1. Surgical Management of Primary Neuroendocrine Tumors
Carl R. Schmidt, MD, FACS
Set B1 – Title Slide

2. Objectives Discuss goals of surgical resection
Management of GI primary tumors
Management of pancreatic primary tumors (PNET)

3. Goals of Surgical Resection
Cure
Survival
Palliation

4. 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. Survival Typically indolent Long-term survival common
Management of symptoms is important

6. Palliation Hormone syndromes Jaundice Bowel obstruction Pain
Carcinoid syndrome usually liver metastases
Jaundice
Bowel obstruction
Pain

7. GI Carcinoid Locations
Distal small bowel (25-30%)
Colon/appendix
Rectum
Stomach

8. 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. Gastric Carcinoids Type Etiology Gastrin Manifestations
Treatment options I
70-80%
Autoimmune first, atrophic gastritis, achlorhydria
High
Low grade, multiple small <5% mets
Surveillance EGD; EMR or antrectomy * II
5-10%
ZES (neoplasia first)
MEN1
Ulcers multicentric 10% metastasize
Call Dr. Ellison
III
15-20%
Sporadic malignancy
Normal
Solitary mass 50% metastasize
Radical operation
*rarely associated with gastric adenocarcinoma
Gladdy Annals Surg Onc 2009

10. Type III Gastric NEC 4 cm well-diff One + LN No adjuvant therapy
Cancer surveillance

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

12. 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 mets
Mesentery
Liver
Extra-abdominal
None (N=8)
0.25
0.13
Mesentery (N=37)
0.56
0.05
Liver (N=15)
0.27
0.60
Mesentery and Liver (N=59)
0.22

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

14. 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. Rectal Primary Need colonoscopy and CT, consider EUS
< 2 cm - transanal or EMR if possible
> 2cm – LAR or APR

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

17. Functional PNET Tumor Sx Hormone Malignant Other Gastrinoma PUD
Very
Diarrhea
Insulinoma (70%)
Hypo-glycemia
Insulin
Low
Catecholamine
Excess
Glucagonoma
DM, rash
Glucagon
DVT/PE
Weight loss
VIPoma
Watery diarrhea, hypoK
achlorhydria
VIP
High
Met. Acidosis
Hyperglycemia
HyperCa
Flushing
Somatostatinoma DM
Somatostatin
PPoma
Hepatomeg
Pain
Pancreatic polypeptide
Watery diarrhea

18. Glucagonoma

19. Surgical Approach - PNET
Locoregional disease
Radical resection
Enucleation (small, localized lesions)
Advanced disease
Cytoreduction
Optimal management unclear

20. Bloomston J GI Surg 2006

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

22. 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. Insulinoma Basic stats Multicentric ~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. 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
O’Riordain World J Surg 1994

25. 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. Leave Primary Alone? Courtesy M. Bloomston, unpublished
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. 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. The Horizon is Here Minimally-invasive operations Focal radiation
Ablation

29. Intraoperative detection
SPECT/CT

30. Robotic Distal Pancreatectomy/Splenectomy

31. Video

32. Robotic Distal Pancreatectomy/Splenectomy