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Management of Primary Neuroendocrine Tumors
Surgical Management of Primary Neuroendocrine Tumors
Carl R. Schmidt, MD, FACS
Management of Primary Neuroendocrine Tumors
Objectives
• Discuss goals of surgical resection
• Management of GI primary tumors
• Management of pancreatic primary tumors (PNET)
Management of Primary Neuroendocrine Tumors
Goals of Surgical Resection
• Cure
• Survival
• Palliation
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
Management of Primary Neuroendocrine Tumors
Survival
• Typically indolent
• Long-term survival common
• Management of symptoms is important
Management of Primary Neuroendocrine Tumors
Palliation
• Hormone syndromes– Carcinoid syndrome usually liver metastases
• Jaundice
• Bowel obstruction
• Pain
Management of Primary Neuroendocrine Tumors
GI Carcinoid Locations
• Distal small bowel (25-30%)
• Colon/appendix
• Rectum
• Stomach
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
Management of Primary Neuroendocrine Tumors
Gastric Carcinoids
Type Etiology Gastrin Manifestations Treatment options
I70-80%
Autoimmune first, atrophic gastritis, achlorhydria
High Low grade, multiple small <5% mets
Surveillance EGD; EMR or antrectomy *
II5-10%
ZES (neoplasia first)MEN1
High Ulcers multicentric 10% metastasize
Call Dr. Ellison
III15-20%
Sporadic malignancy Normal Solitary mass 50% metastasize
Radical operation
Gladdy Annals Surg Onc 2009
*rarely associated with gastric adenocarcinoma
Management of Primary Neuroendocrine Tumors
Type III
• Gastric NEC• 4 cm well-diff• One + LN• No adjuvant
therapy• Cancer
surveillance
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
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 mets Mesentery Liver Extra-abdominal
None (N=8) 0.25 0.13 0
Mesentery (N=37) 0.56 0.05
Liver (N=15) 0.27 0.60
Mesentery and Liver (N=59)
0.22
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
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
Management of Primary Neuroendocrine Tumors
Rectal Primary
• Need colonoscopy and CT, consider EUS
• < 2 cm - transanal or EMR if possible
• > 2cm – LAR or APR
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?
Management of Primary Neuroendocrine Tumors
Functional PNETTumor Sx Hormone Maligna
ntOther
Gastrinoma PUD Gastrin Very Diarrhea
Insulinoma (70%)
Hypo-glycemia
Insulin Low CatecholamineExcess
Glucagonoma DM, rash Glucagon Very DVT/PEWeight loss
VIPoma Watery diarrhea, hypoKachlorhydria
VIP High Met. AcidosisHyperglycemiaHyperCaFlushing
Somatostatinoma
DMDiarrhea
Somatostatin
Very Weight loss
PPoma HepatomegPain
Pancreatic polypeptide
Very Watery diarrhea
Management of Primary Neuroendocrine Tumors
Glucagonoma
Management of Primary Neuroendocrine Tumors
Surgical Approach - PNET
• Locoregional disease– Radical resection– Enucleation (small,
localized lesions)
• Advanced disease– Cytoreduction– Optimal management
unclear
Management of Primary Neuroendocrine Tumors
Bloomston J GI Surg 2006
Management of Primary Neuroendocrine Tumors
Advanced PNET
Mortality: R2 > R0/1 (21% vs. 2%, p=0.009)
Bloomston J GI Surg 2006
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
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
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
O’Riordain World J Surg 1994
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
• PancreaticTumorClinic@OSUMC.edu
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 PrimariesDeveloped Symptoms
89 PrimariesRemained
Asymptomatic
Courtesy M. Bloomston, unpublished
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
Management of Primary Neuroendocrine Tumors
The Horizon is Here
• Minimally-invasive operations• Focal radiation• Ablation
Management of Primary Neuroendocrine Tumors
Intraoperative detectionSPECT/CT
Management of Primary Neuroendocrine Tumors
Robotic Distal Pancreatectomy/Splenectomy
Management of Primary Neuroendocrine Tumors
Video
Management of Primary Neuroendocrine Tumors
Robotic Distal Pancreatectomy/Splenectomy
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