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Surgical resection of double advanced pancreatic neuroendocrine tumors with multiple renal cell carcinoma associated with von Hippel–Lindau disease

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Abstract

Von Hippel–Lindau (VHL) disease, an autosomal dominant genetic disorder caused by a germline mutation, is associated with non-functional and slow-growing pancreatic neuroendocrine tumor (PNET) and kidney cancer. We describe the case of a 46 year-old man with a 35 mm mass in the pancreatic head causing stricture of the bile duct and main pancreatic duct, a 55 mm mass in the pancreatic tail causing obstruction of the splenic vein (SV), and multiple masses of > 36 mm on both kidneys. We performed a two-stage resection. First, a total pancreatectomy with superior mesenteric vein (SMV) resection and reconstruction and retroperitoneoscopic right partial nephrectomy (NP) for five lesions was performed, followed by retroperitoneoscopic left partial NP of the five lesions 6 months later. Postoperative histopathological examination revealed NET G2 in the pancreatic head with SMV invasion and somatostatin receptor type 2A (SSTR2A) positivity, NET G2 in the pancreatic tail showed SV invasion and negative SSTR2A, and multiple clear cell renal cell carcinomas (RCC) were also noted. Multiple liver recurrences occurred 22 months after primary surgery. The patient remains alive 41 months after primary surgery. Kidney cancer generally determines VHL prognosis; however, we experienced dual-advanced PNETs with a more defined prognosis than multiple RCC associated with VHL.

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Authors and Affiliations

  1. Department of Surgery, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan

    Yoshiyuki Shibata, Takeshi Sudo, Sho Tazuma, Takashi Onoe & Hirotaka Tashiro

  2. Department of Gastroenterology, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan

    Atsushi Yamaguchi

  3. Department of Urology, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan

    Masanobu Shigeta

  4. Department of Anatomical Pathology, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan

    Kazuya Kuraoka & Rie Yamamoto

  5. Department of Surgery, Graduate School of Biochemical and Health Science, Hiroshima University, 1-2-3 Kasumi, Minami-Ku, Hiroshima, 734-8551, Japan

    Shinya Takahashi

Authors
  1. Yoshiyuki Shibata
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  2. Takeshi Sudo
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  3. Sho Tazuma
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  4. Takashi Onoe
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  7. Kazuya Kuraoka
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  8. Rie Yamamoto
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  9. Shinya Takahashi
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Contributions

Study conception and design: Yoshiyuki Shibata, Takeshi Sudo. Acquisition of data: Yoshiyuki Shibata, Takeshi Sudo, Sho Tazuma, Takashi Onoe, Atsushi Yamaguchi, Masanobu Shigeta, Kazuya Kuraoka, Rie Yamamoto, Hirotaka Tashiro. Analysis and interpretation of data: Yoshiyuki Shibata, Takeshi Sudo, Kazuya Kuraoka, Rie Yamamoto. Drafting of manuscript: Yoshiyuki Shibata, Takeshi Sudo. Critical revision of manuscript: Yoshiyuki Shibata, Takeshi Sudo, Shinya Takahashi.

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Correspondence to Yoshiyuki Shibata.

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All procedures followed in this study were performed in accordance with the ethical standards of the 1964 Declaration of Helsinki and its subsequent amendments.

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Shibata, Y., Sudo, T., Tazuma, S. et al. Surgical resection of double advanced pancreatic neuroendocrine tumors with multiple renal cell carcinoma associated with von Hippel–Lindau disease. Clin J Gastroenterol (2024). https://doi.org/10.1007/s12328-024-01967-2

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  • DOI: https://doi.org/10.1007/s12328-024-01967-2

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