Metastatic Phaeochromocytoma: Spinning Towards More Promising Treatment Options

Nölting, Svenja; Grossman, Ashley; Pacák, Karel

doi:10.1055/a-0715-1888

Cited by 44 publications

(35 citation statements)

References 108 publications

(155 reference statements)

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“…The prevalence of metastasis ranges from 2–13% in PCC to 2.4–50% in PGL ( 1 , 2 ). In patients with unresectable, locally advanced or metastatic PPGL, symptomatic or progressive disease is usually treated with chemotherapy, radionuclide therapy ( 131 I-metaiodobenzylguanidine ( 131 I-MIBG) and peptide receptor radionuclide therapy (PRRT)), external radiotherapy, radiofrequency ablation therapy or tyrosine kinase inhibitors ( 3 , 4 ). There is no head to head trials that compare the superiority of one modality of therapy over the other.…”

Section: Introductionmentioning

confidence: 99%

177Lu-DOTATATE therapy in metastatic/inoperable pheochromocytoma-paraganglioma

Jaiswal¹,

Sarathi

Memon³

et al. 2020

Endocrine Connections

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Introduction: 177Lu-DOTATATE-based peptide receptor radionuclide therapy (PRRT) is a promising therapy for metastatic and/ inoperable pheochromocytoma and paraganglioma (PPGL). We aim to evaluate the efficacy and safety of and identify predictors of response to 177Lu-DOTATATE therapy in metastatic and/ inoperable PPGL. Methods: This retrospective study involved 15 patients of metastatic or unresectable PPGL, who received 177Lu-DOTATATE PRRT therapy. Clinical, biochemical (Plasma free normetanephrine), and radiological (anatomical and functional) responses were compared before and after the last therapy. Results: A total of 15 patients PCC (4), sPGL (4), HNPGL (5), PCC+sPGL (1), HNPGL+sPGL (1) were included. The median duration of follow up was 27 (range: 11-62) months from the start of PRRT. Based on the RECIST (1.1) criteria, progressive disease was seen in three (20%), stable disease in eight (53 %), partial response in one (7%), and minor response in three (20%) and controlled disease in 12 (80%). On linear regression analysis presence of PGL (p= 0.044) and baseline SUVmax >21 (p < 0.0001) were significant positive predictors of early response to PRRT. Encouraging safety profiles were noted with no long term nephrotoxicity and haematotoxicity. Conclusion: 177Lu-DOTATATE therapy is an effective and safe modality of treatment for patients with metastatic/inoperable PPGL. Although it is not prudent to withhold PRRT in metastatic PPGL with baseline SUVmax < 21, baseline SUVmax >21 can be used to predict early response to PRRT.

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Section: Introductionmentioning

confidence: 99%

177Lu-DOTATATE therapy in metastatic/inoperable pheochromocytoma-paraganglioma

Jaiswal¹,

Sarathi

Memon³

et al. 2020

Endocrine Connections

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“…In the present study, we demonstrated the anti-tumor activity of Aerothionin and Homoaerothionin on pheochromocytoma cells. For non-metastatic pheochromocytomas and paragangliomas, surgery is the treatment of choice, but if metastases already occur treatment is challenging [39]. Combination therapy with BYL719, a phosphatidylinositol-3-kinase α inhibitor, and everolimus, a mammalian target of rapamycin inhibitor, showed synergistic effects on PPGLs in vitro [40].…”

Section: Discussionmentioning

confidence: 99%

Anti-Tumor Activity vs. Normal Cell Toxicity: Therapeutic Potential of the Bromotyrosines Aerothionin and Homoaerothionin In Vitro

et al. 2020

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Novel strategies to treat cancer effectively without adverse effects on the surrounding normal tissue are urgently needed. Marine sponges provide a natural and renewable source of promising anti-tumor agents. Here, we investigated the anti-tumor activity of Aerothionin and Homoaerothionin, two bromotyrosines isolated from the marine demosponge Aplysina cavernicola, on two mouse pheochromocytoma cells, MPC and MTT. To determine the therapeutic window of these metabolites, we furthermore explored their cytotoxicity on cells of the normal tissue. Both metabolites diminished the viability of the pheochromocytoma cell lines significantly from a concentration of 25 µM under normoxic and hypoxic conditions. Treatment of MPC cells leads moreover to a reduction in the number of proliferating cells. To confirm the anti-tumor activity of these bromotyrosines, 3D-pheochromocytoma cell spheroids were treated with 10 µM of either Aerothionin or Homoaerothionin, resulting in a significant reduction or even complete inhibition of the spheroid growth. Both metabolites reduced viability of normal endothelial cells to a comparable extent at higher micromolar concentration, while the viability of fibroblasts was increased. Our in vitro results show promise for the application of Aerothionin and Homoaerothionin as anti-tumor agents against pheochromocytomas and suggest acceptable toxicity on normal tissue cells.

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“…In metastatic PGLs, 131I-MIBG is the most studied treatment, while radiolabeled somatostatin analogs have been used in small retrospective and prospective studies [9,45]. Chemotherapy (cyclophosphamide, vincristine, and dacarbazine) is indicated in case of rapidly progressive metastatic disease [46]. More recently, the antiangiogenic drug sunitinib, an inhibitor of multiple tyrosine kinase receptors, has been used in metastatic PGLs and 8/17 patients had partial response or stable disease [47].…”

Section: Therapymentioning

confidence: 99%

Natural History and Management of Familial Paraganglioma Syndrome Type 1: Long-Term Data from a Large Family

Puliani

Sesti

Feola

et al. 2020

JCM

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Head and neck paragangliomas are the most common clinical features of familial paraganglioma syndrome type 1 caused by succinate dehydrogenase complex subunit D (SDHD) mutation. The clinical management of this syndrome is still unclear. In this study we propose a diagnostic algorithm for SDHD mutation carriers based on our family case series and literature review. After genetic diagnosis, first evaluation should include biochemical examination and whole-body imaging. In case of lesion detection, nuclear medicine examination is required for staging and tumor characterization. The study summarizes the diagnostic accuracy of different functional imaging techniques in SDHD mutation carriers. 18F-3,4-dihydroxyphenylalanine (18F-DOPA) positron emission tomography (PET)-computed tomography (CT) is considered the gold standard. If it is not available, 123I-Metaiodobenzylguanidine (MIBG) could be used also for predicting response to radiometabolic therapy. 18F-fluoro-2-deoxy-D-glucose (18F-FDG) PET-CT has a prognostic role since high uptake identifies more aggressive cases. Finally, 68Ga-peptides PET-CT is a promising diagnostic technique, demonstrating the best diagnostic accuracy in our and in other published case series, even if this finding still needs to be confirmed in larger studies. Periodic follow-up should consist of annual biochemical and ultrasonographic screening and biannual magnetic resonance examination to identify biochemical silent tumors early.

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Metastatic Phaeochromocytoma: Spinning Towards More Promising Treatment Options

Cited by 44 publications

References 108 publications

177Lu-DOTATATE therapy in metastatic/inoperable pheochromocytoma-paraganglioma

177Lu-DOTATATE therapy in metastatic/inoperable pheochromocytoma-paraganglioma

Anti-Tumor Activity vs. Normal Cell Toxicity: Therapeutic Potential of the Bromotyrosines Aerothionin and Homoaerothionin In Vitro

Natural History and Management of Familial Paraganglioma Syndrome Type 1: Long-Term Data from a Large Family

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