PRRT for Malignant Pheochromocytomas and Paragangliomas: The Singapore General Hospital Experience Abstract #1305

Introduction: In recent years, the use of 177Lu-DOTATATE radioisotope therapy has been expanded to patients with metastatic pheochromocytomas and paragangliomas (PCC/PGL). Although this is not the standard of care, treatment options are limited for those with MIBG negative disease. Our centre has treated over 40 patients with NETS with PRRT since 2012.
Aim(s): We share our clinical experience of PRRT in 4 patients with functional metastatic PCC/PGL.
Materials and methods: Three PGL patients and 1 PCC patient have undergone PRRT since 2014.
Conference: 13th Annual ENETS conference (2016)
Category: PRRT-Ablative therapies-Endoscopic treatment
Presenting Author: Dr Lih Ming Loh

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#2788 Favourable Outcome in Patients with Metastatic Pheochromocytomas and Paragangliomas Treated with 177Lu-DOTATATE
Introduction: There is limited previous data on Peptide receptor radiotherapy (PRRT) in pheochromocytoma (PCC) and paraganglioma (PGL).
Conference: 17th Annual ENETS Conference (2020)
Category: Nuclear Medicine - Imaging and Therapy (PRRT)
Presenting Author: Prof. Anders Sundin
#22 A Case Illustrative of Phenotypic Heterogeneity and Challenges in the Management of Paraganglioma
Introduction: Paragangliomas (PGLs) are extra-adrenal, usually benign, highly vascularized tumors that originate from neural-crest-derived chromaffin cells. These tumors are subdivided as either sympathetic or parasympathetic, depending on their location and catecholamine production. Sympathetic PGLs are situated along the abdominal sympathetic trunk and usually produce catecholamines, whereas parasympathetic PGLs are located in the head and neck, and these usually do not produce catecholamines. PGLs may present as sporadic or inherited tumor syndrome, including MEN 2, with RET germline mutations, von Hippel-Lindau (VHL) disease due to germline mutations in VHL gene, and pheochromocytoma-PGL syndrome. The latter is frequently a hereditary condition and is caused by germline mutations in the SDHB, SDHC, or SDHC genes. Patients with familial PGLs may present at a younger age, often as multifocal tumors, with an increased risk of recurrence and a higher frequency of malignancy in those with SDHB mutations. SDH mutations induce angiogenesis and tumorogenesis through the inhibition of hypoxia-inducible factors (HIF)-propyl hyroxylase. A younger age at onset, malignancy, and a positive family history are clinical parameters of high specificity, but low sensitivity for diagnosis. Genetic analysis for mutations in SDH genes for the patient and family members, and surveillance for the affected patient and family members, are necessary where there are no clear clinical or family indicators for the syndrome. We present a case of a large abdominal malignant PGL in a 20-year-old pt. that went on without clinical detection for at least three years.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
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#941 Comparison of Radiolableled Somatostatin Analogues and (131) I-MIBG Treatment for the Management of Patients with Metastatic/Progressive Phaeochromocytomas and Paragangliomas
Introduction: Radionuclide therapy has been used to treat patients with progressive/metastatic paragangliomas (PGGs) and pheochromocytomas (PCCs). To date, there is no study comparing (131)I-MIBG and peptide receptor radionuclide treatment (PRRT) in those patients.
Conference: 11th Annual ENETS Conference (2014)
Category: PRRT-Ablative therapies-Endoscopic treatment
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#590 Imaging of Metastatic-Recurrent Paragangliomas and Pheochromocytomas with Ga-68 DOTATATE PET/CT
Introduction: Approximately 73% of Pheochromocytomas(Pheo) and 93% of paragangliomas(PGL) express somatostatin receptors and Ga-68 DOTATATE PET/CT may have a role in metastatic PHEO/PGLs for determination of extent of disease.
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#1173 Investigation of the Effects of Sunitinib on Pheochromocytoma and Paraganglioma Primary Cultures
Introduction: The main treatment for Pheochromocytoma and Paraganglioma is surgery. However these tumors are highly vascularized, suggesting the possible role for anti-angiogenic agents in the medical therapy. Sunitinib is a multi-targeted receptor tyrosine kinase inhibitor (TKI), mainly described to inhibit VEGFR
Conference: 12th Annual ENETS Conference (2015)
Category: Basic Science - In vitro models, tumor growth, CTCs
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