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Management of Primary Neuroendocrine Tumors Surgical Management of Primary Neuroendocrine Tumors Carl R. Schmidt, MD, FACS.

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Presentation on theme: "Management of Primary Neuroendocrine Tumors Surgical Management of Primary Neuroendocrine Tumors Carl R. Schmidt, MD, FACS."— Presentation transcript:

1 Management of Primary Neuroendocrine Tumors Surgical Management of Primary Neuroendocrine Tumors Carl R. Schmidt, MD, FACS

2 Management of Primary Neuroendocrine Tumors Objectives Discuss goals of surgical resection Management of GI primary tumors Management of pancreatic primary tumors (PNET)

3 Management of Primary Neuroendocrine Tumors Goals of Surgical Resection Cure Survival Palliation

4 Management of Primary Neuroendocrine Tumors Cure Carcinoid = cancer Five-year survival 67% Death often due to metastatic disease –Extent of disease workup –Multi-phase CT or MRI (liver mets) –Octreoscan

5 Management of Primary Neuroendocrine Tumors Survival Typically indolent Long-term survival common Management of symptoms is important

6 Management of Primary Neuroendocrine Tumors Palliation Hormone syndromes –Carcinoid syndrome usually liver metastases Jaundice Bowel obstruction Pain

7 Management of Primary Neuroendocrine Tumors GI Carcinoid Locations Distal small bowel (25-30%) Colon/appendix Rectum Stomach

8 Management of Primary Neuroendocrine Tumors GI Carcinoid Symptoms Diagnosis difficult –2-3 years from symptom onset to diagnosis –Normal exam, labs, endoscopy Abdominal pain (mesenteric ischemia) Partial small bowel obstruction

9 Management of Primary Neuroendocrine Tumors Gastric Carcinoids TypeEtiologyGastrinManifestationsTreatment options I 70-80% Autoimmune first, atrophic gastritis, achlorhydria HighLow grade, multiple small <5% mets Surveillance EGD; EMR or antrectomy * II 5-10% ZES (neoplasia first) MEN1 HighUlcers multicentric 10% metastasize Call Dr. Ellison III 15-20% Sporadic malignancyNormalSolitary mass 50% metastasize Radical operation Gladdy Annals Surg Onc 2009 *rarely associated with gastric adenocarcinoma

10 Management of Primary Neuroendocrine Tumors Type III Gastric NEC 4 cm well-diff One + LN No adjuvant therapy Cancer surveillance

11 Management of Primary Neuroendocrine Tumors Small Bowel Primary Curative intent: –Bowel resection with regional LND –Multicentric (20-40%) –Consider prophylactic cholecystectomy (gallstones associated with Octreotide) Palliation –Small bowel bypass

12 Management of Primary Neuroendocrine Tumors Small Bowel Primary >80% risk of recurrence after initial resection –Moertel J Clin Oncol 1987 Probability of developing metastases to new sites –Follow-up 1-11 years (mean 5.2) –Makridis World J Surg 1996 Initial metsMesenteryLiverExtra- abdominal None (N=8) Mesentery (N=37) Liver (N=15) Mesentery and Liver (N=59) 0.22

13 Management of Primary Neuroendocrine Tumors Appendiceal Primary Incidence decreasing –?less incidental appendectomies Generally good prognosis –5-30% localized –86% 5-yr survival Sandor Am J Gastro 1998

14 Management of Primary Neuroendocrine Tumors NCCN - Appendix 2cm, confined to appendix –Appendectomy, no surveillance > 2cm, incomplete resection, nodal spread –Abdomen/pelvis CT or MRI –Right hemicolectomy Goblet cell or adenocarcinoid – manage as colon adenocarcinoma

15 Management of Primary Neuroendocrine Tumors Rectal Primary Need colonoscopy and CT, consider EUS < 2 cm - transanal or EMR if possible > 2cm – LAR or APR

16 Management of Primary Neuroendocrine Tumors Pancreatic Primary (PNET) Family history – MEN1 (gastrinoma and insulinoma) 60% functional 90% of non-functional are malignant –Chromogranin A (pancreastatin) –CT, MRI –Octreoscan?

17 Management of Primary Neuroendocrine Tumors Functional PNET TumorSxHormoneMalignantOther GastrinomaPUDGastrinVeryDiarrhea Insulinoma (70%)Hypo-glycemiaInsulinLowCatecholamin e Excess GlucagonomaDM, rashGlucagonVeryDVT/PE Weight loss VIPomaWatery diarrhea, hypoK achlorhydria VIPHighMet. Acidosis Hyperglycemi a HyperCa Flushing SomatostatinomaDM Diarrhea Somatostati n VeryWeight loss PPomaHepatomeg Pain Pancreatic polypeptide VeryWatery diarrhea

18 Management of Primary Neuroendocrine Tumors Glucagonoma

19 Management of Primary Neuroendocrine Tumors Surgical Approach - PNET Locoregional disease –Radical resection –Enucleation (small, localized lesions) Advanced disease –Cytoreduction –Optimal management unclear

20 Management of Primary Neuroendocrine Tumors Bloomston J GI Surg 2006

21 Management of Primary Neuroendocrine Tumors Advanced PNET Mortality: R2 > R0/1 (21% vs. 2%, p=0.009) Bloomston J GI Surg 2006

22 Management of Primary Neuroendocrine Tumors Advanced PNET Long-term survival possible with complete resection of PNET –5 year survival 74% with R0 resection Noncurative pancreatectomy requires extensive resection resulting in substantial morbidity and mortality –Approach cautiously

23 Management of Primary Neuroendocrine Tumors Insulinoma Basic stats –~10% malignant –~10% >2 cm –~10% multiple* –<10% associated with MEN1* Multicentric –6 of 207 (3%) non-MEN1 –10 of 17 (59%) MEN1 Service Mayo Clinic Proceedings 1991

24 Management of Primary Neuroendocrine Tumors Insulinoma Surgical Approach Non-MEN1 –enucleation if small >2-3 mm from PD –partial pancreatectomy for large/deep tumors MEN1 –subtotal pancreatectomy + enucleation of head lesions ORiordain World J Surg 1994

25 Management of Primary Neuroendocrine Tumors Pancreas Bottom Line PNET require complex management like any pancreas mass or malignancy Multidisciplinary approach – HPB, Surg Onc, GI, Med Onc, Rad Onc, Radiology, Pathology

26 Management of Primary Neuroendocrine Tumors Leave Primary Alone? 197 Patients Undergoing TACE 100 Primary Tumors Intact at TACE 97 Primary Tumors Not Intact at TACE 7 Primaries Symptomatic at TACE 93 Primaries Asymptomatic at TACE 67 Primaries Symptomatic at Resection 30 Primaries Asymptomatic at Resection 4 Primaries Developed Symptoms 89 Primaries Remained Asymptomatic Courtesy M. Bloomston, unpublished

27 Management of Primary Neuroendocrine Tumors Surveillance NCCN guidelines exist Data does not Generally –Exams, labs and imaging within 3-12 months –Endoscopy (gastric or rectal) –Exams and labs every 6 months or annual after first year – imaging when indicated

28 Management of Primary Neuroendocrine Tumors The Horizon is Here Minimally-invasive operations Focal radiation Ablation

29 Management of Primary Neuroendocrine Tumors Intraoperative detection SPECT/CT

30 Management of Primary Neuroendocrine Tumors Robotic Distal Pancreatectomy/Splenectomy

31 Management of Primary Neuroendocrine Tumors Video

32 Management of Primary Neuroendocrine Tumors Robotic Distal Pancreatectomy/Splenectomy


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