Management of Primary Neuroendocrine Tumors Surgical Management of Primary Neuroendocrine Tumors...

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Management of Primary Neuroendocrine Tumors

Surgical Management of Primary Neuroendocrine Tumors

Carl R. Schmidt, MD, FACS

Objectives

• Discuss goals of surgical resection

• Management of GI primary tumors

• Management of pancreatic primary tumors (PNET)

Goals of Surgical Resection

• Cure

• Survival

• Palliation

• Carcinoid = cancer

• Five-year survival 67%

• Death often due to metastatic disease– Extent of disease workup– Multi-phase CT or MRI (liver mets)– Octreoscan

Survival

• Typically indolent

• Long-term survival common

• Management of symptoms is important

Palliation

• Hormone syndromes– Carcinoid syndrome usually liver metastases

• Jaundice

• Bowel obstruction

• Pain

GI Carcinoid Locations

• Distal small bowel (25-30%)

• Colon/appendix

• Rectum

• Stomach

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

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

Type III

• Gastric NEC• 4 cm well-diff• One + LN• No adjuvant

therapy• Cancer

surveillance

Small Bowel Primary

• Curative intent:– Bowel resection with regional LND– Multicentric (20-40%)– Consider prophylactic cholecystectomy

(gallstones associated with Octreotide)

• Palliation– Small bowel bypass

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)

Appendiceal Primary

• Incidence decreasing – ?less incidental

appendectomies

• Generally good prognosis– 5-30% localized– 86% 5-yr survival

Sandor Am J Gastro 1998

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

Rectal Primary

• Need colonoscopy and CT, consider EUS

• < 2 cm - transanal or EMR if possible

• > 2cm – LAR or APR

Pancreatic Primary (PNET)

• Family history – MEN1 (gastrinoma and insulinoma)

• 60% functional• 90% of non-functional

are malignant– Chromogranin A

(pancreastatin)– CT, MRI– Octreoscan?

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

Glucagonoma

Surgical Approach - PNET

• Locoregional disease– Radical resection– Enucleation (small,

localized lesions)

• Advanced disease– Cytoreduction– Optimal management

unclear

Bloomston J GI Surg 2006

Advanced PNET

Mortality: R2 > R0/1 (21% vs. 2%, p=0.009)

Bloomston J GI Surg 2006

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

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

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

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

Leave Primary Alone?

197 Patients Undergoing

100 Primary Tumors Intact at

97 Primary Tumors Not

Intact at TACE

7 Primaries Symptomatic at

93 Primaries Asymptomatic at

67 Primaries Symptomatic at

Resection

30 Primaries Asymptomatic at

Resection

4 PrimariesDeveloped Symptoms

89 PrimariesRemained

Asymptomatic

Courtesy M. Bloomston, unpublished

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

The Horizon is Here

• Minimally-invasive operations• Focal radiation• Ablation

Intraoperative detectionSPECT/CT

Robotic Distal Pancreatectomy/Splenectomy

Management of Primary Neuroendocrine Tumors Surgical Management of Primary Neuroendocrine Tumors...

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