title: Dopamine-Secreting Paraganglioma in the Retroperitoneum.
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.
authors.affiliation: Department of Endocrinology and Diabetology, Ome Municipal General Hospital, Tokyo, 198-0042, Japan.|Department of Diabetology, Endocrinology and Metabolism, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Matsuda
authors.affiliation: Department of Clinical Research Pathology Division, National Hospital Organization Hakodate Hospital, Hakodate, Hokkaido, 041-8512, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Kimura
authors.affiliation: Department of Diabetology, Endocrinology and Metabolism, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan. tyoshimoto.mem@tmd.ac.jp.
authors.forename: Takanobu
authors.initials: T
authors.lastname: Yoshimoto
authors.affiliation: Department of Endocrinology and Diabetology, Ome Municipal General Hospital, Tokyo, 198-0042, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Sekiguchi
authors.affiliation: Department of Urology, Ome Municipal General Hospital, Tokyo, 198-0042, Japan.
authors.forename: Junzo
authors.initials: J
authors.lastname: Tomoishi
authors.affiliation: Department of Pathology, Ome Municipal General Hospital, Tokyo, 198-0042, Japan.
authors.forename: Ichiro
authors.initials: I
authors.lastname: Kasahara
authors.affiliation: Department of Endocrinology and Diabetology, Ome Municipal General Hospital, Tokyo, 198-0042, Japan.
authors.forename: Yoshihito
authors.initials: Y
authors.lastname: Hara
authors.affiliation: Department of Diabetology, Endocrinology and Metabolism, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Ogawa
mesh_terms: D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D004298:Dopamine / Q000378:metabolism*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D010236:Paraganglioma, Extra-Adrenal / Q000378:metabolism* / Q000473:pathology; D012186:Retroperitoneal Neoplasms / Q000378:metabolism* / Q000473:pathology
chemical_list: D014408:Biomarkers, Tumor; D004298:Dopamine
keywords: Chromogranin a; Dopamine; Immunohistochemistry; Paraganglioma; Pheochromocytoma