BACKGROUND: Measurement of plasma/urinary catecholamine metabolites--especially normetanephrine (NMN)--represents a gold standard in biochemical detection of succinate dehydrogenase subunit B (SDHB) and D (SDHD)-related pheochromocytomas (PHEO) and paragangliomas (PGL). This study was designed to assess diagnostic utility of chromogranin A (CgA) alone or in combination with NMN in patients with PHEO/PGL related to mutations in SDHB and SDHD. MATERIALS AND METHODS: A retrospective study of SDHB and SDHD NIH patients' cohort, which included 41 patients with SDHB mutation-related PHEO/sPGL and 18 patients with either SDHD or SDHB mutation-related head and neck PGL (HNPGL) with both CgA and NMN measured at the time of diagnosis at NIH. RESULTS: In the SDHB group, CgA showed sensitivity of 73.2% and specificity of 95.9%, while for NMN they were 70.7% and 98.6%, respectively. Elevations in CgA and NMN were complementary in 92.7% of patients with proven tumors. Both tests performed well on receiver operating characteristic curve analysis. CgA levels were elevated in 76.9% of SDHB patients and in 80% of patients with metastatic disease and normal NMN levels. CgA values in patients with HNPGL were significantly lower than in patients with PHEO/sPGL. CONCLUSION: CgA is a valuable complementary biomarker in work-up of SDHB-related PHEO/sPGL. In combination with plasma NMN, CgA further enhances tumor detection by 22.0% with minimal loss in specificity. Although non-specific for PHEO/PGL, CgA may well supplement plasma NMN to facilitate diagnostic evaluation of SDHB-related PHEO/sPGL, especially where the measurement of plasma metanephrines could otherwise be delayed by decreased availability or cost restriction.
BACKGROUND: Measurement of plasma/urinary catecholamine metabolites--especially normetanephrine (NMN)--represents a gold standard in biochemical detection of succinate dehydrogenase subunit B (SDHB) and D (SDHD)-related pheochromocytomas (PHEO) and paragangliomas (PGL). This study was designed to assess diagnostic utility of chromogranin A (CgA) alone or in combination with NMN in patients with PHEO/PGL related to mutations in SDHB and SDHD. MATERIALS AND METHODS: A retrospective study of SDHB and SDHD NIH patients' cohort, which included 41 patients with SDHB mutation-related PHEO/sPGL and 18 patients with either SDHD or SDHB mutation-related head and neck PGL (HNPGL) with both CgA and NMN measured at the time of diagnosis at NIH. RESULTS: In the SDHB group, CgA showed sensitivity of 73.2% and specificity of 95.9%, while for NMN they were 70.7% and 98.6%, respectively. Elevations in CgA and NMN were complementary in 92.7% of patients with proven tumors. Both tests performed well on receiver operating characteristic curve analysis. CgA levels were elevated in 76.9% of SDHBpatients and in 80% of patients with metastatic disease and normal NMN levels. CgA values in patients with HNPGL were significantly lower than in patients with PHEO/sPGL. CONCLUSION:CgA is a valuable complementary biomarker in work-up of SDHB-related PHEO/sPGL. In combination with plasma NMN, CgA further enhances tumor detection by 22.0% with minimal loss in specificity. Although non-specific for PHEO/PGL, CgA may well supplement plasma NMN to facilitate diagnostic evaluation of SDHB-related PHEO/sPGL, especially where the measurement of plasma metanephrines could otherwise be delayed by decreased availability or cost restriction.
Authors: Joseph M Pappachan; Nyo Nyo Tun; Ganesan Arunagirinathan; Ravinder Sodi; Fahmy W F Hanna Journal: Curr Hypertens Rep Date: 2018-01-22 Impact factor: 5.369
Authors: Laurence Amar; Karel Pacak; Olivier Steichen; Scott A Akker; Simon J B Aylwin; Eric Baudin; Alexandre Buffet; Nelly Burnichon; Roderick J Clifton-Bligh; Patricia L M Dahia; Martin Fassnacht; Ashley B Grossman; Philippe Herman; Rodney J Hicks; Andrzej Januszewicz; Camilo Jimenez; Henricus P M Kunst; Dylan Lewis; Massimo Mannelli; Mitsuhide Naruse; Mercedes Robledo; David Taïeb; David R Taylor; Henri J L M Timmers; Giorgio Treglia; Nicola Tufton; William F Young; Jacques W M Lenders; Anne-Paule Gimenez-Roqueplo; Charlotte Lussey-Lepoutre Journal: Nat Rev Endocrinol Date: 2021-05-21 Impact factor: 43.330
Authors: Abhishek Jha; Kristine de Luna; Charlene Ann Balili; Corina Millo; Cecilia Angela Paraiso; Alexander Ling; Melissa K Gonzales; Bruna Viana; Rami Alrezk; Karen T Adams; Isabel Tena; Alice Chen; Jiri Neuzil; Margarita Raygada; Electron Kebebew; David Taieb; M Sue O'Dorisio; Thomas O'Dorisio; Ali Cahid Civelek; Constantine A Stratakis; Leilani Mercado-Asis; Karel Pacak Journal: Front Oncol Date: 2019-02-22 Impact factor: 6.244
Authors: Annika M A Berends; Graeme Eisenhofer; Lauren Fishbein; Anouk N A V D Horst-Schrivers; Ido P Kema; Thera P Links; Jacques W M Lenders; Michiel N Kerstens Journal: Cancers (Basel) Date: 2019-08-06 Impact factor: 6.639