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

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18 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)511-516
Number of pages6
JournalJournal of gastroenterology
Volume49
Issue number3
DOIs
Publication statusPublished - Jan 1 2014

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von Hippel-Lindau Disease
Neoplasms
Surveys and Questionnaires
Neoplasm Metastasis
Pancreatectomy
Pheochromocytoma

All Science Journal Classification (ASJC) codes

  • Gastroenterology

Cite this

Igarashi, H., Ito, T., Nishimori, I., Tamura, K., Yamasaki, I., Tanaka, M., & Shuin, T. (2014). Pancreatic involvement in Japanese patients with von Hippel-Lindau disease: Results of a nationwide survey. Journal of gastroenterology, 49(3), 511-516. https://doi.org/10.1007/s00535-013-0794-1

Pancreatic involvement in Japanese patients with von Hippel-Lindau disease : Results of a nationwide survey. / Igarashi, Hisato; Ito, Tetsuhide; Nishimori, Isao; Tamura, Kenji; Yamasaki, Ichiro; Tanaka, Masao; Shuin, Taro.

In: Journal of gastroenterology, Vol. 49, No. 3, 01.01.2014, p. 511-516.

Research output: Contribution to journalArticle

Igarashi, H, Ito, T, Nishimori, I, Tamura, K, Yamasaki, I, Tanaka, M & Shuin, T 2014, 'Pancreatic involvement in Japanese patients with von Hippel-Lindau disease: Results of a nationwide survey', Journal of gastroenterology, vol. 49, no. 3, pp. 511-516. https://doi.org/10.1007/s00535-013-0794-1
Igarashi, Hisato ; Ito, Tetsuhide ; Nishimori, Isao ; Tamura, Kenji ; Yamasaki, Ichiro ; Tanaka, Masao ; Shuin, Taro. / Pancreatic involvement in Japanese patients with von Hippel-Lindau disease : Results of a nationwide survey. In: Journal of gastroenterology. 2014 ; Vol. 49, No. 3. pp. 511-516.
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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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AU - Tamura, Kenji

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AU - Tanaka, Masao

AU - Shuin, Taro

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N2 - 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.

AB - 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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