EndocrinologyTier 2Disease (DEADMAN)

Neuroendocrine tumours

Description

Neuroendocrine tumours arise from cells of the diffuse neuroendocrine system (found throughout the gastrointestinal tract, pancreas, lungs, and elsewhere) - ranging from indolent, slow-growing tumours to aggressive, poorly differentiated neuroendocrine carcinomas, with functional status (hormone-secreting vs non-functioning) shaping clinical presentation independent of tumour grade

Relatively uncommon but with rising detected incidence, partly reflecting increased incidental detection on imaging performed for other indications

Tumour behaviour is characterised by grade (differentiation and proliferation index, e.g. Ki-67) rather than site alone - well-differentiated, low-grade tumours can behave indolently for years even with metastatic spread, while poorly differentiated neuroendocrine carcinomas behave aggressively similar to other high-grade malignancies, meaning grade (not just the "neuroendocrine tumour" label) should guide prognosis and treatment intensity discussions

Biochemical markers (chromogranin A, and syndrome-specific markers such as 5-HIAA for carcinoid or specific hormone levels for functioning pancreatic neuroendocrine tumours) support diagnosis and monitoring
Somatostatin receptor imaging (e.g. Ga-68 DOTATATE PET) is particularly sensitive for localising well-differentiated neuroendocrine tumours, exploiting their characteristic somatostatin receptor expression, and also identifies candidates for peptide receptor radionuclide therapy

Somatostatin analogues (octreotide, lanreotide) control hormone-related symptoms in functioning tumours and have anti-proliferative activity in well-differentiated disease
Peptide receptor radionuclide therapy (radiolabelled somatostatin analogues) is used for somatostatin receptor-positive, progressive disease
Surgical resection remains central for localised disease; poorly differentiated neuroendocrine carcinoma is generally treated more like other aggressive malignancies (platinum-based chemotherapy)

Carcinoid tumours and carcinoid syndrome (a specific functioning subtype), pancreatic neuroendocrine tumours (functioning - insulinoma, gastrinoma; non-functioning), MEN1 association (see ), somatostatin receptor imaging and peptide receptor radionuclide therapy

Tumour grade (differentiation and proliferation index), not the "neuroendocrine tumour" label alone, determines expected behaviour and appropriate treatment intensity - a well-differentiated, low-grade tumour with metastases can still follow an indolent course over many years, in clear contrast to a poorly differentiated neuroendocrine carcinoma, which behaves aggressively regardless of site

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