Abdominal Neuroendocrine Tumors

Description:

This book aims to equip readers with a better understanding of neuroendocrine tumors of the abdomen and explains how to manage them optimally by making use of novel therapeutic options that represent major advances on previous treatments. The most recent advances in epidemiology, genetics, molecular biology, biomarkers, pathology, diagnostics, clinical assessment, medical therapy, and surgical treatment are presented. The information and data provided will stimulate readers to develop their personal opinions on significant issues and assist in decision making in individual patients during routine clinical practice. The book features a multidisciplinary approach and is designed to meet the needs of all physicians seeking comprehensive guidance from experts on the management of patients with neuroendocrine tumors. It will also be of value for researchers wishing to acquaint themselves with the state of the art in the field and emerging research avenues. Abdominal Neuroendocrine Tumors is published in Springer’s highly successful Updates in Surgery series, which now comprises more than 20 titles.

Table of Contents:

Part I Nosography

1 Neuroendocrine Tumors: a Nosologic Framework

1.1 Neuroendocrine Cells of the Digestive System

1.2 Tumors Arising from Neuroendocrine Cells

1.2.1 Tumors by Site

1.2.1.1 Esophagus

1.3 NET Epidemiology

1.4 NET Natural History

1.5 Mortality

References

2 Classification of Abdominal Neuroendocrine Tumors

2.1 Introduction

2.2 Grading

2.3 Staging

2.4 Conclusive Remarks

References

3 Hereditary Syndromes and Abdominal Neuroendocrine Tumors

3.1 Multiple Endocrine Neoplasia Type 1 (MEN1)

3.1.1 General Overview

3.1.2 Clinical Characteristics of MEN1-Associated Abdominal NETs

3.1.3 Diagnosis

3.1.4 Molecular Tumorigenesis

3.1.5 Pharmacological Therapy of MEN1-associated Abdominal NETs

3.1.6 Surgical Therapy of MEN1-associated Abdominal NETs

3.2 Multiple Endocrine Neoplasia Type 4 (MEN4)

3.2.1 General Overview

3.2.2 Clinical Characteristics of MEN4-associated Abdominal NETs

3.2.3 Diagnosis

3.2.4 Molecular Tumorigenesis

3.2.5 Pharmacological and Surgical Therapy of MEN4-associated Abdominal NETs

3.3 Von Hippel-Lindau Syndrome (VHL)

3.3.1 General Overview

3.3.2 Clinical Characteristics of VHL-associated Abdominal NETs

3.3.3 Diagnosis

3.3.4 Molecular Tumorigenesis

3.3.5 Pharmacological and Surgical Therapy of VHL-associated Abdominal NETs

3.4 Tuberous Sclerosis (TSC)

3.4.1 General Overview

3.4.2 Clinical Characteristics of TSC-associated Abdominal NETs

3.4.3 Diagnosis

3.4.4 Molecular Tumorigenesis

3.4.5 Pharmacological Therapy of TSC-associated Abdominal NETs

3.4.6 Surgical Therapy of TSC-associated Abdominal NETs

3.5 Neurofibromatosis Type 1 (NF1)

3.5.1 General Overview

3.5.2 Clinical Characteristics of NF1-associated Abdominal NETs

3.5.3 Diagnosis

3.5.4 Molecular Tumorigenesis

3.5.5 Pharmacological Therapy of NF1-associated Abdominal NETs

3.5.6 Surgical Therapy of NF1-associated Abdominal NETs

3.6 Mahvash disease

3.6.1 General Overview

3.6.2 Clinical Characteristics of MD-associated Abdominal NETs

3.6.3 Diagnosis

3.6.4 Molecular Tumorigenesis

3.6.5 Therapy

References

4 Sporadic Gastroenteropancreatic Neuroendocrine Tumors

4.1 Functioning GEP-NETs

4.1.1 Carcinoid

4.1.2 Gastrinoma

4.1.3 Insulinoma

4.1.4 Established Rare Functioning P-NETs (RFTs)

4.1.5 Possible Rare Functioning P-NET Syndromes

4.2 Non-Functioning GEP-NETs

References

Part II Diagnosis

5 Neuroendocrine Tumors Biomarkers

5.1 Non-Specific Biomarkers

5.2 Specific Biomarkers

5.2.1 Serotonin

5.2.2 Gastrin

5.2.3 Insulin

5.2.4 Glucagon

5.2.5 Somatostatin (SS)

5.2.6 Vasoactive Intestinal Peptide (VIP)

References

6 Endoscopic Diagnosis of Gastrointestinal and Pancreatic Neuroendocrine Tumors

6.1 Introduction

6.2 Neuroendocrine Tumors of the Gastrointestinal Wall

6.2.1 Esophagus

6.2.2 Stomach

6.2.3 Duodenum

6.2.4 Small Intestine

6.2.5 Colon and Rectum

6.3 Pancreatic NETs

6.4 Conclusions

References

7 Role of Non-Functional Imaging in the Diagnosis of Abdominal Neuroendocrine Tumors

7.1 Introduction

7.2 Pancreatic Neuroendocrine Tumors

7.2.1 Transabdominal Ultrasound

7.2.2 Computed Tomography

7.2.3 Magnetic Resonance Imaging

7.2.4 Endoscopic Ultrasound

7.2.5 Angiography

7.3 Extrapancreatic Neuroendocrine Tumors

7.3.1 Transabdominal Ultrasound

7.3.2 Computed Tomography

7.3.3 Magnetic Resonance Imaging

7.3.4 Endoscopic Ultrasound

7.4 Imaging of Metastatic Disease

References

8 Role of Functional Imaging in the Diagnosis of Neuroendocrine Tumors

8.1 Background

8.2 Acquisition Techniques

8.3 18F-DOPA

8.4 123/131I-MIBG

8.4.1 131I-MIBG Therapy

8.5 18F-FDG PET

8.6 5-Hydroxytryptophan (11C-5HTP)

8.7 111In-Octreoscan

8.8 Somatostatin Receptor PET and Post-PRRT Imaging

References

9 Pathological Analysis of Abdominal Neuroendocrine Tumors

9.1 Introduction

9.2 Fine-needle Aspiration Cytology

9.3 Small Biopsy

9.4 Surgical Specimens

9.4.1 Gastric NETs

9.4.2 Pancreatic NETs

9.4.3 Small Bowel NETs

9.4.4 Appendix NETs

9.4.5 Large Bowel NETs

9.5 Conclusive Remarks

References

Part III Management

10 Management of Gastric Neuroendocrine Tumors

10.1 Type 1 G-NETs

10.1.1 Endoscopy

10.1.2 Surgery

10.2 Type 2 G-NETs

10.2.1 Surgery and Endoscopy

10.2.2 Medical Treatments

10.3 Type 3 G-NETs

10.3.1 Surgery

10.3.2 Medical Treatments

10.4 Type 4 G-NETs

10.4.1 Surgery

10.4.2 Medical Treatments

References

11 Management of Pancreatic and Duodenal Neuroendocrine Tumors

11.1 Surgical Treatments for Pancreatic NeuroendocrineTumors

11.1.1 Surgery for Functioning P-NETs

11.1.2 Surgery for Non-Functioning P-NETs

11.1.3 Conservative Treatment for Non-Functioning P-NETs

11.1.4 Surgery in Advanced-Disease Scenarios

11.1.5 Minimally Invasive Approach

11.2 Surgery for Duodenal Neuroendocrine Tumors

11.2.1 Endoscopic Treatment

11.2.2 Surgical Treatment

11.3 Ampullary Neuroendocrine Tumors

11.4 Medical Treatments

11.4.1 The Role of Chemotherapy

11.4.2 Targeted Therapies

11.4.3 The Role of Peptide Receptor Radionuclide Therapy (PRRT)

References

12 Management of Ileal, Appendiceal and Colorectal Neuroendocrine Tumors

12.1 Ileal Neuroendocrine Tumors

12.1.1 Surgery

12.1.2 Medical Treatments

12.1.3 Chemotherapy

12.1.4 Peptide Receptor Radionuclide Therapy

12.2 Appendiceal Neuroendocrine Tumors

12.2.1 Surgery

12.2.2 Medical Treatment

12.2.3 Chemotherapy

12.3 Colorectal Neuroendocrine Tumors

12.3.1 Surgery

12.3.2 Medical Treatment

12.3.3 Chemotherapy

References

13 Management of Liver Metastases from Gastroenteropancreatic Neuroendocrine Tumors

13.1 Hepatic Resections

13.1.1 Indications, Techniques and Results

13.1.2 Synchronous Liver Metastases

13.2 Neoadjuvant and Adjuvant Treatments

13.3 Liver Transplantation

13.4 Locoregional Treatments

13.4.1 Peptide Receptor Radionuclide Therapy (PRRT)

13.4.2 Radioembolization with 90Y-Spheres

13.4.3 Hepatic Artery Embolization and TransarterialChemoembolization

13.4.4 Radiofrequency Ablation

13.5 Systemic Therapy

13.5.1 Somatostatin Analogs

13.5.2 Chemotherapy

13.5.3 Interferon

13.5.4 Targeted Therapy