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MALIGNANT PHAEOCHROMOCYTOMA: CLINICAL, BIOCHEMICAL AND SCINTIGRAPHIC CHARACTERIZATION
Author(s) -
SHAPIRO B.,
SISSON J. C.,
LLOYD R.,
NAKAJO M.,
SATTERLEE W.,
BEIERWALTES W. H.
Publication year - 1984
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/j.1365-2265.1984.tb00074.x
Subject(s) - medicine , scintigraphy , pathology , pheochromocytoma , histology , lung , vanillylmandelic acid , peritoneum , radiology , homovanillic acid , receptor , serotonin
SUMMARY We have evaluated thirty patients with malignant metastatic phaeochromocytoma with regard to clinical features, indices of catecholamine secretion, histology of lesions and a number of imaging procedures including scintigraphy with the recently developed sympathetic tissue‐seeking radiopharmaceutical 131 I‐metaiodobenzylguanidine ( 131 I‐MIBG). The primary tumour was extraadrenal in 13 cases. The commonest site of metastases was the axial skeleton (20 cases), followed by liver (four cases), lymph nodes (four cases), peritoneum (two cases) and lung (three cases). The malignancies were indolent, the mean time following the initial diagnosis was 9·18 years (range 0 to 33 years) and the mean duration of known metastases 3·71 years (range 0 to 18 years). There was a wide range of abnormalities in plasma and urinary catecholamines which did not correlate with the extent of tumour spread, histological pattern (mitotic index, Zellballen pattern, capsular or vascular invasion pleomorphism or necrosis) or 131 I‐MIBG uptake by tumour deposits. 131 I‐MIBG scintigraphy was found to be a useful technique for determining the extent of metastatic disease in most cases (26 of 30) and in some patients (16 of 30) was more sensitive than other radiological procedures. No false positive scans were encountered.