Management of metastatic phaeochromocytoma and paraganglioma: use of iodine-131-meta-iodobenzylguanidine therapy in a tertiary referral centre

Rutherford, Matthew A.; Rankin, Alastair; Yates, TM; Mark, Patrick B.; Perry, CG; Reed, NS; Freel, E Marie

doi:10.1093/qjmed/hcu208

Cited by 27 publications

(24 citation statements)

References 20 publications

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“…The absence of mutations in the reported case, especially SDHB, may also be related to long-term survival, since SDHB mutations are independently related to shorter survival in patients with malignant pheochromocytoma (26). Intriguingly, there are few reports of malignant pheochromocytoma patients harboring SDHB mutations with prolonged survival (17,22). Whether the absence of germline mutations in the genes previously associated with a malignant behavior represents a protective factor against aggressive disease must be further investigated.…”

Section: Discussionmentioning

confidence: 89%

“…Nevertheless, there are few reports of patients with malignant PCC surviving for Pheochromocytoma with eighteen-year survival Arch Endocrinol Metab. 2018;62/2 more than 15 years after diagnosis (6,(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24). We report a case of malignant PCC with 18-year survival and discuss which factors may be related to mortality and long-term survival in malignant pheochromocytoma.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

What determines mortality in malignant pheochromocytoma? – Report of a case with eighteen-year survival and review of the literature

Andrade¹,

Cunha²,

Oliveira³

et al. 2018

Archives of Endocrinology and Metabolism

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Pheochromocytoma (PCC) is a tumor derived from adrenomedullary chromaffin cells. Prognosis of malignant PCC is generally poor due to local recurrence or metastasis. We aim to report a case of malignant PCC with 18-year survival and discuss which factors may be related to mortality and long-term survival in malignant pheochromocytoma. The patient, a 45-year-old man, reported sustained arterial hypertension with paroxysmal episodes of tachycardia, associated with head and neck burning sensation, and hand and foot tremors. Diagnosis of PCC was established biochemically and a tumor with infiltration of renal parenchyma was resected. No genetic mutation or copy number variations were identified in SDHB, SDHD, SDHC, MAX and VHL. Over 18 years, tumor progression was managed with 131I-MIBG (iodine-metaiodobenzylguanidine) and 177Lutetium-octreotate therapy. Currently, the patient is asymptomatic and presents sustained stable disease, despite the presence of lung, para-aortic lymph nodes and femoral metastases. Adequate response to treatment with control of tumor progression, absence of significant cardiovascular events and other neoplasms, and lack of mutations in the main predisposing genes reported so far may be factors possibly associated with the prolonged survival in this case. Early diagnosis and life-long follow-up in patients with malignant pheochromocytoma are known to be crucial in improving survival.

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Section: Discussionmentioning

confidence: 89%

Section: Introductionmentioning

confidence: 99%

What determines mortality in malignant pheochromocytoma? – Report of a case with eighteen-year survival and review of the literature

Andrade¹,

Cunha²,

Oliveira³

et al. 2018

Archives of Endocrinology and Metabolism

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“…131 I-MIBG doses with a weightbased dose of greater than 2 Gy caused a higher number of grade 3 or 4 hematologic toxicity (107). Biochemical hypothyroidism has been reported in 11%-20% despite thyroid blockade with potassium iodide but symptomatic hypothyroidism is rare (113). Those receiving high single doses of MIBG therapy have a higher incidence of toxicities.…”

Section: I/ 131 I-mibg Theranostics For Pheo and Pglmentioning

confidence: 99%

Norepinephrine Transporter as a Target for Imaging and Therapy

Pandit‐Taskar

Modak

2017

J Nucl Med

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The norepinephrine transporter (NET) is essential for norepinephrine uptake at the synaptic terminals and adrenal chromaffin cells. In neuroendocrine tumors, NET can be targeted for imaging as well as therapy. One of the most widely used theranostic agents targeting NET is metaiodobenzylguanidine (MIBG), a guanethidine analog of norepinephrine. 123 I/ 131 I-MIBG theranostics have been applied in the clinical evaluation and management of neuroendocrine tumors, especially in neuroblastoma, paraganglioma, and pheochromocytoma. 123 I-MIBG imaging is a mainstay in the evaluation of neuroblastoma, and 131 I-MIBG has been used for the treatment of relapsed high-risk neuroblastoma for several years, however, the outcome remains suboptimal. 131 I-MIBG has essentially been only palliative in paraganglioma/pheochromocytoma patients. Various techniques of improving therapeutic outcomes, such as dosimetric estimations, high-dose therapies, multiple fractionated administration and combination therapy with radiation sensitizers, chemotherapy, and other radionuclide therapies, are being evaluated. PET tracers targeting NET appear promising and may be more convenient options for the imaging and assessment after treatment. Here, we present an overview of NET as a target for theranostics; review its current role in some neuroendocrine tumors, such as neuroblastoma, paraganglioma/ pheochromocytoma, and carcinoids; and discuss approaches to improving targeting and theranostic outcomes.

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“…Antiemetics are routinely recommended before the infusion starts. Its incidence has been reported between 4 and 40% [20,[69][70][71][72][73]. During I-131 MIBG infusion, catecholamine discharge may cause hypertension and tachycardia in 20% of the patients [11].…”

Section: Toxicitymentioning

confidence: 99%

“…However, this effect is usually transient and aggravated by combination chemo-radiotherapies [81,86]. Hypogonadism has been reported in higher frequencies with higher I-131 MIBG doses [11,73,87].…”

Section: Radionuclide Treatmentsmentioning

confidence: 99%

I-131 Metaiodobenzylguanidine Therapy of Neuroectodermal Tumors

Araz¹,

Çayir²

2017

Radionuclide Treatments

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I-131 metaiodobenzylguanidine (MIBG) is a noradrenaline analogue and acts as an adrenergic neuron blocker. It is commonly used in the radionuclide treatment of neuroectodermal-derived tumors (Stage III-IV neuroblastoma, inoperable pheochromocytoma, paraganglioma and carcinoid tumor, metastatic or recurrent medullary thyroid cancer). These are rare tumors and clinical data about therapeutic options accumulate slowly. I-131 MIBG has a well-known role in the salvage therapy of these tumors; however, recent data suggest that it may also be beneficial to use as the first-line method. Here, we define characteristics of the radiopharmaceutical, mention cautions during administration and discuss clinical applications of I-131 MIBG therapy of the neuroectodermal tumors.

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Management of metastatic phaeochromocytoma and paraganglioma: use of iodine-131-meta-iodobenzylguanidine therapy in a tertiary referral centre

Cited by 27 publications

References 20 publications

What determines mortality in malignant pheochromocytoma? – Report of a case with eighteen-year survival and review of the literature

What determines mortality in malignant pheochromocytoma? – Report of a case with eighteen-year survival and review of the literature

Norepinephrine Transporter as a Target for Imaging and Therapy

I-131 Metaiodobenzylguanidine Therapy of Neuroectodermal Tumors

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