Journal of Gastroenterology

, Volume 49, Issue 3, pp 511–516

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

  • Hisato Igarashi
  • Tetsuhide Ito
  • Isao Nishimori
  • Kenji Tamura
  • Ichiro Yamasaki
  • Masao Tanaka
  • Taro Shuin
Original Article—Liver, Pancreas, and Biliary Tract

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.

Keywords

Pancreas von Hippel-Lindau disease Neuroendocrine tumor Pancreatic cystic tumor 

Copyright information

© Springer Japan 2013

Authors and Affiliations

  • Hisato Igarashi
    • 1
  • Tetsuhide Ito
    • 1
  • Isao Nishimori
    • 2
  • Kenji Tamura
    • 4
  • Ichiro Yamasaki
    • 4
  • Masao Tanaka
    • 3
  • Taro Shuin
    • 4
  1. 1.Department of Medicine and Bioreguratory Science, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  2. 2.Nishimori ClinicKochiJapan
  3. 3.Department of Surgery and Oncology, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  4. 4.Department of UrologyKochi Medical SchoolNankokuJapan

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