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Dopamine-Secreting Paraganglioma in the Retroperitoneum

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Abstract

Pheochromocytomas and paragangliomas, which exclusively produce dopamine, are very rare. Herein, we report for the first time a Japanese case of an exclusively dopamine-producing paraganglioma accompanied by detailed immunohistochemical analyses. A 70-year-old Japanese woman was referred to our hospital for functional examination of her left retroperitoneal mass. Her adrenal functions were normal, except for excessive dopamine secretion. After the tumorectomy, her dopamine level normalized. The histopathological diagnosis of the tumor was paraganglioma; this was confirmed by positive immunostaining of chromogranin A (CgA), tyrosine hydroxylase (TH), dopamine β-hydroxylase (DBH), and succinate dehydrogenase gene subunit B (SDHB). However, the immunostaining of CgA in the tumor cells showed peculiar dot-like staining located corresponding to Golgi complex in the perinuclear area, rather than the diffuse cytoplasmic staining usually observed in epinephrine- or norepinephrine-producing functional pheochromocytomas and paragangliomas. The immunohistochemical results suggested that the tumor cells had sparse neuroendocrine granules in the cytoplasm, resulting in inhibition of catecholamine synthesis from dopamine to norepinephrine in neurosecretory granules. This may be the mechanism responsible for exclusive dopamine secretion in the present case.

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Acknowledgments

We thank all members of the Department of Endocrinology and Diabetology of Ome Municipal General Hospital for their administrative and clinical assistance and Dr. Mitsuhide Naruse at the National Hospital Organization Kyoto Medical Center for helpful discussion.

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Authors and Affiliations

  1. Department of Endocrinology and Diabetology, Ome Municipal General Hospital, Tokyo, 198-0042, Japan

    Yusuke Matsuda, Yoshihiro Sekiguchi & Yoshihito Hara

  2. Department of Clinical Research Pathology Division, National Hospital Organization Hakodate Hospital, Hakodate, Hokkaido, 041-8512, Japan

    Noriko Kimura

  3. Department of Diabetology, Endocrinology and Metabolism, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan

    Yusuke Matsuda, Takanobu Yoshimoto & Yoshihiro Ogawa

  4. Department of Urology, Ome Municipal General Hospital, Tokyo, 198-0042, Japan

    Junzo Tomoishi

  5. Department of Pathology, Ome Municipal General Hospital, Tokyo, 198-0042, Japan

    Ichiro Kasahara

Authors
  1. Yusuke Matsuda
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  2. Noriko Kimura
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  3. Takanobu Yoshimoto
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  4. Yoshihiro Sekiguchi
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  5. Junzo Tomoishi
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  6. Ichiro Kasahara
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  7. Yoshihito Hara
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  8. Yoshihiro Ogawa
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Corresponding author

Correspondence to Takanobu Yoshimoto.

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Conflict of Interest

None of the authors have any potential conflicts of interest associated with this research. This case was presented at the 72nd Tama Society of Clinical Endocrinology and Metabolism meeting, Japan.

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Matsuda, Y., Kimura, N., Yoshimoto, T. et al. Dopamine-Secreting Paraganglioma in the Retroperitoneum. Endocr Pathol 28, 36–40 (2017). https://doi.org/10.1007/s12022-016-9457-0

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  • DOI: https://doi.org/10.1007/s12022-016-9457-0

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