ENDOCRINE Flashcards
Pnet men or women more common
M>f Median age -60 64% distant mets at presentation 14%local disease 22% regional disease Men1 Tsc Vhl exon 3 aggressive Nf1- somatostatinoma
Ghrelin from which cell
P/D1 cells
General Markers of NETs
Chromogranin
Synaptophysin
NSE
protein gene product 9.5( PGP)
Markers diff bw PNET &NET
NESP55a pnet and pheo
PDX1
Pnet main pathways
Men1
Daxx or atrx
Mtor
Whicbtumor acts like exocrine
Mixed nets
Grades of pnet
Mitotic count and ki67
G1-3
Nonfunctional or nonsecretory pnet
Pancreatic polypeptide- no clincial syndrome
Rarely diarhea dm ulcer
Drugs for pnet
Sunitinib Everolimus Somatostatin analogues Streptozocin based ct doxo fu capacetabine Dacarbazine and temozolomide
Somatostatin analogues
Octreo lanreo- sstr 2 >sstr5
Pasireotide- ssrt1,2,3,5
Peptide receptir rt
Lu 177 dotao
In 111 dtoa
Y90 dota octreo
Mc functioning pnet
Insulinoma
Gastrinoma in duo
Secretin stimulation level and ca stimulation level in equivocal gastrin levels
Secretin -200
Ca-395
Gastrinoma in pancreas mc site
Head or uncinate
In MEN 1 with ZES which type of gastric
carcinoid occurs
Type 2
Insulinoma diagnostic level
> 3microIU insulin
Verner morrison syndrome
Vipoma
Watery diarrhoea
Hypokalemia
Achlorhydria
Necrolytic migratory erythema
Glucagonoma T/t oha and insulin Nem -100% Dm- 83% Stomatitis and wt loss 67%
Von rechlingausen asso with net?
Somatostatinoma
Mc carcinoid
Gi> lung
SI>
Risk factors for midgut carcinoid
Age
Male sex
Increased bmi
Menopausal hrt
Carcinoid markers
Specific- 5hiaa
Chg A
Which carcinoid produce acth
Thymic
Mc cause of ectopic ACTH syndrome
Bronchial carcinoid
100 %specific for midgut carcinoid
Ct enteroclysis
Which test identifies whether pt will
respond to therapy( lu 177) or not?
Ga 68 dotate
Mc site of esophageal carcinoid
Lower
Mc gastric net
Type 1>type 3
Type 3- no hypergastrinemia and has high mets chance
Carcinoid symptom mc
Flushing 94%
Diarrhoea 80%
Heart disease 40% rt heart valvular
Increased cardiac disease of 5hiaa more than
100
3 non classical men2A
Men 2a with cutaneous lichen amyloidsosis, with hirschspring disease
And familial MTC
Wagermann frobose
Men 2b or 3
Collagenomas and angiofibromas common in which men
1
Cdk1b mutation in which men
4 - kidney and reproductive organs
Mcc of death un men1
Pnet malignan
> thymic carcinoid
Inability to cry
Men 3
P27 protein product is seen in whcih men
Men 4
Cla men 2a codon
634 in exon 11 609 611 618 620 in exon 10 in hirchsprung
Men1 genetic screening when
.2/> men1 related tumors
Multiple abnormal pth gland before 30 yrs
Recurrent hot at young age
Gastrinoma and hot or multiple pnetd at any age
Fho renal stones endocrine tumors
Phpt men1 surgery
3.5 gland removed + thymectomy
Basal acid output > ….. dx of ZES
15 mEq /hr
All cases surgery
Insulinoma
Codon in men2b
16 M918T
15 A883F
Local adv ileal net treatment
Ileal resection + nodes+ cholecystectomy ( octreotide causes stone)
Ssr pet
68 ga dotatate
68ga dotatoc
64cu dotatate
Carcinoid appendix mgt
When is rt hemi needed
=2 cm - simple appendectomy
>2 or incomplete or positive node or margin— rt hemicolectomy
Nodes for appendectomy net in > 2 cm
12
Telotristat
Im carcinoid when its uncontrolled with lanreo and octreo to control diarrhoea
Pnet cam be observed if
Low grade
less than 2 cm
Incidental
Non functional
Glucagonoma mc site
Tail
Mitotic rate per
10hpf or 2 mm squared
Typical and atypical carcinoid in bronchus
Bronchus
Typical <2 mitosis
Atypical 2-10
Small and large cell >10
Pancreatic net origin identified by
Isl 1 and PAX 8
Lung -TTF
Bowel- CDX2
Glucagonoma syndrome
Diabetes
Dermatitis
Deep vein thrombosis
Depression.
