Paraneoplastic endocrine syndromes

Abstract

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.

Bone Metabolism and Vitamin D Implication in Gastroenteropancreatic Neuroendocrine Tumors

“…ECS represents only 10% of all causes of Cushing's, and among these pNET, represents only a small percentage of cases. However, ECS is the most frequent endocrine paraneoplastic syndrome reported in NET [47]. It has well demonstrated that cortisol excess has a negative impact on bone health [48][49][50].…”

The Diagnosis of Neuroendocrine Tumours: An Endocrine Perspective

“…At present, this classification officially only applies to pancreatic NETs, but can probably be applied informally to all NETs (27). Because NE cells can produce a number of hormonal peptides and neuroamines (29,30), an-other classification system for NETs may be based on secretory products. However, a NET may start as a silent tumour then become secretory, may co-secrete several hormones/amines (31), and on recurrence may secrete another product (32); even its metastases may secrete different peptides from the parent tumour.…”

Paraneoplastic syndrome in undifferentiated embryonic sarcoma of the liver

“…Besides general manifestations such as fever, loss of body weight, and night sweats, special paraneoplastic symptoms may occur, which can be very specific in pointing to an underlying cancer. Identifying a patient's symptoms as paraneoplastic allows for early diagnosis of cancer and can be used to monitor response to treatment or to detect recurrence [1,2]. In this report, we describe the case of a 46-year-old patient with undifferentiated embryonic liver sarcoma (UESL).…”