Paraneoplastic endocrine syndromes.
The majority of neoplasms are responsible for symptoms caused by mass effects to surrounding tissues and/or through the development of metastases. However, occasionally neoplasms, with or without endocrine differentiation, acquire the ability to secrete a variety of bioactive substances or induce immune cross-reactivity with the normal tissues that can lead to the development of characteristic clinical syndromes. These syndromes are named endocrine paraneoplastic syndromes when the specific secretory components (hormones, peptides or cytokines) are unrelated to the anticipated tissue or organ of origin. Endocrine paraneoplastic syndromes can complicate the patient's clinical course, response to treatment, impact prognosis and even be confused as metastatic spread. These syndromes can precede, occur concomitantly or present at a later stage of tumour development, and along with the secreted substances constitute the biological 'fingerprint' of the tumour. Their detection can facilitate early diagnosis of the underlying neoplasia, monitor response to treatment and/or detect early recurrences following successful initial management. Although when associated with tumours of low malignant potential they usually do not affect long-term outcome, in cases of highly malignant tumours, endocrine paraneoplastic syndromes are usually associated with poorer survival outcomes. Recent medical advances have not only improved our understanding of paraneoplastic syndrome pathogenesis in general but also enhanced their diagnosis and treatment. Yet, given the rarity of endocrine paraneoplastic syndromes, there is a paucity of prospective clinical trials to guide management. The development of well-designed prospective multicentre trials remains a priority in the field in order to fully characterise these syndromes and provide evidence-based diagnostic and therapeutic protocols.
The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism (WISDEM), University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK g.dimitriadis@warwick.ac.uk.
Georgios K
GK
Dimitriadis
Division of PathophysiologyNational and Kapodistrian University of Athens Medical School, Athens, Greece.
Anna
A
Angelousi
The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism (WISDEM), University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK.
Martin O
MO
Weickert
The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism (WISDEM), University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK.
Harpal S
HS
Randeva
The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism (WISDEM), University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK.|Division of PathophysiologyNational and Kapodistrian University of Athens Medical School, Athens, Greece.|Oxford Centre for DiabetesEndocrinology and Metabolism, University of Oxford, Oxford, UK.
Gregory
G
Kaltsas
Oxford Centre for DiabetesEndocrinology and Metabolism, University of Oxford, Oxford, UK.
Ashley
A
Grossman
D000818:Animals; D006801:Humans; D009369:Neoplasms / Q000378:metabolism; D009384:Paraneoplastic Endocrine Syndromes / Q000175:diagnosis / Q000209:etiology / Q000378:metabolism; D036361:Peptide Hormones / Q000378:metabolism
D036361:Peptide Hormones
Cushing’s syndrome; SIADH; acromegaly; endocrine tumours; hypercalcaemia; hypoglycaemia; paraneoplastic syndromes