A case of MEN1 presenting with multiple pancreatic neuroendocrine tumors with large cyst-like morphology difficult to differentiate from mucinous cystic neoplasm (MCN)

DOI Web Site 13 References
  • OHTA Yasuharu
    Department of Endocrinology, Metabolism, Hematological Science and Therapeutics, Yamaguchi University Graduate School of Medicine
  • AKIYAMA Masaru
    Department of Gastroenterological, Breast and Endocrine Surgery, Yamaguchi University Graduate School of Medicine
  • YODOKAWA Chika
    Department of Gastroenterological, Breast and Endocrine Surgery, Yamaguchi University Graduate School of Medicine
  • NAKABAYASHI Hiroko
    Department of Gastroenterological, Breast and Endocrine Surgery, Yamaguchi University Graduate School of Medicine
  • TAGUCHI Akihiko
    Department of Gastroenterological, Breast and Endocrine Surgery, Yamaguchi University Graduate School of Medicine
  • SHINDO Yoshitaro
    Department of Gastroenterological, Breast and Endocrine Surgery, Yamaguchi University Graduate School of Medicine
  • AMANO Shogo
    Department of Gastroenterology and Hepatology, Yamaguchi University Graduate School of Medicine
  • SUENAGA Shigeyuki
    Department of Molecular Pathology, Yamaguchi University Graduate School of Medicine
  • KAWATANI Yuki
    Department of Molecular Pathology, Yamaguchi University Graduate School of Medicine
  • OGA Atsunori
    Division of Surgical Pathology, Yamaguchi University Hospital
  • HOSHI Yoshinobu
    Division of Surgical Pathology, Yamaguchi University Hospital
  • ITO Hiroshi
    Department of Molecular Pathology, Yamaguchi University Graduate School of Medicine
  • TAKAMI Taro
    Department of Gastroenterology and Hepatology, Yamaguchi University Graduate School of Medicine
  • NAGANO Hiroaki
    Department of Gastroenterological, Breast and Endocrine Surgery, Yamaguchi University Graduate School of Medicine

Bibliographic Information

Other Title
  • MEN1に合併し，粘液性嚢胞腫瘍（MCN）との鑑別困難な大型の嚢胞様形態を伴った多発性膵神経内分泌腫瘍の1例

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<p>After undergoing prolactin-producing pituitary adenoma removal surgery at age 9, a 34-year-old female was diagnosed with adult growth hormone deficiency and began hormone replacement therapy. Four months later, abdominal CT showed an incidental cystic lesion 50mm in diameter in the pancreatic tail. In addition to hypergastrinemia and hyperglucagonemia, the patient had mildly elevated intact PTH. Somatostatin receptor scintigraphy revealed high levels of accumulation in the lesion. Although pancreatic neuroendocrine tumor (pNET) was suspected, a definitive diagnosis by EUS-FNA was difficult because the lesion was predominantly cystic. Based on ultrasound endoscopy, the preoperative diagnosis was mucinous cystic tumor followed by laparoscopic resection of the pancreatic body tail tumor. Because gastrin-producing tumors were observed in areas other than the thickened wall and limbs of the cyst, the patient was diagnosed with MEN1 presenting with pNET. Differential diagnosis of pancreatic tumors with cysts is difficult and requires multiple viewpoints. A large number of cases of pNET should be accumulated to build up evidence for postoperative adjuvant treatment.</p>

Journal

  • Suizo

    Suizo 38 (5), 328-336, 2023-10-31

    Japan Pancreas Society

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