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Surgical resection of double advanced pancreatic neuroendocrine tumors with multiple renal cell carcinoma associated with von Hippel-Lindau disease.
Shibata, Yoshiyuki; Sudo, Takeshi; Tazuma, Sho; Onoe, Takashi; Yamaguchi, Atsushi; Shigeta, Masanobu; Kuraoka, Kazuya; Yamamoto, Rie; Takahashi, Shinya; Tashiro, Hirotaka.
Afiliación
  • Shibata Y; Department of Surgery, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan. dash.dash.try@gmail.com.
  • Sudo T; Department of Surgery, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan.
  • Tazuma S; Department of Surgery, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan.
  • Onoe T; Department of Surgery, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan.
  • Yamaguchi A; Department of Gastroenterology, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan.
  • Shigeta M; Department of Urology, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan.
  • Kuraoka K; Department of Anatomical Pathology, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan.
  • Yamamoto R; Department of Anatomical Pathology, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan.
  • Takahashi S; Department of Surgery, Graduate School of Biochemical and Health Science, Hiroshima University, 1-2-3 Kasumi, Minami-Ku, Hiroshima, 734-8551, Japan.
  • Tashiro H; Department of Surgery, Chugoku Cancer Center, National Hospital Organization Kure Medical Center, 3-1 Aoyama, Kure, Hiroshima, 737-0023, Japan.
Clin J Gastroenterol ; 17(4): 697-704, 2024 Aug.
Article en En | MEDLINE | ID: mdl-38693425
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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Pancreatectomía / Neoplasias Pancreáticas / Carcinoma de Células Renales / Tumores Neuroendocrinos / Enfermedad de von Hippel-Lindau / Neoplasias Renales Límite: Humans / Male / Middle aged Idioma: En Revista: Clin J Gastroenterol Año: 2024 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Japón
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Pancreatectomía / Neoplasias Pancreáticas / Carcinoma de Células Renales / Tumores Neuroendocrinos / Enfermedad de von Hippel-Lindau / Neoplasias Renales Límite: Humans / Male / Middle aged Idioma: En Revista: Clin J Gastroenterol Año: 2024 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Japón