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Surgical Management of Primary Neuroendocrine Tumors

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Presentation on theme: "Surgical Management of Primary Neuroendocrine Tumors"— Presentation transcript:

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

29 Intraoperative detection

30 Robotic Distal Pancreatectomy/Splenectomy

31 Video

32 Robotic Distal Pancreatectomy/Splenectomy

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