Neuroendocrine Tumours. Heterogenous group of neoplasms Share certain characteristic features...
-
Upload
hannah-griffith -
Category
Documents
-
view
216 -
download
1
Transcript of Neuroendocrine Tumours. Heterogenous group of neoplasms Share certain characteristic features...
Neuroendocrine TumoursNeuroendocrine Tumours
Neuroendocrine TumoursNeuroendocrine Tumours
Heterogenous group of neoplasmsHeterogenous group of neoplasms Share certain characteristic featuresShare certain characteristic features
– Originate from neuroendocrine cellsOriginate from neuroendocrine cells– Have secretory characteristicsHave secretory characteristics– Frequently present with hypersectretory Frequently present with hypersectretory
syndromessyndromes
Neuroendocrine TumoursNeuroendocrine Tumours
Pancreatic islet cells Pancreatic islet cells Gastroenteric tissueGastroenteric tissue Respiratory epitheliumRespiratory epithelium
Gastroenteropancreatic NETsGastroenteropancreatic NETs Includes carcinoid (serotonin Includes carcinoid (serotonin
secreting tumour)secreting tumour)
PresentationPresentation
AsymptomaticAsymptomatic– Present with obstructive symptomsPresent with obstructive symptoms
SymptomsSymptoms– Usually due to liver metastases Usually due to liver metastases – Release of hormones into circulation e.g. Release of hormones into circulation e.g.
Serotonin, tachykininSerotonin, tachykinin
SymptomsSymptoms
Intermittent abdo pain Intermittent abdo pain 70%70% DiarrhoeaDiarrhoea 50%50% FlushingFlushing 30%30% LacrimationLacrimation RhinorrhoeaRhinorrhoea Episodic palpitationsEpisodic palpitations Wheezing Wheezing PellagraPellagra
““Carcinoid crisis”Carcinoid crisis”
Precipitated byPrecipitated by Anaesthetic inductionAnaesthetic induction
Intraoperative tumour handlingIntraoperative tumour handlingTherapeutic procedures, eg embolisationTherapeutic procedures, eg embolisation
Profound flushingProfound flushingBronchospasmBronchospasmTachycardiaTachycardiaWidely fluctuating BPWidely fluctuating BP
Aetiology and GeneticsAetiology and Genetics
Poorly understoodPoorly understood Most are sporadicMost are sporadic Small increased familial risk for small Small increased familial risk for small
intestinal / colonic tumoursintestinal / colonic tumours Aim to exclude complex cancer Aim to exclude complex cancer
syndromes (e.g. MEN 1, MEN 2, NF1)syndromes (e.g. MEN 1, MEN 2, NF1)
DiagnosisDiagnosis
Clinical symptomsClinical symptoms Hormone concentrationsHormone concentrations RadiologyRadiology HistologyHistology – gold standard – gold standard
Hormone concentrationsHormone concentrations Plasma chromagranin A (CgA)Plasma chromagranin A (CgA)
– May correlate with response and relapseMay correlate with response and relapse– Fast rising levels = poor prognosisFast rising levels = poor prognosis
Urine 5-HIAA (24 hrs)Urine 5-HIAA (24 hrs)– Certain foods affect urinary excretion and may Certain foods affect urinary excretion and may
cause false positivescause false positives Pancreatic polypeptidePancreatic polypeptide
– High concentration in 80% pancreatic and 50% High concentration in 80% pancreatic and 50% carcinoidcarcinoid
Should also measure other hormones for Should also measure other hormones for MEN syndromes. MEN syndromes.
ImagingImaging Sensitivites (%) of various imaging modalities for Sensitivites (%) of various imaging modalities for
locating specific neuroendocrine tumourslocating specific neuroendocrine tumours
Primary carcinoid Primary carcinoid tumourtumour
Carcinoid liver Carcinoid liver metsmets
UltrasoundUltrasound 4646 8383
CTCT 6464 8888
MRIMRI 5656 8585
SSRSSSRS 8080 9090
ImagingImaging
40-70% 40-70% of patients have nodal or of patients have nodal or liver metastases at time of liver metastases at time of presentationpresentation
If <2 cm diameter: low incidence of If <2 cm diameter: low incidence of metastasesmetastases
Small intestinal carcinoid: Small intestinal carcinoid: TreatmentTreatment
Aim should be curativeAim should be curative Palliation in majority of casesPalliation in majority of cases Majority are malignantMajority are malignant Resection of primary and mesenteric Resection of primary and mesenteric
lymph nodes despite liver metslymph nodes despite liver mets– For cure / delay progression (could For cure / delay progression (could
endanger small bowel)endanger small bowel)– Can alleviate symptomsCan alleviate symptoms– ? Prolong survival ? Prolong survival
Symptomatic TreatmentSymptomatic Treatment
Used in patient with secretory symptomsUsed in patient with secretory symptoms Somatostatin analogues (e.g. Octreotide)Somatostatin analogues (e.g. Octreotide)
– Inhibits release of many hormonesInhibits release of many hormones– Can impair some exocrine functionsCan impair some exocrine functions
Hormone response in 30-70% patientsHormone response in 30-70% patients Symptom control in majoritySymptom control in majority Rarely tumour shrinkageRarely tumour shrinkage
Additional medicationAdditional medication
Ondansatron Ondansatron – nauseanausea
CyproheptadineCyproheptadine CholestyramineCholestyramine CREONCREON
– Control of diarrhoea, esp after intestinal Control of diarrhoea, esp after intestinal resectionresection
Interferon alphaInterferon alpha
Sole use or with somatostatin Sole use or with somatostatin analoguesanalogues
? Efficacy (conflicting evidence)? Efficacy (conflicting evidence) Some evidence better in tumour with Some evidence better in tumour with
low mitotic rateslow mitotic rates Biochemical response 40-60% Biochemical response 40-60% Symptomatic improvement 40-70%Symptomatic improvement 40-70% Significant tumour shrinkage 10-15% Significant tumour shrinkage 10-15%
Other optionsOther options
Chemotherapy Chemotherapy – Role uncertain but being actively Role uncertain but being actively
researchedresearched– Response short lived (8-10 months)Response short lived (8-10 months)
Hepatic artery embolisationHepatic artery embolisation– Indicated for non resectable & multiple Indicated for non resectable & multiple
hormone secreting tumourshormone secreting tumours– Causes ischaemia of tumour cellsCauses ischaemia of tumour cells– Symptomatic response 40-80%Symptomatic response 40-80%– 5 year survival 50-60%5 year survival 50-60%
Radionucleotide therapyRadionucleotide therapy– Palliative optionPalliative option– Exploits increased uptake of Exploits increased uptake of
radiolabelled isotopes I-MIBG or radiolabelled isotopes I-MIBG or octreotideoctreotide
– Symptom control 80%Symptom control 80% Radiotherapy Radiotherapy
– Carcinoid – radioresistantCarcinoid – radioresistant– Relief of pain from bone metsRelief of pain from bone mets
PrognosisPrognosis
No TNM classificationNo TNM classification Slow growing, but survival depends Slow growing, but survival depends
on on – Histological type Histological type – Degree of differentiationDegree of differentiation– Mitotic rateMitotic rate– Tumour sizeTumour size– Depth and locationDepth and location– Lymph node/ liver metastasesLymph node/ liver metastases
Prognosis – 5 year survivalPrognosis – 5 year survival