Yoo

1. Neuroendocrine Tumours

2. Neuroendocrine Tumours
 Heterogenous group of neoplasms
 Share certain characteristic features
– Originate from neuroendocrine cells
– Have secretory characteristics
– Frequently present with hypersectretory
syndromes

3. Neuroendocrine Tumours
 Pancreatic islet cells
 Gastroenteric tissue
 Respiratory epithelium
 Gastroenteropancreatic NETs
 Includes carcinoid (serotonin
secreting tumour)

4. Presentation
 Asymptomatic
– Present with obstructive symptoms
 Symptoms
– Usually due to liver metastases
– Release of hormones into circulation
e.g. Serotonin, tachykinin

5. Symptoms
 Intermittent abdo pain 70%
 Diarrhoea
50%
 Flushing
30%
 Lacrimation
 Rhinorrhoea
 Episodic palpitations
 Wheezing

6. “Carcinoid crisis”
Precipitated by
Anaesthetic induction
Intraoperative tumour handling
Therapeutic procedures, eg embolisation
Profound flushing
Bronchospasm
Tachycardia
Widely fluctuating BP

7. Aetiology and Genetics
 Poorly understood
 Most are sporadic
 Small increased familial risk for small
intestinal / colonic tumours
 Aim to exclude complex cancer
syndromes (e.g. MEN 1, MEN 2, NF1)

8. Diagnosis
 Clinical symptoms
 Hormone concentrations
 Radiology
 Histology – gold standard

9. Hormone concentrations
 Plasma chromagranin A (CgA)
– May correlate with response and relapse
– Fast rising levels = poor prognosis
 Urine 5-HIAA (24 hrs)
– Certain foods affect urinary excretion and may
cause false positives
 Pancreatic polypeptide
– High concentration in 80% pancreatic and 50%
carcinoid
 Should also measure other hormones for
MEN syndromes.

10. 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

11. Imaging
 40-70% of patients have nodal or
liver metastases at time of
presentation
 If <2 cm diameter: low incidence of
metastases

12. Small intestinal carcinoid:
Treatment
 Aim should be curative
 Palliation in majority of cases
 Majority are malignant
 Resection of primary and mesenteric
lymph nodes despite liver mets
– For cure / delay progression (could
endanger small bowel)
– Can alleviate symptoms
– ? Prolong survival

13. Symptomatic Treatment
 Used in patient with secretory
symptoms
 Somatostatin analogues (e.g.
Octreotide)
– Inhibits release of many hormones
– Can impair some exocrine functions
 Hormone response in 30-70%
patients
 Symptom control in majority

14. Additional medication
 Ondansatron
– nausea
 Cyproheptadine
 Cholestyramine
 CREON
– Control of diarrhoea, esp after intestinal
resection

15. Interferon alpha
 Sole use or with somatostatin
analogues
 ? Efficacy (conflicting evidence)
 Some evidence better in tumour with
low mitotic rates
 Biochemical response 40-60%
 Symptomatic improvement 40-70%
 Significant tumour shrinkage 10-
15%

16. Other options
 Chemotherapy
– Role uncertain but being actively
researched
– Response short lived (8-10 months)
 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%

17.  Radionucleotide therapy
– Palliative option
– Exploits increased uptake of
radiolabelled isotopes I-MIBG or
octreotide
– Symptom control 80%
 Radiotherapy
– Carcinoid – radioresistant
– Relief of pain from bone mets

18. Prognosis
 No TNM classification
 Slow growing, but survival depends
on
– Histological type
– Degree of differentiation
– Mitotic rate
– Tumour size
– Depth and location
– Lymph node/ liver metastases

19. Prognosis – 5 year survival