OncologyTier 2Disease (DEADMAN)

Hormone secreting tumours

Description

A subset of tumours produce excess hormone, causing a distinct clinical syndrome from the hormonal effect itself, independent of (and sometimes preceding) symptoms from tumour bulk

Individually rare, but important to recognise given the syndrome itself can be the presenting/most disabling feature, and specific treatment of the hormonal effect is often needed alongside oncological treatment

Tumour	Hormone	Syndrome
Carcinoid tumour (usually midgut, with liver metastases)	Serotonin and other vasoactive substances	Carcinoid syndrome - flushing, diarrhoea, bronchospasm, right heart valve fibrosis
Phaeochromocytoma	Catecholamines	Episodic hypertension, palpitations, headache, sweating
Insulinoma	Insulin	Fasting hypoglycaemia (Whipple's triad)
Gastrinoma	Gastrin	Zollinger-Ellison syndrome - refractory peptic ulcer disease
Small cell lung cancer	ACTH (ectopic)	Cushing's syndrome, often rapid-onset
Small cell lung cancer	ADH (ectopic)	SIADH, hyponatraemia

Carcinoid syndrome requires hepatic metastasis (or a non-portal-drained primary) to become clinically apparent - the liver normally metabolises serotonin from a midgut primary before it reaches the systemic circulation via the portal vein
Biochemical confirmation is tumour/hormone-specific: 24-hour urinary 5-HIAA (carcinoid), plasma free metanephrines (phaeochromocytoma), supervised fasting test with insulin/C-peptide (insulinoma)

Functional imaging (e.g. somatostatin receptor scintigraphy/PET for neuroendocrine tumours, MIBG scan for phaeochromocytoma) localises the tumour by exploiting receptor/uptake characteristics specific to the tumour type
Symptom control often requires specific hormonal blockade alongside oncological treatment - e.g. somatostatin analogues (octreotide) for carcinoid syndrome, alpha-blockade before beta-blockade for phaeochromocytoma (beta-blockade first risks unopposed alpha-mediated hypertensive crisis)
Definitive treatment is tumour removal/debulking where feasible, alongside hormonal control

Multiple endocrine neoplasia syndromes (MEN1 - gastrinoma, insulinoma; MEN2 - phaeochromocytoma), neurofibromatosis type 1 (phaeochromocytoma)

Hormonal crises (e.g. carcinoid crisis, phaeochromocytoma hypertensive crisis) can be triggered by anaesthesia/tumour manipulation during surgery - specific perioperative prophylaxis (octreotide cover, alpha-blockade) is required to prevent this

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