Metastatic pheochromocytomas and paragangliomas: where are we?

Prinzi, Natalie; Corti, Francesca; Torchio, Martina; Niger, Monica; Antista, Maria; Pagani, Filippo; Beninato, Teresa; Pulice, Iolanda; Rossi, Roberta; Coppa, Jorgelina; Cascella, Tommaso; Giacomelli, Luca; Bartolomeo, Maria Di; Milione, Massimo; Braud, Filippo de; Pusceddu, Sara

doi:10.1177/03008916221078621

Cited by 5 publications

(4 citation statements)

References 149 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Last, given the low toxicity profile, PRRT may also be combined with other innovative systemic therapies, including tyrosine kinase inhibitors (sunitinib) and immunotherapy (pembrolizumab). 28 Limitations of this retrospective investigation includes the small number of patients per subgroup. Nevertheless, significant differences for SUV max and TBR were recorded.…”

Section: Discussionmentioning

confidence: 99%

“…Further strategies to enhance therapeutic efficacy may then also include shorter intervals between cycles or individualized, dosimetry-based approach concepts, 26 for example, by applying higher administered activities instead of fixed regimens using 7.4 GBq. Last, given the low toxicity profile, PRRT may also be combined with other innovative systemic therapies, including tyrosine kinase inhibitors (sunitinib) 27 and immunotherapy (pembrolizumab) 28 …”

Section: Discussionmentioning

confidence: 99%

“…Last, given the low toxicity profile, PRRT may also be combined with other innovative systemic therapies, including tyrosine kinase inhibitors (sunitinib) 27 and immunotherapy (pembrolizumab). 28 …”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Somatostatin Receptor–Directed PET/CT Can Differentiate Between Different Subtypes of Head and Neck Paragangliomas

Zhi,

Gerhard-Hartmann,

Hartrampf

et al. 2023

Clin Nucl Med

View full text Add to dashboard Cite

Background Given their neuroendocrine origin, head and neck paragangliomas (HNPGLs) can be imaged with somatostatin receptor (SSTR)–directed PET/CT. We aimed to determine whether the in vivo PET signal can differentiate between varying HNPGL subtypes. Patients and Methods Fourteen patients with HNPGL received pretherapeutic SSTR-PET/CTs using 68Ga-DOTATOC. Six (42.9%) patients had a jugular paraganglioma (PGL-J), 5 (35.7%) were diagnosed with carotid paraganglioma (PGL-Cs), and the remaining 3 patients (21.4%) had PGL-C with pathogenic SDHx germline variants (PGL-C-SDH). A visual and quantitative assessment of the primary tumor on SSTR-PET was performed, including SUVmax and target-to-background ratio (TBR). Quantitative values were then compared between subgroups of patients affected with different HNPGL entities. Results On visual assessment, all primary HNPGLs could be identified on SSTR-PET/CT. Quantification of HNPGL revealed substantially elevated SUVmax in PGL-J (101.7 ± 58.5) when compared with PGL-C-SDH (13.4 ± 5.6, P < 0.05), but not when compared with PGL-C (66.7 ± 27.3, P = 0.4; PGL-C vs PGL-C-SDH, P = 0.2). TBR of PGL-J (202.9 ± 82.2), however, further differentiated between PGL-C (95.7 ± 45.4, P < 0.05) and PGL-C-SDH (20.4 ± 12.2, P < 0.01; PGL-C vs PGL-C-SDH, P = 0.3). Moreover, whole-body readout revealed metastases in 2/3 (66.7%) of PGL-C-SDH patients, with a single SSTR-expressing skeletal lesion in one subject and bipulmonary lesions in the other patient. Conclusions In patients with HNPGL, SSTR-PET/CT identified the primary and metastatic disease and provides substantially elevated TBR, indicating excellent image contrast. PET-based quantification can also differentiate between varying HNPGL subtypes.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Somatostatin Receptor–Directed PET/CT Can Differentiate Between Different Subtypes of Head and Neck Paragangliomas

Zhi,

Gerhard-Hartmann,

Hartrampf

et al. 2023

Clin Nucl Med

View full text Add to dashboard Cite

show abstract

“…Grade 3/4 toxicities were represented by fatigue (16%), thrombocytopenia (16%), cardiac events (8%), and hypertension (4%) [ 32 ]. Clinical studies on cabozantinib, axitinib, lenvatinib, and everolimus are ongoing, and available results are too few to provide definitive indications on the role of TKIs (tyrosin-kinases inhibitors) in PCCs and PGLs [ 33 , 34 , 35 ].…”

Section: Discussionmentioning

confidence: 99%

Response to Peptide Receptor Radionuclide Therapy in Pheocromocytomas and Paragangliomas: A Systematic Review and Meta-Analysis

Marretta

Ottaiano

Iervolino

et al. 2023

JCM

View full text Add to dashboard Cite

Introduction. Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE and 90Y-DOTATOC showed efficacy in the metastatic setting of pheocromocytomas (PCCs) and paragangliomas (PGLs) where no standard therapies have been established. Background. A search of peer-reviewed and English articles reporting on 177Lu-DOTATATE and 90Y-DOTATOC efficacy was performed through Medline and Scopus. A subsequent meta-analysis was performed to evaluate the pooled effect size on disease control rate (DCR) with PRRT. Secondary endpoints were description of patients’ genetic characteristics, hematologic toxicity, and time-to-outcome. The pooled effect was estimated with both a mixed-effects model and a random-effects model. Results. Twelve studies met the criteria for this meta-analysis: ten with 177Lu- and two with 90Y-PRRTs (213 patients). The largest one included 46 patients. Median ages ranged from 32.5 to 60.4 years. When reported, mutations of SDHB were the most frequent genetic alterations. The pooled DCRs were 0.83 (95% CI: 0.75–0.88) and 0.76 (95% CI: 0.56–0.89) for 177Lu- and 90Y-PRRT, respectively. The pooled DCR for PRRT was 0.81 (95% CI: 0.74–0.87). Conclusions. We report an updated and solid estimate of DCR achieved with 177Lu- and 90Y-PRRT in PCCs and PGLs, showing that these therapies can be considered in the multidisciplinary treatment of PCCs and PGLs as alternatives to I-131 MIBG and chemotherapy.

show abstract