SFEBES2013 Poster Presentations Neoplasia, cancer and late effects (26 abstracts)
The relationship between anatomical location of phaeochromocytoma and paraganglioma and their secretory properties
Chung Thong Lim 1 , Jasdeep Gill 1 , Radha Ramachandran 1 , Susan E Brook 1 , Niamh M Martin 1 , Waljit Dhillo 1 , Jeannie Todd 1 , Fausto Palazzo 1 , Martyn Caplin 2 , Pierre Bouloux 3 , Karim Meeran 1 , Tricia Tan 1 & Bernard Khoo 2,
1Imperial Centre for Endocrinology, Imperial College Healthcare NHS Trust, London, UK; 2Neuroendocrine Tumour Unit, Royal Free London NHS Foundation Trust, London, UK; 3Endocrinology, Royal Free London NHS Foundation Trust, London, UK.
Background: Phaeochromocytomas (PCC) and paragangliomas (PGL) are neuroendocrine tumours that can secrete catecholamines. PCC are found in the adrenals, whereas PGL are found at any level between the skull base to the pelvis. More than 25% of cases are associated with mutations in susceptibility genes such as the SDH subunits. Screening for PCC/PGL crucially depends on detection of biochemical markers such as catecholamines and metanephrines (catecholamine metabolites).
Study design: This retrospective study looked at the influence of the site of PGL/PCC on their secretory properties. The relationship between the type of genetic mutations and the secretory properties of PGL/PCC was also investigated. 103 patients with PGL/PCC were identified. Secretory tumours were those with pre-operative levels of catecholamine or metanephrines above normal reference ranges.
Results: PCC are almost exclusively secretory (97%, P<0.0001) while PGL can be secretory (52%) or non-secretory (48%). The majority of the secretory tumours were located in the thorax/abdomen (97%, P<0.0001). In contrast, most H&N PGL are non-secretory (80%). Interestingly, both patients with secretory H&N tumours in this study carried germline mutations in SDH-B, which may lead to metachronous tumours or undetected metastases. In addition, as shown in this study, PGL/PCC that occur in patients with SDH-B mutation tend to be secretory (77%), whereas the tumours could be either secretory (50%) or non-secretory (50%) in patients with SDH-D mutations. Although no additional tumours have been detected in these two patients on follow-up, further and close monitoring is necessary.
Conclusions: PCC are usually secretory whereas PGL can be either secretory or non-secretory. Secretory PGL/PCC tend to be sited in the abdomen. H&N PGL are generally non-secreting tumours. Most importantly, normal catecholamine or metanephrine results do not exclude the diagnosis of PGL/PCC.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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