High-Specific-Activity-131I-MIBG versus 177Lu-DOTATATE Targeted Radionuclide Therapy for Metastatic Pheochromocytoma and Paraganglioma

Jha, Abhishek; Taïeb, David; Carrasquillo, Jorge A.; Pryma, Daniel A.; Patel, Mayank; Millo, Corina; Herder, Wouter W. de; Rivero, Jaydira Del; Crona, Joakim; Shulkin, Barry L.; Virgolini, Irene; Chen, Alice; Mittal, Bhagwant Rai; Basu, Sandip; Dillon, Joseph S.; Hope, Thomas A.; Aparici, Carina Marí; Iagaru, Andrei; Hicks, Rodney J.; Avram, Anca M.; Strosberg, Jonathan R.; Civelek, A. Cahid; Lin, Frank I.; Pandit‐Taskar, Neeta

doi:10.1158/1078-0432.ccr-20-3703

Cited by 51 publications

(31 citation statements)

References 22 publications

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“…131 I-MIBG could be used in patients with a good bone marrow reserve. In patients with elevated cardiac risk, 131 I-MIBG might be preferred over DOTATOC, as this is not considered to be associated with increased secretion of metanephrines [ 130 ].…”

Section: Resultsmentioning

confidence: 99%

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Calissendorff

Juhlin

Bancos

et al. 2022

Cancers

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Pheochromocytomas and abdominal paragangliomas (PPGLs) are rare tumors arising from the adrenal medulla or the sympathetic nervous system. This review presents a practical guidance for clinicians dealing with PPGLs. The incidence of PPGLs has risen. Most cases are detected via imaging and less present with symptoms of catecholamine excess. Most PPGLs secrete catecholamines, with diffuse symptoms. Diagnosis is made by imaging and tests of catecholamines. Localized disease can be cured by surgery. PPGLs are the most heritable of all human tumors, and germline variants are found in approximately 30–50% of cases. Such variants can give information regarding the risk of developing recurrence or metastases as well as the risk of developing other tumors and may identify relatives at risk for disease. All PPGLs harbor malignant potential, and current histological and immunohistochemical algorithms can aid in the identification of indolent vs. aggressive tumors. While most patients with metastatic PPGL have slowly progressive disease, a proportion of patients present with an aggressive course, highlighting the need for more effective therapies in these cases. We conclude that PPGLs are rare but increasing in incidence and management should be guided by a multidisciplinary team.

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Section: Resultsmentioning

confidence: 99%

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Calissendorff

Juhlin

Bancos

et al. 2022

Cancers

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“…Given the lack of controlled studies or comparative studies between 131 I-MIBG and 177 Lu-DOTATATE, it is not possible to determine the superiority of either agent nor can definitive conclusion be drawn from this current case report. The best approach for now is based on tumor characterisation from SSTR and MIBG imaging, opting for the treatment with the highest uptake, considering the toxicity profiles, the characteristics and comorbidities of the patient, and local availability (19). In the case of our patient, 177 Lu-DOTATATE was the only remaining radiopharmaceutical therapeutic agent in view of the lack of 131 I-MIBG uptake.…”

Section: Discussionmentioning

confidence: 99%

Highly Symptomatic Progressing Cardiac Paraganglioma With Intracardiac Extension Treated With 177Lu-DOTATATE: A Case Report

et al. 2021

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IntroductionPrimary cardiac paragangliomas are rare tumors. Metastatic disease is even rarer. Surgical management is technically challenging, and sometimes even impossible. Available therapeutic modalities for metastatic disease include external beam radiation therapy as well as systemic treatments, namely 131I-MIBG and more recently, peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE. To our knowledge, this is the first case of progressive unresectable cardiac paraganglioma with intracardiac extension treated with dosimetry based personalized PRRT to be reported. This case is of particular interest since it documents for the first time the efficacy, and especially the safety of the 177Lu-DOTATATE PRRT in this precarious context for which therapeutic options are limited.Case PresentationA 47-year-old man with no medical history consulted for rapidly decreasing exercise tolerance. The investigation demonstrated an unresectable progressing metastatic cardiac paraganglioma with intracardiac extension. The patient was treated with personalized 177Lu-DOTATATE PRRT and showed complete symptomatic and partial anatomical responses, with a progression-free survival of 13 months.ConclusionsPRRT with 177Lu-DOTATATE should be considered for inoperable cardiac paraganglioma. No major hemodynamic complications were experienced. Therapy resulted in safety and substantially improved quality of life.

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“…Patients with metastatic neuroendocrine tumors now have many systemic treatment options (1). These include not only FDA-approved chemotherapy options such as mTOR inhibitors such as everolimus and sunitinib (37)(38)(39), but also lanreotide (6,40), radiopharmaceuticals 131-MIBG, and 177-Lu-DOTATATE (3,41). Agents to possibly consider for future study could include checkpoint inhibitors (14) and ascorbic acid (42).…”

Section: Discussionmentioning

confidence: 99%

Phase II Study of ONC201 in Neuroendocrine Tumors including Pheochromocytoma-Paraganglioma and Desmoplastic Small Round Cell Tumor

Anderson

Trucco

Tarapore

et al. 2022

Clinical Cancer Research

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◥Purpose: Tumor dopamine-like DRD2 receptor expression is higher in pheochromocytoma-paraganglioma (PC-PG) compared with other cancers. ONC201 is a bitopic DRD2 antagonist with preclinical ONC201 activity in desmoplastic small round cell tumor (DSRCT).Patients and Methods: Patients (N ¼ 30) with neuroendocrine tumors were treated on this investigator-initiated trial (NCT03034200). ONC201 dose and schedule were 625 mg orally weekly in cohorts A (PC-PG) þ B (other neuroendocrine tumors) and 625 mg orally on 2 consecutive days each week in cohort C, which included 5 responding patients. The primary endpoint was radiographic response measured using RECIST. Secondary endpoints included progression-free survival, overall survival, and safety.Results: In arm A (n ¼ 10; all PC-PG), 50% (5/10) exhibited a partial response (PR) and 2 additional patients had stable disease (SD) >3 months. Median duration of therapy for arm A patients was 9 months (range: 1.5-33 months) with 5 patients treated >1 year. In arm B (n ¼ 12), there were 1 PR (DSRCT) and 2 SD (DSRCT; neuroblastoma) >3 months. Median duration of therapy in arm A was 18 months (range: 1-33 months) and arm B was 3 months (range: 1.5-33 months). Arm C PC-PG (N ¼ 8) showed 1 PR and 7 SD at 3 months, with median duration of therapy >10 months. There was no decline in Karnofsky performance status at week 12 for 28 of 30 patients and no dose modification due to treatment-related adverse events.Conclusions: Oral ONC201 was well tolerated in patients with metastatic neuroendocrine tumors and associated with clinical benefit, including tumor responses, particularly in some patients with DSRCT and the majority of patients with PC-PG.

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High-Specific-Activity-131I-MIBG versus 177Lu-DOTATATE Targeted Radionuclide Therapy for Metastatic Pheochromocytoma and Paraganglioma

Cited by 51 publications

References 22 publications

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Highly Symptomatic Progressing Cardiac Paraganglioma With Intracardiac Extension Treated With 177Lu-DOTATATE: A Case Report

Phase II Study of ONC201 in Neuroendocrine Tumors including Pheochromocytoma-Paraganglioma and Desmoplastic Small Round Cell Tumor

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