Dopamine-Secreting Paraganglioma in the Retroperitoneum

Yusuke Matsuda, Noriko Kimura, Takanobu Yoshimoto, Yoshihiro Sekiguchi, Junzo Tomoishi, Ichiro Kasahara, Yoshihito Hara, Yoshihiro Ogawa

研究成果: Contribution to journalArticle査読

3 被引用数 (Scopus)


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.

ジャーナルEndocrine Pathology
出版ステータス出版済み - 3 1 2017

All Science Journal Classification (ASJC) codes

  • 病理学および法医学
  • 内分泌学、糖尿病および代謝内科学
  • 内分泌学


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