NET NM Flashcards
NET of Pancreas inoperable
Celiac trunk
SMA
NET of Pancreas M
Liver
Bone
Abdomen
Lung
NET of Pancreas G
G1 Ki67 <3%
G2 Ki67 3-20%
G3 Ki67 >20%
NET of Pancreas Syndromic
20%
Early detection
Small size
Insulinoma
Gastrinoma
Glucagonoma
VIPoma
Somatostatinoma
Carcinoid
Insulinoma
Most common
6-10% malignancy
Solitary
25% MEN1
10% Islet cell hyperplasia
90% < 2 cm
Whipple triad
Insulinoma
Fasting glucose <50 mg/dL
Hypoglycemia
Relief of symptoms after glucose administration
Gastrinoma triangle
Cystic duct confluence
Junction of pancreatic head and body
Junction of second and third parts of duodenum
Gastrinoma
60-90% malignant
30% with liver MTS
Multiple
75% MEN1
Zollinger-Ellinson sy - - excess of gastrin - - ulcer–PPI
Epigastric pain
Diarrhoea
Glucagonoma
60% malignant
Usually large 6 cm
Necrolytic migratory erythema (usually genitals)
4D: dermatosis, diarrhea, depression, DVT
Stomatitis, anemia, weight loss
VIPoma
10-20% extrapancreatic
80% malignant
VIP - vasoactive intestinal peptide
WDHA sy: watery diarrhea, Hypokalemia, achlorhydria
MEN1
Pituitary adenoma
Parathyroid hyperplasia
Pancreatic tumor - 40-70% NET
NET of Pancreas genetic
MEN1 - 40-70%
Von Hippel Landau - 10-17%
Neurofibromatosis type 1
Tuberous Sclerosis
NET of Pancreas Octreoscan
Negative doesn’t exclude gastrinoma or insulinoma
Glucagonoma 100%
VIPoma 88%
Carcinoid 87%
Gastrinoma 73%
Insulinoma <25%
NET of Pancreas Ga-DOTA-TATE
Gold Standard
Localisation of unknown primary
More accurate than octreoscan
ASVS = arterial stimulation with venous sampling
Intraarterial calcium stimulate insulin production
Localised insulin-secreting NET
NET from neuroendocrine organ
Medullary thyroid cancer
Pancreatic
Pheo
Paraganglioma
NET from dispersed neuroendocrine cells
Pulmonary
Gastro-entero-pancreatic
NET from non endocrine organ
Thymus
Cutaneous NET
Pheo and paraganglioma M
Bone
Liver
Lung
Pheo genetic
MEN2A, MEN2B
VHL
Neurofibromatosis type 1
Pheo 10%
Bilateral
Extra adrenal
Children
Malignant (>5 cm, SDHB mutation)
Pheo image
DOTA - most sensitive, highest detection rate of MTS, SDHB mutation
MRI - 100%
Octreotide <30%
MIBG Pheo and paraganglioma %
Specificity 100%
Sensitivity 80% 50%
MTS avid 60%
Preoperative biopsy not recommended
Pheo
Catecholamine crisis
Tumor rupture
Seeding
Complications: severe hypertension, hematoma, inadequate biopsy, delay in surgery
Low suspicion Pheo
24h urine test
Normetanephrine >900 mcg/24h
Metanephrine >400 mcg/24h
Norepinephrine >170 mcg/24h
Dopamine >700 mcg/24h
High suspicion Pheo
Plasma test
Metanephrine <0.5 nmol/L
Normetanephrine <0.9 nmol/L
Type I gastric Carcinoid
80-90%
Ass with chronic autoimmune gastritis and pernicious anemia
Fundus and body
<2 cm, multiple, localized in gastric wall
Type II gastric Carcinoid
5-7%
Hypergastrinemia from Zollinger-Ellinson sy or small intestine
Recurrent peptic ulcers
<2 cm, multiple, localized in gastric wall
Type III gastric Carcinoid
10-15%
>2 cm, single, infiltrate Perigastric tissue
Well diff - - MTS 50-70%
Duodenal NET
Gastrin producing G-cells 62% - - proximal duodenum - - MEN1
Somatostatin producing D-cells - periampullary - - somatostatinoma - - Neurofibromatosis type 1
<1 cm duodenal gastrinoma - - bulky adenopathy
Best prognosis among gastrointestinal NET
Rectum
Worst prognosis among gastrointestinal NET
Colon
Octreoscan in jejunum and ileum
Distinguish sclerosing mesenteritis from metastatic carcinoid
DOTA in colon and rectum NET
Localization if unknown primary
Octreotide highest affinity
SSTR2a/b
SSTR density in malignant tissue
80-2000 fmol/mg protein
Octreoscan protocol and preparation
IN111-DTPA octreotide
4h and 24h
No consensus - Stop cold octreotide: 1 day for short lived molecule, 3-4 weeks for long-acting
Mild oral laxative 1 day before and continue
120-200 MBq, SPECT
Medium energy collimator
Dual peak energy 172 and 245 keV
Octreoscan indication
GEP-NET, pulmonary NET
Less - skin, paraganglioma, genital tract
Liver MTS isointense, hard to detect
Intense uptake - - higher response to PRRNT
Small insulinoma
No SSTR expression
Tc99-EDDA/HYNIC-TOC octreotide dose
370-740 MBq
VS Octreoscan:
Better distribution pattern
Higher tumor-non-tumor ratio
Lower radiation dose
Better spatial resolution
Ga68-DOTA PET protocol
T1/2 68 min
100-200 MBq
TOC - - SSTR2
TATE - - 10*SSTR2
NOC–SSTR2, 3, 5
Ga - DOTA uptake
Pituitary
Thyroid
Liver
Adrenal
Kidney
Spleen
Excrete - - bladder, intestine
Ga-DOTA indication
GEP-NET and pulmonary NET
Congenital hyperinsulinism,
breast ca,
medulloblastoma,
supratentorial primitive neuroectodermal tumor,
Meningioma
Neuroblastoma
Pheo
Paraganglioma
Catecholamine analog
C11-epinephrine
C11-hydroxyephedrine
F18-dopamine
F18-DOPA
I123-MIBG
NET synthesize catecholamine
Transported by VMAT 1 and 2
VMAT2 in head and neck
I123-MIBG protocol
At least 24h
Option 4-6h
VMAT 1
200-400 MBq
Slow IV to avoid tachycardia, pallor, vomit, abd pain
WBS speed 5-7 cm/min
Spot images 10 min per view
Medium energy high resolution collimator, 159 keV, 20% window
I123-MIBG vs I131-MIBG
Imaging quality better than I131
Better spatial resolution
Higher count rate
Stop drugs 1-3 days before MIBG
Sympathomimetics and cardiovascular
Antiarrhythmics
Alpha and beta blockers
Adrenergic neuron blockers
Ca-channel blockers
Vasoconstricters
Nasal decongestant
Glaucoma Sympathomimetics
Stop neurological drugs 1-3 days before MIBG
Antipsychotics
Sedating antihistamine
Opioids
Tricyclic antidepressants
CNS stimulants
Before MIBG thyroid block
Lugol solution 1 day before, 2 days after
K-perchlorate 4h before, 2 days after 400-600 mg/day
MIBG uptake
Salivary glands
Lung
Heart
Liver
Spleen
Normal adrenal (late image)
Excrete - - kidneys, fecal
MIBG image indication
Paraganglioma – uptake in heart can be very low (down regulation in response to elevated catecholamine)
High uptake in enlarged adrenal - - adrenal paraganglioma
Reduced sensitivity in extra adrenal lesions due to close to physiological uptake
Low sensitivity in paraganglioma of head and neck
F18-DOPA preparation
Fast 4h
200 mg carbidopa 1h before - - increase uptake
F18-DOPA vs MIBG
Higher spatial resolution
Low background activity - - detect smaller lesions
30-60 min vs 24h
No need for thyroid block and stop medication
Both VMAT1 and 2 vs VMAT1
Esp VMAT2 in head and neck paraganglioma
F18-DOPA uptake
Striatum
Kidneys
Pancreas
Liver
Gallbladder
Biliary tract
Duodenum
Adrenal (high uptake in enlarged - - paraganglioma
F18-DOPA indication
Highly sensitive for extra adrenal paraganglioma, esp head and neck
Residual MTC, rising markers
Pancreatic NET in patients with VHL sy
Insulinoma - preop Localisation if congenital hyperinsulinism
Children - - affected Pancreas takes up DOPA and convert to dopamine
Adults - - no dd affected vs normal pancreas due to high physiologic uptake
F18-FDG GLUT
GLUT
Well-diff NET overexpress SSTR and not GLUT
poorly - diff NET - - decline tin SSTR, increase in GLUT expression
F18-FDG indication
Not to localize paraganglioma
Preferred for staging and treatment monitor of metastatic adrenal paraganglioma with SDHB mutation
GEP-NET with positive FDG - - more aggressive
Pulmonary PET - - uptake depends on mitotic index and tumor proliferation rate
Typical Carcinoid
Low proliferative index
Overexpress SSTR
High DOTA
low FDG
Well-diff
Slow grow
Chemo ineffective
Biotherapy with somatostatin analog
Tachypylaxis early after initiation of therapy
<10% partial response
Radionuclide therapy indication
All patients affected by metastatic/unresectable SSTR2-expressing NET
GEP - - frequent liver MTS
Pulmonary
Pheo
Paraganglioma
Neuroblastoma
MTC
Y90-DOTA-TOC and Lu177-DOTA-TATE contra
Abs:
Pregnancy
Severe acute illness
Unmanageable psychiatric disorder
Relative:
Breastfeeding
Creatinine >1.7 (esp Y)
RBC <3, WBC <3, PLT <90 (TOC), <75 (TATE)
Y90-DOTA-TOC and Lu111-DOTA-TATE pretreatment
Renal protection - L-lysine / L-arginine
Inhibit tubular reabsorption
Reduce renal absorbed dose
Hydration - over 4h,starting 30-60 min before PRRNT
25g lysine or arginine in 2L Saline
Amino acids + Gelofusine infusion (plasma expander)
Y90-DOTA-TOC administration
Beta emitters
100 mCi per m^2 of body surface
Second cycle in 6-12 weeks
Or 75-120 mCi in 2-4 cycles at 6-12 weeks interval
LU177-DOTA-TATE administration
150-200 mCi 3-5 cycles at 6-12 weeks interval
Y90-DOTA-TOC vs Lu177-DOTA-TATE
Y90 - - higher energy beta - - more penetrating
Y90 shorter T1/2 - - higher dose rate to tumor
Lu177 - - lower energy and penetration, gamma camera suitable, shorter beta range (higher dose rate to small tumor), lower nephrotoxicity
Y90-DOTA-TOC indication
Bulky and lesions with inhomogenous SSTR
Lu177-DOTA-TATE therapy indication
Smaller tumors
I131-MIBG Contra
Abs
Pregnancy
Life expectancy <3 months (unless palliative)
Severe renal failure + planned dialysis
Relative
Unacceptable medical risk for isolation, urinary incontinence
GFR <30 mL/min
Severe toxycities from prior treatment
WBC <3, PLT <100
I131-MIBG treatment preparation
stop medication
Thyroid block 1-2 days before, over 14 days after
I131-MIBG administration
No consensus - 268 or 149 mCi
Higher activity deliver dose faster with modest increase in toxicity but similar response
Slow IV 45 min-4h to avoid nausea, vomit, hypertension
Monitor vital signs over next 2-3 days
Specific activity for therapy should be higher than for diagnostic purpose
I131-MIBG therapy indication
Inoperable pheo, paraganglioma, carcinoid
Stage III or IV neuroblastoma
Metastatic or recurrent MTC
GEP-NET - - palliative in SSTR-negative, borderline renal function, resistant to somatostatin therapy
Assess SSTR overexpression and select patients for PRRNT
Ga68-DOTA-TATE or Ga68-DOTA-TOC
MEN 2a
Parathyroid hyperplasia
Medullary thyroid carcinoma
Pheo
MEN2b
Mucosal neuroma
Marfanoid body habitus
Medullary thyroid carcinoma
Pheo
Familial Pheo
Hippel-Landau gene - - VHL
RET gene - - MEN2
Neurofibromatosis type 1 gene - - Von Recklinghausen disease
SDHB and SDHD genes - - familial Pheo and paraganglioma
