Malignant insulinoma

Spectrum of unusual clinical features

Authors

  • Boaz Hirshberg M.D.,

    Corresponding author
    1. Division of Intramural Research, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland
    • PFIZER, Inc., Eastern Point Road MS 8260-2502, Groton, CT 06340
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  • Craig Cochran R.N.,

    1. Division of Intramural Research, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland
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  • Monica C. Skarulis M.D.,

    1. Division of Intramural Research, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland
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  • Steven K. Libutti M.D.,

    1. Surgical Metabolism Branch, National Cancer Insitute, National Institutes of Health, Bethesda, Maryland
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  • H. Richard Alexander M.D.,

    1. Surgical Metabolism Branch, National Cancer Insitute, National Institutes of Health, Bethesda, Maryland
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  • Bradford J. Wood M.D.,

    1. Department of Radiology, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland
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  • Richard Chang M.D.,

    1. Department of Radiology, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland
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  • David E. Kleiner M.D. Ph.D.,

    1. Department of Anatomical Pathology, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland
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  • Phillip Gorden M.D.

    1. Division of Intramural Research, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland
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  • This article is a US Government work and, as such, is in the public domain in the United States of America.

Abstract

BACKGROUND

Malignant insulinoma occurs in a few patients with insulinoma. Due to the small sample of patients, there are little data regarding their clinical manifestation as well as the preferred treatment modalities. The aims of the current study were to summarize the National Institutes of Health experience during the last two decades and to conduct a critical review of the current literature.

METHODS

The authors identified 10 patients with metastatic insulinoma.

RESULTS

The patients presented with four patterns of clinical behavior. First, four patients presented with lymph node metastasis and, after surgical excision, maintained a prolonged tumor-free survival. Second, four patients presented with metastatic disease to the liver, which appeared years after the initial diagnosis and presumed curative surgery. Third, one patient presented with a large α-fetoprotein-secreting liver mass. Finally, 9 of the 10 patients had a prolonged survival. Various treatment modalities were used to control hypoglycemia. Short-term benefits were most often achieved with embolization and diazoxide. Less successful modalities included radiofrequency ablation, radical debulking surgery, verapamil therapy, octreotide therapy, and chemotherapy.

CONCLUSIONS

The current study, as well as others, suggested that metastatic insulinoma may have a variable natural history. After the initial surgical resection, the biology of the tumor, rather than any treatment modality, was most likely the major determinant of long-term survival. Cancer 2005. © Published 2005 American Cancer Society.

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