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Purpose: This study examined the usefulness of the maximum standardized uptake value (SUVmax) of myocardial [ 123 I]-metaiodobenzylguanidine ([ 123 I]-MIBG) to characterize myocardial function by comparing it with echocardiographic parameters in patients with pheochromocytoma., Materials and Methods: This study included 18 patients with pheochromocytoma who underwent both planar and [ 123 I]-MIBG single-photon emission computed tomography/computed tomography scans and echocardiography before surgery. Myocardial [ 123 I]-MIBG visibility and SUVmax were compared with echocardiographic parameters related to systolic and diastolic functions. The Mann-Whitney U test, Fisher exact test, or Spearman rank correlation assessed differences or relationships between two quantitative variables., Results: On visual analysis, 6 patients showed normal myocardial [ 123 I]-MIBG uptake, whereas 12 patients showed decreased myocardial [ 123 I]-MIBG uptake. No patients showed systolic dysfunction. A significant difference was observed in the incidence of diastolic dysfunction between the groups with normal and decreased uptake (p = 0.009), and left ventricular (LV) diastolic dysfunction was observed in 9 (75%) of 12 patients with decreased myocardial uptake. The myocardial SUVmax was significantly lower in 9 patients with LV diastolic dysfunction than in 9 patients with normal cardiac function (1.67 ± 0.37 vs. 3.03 ± 1.38, p = 0.047). Myocardial SUVmax was positively correlated with septal e' (early diastolic velocity of septal mitral annulus) (ρ = 0.51, p = 0.031) and negatively correlated with the septal E/e' ratio (early mitral E-velocity to early diastolic velocity of septal mitral annulus; ρ =  - 0.64, p = 0.004), respectively., Conclusions: LV diastolic dysfunction was inversely related to myocardial [ 123 I]-MIBG uptake. Myocardial [ 123 I]-MIBG SUVmax may be useful for characterizing cardiac function in patients with pheochromocytoma. Second abstract. The semiquantitative analysis using the myocardial SUVmax in 123 I-MIBG SPECT/CT was found to be potentially useful for characterizing cardiac function in patients with pheochromocytoma., (© 2022. The Author(s).)

Objective: In this phase II study, we aimed to investigate the efficacy and safety of single-dose [ 131 I]meta-iodobenzylguanidine ( 131 I-mIBG) therapy in patients with refractory pheochromocytoma and paraganglioma (PPGL)., Patients and Methods: This study was designed as an open-label, single-arm, multi-center, phase II clinical trial. The enrolled patients were administered 7.4 GBq of 131 I-mIBG. Its efficacy was evaluated 12 and 24 weeks later, and its safety was monitored continuously until the end of the study. We evaluated the biochemical response rate as the primary endpoint using the one-sided exact binomial test based on the null hypothesis (≤ 5%)., Results: Seventeen patients were enrolled in this study, of which 16 were treated. The biochemical response rate (≥ 50% decrease in urinary catecholamines) was 23.5% (90% confidence interval: 8.5-46.1%, p = 0.009). The radiographic response rates, determined with CT/MRI according to the response evaluation criteria in solid tumors (RECIST) version 1.1 and 123 I-mIBG scintigraphy were 5.9% (0.3%-25.0%) and 29.4% (12.4%-52.2%), respectively. The most frequent non-hematologic treatment-emergent adverse events (TEAEs) were gastrointestinal symptoms including nausea, appetite loss, and constipation, which were, together, observed in 15 of 16 patients. Hematologic TEAEs up to grade 3 were observed in 14 of 16 patients. No grade 4 or higher TEAEs were observed. All patients had experienced at least one TEAE, but no fatal or irreversible TEAEs were observed., Conclusion: A single dose 131 I-mIBG therapy was well tolerated by patients with PPGL, and statistically significantly reduced catecholamine levels compared to the threshold response rate, which may lead to an improved prognosis for these patients., (© 2021. The Author(s).)

Refractory pheochromocytoma and paraganglioma (PPGL) have a poor prognosis and the treatment strategy remains to be established. This multi-institutional phase I study was performed to determine the safety, dose-limiting toxicity (DLT), and efficacy of [ 131 I]-meta-iodobenzylguanidine ( 131 I-mIBG) therapy for refractory PPGLs. Twenty patients with refractory PPGL were enrolled in this study. We administered fixed doses of 131 I-mIBG to all patients, delivering a second and third course of 131 I-mIBG to eight and three patients, respectively. During the 20 weeks after 131 I-mIBG injection, the authors surveyed the adverse events in accordance with the Common Terminology Criteria for Adverse Events. All patients experienced adverse events and adverse reactions, but none experienced a grade 4 adverse event. Twelve weeks after 131 I-mIBG injection, examinations for the evaluation of therapeutic effects was performed in accordance with the Response Evaluation Criteria in Solid Tumours (RECIST). The best overall response rates (based on RECIST categories) were 10% (complete response), 65% (stable disease), 15% (progressive disease), and 10% (not all evaluated). The efficacy and safety of 131 I-mIBG therapy was shown in patients with refractory PPGL, and DLT was observed in neither single nor repeated 131 I-mIBG therapy, indicating a tolerability for 131 I-mIBG therapy.

Objective Pheochromocytoma and paraganglioma (PPGLs) are rare neuroendocrine tumors derived from the adrenal medulla or extra-adrenal paraganglioma from extra-adrenal chromaffin tissue. Although malignant PPGLs has miserable prognosis, the treatment strategy remains to be established. An internal radiation therapy using [ 131 I]meta-iodobenzylguanidine ( 131 I-mIBG) called MIBG therapy has been attempted as one of the systemic treatment of malignant PPGLs. The aim of this study is therefore to evaluate the safety and the efficacy of MIBG therapy for refractory PPGLs. Methods Patients with refractory PPGLs will be enrolled in this study. The total number of patients for registration is 20. The patients receive a fixed dose of 7,400 MBq of 131 I-mIBG. Adverse events are surveyed during 20 weeks after 131 I-mIBG injection and all severe adverse events will be documented and reported in detail in accordance with the Common Terminology Criteria for Adverse Events (CTCAE). Examination and imaging diagnosis are performed in 12 weeks after 131 I-mIBG injection for the evaluation of therapeutic effect in accordance with the Response Evaluation in Solid Tumours (RECIST). Conclusion The current study is the first multi-institutional prospective study of MIBG therapy and thereby will play a significant role in improving the patients' prognosis of refractory PPGLs. J. Med. Invest. 64: 205-209, August, 2017.

Purpose: The purpose of this study is to compare diagnostic performances of (18)F-fluorodeoxyglucose (FDG) visual score, maximum standardized uptake value (SUVmax), ratio of adrenal SUVmax to liver SUVmax (A/L SUVmax), apparent diffusion coefficient (ADC) from diffusion-weighted imaging, and SUVmax/ADC ratio to differentiate adrenal pheochromocytoma from other benign tumors., Methods: Eleven pheochromocytomas and 22 other benign tumors in 30 patients were included. FDG-based indices, ADC, and SUVmax/ADC ratio were compared between groups using the Mann-Whitney U test, and sensitivity, specificity, accuracy, and area under the curve (AUC) for diagnosing pheochromocytoma by receiver operating characteristic analyses. The correlation between SUVmax and ADC was analyzed using the Spearman's rank test., Results: Pheochromocytoma showed significantly higher visual score (2.8 ± 0.4 vs. 1.3 ± 0.9), SUVmax (11.0 ± 8.9 vs. 3.2 ± 1.4), A/L SUVmax ratio (3.96 ± 3.48 vs. 0.96 ± 0.51), and SUVmax/ADC ratio (10.6 ± 8.09 vs. 2.28 ± 0.98) (each P < 0.001) and significantly lower ADC (1.08 ± 0.23 × 10(-3) mm(2)/s vs. 1.43 ± 0.29 × 10(-3) mm(2)/s, P = 0.003) than other benign tumors. Sensitivity, specificity, and accuracy for diagnosing pheochromocytoma were 100, 73, and 82% for visual score, 100, 86, and 91% for both SUVmax and A/L SUVmax ratio, and 64, 100, and 88% for ADC and 82, 95, and 91% for SUVmax/ADC ratio. No significant differences in AUC were found between FDG-based indices, ADC, and SUVmax/ADC ratio. A significant negative correlation was noted between SUVmax and ADC (ρ = -0.36, P = 0.039)., Conclusion: FDG-based indices and ADC appear comparably useful for differentiating pheochromocytoma from other benign adrenal tumors.

Effective treatments for malignant neuroendocrine tumors are under development. While iodine-131 metaiodobenzylguanidine (¹³¹I-MIBG) radiotherapy has been used in the treatment of malignant neuroendocrine tumors, there are few studies evaluating its therapeutic effects and safety in a multicenter cohort. In the current study, we sought to evaluate the effects and safety of ¹³¹I-MIBG therapy for conditions including malignant pheochromocytoma and paraganglioma within a multicenter cohort. Forty-eight malignant neuroendocrine tumors (37 pheochromocytoma and 11 paraganglioma) from four centers underwent clinical ¹³¹I-MIBG radiotherapy. The tumor responses were observed before and 3 to 6 months after the ¹³¹I-MIBG radiotherapy in accordance with RECIST criteria. We also evaluated the data for any adverse effects. The four centers performed a total of 87 ¹³¹I-MIBG treatments on 48 patients between January 2000 and March 2009. Of the treatments, 65 were evaluable using RECIST criteria. One partial response (PR), 40 stable disease (SD), and 9 progressive disease (PD) in malignant pheochromocytoma were observed after each treatment. Fourteen SD and one PD-were observed in paraganglioma. Patients with normal hypertension (systolic blood pressure (BP) > 130 mmHg) showed significantly reduced systolic BP after the initial follow-up (n=10, 138.1±8.2 to 129.5±13.5 mmHg, P=0.03). In adult neuroendocrine tumors with a treatment-basis analysis, there were side effects following 41 treatments (47.1%) and most of them (90.2%) were minor. In this multicenter registry, PR or SD was achieved in 84.6% of the treatment occasions in adult neuroendocrine tumors through ¹³¹I-MIBG radiotherapy. This indicated that most of the ¹³¹I-MIBG radiotherapy was performed safely without significant side effects.