ISSN:
1432-2307
Keywords:
Phaeochromocytoma
;
Tyrosine hydroxylase
;
Aromaticl-amino acid decarboxylase
;
Dopamineβ hydroxylase
;
Phenylethanolaminen-methyltransferase
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary Immunohistochemical localization of the catecholamine synthesizing enzymes, tyrosine hydroxylase (TH), aromatic L-amino acid decarboxylase (AADC), dopamine-β-hydroxylase (DBH) and phenylethanolamineN-methyltransferase (PNMT), was investigated in 70 cases of functioning and non-functioning phaeochromocytomas comprising 52 of adrenal and 18 of extra-adrenal origin. Of 59 functioning tumours, 30 were mixed epinephrine and norepinephrine-producing (mixed type) and 29 were norepinephrine-producing tumours. TH, AADC and DBH were detected in all functioning phaeochromocytomas, but PNMT was limited to the mixed-type phaeochromocytomas. Non-functioning phaeochromocytomas were divided into two groups, comprising a complete type, which induced neither elevated plasma catecholamines nor their metabolites in urine, and an incomplete type which exhibited no elevated plasma catecholamines, but showed a slightly high urinary vanillylmandelic acid level. In the non-functioning complete-type tumours, immunoreactive TH was negative, but the incomplete tumours of the adrenal medulla had all four enzymes, and corresponded to a mixed-type phaeochromocytoma. AADC and DBH were present universally in all functioning and non-functioning tumours, including TH-negative tumours. TH is a rate-limiting enzyme of catecholamine biosynthesis and deficiency of TH is an important feature of extraadrenal non-functioning phaeochromocytomas.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01607135
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