Catecholamine-Synthesizing Enzymes in Pheochromocytoma and Extraadrenal Paraganglioma

Sachiko Konosu-Fukaya, Kei Omata, Yuta Tezuka, Yoshikiyo Ono, Yayoi Aoyama, Fumitoshi Satoh, Fumiyoshi Fujishima, Hironobu Sasano, Yasuhiro Nakamura

研究成果: Article査読

12 被引用数 (Scopus)


In chromaffin cells, tyrosine hydroxylase (TH), aromatic l-amino acid decarboxylase (AADC), dopamine β-hydroxylase (DBH), and phenylethanolamine N-methyltransferase (PNMT) are mainly involved in catecholamine synthesis. In this study, we evaluated the association between the status of catecholamine-synthesizing enzymes and histopathological features of pheochromocytoma and extraadrenal paraganglioma with special emphasis upon their postoperative clinical behavior. Immunohistochemical evaluation of TH, DBH, AADC, PNMT, Ki 67, and S-100 was performed in 29 pheochromocytoma and 10 extraadrenal paraganglioma and one lymph node harboring metastatic pheochromocytoma. Among these cases, metastasis was subsequently developed in three cases. Urinary normetanephrine (U-NM) levels were significantly higher in clinical metastatic cases than non-metastatic ones. Ki 67 labeling index was significantly higher in both clinical metastatic cases and the Adrenal Gland Scaled Score (PASS) score of ≧ 4 cases than PASS < 4 cases, although this score was originally used in pheochromocytoma. H-score of AADC and DBH were significantly lower in PASS ≧ 4 cases than those with < 4 cases, and in the cases associated with intratumoral necrosis (n = 4), the presence of spindle shaped tumor cells (n = 4), and large nests of cells or diffuse growth (n = 5). Lower status of intratumoral AADC could be related to poor differentiation of tumor cells in both catecholamine production and morphology and could be related to aggressive biological behavior of both pheochromocytoma and extraadrenal paraganglioma.

ジャーナルEndocrine Pathology
出版ステータスPublished - 2018 12月 1

ASJC Scopus subject areas

  • 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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