Abstract Library

#4468 Prevalence of familial pheochromocytoma and paraganglioma syndromes – A large multicentre study

Introduction: Pheochromocytoma and paraganglioma (PPGLs) are rare tumours. Depending on the studied population, approximately 30% of PPGLs are caused by a germline pathogenic variant (PV).

Conference:

Presenting Author: Halperin R

Authors: Halperin R, Reznick-Levi G, Khalaileh A, Svirsky-Frayden R, Reish O,

Keywords: paraganglioma, pheochromocytoma, genetic evaluation, catecholamine, sdhb, vhl,

#4449 Treatment of MiNEN with peptide receptor radionuclide therapy

Introduction: Mixed non-neuroendocrine and neuroendocrine neoplasms (MiNENs) of gastrointestinal tract are rare tumours with characteristic histological features include at least 30% of both neuroendocrine and non-neuroendocrine components of the whole neoplasm. They are usually treated with surgical resection and chemotherapy in advanced disease. However, there is scarcity of data for the use of peptide receptor radionuclide therapy (PRRT) in these tumours.

Conference:

Presenting Author: Shekhda K

Authors: Shekhda K, Luong T, Paterson A, Navalkissoor S, Krell D,

Keywords: MiNEN, PRRT, DOTATATE,

#4385 SDHB-related metastatic paragangliomas treated with capecitabine and temozolomide: Experience of a tertiary centre

Introduction: SDHB pathogenic variants predispose to aggressive and metastatic paragangliomas (mPGLs). International consensus recommends temozolomide treatment (low-grade evidence). Capecitabine and temozolomide (CAPTEM) has shown advantage over temozolomide in the treatment of neuroendocrine tumours (NETs). Evidence for its use in mPGLs remains scarce.

Conference:

Presenting Author: G. Martins R

Authors: Silva L, Martins R, Couto J, Garcia R, Martins Fernandes A,

Keywords: mPGL, SDHB, captem,

#4224 Uncommon manifestations in type 4 familial paraganglioma syndrome – A large cohort of patients harbouring the SDHB p.Q214Ter variant

Introduction: Familial paraganglioma type 4 syndrome (PPGL4) is caused by a germline pathogenic variant (PV) in the SDHB gene. Patients harbouring germline SDHB PV have a higher risk of developing paragangliomas and pheochromocytomas. PPGL4 is considered a higher-risk syndrome for aggressive, and metastatic, abdominal-thoracic paragangliomas compared with other familial paraganglioma syndromes.

Conference:

Presenting Author: Halperin R

Authors: Halperin R, Jabarin A, Tirosh A,

Keywords: paraganglioma, pheochromocytoma, sdhb, hereditary,

#4208 The somatostatin system – A silent messenger in pheochromocytomas and paragangliomas?

Introduction: Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine neoplasms (NENs) arising from neural crest-derived chromaffin cells. Due to the abundance of somatostatin receptors (SSTs) on the surface of most NENs, somatostatin analogues (SSAs) have become a cornerstone in their diagnosis and treatment. However, while PPGLs exhibit this feature, their functional responsiveness to SSAs remains limited, with the underlying cause of this resistance being still elusive.

Conference:

Presenting Author:

Authors: García Vioque V, Moreno-Montilla M, Blázquez-Encinas R, Barlier A, Arroba E,

Keywords: pheochromocytomas, paragangliomas, neuroendocrine tumor, somatostatin analogues, treatment resistance,