Neuroendocrine Tumours Neuroendocrine Tumours u Heterogenous group of
- Slides: 19
Neuroendocrine Tumours
Neuroendocrine Tumours u Heterogenous group of neoplasms u Share certain characteristic features – Originate from neuroendocrine cells – Have secretory characteristics – Frequently present with hypersectretory syndromes
Neuroendocrine Tumours u Pancreatic islet cells u Gastroenteric tissue u Respiratory epithelium u Gastroenteropancreatic NETs u Includes carcinoid (serotonin secreting tumour)
Presentation u Asymptomatic – Present with obstructive symptoms u Symptoms – Usually due to liver metastases – Release of hormones into circulation e. g. Serotonin, tachykinin
Symptoms u Intermittent u Diarrhoea abdo pain 50% u Flushing 30% u Lacrimation u Rhinorrhoea u Episodic palpitations u Wheezing 70%
“Carcinoid crisis” Precipitated by Anaesthetic induction Intraoperative tumour handling Therapeutic procedures, eg embolisation Profound flushing Bronchospasm Tachycardia Widely fluctuating BP
Aetiology and Genetics u Poorly understood u Most are sporadic u Small increased familial risk for small intestinal / colonic tumours u Aim to exclude complex cancer syndromes (e. g. MEN 1, MEN 2, NF 1)
Diagnosis u Clinical symptoms u Hormone concentrations u Radiology u Histology – gold standard
Hormone concentrations u Plasma chromagranin A (Cg. A) u Urine 5 -HIAA (24 hrs) u Pancreatic polypeptide u Should also measure other hormones for MEN syndromes. – May correlate with response and relapse – Fast rising levels = poor prognosis – Certain foods affect urinary excretion and may cause false positives – High concentration in 80% pancreatic and 50% carcinoid
Imaging Sensitivites (%) of various imaging modalities for locating specific neuroendocrine tumours Primary carcinoid tumour Carcinoid liver mets Ultrasound 46 83 CT 64 88 MRI 56 85 SSRS 80 90
Imaging u 40 -70% of patients have nodal or liver metastases at time of presentation u If <2 cm diameter: low incidence of metastases
Small intestinal carcinoid: Treatment u Aim should be curative u Palliation in majority of cases u Majority are malignant u Resection of primary and mesenteric lymph nodes despite liver mets – For cure / delay progression (could endanger small bowel) – Can alleviate symptoms – ? Prolong survival
Symptomatic Treatment u Used in patient with secretory symptoms u Somatostatin analogues (e. g. Octreotide) – Inhibits release of many hormones – Can impair some exocrine functions u Hormone response in 30 -70% patients u Symptom control in majority
Additional medication u Ondansatron – nausea u Cyproheptadine u Cholestyramine u CREON – Control of diarrhoea, esp after intestinal resection
Interferon alpha u Sole use or with somatostatin analogues u ? Efficacy (conflicting evidence) u Some evidence better in tumour with low mitotic rates u Biochemical response 40 -60% u Symptomatic improvement 40 -70% u Significant tumour shrinkage 1015%
Other options u Chemotherapy – Role uncertain but being actively researched – Response short lived (8 -10 months) u Hepatic artery embolisation – Indicated for non resectable & multiple hormone secreting tumours – Causes ischaemia of tumour cells – Symptomatic response 40 -80% – 5 year survival 50 -60%
u Radionucleotide therapy – Palliative option – Exploits increased uptake of radiolabelled isotopes I-MIBG or octreotide – Symptom control 80% u Radiotherapy – Carcinoid – radioresistant – Relief of pain from bone mets
Prognosis u No TNM classification u Slow growing, but survival depends on – Histological type – Degree of differentiation – Mitotic rate – Tumour size – Depth and location – Lymph node/ liver metastases
Prognosis – 5 year survival
- Tumours
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- Heterogenous
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- Heterogeneous audience
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