Duodenal adenocarcinoma with neuroendocrine features in a patient with acromegaly and thyroid papillary adenocarcinoma: a unique combination of endocrine neoplasia

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  • Kato Ken-ichiro
    Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan
  • Takeshita Yumie
    Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan
  • Misu Hirofumi
    Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan
  • Ishikura Kazuhide
    Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan
  • Kakinoki Kaheita
    Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan
  • Kitamura Seiko Sawada-
    Department of Pathology, Kanazawa University Hospital, Kanazawa 920-8641, Japan
  • Kaneko Shuichi
    Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan
  • Takamura Toshinari
    Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan

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Abstract

A 67-year-old woman with familial clustering of thyroid papillary adenocarcinoma was diagnosed with acromegaly due to pituitary macroadenoma. She had multiple skin vegetations, but had no parathyroid and pancreas diseases. Before transsphenoidal surgery, she was further diagnosed as having a duodenal tumor and multiple hypervascular liver nodules. Biopsy specimens from the duodenal tumor and liver nodules were diagnosed histologically as moderately differentiated adenocarcinoma. Immunohistochemically, the tumor cells were positive for chromogranin, synaptophysin and somatostatin receptor 2a, suggestive for neuroendocrine features. After surgery, the patient was not in biochemical remission, and octreotide treatment was initiated. The duodenal cancer was treated with chemotherapy (neoadjuvant cisplatin and S-1). After 24 months, the patient’s insulin-like growth factor I level had been normalized, and her liver tumors had not progressed macroscopically. This is a rare case of acromegaly associated with multiple endocrine tumors, not being categorized as conventional multiple endocrine neoplasia. Octreotide treatment might have had beneficial effects on our patient’s duodenal adenocarcinoma and liver metastases, both directly via SSTR2a and indirectly via GH suppression, thereby contributing to their slow progression.

Journal

  • Endocrine Journal

    Endocrine Journal 59 (9), 791-796, 2012

    The Japan Endocrine Society

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