Akiyuki Kawashima1, Masakatsu Sone2,3, Nobuya Inagaki1, Kentaro Okamoto1, Mika Tsuiki4, Shoichiro Izawa5, Michio Otsuki6, Shintaro Okamura7, Takamasa Ichijo8, Takuyuki Katabami9, Yoshiyu Takeda10, Takanobu Yoshimoto11,12, Mitsuhide Naruse13, Akiyo Tanabe14. 1. Department of Diabetes, Endocrinology and Nutrition, Kyoto University, Kyoto, Japan. 2. Department of Diabetes, Endocrinology and Nutrition, Kyoto University, Kyoto, Japan. sonemasa@kuhp.kyoto-u.ac.jp. 3. Division of Metabolism and Endocrinology, Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa, Japan. sonemasa@kuhp.kyoto-u.ac.jp. 4. Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center, Kyoto, Japan. 5. Division of Endocrinology and Metabolism, Tottori University Faculty of Medicine, Yonago, Japan. 6. Department of Metabolic Medicine, Osaka University Graduate School of Medicine, Osaka, Japan. 7. Department of Endocrinology, Tenri Hospital, Tenri, Japan. 8. Department of Diabetes and Endocrinology, Saiseikai Yokohamashi Tobu Hospital, Yokohama, Japan. 9. Division of Metabolism and Endocrinology, Department of Internal Medicine, St. Marianna University School of Medicine Yokohama City Seibu Hospital, Yokohama, Japan. 10. Department of Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan. 11. Department of Diabetes and Endocrinology, Tokyo Metropolitan Hiroo Hospital, Tokyo, Japan. 12. Department of Molecular Endocrinology and Metabolism, Tokyo Medical and Dental University, Tokyo, Japan. 13. Clinical Research Institute of Endocrinology and Metabolism, NHO Kyoto Medical Center and Endocrine Center, Ijinkai Takeda General Hospital, Kyoto, Japan. 14. Division of Endocrinology, National Center for Global Health and Medicine, Tokyo, Japan.
Abstract
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 < 0.001), the presence of metastatic disease (OR 4.22, 95% CI 1.58-11.3, p = 0.004), and negativity on meta-iodobenzylguanidine (MIBG) scintigraphy (OR 0.15, 95% CI 0.03-0.77, p = 0.023). CONCLUSIONS: Our findings demonstrate that PPGL patients with negative results for urinary metanephrines are associated with extra-adrenal lesions, metastatic disease, and negative MIBG findings. This suggests that PPGL patients with negative results for urinary metanephrines have a greater need for systemic whole-body imaging other than MIBG scintigraphy and close follow-up to monitor for metastasis than do patients with PPGL overtly producing excessive catecholamine.
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 PPGLpatients 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 PPGLpatients with negative results for urinary metanephrines and those with catecholamine-producing PPGL. RESULTS: Five hundred PPGLpatients 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 < 0.001), the presence of metastatic disease (OR 4.22, 95% CI 1.58-11.3, p = 0.004), and negativity on meta-iodobenzylguanidine (MIBG) scintigraphy (OR 0.15, 95% CI 0.03-0.77, p = 0.023). CONCLUSIONS: Our findings demonstrate that PPGLpatients with negative results for urinary metanephrines are associated with extra-adrenal lesions, metastatic disease, and negative MIBG findings. This suggests that PPGLpatients with negative results for urinary metanephrines have a greater need for systemic whole-body imaging other than MIBG scintigraphy and close follow-up to monitor for metastasis than do patients with PPGL overtly producing excessive catecholamine.
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