.2 % underwent surgery or drainage. According to the classification system without or with adrenal pheochromocytoma, the VHL patients studied herein were subdivided into 313 (83 %) with VHL type 1 and 64 (17 %) with VHL type 2; 29 (9.3 %) and 24 (37.5 %) patients had PNET with VHL type 1 and 2, suggesting that patients with VHL type 2 were significantly more related to PNET than those with VHL type 1 (P < 0.01).

Conclusions

This study showed no significant difference in the epidemiology of pancreatic involvement between Japanese and non-Japanese VHL patients. Concerning the prognosis, follow-up study is needed.

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Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Medicine and Bioreguratory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Hisato Igarashi & Tetsuhide Ito

  2. Nishimori Clinic, Nakagami, 49-4, Sakawa, Kochi, 789-1233, Japan

    Isao Nishimori

  3. Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Masao Tanaka

  4. Department of Urology, Kochi Medical School, Nankoku, Kochi, 783-8505, Japan

    Kenji Tamura, Ichiro Yamasaki & Taro Shuin

Authors
  1. Hisato Igarashi
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  2. Tetsuhide Ito
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  3. Isao Nishimori
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  4. Kenji Tamura
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  5. Ichiro Yamasaki
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  6. Masao Tanaka
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  7. Taro Shuin
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Correspondence to Tetsuhide Ito.

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Igarashi, H., Ito, T., Nishimori, I. et al. Pancreatic involvement in Japanese patients with von Hippel-Lindau disease: results of a nationwide survey. J Gastroenterol 49, 511–516 (2014). https://doi.org/10.1007/s00535-013-0794-1

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  • DOI: https://doi.org/10.1007/s00535-013-0794-1

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