Neuroendocrine Tumours Neuroendocrine Tumours u Heterogenous group of

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Neuroendocrine Tumours

Neuroendocrine Tumours

Neuroendocrine Tumours u Heterogenous group of neoplasms u Share certain characteristic features – Originate

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

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

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

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

“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

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

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)

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

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

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

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.

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

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)

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

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

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

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

Prognosis – 5 year survival