Abstract Library

#946 Immunohistochemical Staining of Catecholamine-Synthesizing Enzymes in Head and Neck Paraganglioma Tissue

Introduction: Head and neck paragangliomas (HNPGLs) are non-producing neuroendocrine tumors, alhough 19-28% of patients have an increased dopamine production. The high sensitivity of 18F-DOPA PET also shows that these tumors might be able to synthesize catecholamines.

Conference: 11th Annual ENETSConcerence (2014)

Presenting Author:

Authors: Van der Horst-Schrivers A, Osinga T, Korpershoek E, De Krijger R, Kerstens M,

Keywords: paraganglioma, dopamine,

#510 Salivary-Free Catecholamines Metabolites in Pheochromocytoma

Introduction: Salivary monitoring of hormone levels have many advantages over the more conventional serum/plasma analysis. Salivary-free metanephrines (MN) and normetanephrines (NMN) could improve biochemical diagnosis of pheochromocytoma (PHEO) as an alternative to plasma metabolites.

Conference: 9th Annual ENETSConcerence (2012)

Presenting Author:

Authors: Stefanescu A, Schipor S, Jercalau S, Badiu C,

Keywords: pheochromocytoma, free metabolites,

#287 Lutetium-177 DOTATATE Therapy in the Management of Neuroendocrine Tumors

Introduction: Peptide receptor radionuclide therapy (PRRT) is a specific targeted treatment for NETs expressing somatostatin receptors. 177Lu-DOTATATE is one of several newly available compounds for this treatment.

Conference: 8th Annual ENETSConcerence (2011)

Presenting Author:

Authors: Carroll R, Tan T, Todd J, Al-Nahhas A, Bomanji J,

Keywords: 177Lutetium-DOTATATE therapy, neuroendocrine tumor, paraganglioma, SDHB,

#64 Sustained response to anti IGFR-1R monoclonal antibody combined with docetaxel in a patient with metastatic adrenocortical carcinoma

Introduction: Adrenocortical carcinoma (ACC) is a rare neoplasm with poor prognosis. Treatment results in advanced disease are disappointing and median survival is less than 12 months. There is no standard chemotherapy, even though mitotane and cisplatin-based regimens are frequently used with limited efficacy. In vitro data suggest that overexpressed insulin growth factor-2 (IGF-2) acting via the IGF-1 receptor (IGF-1R) is relevant for ACC and that the IGF-1R and 2R pathway is a promising target.

Conference: 7th Annual ENETSConcerence (2010)

Presenting Author:

Authors: De Dosso S, Saletti P, Gallerani E, Terrot T, Sessa C,

Keywords: adrenocortical carcinoma, anti IGFR-1R monoclonal antibody,

#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 ENETSConcerence (2010)

Presenting Author: Ahmed D

Authors: Ahmed D, Amin D, Al Faraj D, Al Qahtani D,

Keywords: paraganglioma, succinate dehydrogenase B mutation, abdominal tumor, malignant/metastatic paraganglioma, serum urinary, catecholamines,