You are on page 1of 3

GEP-NETs short note by S.

Wichien (SNG KKU)


GEP-NETs -Gastro-Entero-Pancreatic NE Tumor -Enterochromaffin cell (Kulchitsky cell, Argentaffin cell) -ectoderm -serotonin, chromogranin, histamine Location 1.foregut (7%) -esophagus, stomach, duodenum GB, pancreas, trachea, bronchus, lung 2.midgut (63%) -jejunum, ileum (most common), meckel, appendix, ovary, testis 3.hindgut (30%) -colon, rectum -not produce carcinoid syndrome Gross pathology -submucosa -yellow cut surface (hi lipid) -desmoplastic rxn around mass :mesenteric fibrosis :can smb obstruction Histology -uniform small round cell -5 growth pattern 1.Insular=peripheral palisading 2.Trabecular=ribbon like 3.Glandular=acinar/rosette like 4.undiff 5.mixed type Carcinoid syndrome Tryptophan metabolism nicotinic serotonin -normal 99% 1% -carcinoid 40% 60% -low nicotinic acidpellagra -hi serotoninportal v5HIAA (inactive) *if have syndrome (not pass PV) 1.liver metastasis 2.primary tumor outside GI tract Clinical features of carcinoid synd -flushing 85-90% -secretory diarrhea 70% -abdo pain -face telangiectasia -rt sided vulvular heart disease (not left) -pellagra3D=diarrhea/dementia/dermatitis Carcinoid crisis -acute hypotension -tachycardia -arrhythmia -bronchospasm -flushing Tx -iv octreotide 50 mcg then 50 mcg/hr Dx study 1.24 hr urine 5 HIAA -can dx, not prognosis 2.serum chromogranin -universal marker -best screening -can tell prognosis 3.CT 4.octreotide scan Typical imaging findings -hypervascular mass -calcification -central necrosis -not involved ductal system -vascular thrombosis (not encase) WHO classification for NETS Well diff Well dif NE tumor NE CA Behavior low low Metas + Ki 67 (%) <2 2-10 Angioinva + Size (cm) <=2 >2 (Panc) >2 >4 IHC -Chromogranin A (CgA) -Synaptophysin -Neuron Specific Enolase (NSE) Poorly diff NE CA hi malig + >20 + any size any size

GEP-NETs short note by S.Wichien (SNG KKU)


Surgical Tx Objective 1.curative 2.cytoreductive 3.palliative from obstruct/bleeding Pre-op preparation 1.echo assess valve HD 2.CT/scan for localization 3.preop octreotide prevent crisis Intraop assessment -may need IOUS/scope -primary tumor resection -cholecystectomy (may post op octreotide) -debulking other location -liver metastasis :can resect >90% = resect :can resect <90% = OLT 1.Gastric endocrine tumor -incidental finding -body/fundus Type1 (75%) -F>M, 70-80 years -asso atrophic gastritis type A -asso hypergastrinemia -rare carcinoid synd + metas Type2 (5%) -F=M, 60 years -asso ZES -asso hypergastrinemia -rare carcinoid synd, metas < 25% Type3 (20%) -M>F -hi histamine > serotonin (atypical carcinoid syndrome) -sporadic -aggressive -metas 50-60% Sx Type1+2 <1cm -endoscopic resection/limited resection Type1+2 1-2 cm -tumor resection + antrectomy (dec gastrin) -subT gastrectomy + D1LND (aggress type) Type1+2 >2cm -subT gastrectomy + D1LND Type3 -subT gastrectomy + D1LND 2.Duodenal endocrine tumor -1st, 2nd part -incidental finding from EGD Tx <1cm = endoscopic/open excision 1-2cm = controversy >2cm = segmental resection+LND or PPPD 3.SMB carcinoid -most common -2-3 foot from IC valve Tx -segmental resection+LND -margin 5 cm -cholecystectomy 4.Appendiceal NET -80% Tip of appendix -90% finding s/p appendectomy Tx <2cm = appendectomy I/C for rt hemicolectomy 1.>2cm 2.base location 3.+ve margin 4.mesoappendix invasion 5.angiolymphatic invasion 6.serosal involvement 7.globet cell carcinoid 8.hi mitotic index 5.Colon NET -50% caecum -incidental finding s/p colonoscope Tx -as colon adenoca 6.Rectal NET -4-13 cm above dentate line -not produce symptom Tx <1cm, no symp, N0, no muscular invasion -local excisionTransanal excision 1-2cm -controversy >2cm, N+ve -radical oncologic resection -LAR or APR

GEP-NETs short note by S.Wichien (SNG KKU)


7.PNET Tumor malignant potential gastrinoma very hi insulinoma low glucagonoma very hi VIPoma hi somatostatinoma very hi PPoma very hi 1.insulinoma -preserve pancreatic ductenucleation 2.others -PD -distal pancreatectomy -central segmental resection 8.unresectable/ metastasis 1.carcinomatosis -not C/I for sx -should residual < 2.5mm -omentectomy,oophorectomy,appendectomy 2.unresectable metas+resect midgutNET -should resect primary tumor 3.unresectable metas+resectable PNET -not recommend sx 4.poorly diff tumor -not recommend sx Med Tx in GEP-NETs 1.systemic CMT -Streptozotocin -Temozolomide 2.biological Tx -INF -somatostatin analog :Lanreotidelong acting sandostatin :Depot octreotide30d 3.Targeted Tx -mTOR inhibitor -VEGF abbevacizumab -VEGF+PDGF absunitinib,sorafenib 4.RT -painful bony metastasis GI-NET PNET octreotide Everolimus TMZ+cape sunitinib STZ+5FU/Dox CMT = platinum+etoposide

Well diff Poorly diff

You might also like