Pheochromocytoma and paraganglioma with negative results for urinary metanephrines show higher risks for metastatic diseases

Purpose Few studies have assessed the clinical features of pheochromocytoma and paraganglioma (PPGL) not producing excessive catecholamine. We aimed to clarify the clinical characteristics of PPGL patients with negative results for urinary metanephrines. Methods This is a retrospective cross-section...

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Published in:Endocrine 2021-10, Vol.74 (1), p.155-162
Main Authors: Kawashima, Akiyuki, Sone, Masakatsu, Inagaki, Nobuya, Okamoto, Kentaro, Tsuiki, Mika, Izawa, Shoichiro, Otsuki, Michio, Okamura, Shintaro, Ichijo, Takamasa, Katabami, Takuyuki, Takeda, Yoshiyu, Yoshimoto, Takanobu, Naruse, Mitsuhide, Tanabe, Akiyo
Format: Article
Language:English
Subjects:
Catecholamines
Diabetes
Disease
Endocrinology
Humanities and Social Sciences
Internal Medicine
Medicine
Medicine & Public Health
Metastases
Metastasis
multidisciplinary
Original Article
Paraganglioma
Patients
Pheochromocytoma
Science
Scintigraphy
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Summary:Purpose Few studies have assessed the clinical features of pheochromocytoma and paraganglioma (PPGL) not producing excessive catecholamine. We aimed to clarify the clinical characteristics of PPGL patients with negative results for urinary metanephrines. Methods This is a retrospective cross-sectional study. We established a database by combining datasets from the Nationwide Cohort Study on the Development of Diagnosis and Treatment of Pheochromocytoma in Japan (PHEO-J) and the Advancing Care and Pathogenesis of Intractable Adrenal diseases in Japan (ACPA-J). We compared the clinical differences between PPGL patients with negative results for urinary metanephrines and those with catecholamine-producing PPGL. Results Five hundred PPGL patients in the combined database were analyzed. Among them, 31 were negative for metanephrines. PPGL with negative results for urinary metanephrines was significantly associated with extra-adrenal disease (Odds ratio (OR) 6.58, 95% CI (confidence interval) 3.03–14.3, p  
ISSN:1355-008X
1559-0100
DOI:10.1007/s12020-021-02816-9