Endocrine Surgery (eBook)

Johnathan G H Hubbard, MD, FRCS, Consultant Endocrine Surgeon & Clinical Lead Endocrine Surgery, Guys & St Thomas’ NHS Foundation Trust. He is a member of numerous societies, such as International Association Endocrine Surgeons, International Society of Surgery, European Society of Endocrine Surgeons, British Association of Endocrine Surgeons (No 204246), Francophone Association of Endocrine Surgery, Fellow Association of Surgeons of Great Britain and Ireland, Fellow Royal Society of Medicine, BMA (No 7372188)William B. Inabnet, III, MD, Co-Director, New York Thyroid Center, Columbia University Medical Center, New York, NY, USA; Associate Professor of Clinical Surgery, Columbia University College of Physicians & Surgeons, New York, NY; Chief, Endocrine Surgery Section, NewYork-Presbyterian Hospital/Columbia University Medical Center, New York, NY, USA. Fellow American College of Surgeons, Association for Academic Surgery, American Society of General Surgeons, French Surgical Association, American Association of Tissue Banks, American Association of Endocrine Surgeons, American Thyroid Association, Society of American Gastrointestinal Endoscopic Surgeons, Association Francophone de Chirurgie Endocrinienne, Am Society for Bariatric Surgery, International Federation for the Surgery of Obesity, International Association of Endocrine Surgeons, International Society of Surgery, Society for Surgery of the Alimentary Tract, New York Surgical Society, Society of Laparoendoscopic Surgeons, European Society of Endocrine Surgeons. He’s Editorial Board member of the Thyroid journal and guest editor of Surgery, SOAR, World J Surg, Surg Endoscopy, Annals of Surgery journals.Chung-Yau Lo, FRCS, FACS, Chief, Division of Endocrine Surgery, Clinical Professor & Honorary Consultant, Department of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Pokfulam, Hong Kong and Clinical Professor, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Hong Kong. He is on the editorial board of Editorial Board, Asian Journal of Surgery (1 February, 2002 to present) , Chinese Journal of Medicine (Jan 2006), on the International Advisory Board of Chinese Journal of Endocrine Surgery (From 2002), and Associate Editor of Asian Journal of Surgery (since 2005)

Foreword 7
Preface 8
Contents 9
Contributors 13
Section 1 Thyroid 19
Thyroid Embryology, Anatomy, and Physiology: A Review for the Surgeon 20
Thyroid Organogenesis and Anatomy 20
Thyroid Embryogenesis 20
Developmental Abnormalities of the Thyroid 22
Athyreosis 22
Hypoplasia 22
Hemiagenesis 23
Ectopic Thyroid 23
Abnormalities of Thyroid Migration 23
Anatomy of the Thyroid Gland and Related Structures 23
Thyroid Gland 24
Recurrent Laryngeal and Superior Laryngeal Innervation of the Cricothyroid 24
Parathyroid Glands 26
Thyroid Physiology 26
Iodide Metabolism 26
Thyroid Hormone Synthesis and Release 27
Peripheral Transport of Thyroid Hormone 28
Thyroid Hormone Metabolism and Action on Target Cells 29
Regulation of Thyroid Hormone Production 30
Calcitonin Physiology 30
References 31
The Assessment of Thyroid Nodules 34
Introduction 34
The Rapidly Growing Nodule 35
Definition of a Thyroid Nodule 36
History and Clinical Examination 36
Nodule Size and Morphology 36
Thyroid Function 38
Fine Needle Aspiration 38
Freehand and USG FNA 39
Ultrasound in Malignant and Benign Nodules 39
CT and MRI 40
Nuclear Medicine 40
The Role of Calcitonin Screening 41
Thyroid Cancer Gene Expression Profiling and Proteomics 42
2-[18F] fluoro-2-deoxy-d-glucose-Positron Emission Tomography (18) 42
Relationship of Thyroid Nodules with Other Conditions, e.g., Systemic Cancer, Lymphoma, or Septic Diseases 42
Specific Situations 43
Nodules Presenting in Pregnancy 43
Nodules Presenting in Childhood and Adolescence 43
Thyroid Nodules in Secondary Hyperparathyroidism 43
The Nodule Detected on Whole-Body 18F-FDG-PET-CT 43
References 43
Thyroid: Fine-Needle Aspiration Biopsy 46
Introduction 46
Indication and Goal of Thyroid FNA 46
Contraindications and Complications of Thyroid FNA 47
Technical Aspects of Thyroid FNA 47
Selection of Technique 47
Specimen Procurement 48
Specimen Preparation and Staining 49
Immunocytochemistry and Other Ancillary Techniques 50
Immunocytochemistry and Flow Cytometry 50
DNA and Molecular Techniques 50
Specimen Adequacy 51
Cytodiagnosis and Diagnostic Categories 52
Nondiagnostic or Inadequate FNA Specimen (Thy1) 52
Benign or Nonneoplastic Lesions (Thy2) 53
Benign Colloid Nodule or Multinodular Goiter 53
Cysts and Cystic Goiter 54
Thyroiditis 54
Other Benign Lesions 55
Follicular Lesions (Thy3) 55
Cellular Microfollicular Lesions 55
Hurthle Cell Lesion 56
Suspicious of Malignancy (Thy4) 57
Malignant Lesions (Thy5) 57
Papillary Carcinoma 57
Poorly Differentiated Follicular Carcinoma 58
Medullary Carcinoma 58
Anaplastic Carcinoma 59
Lymphoma 60
Secondary Tumors 60
Diagnostic Accuracy and Errors 60
Large-Needle Aspiration Biopsy and Core Needle Biopsy 61
Recommendations for Thyroid FNA Reporting 61
Multidisciplinary Meetings and Quality Assurance 62
References 62
Thyroid Imaging 65
Introduction to Thyroid Imaging 65
Ultrasound and Thyroid Imaging 65
Basics of Ultrasound 65
Set up of office-Based Ultrasound 66
Reporting and Communication of Thyroid Ultrasound 66
Standard Evaluation of the Thyroid by Ultrasound 66
Ultrasound Evaluation of Thyroid Nodules 67
Ultrasound Evaluation for Thyroid Goiter 69
Ultrasound Evaluation of Diffuse Diseases of the Thyroid 70
Ultrasound Evaluation of Thyroid Cysts 71
Ultrasound-Guided Fine-Needle Aspiration Biopsy 71
Ultrasound Evaluation of Thyroid Cancer 71
Ultrasound Evaluation for Thyroid Cancer Recurrence 72
Single Photon Nuclear Medicine Imaging 73
Commonly Used Radionuclides 73
Less Commonly Used Radionuclides 74
Uptake and Thyroid Scintigraphy 74
Thyroid Scintigraphy in Patients with Hyperthyroidism 75
Thyroid Scanning in Patients with a Substernal Goiter 76
Whole-Body Scintigraphy in Patients with Thyroid Cancer 76
Diagnostic Whole-Body Scanning and Thyrogen Scanning 76
PostTreatment Whole-Body Scans 78
Use of Thyroid Scintigraphy in Congenital Thyroid Disorders 78
Positron Emission Tomography 78
Computed Tomography Scan and Thyroid Imaging 79
References 81
Multinodular Goiter 85
Introduction 85
Embryology and Surgical Anatomy of Thyroid Gland 86
Clinical Presentation of Multinodular Goiter 88
Diagnostic Modalities 89
Therapeutic Options 89
Substernal Goiter 90
Classification of Substernal Goiter 91
Operative Techniques 95
Anterior Substernal Goiter 95
Posterior Mediastinal Goiter 96
References 97
Thyrotoxicosis and Thyroiditis: Causes, Investigation, and Management 100
Introduction 100
Clinical Presentation and Systemic Manifestation of Thyrotoxicosis 100
Eye Manifestations of Thyrotoxicosis 100
Thyroid Gland 100
Laboratory Diagnosis of Thyrotoxicosis 101
Antithyroid Antibody 102
Nuclear Medicine Imaging (Thyroid Scintigraphy) 102
Graves’ Disease 102
Pathogenesis 102
Diagnosis 103
Thyroid Eye Disease (Thyroid Ophthalmopathy, Graves’ Ophthalmopathy) 103
Identifying the Etiology of Thyrotoxicosis 104
Treatment of Thyrotoxicosis 105
Antithyroid Drugs 105
Treatment Strategies 105
Side Effects 105
Radioiodine Therapy 105
Surgical Treatment 106
Preparation for Surgery 106
Subtotal Thyroidectomy 106
Thyroid Remnant Size 106
Surgical Objectives 107
Total Thyroidectomy 107
Thyroid Storm 107
Toxic Multinodular Goiter and Toxic Adenoma (Plummer’s Disease) 108
Amiodarone and Thyrotoxicosis 108
Thyroiditis 108
References 109
Molecular Biology of Thyroid Cancer 111
Introduction 111
Common Genetic Changes Found in Thyroid Cancer 111
Medullary Thyroid Cancer 111
RET 111
Thyroid Cancer of Follicular Cell Origin 113
RAS 113
RET/PTC Rearrangement 114
BRAF 115
NTRK Rearrangement 117
PAX8/PPARgamma Fusion 117
P53 117
PTEN 117
Others Molecular Factors Involved in Thyroid Cancer of Follicular Cell Origin 117
Epigenetic Changes in Thyroid Cancer 118
DNA Methylation and Thyroid Tumorigenesis 118
Other Epigenetic Mechanisms in Thyroid Tumorigenesis 119
Summary 120
References 120
Well-Differentiated Thyroid Cancer: An Overview and the Chernobyl Effect 125
Introduction 125
Risk Factors 125
Symptoms 126
Diagnosis 127
Papillary Carcinoma of Thyroid 127
Follicular Carcinoma and Hurthle Cell Carcinoma 128
Operative Management 129
Overall Prognosis 129
Differentiated Thyroid Cancer: The Chernobyl Effect 129
References 132
Poorly Differentiated and Undifferentiated Thyroid Cancer 135
Introduction 135
Poorly Differentiated Thyroid Cancer 135
Insular Carcinoma 136
Pathogenesis of Poorly Differentiated Thyroid Cancer 137
Management and Outcomes of Poorly Differentiated Thyroid Cancers 137
Undifferentiated (Anaplastic) Thyroid Cancer 138
Clinical Features 138
Pathology 139
Assessment and Evaluation 141
Management Strategies 143
Clinical Scenario 1: Incidental Finding of UTC 143
Clinical Scenario 2: Extensive Locoregional Disease 144
Clinical Scenario 3: Distant Metastatic Disease 145
Scenario 4: Acute Airway Compromise 146
Outcomes and Prognosis 147
References 148
Postoperative Management of Well-Differentiated Thyroid Cancer 151
Introduction 151
Initial Risk Stratification 151
Initial Postoperative Visit 153
Goals of Initial Therapy 154
Extent of Initial Thyroid Surgery 155
Radioactive Iodine Remnant Ablation 156
TSH Suppression 157
External Beam Irradiation 157
Systemic Therapy for Distant Metastases 158
Strategy for Detecting Persistent/Recurrent Disease 158
Assessing Response to Therapy 159
Conclusion 160
References 161
Medullary Thyroid Cancer 163
Introduction 163
Diagnosis 164
Serum Markers 165
Associated Conditions 166
Genetic Testing 167
Prognosis 168
Prophylactic Surgery 169
Clinically Evident Disease 170
Postop Surveillance 171
Persistent/Recurrent Disease 172
Radiation Therapy 173
Systemic Therapy 173
Future Therapies 174
References 175
Technique of Thyroidectomy 177
Introduction 177
Isthmolobectomy 177
Skin Incision 178
Strap Muscles 178
Upper Pole 179
Lower Pole 179
Lateral Dissection 179
Parathyroid Autotransplantation 180
Wound Closure 181
Reoperative Thyroid Surgery 181
Substernal Goiter 181
Local or Regional Anesthesia 182
Intraoperative Neuromonitoring 182
Minimally Invasive Thyroidectomy 182
Complete Endoscopic Thyroidectomy 183
Gagner Technique 183
Cougard Technique 183
Video-Assisted Technique (MIVAT) 183
Lateral Approach: Henry Technique 183
Central Approach: Miccoli Technique 184
References 184
Lymph Node Dissection in Thyroid Cancer 186
Introduction 186
Locoregional Lymph Nodes: Surgical Anatomy and Classification Systems 186
Indications for Lymph Node Dissection 189
Papillary Thyroid Carcinoma 189
Follicular Thyroid Carcinoma 190
Poorly Differentiated Thyroid Carcinoma 190
Undifferentiated (Anaplastic) Thyroid Carcinoma 191
Medullary Thyroid Carcinoma 191
Surgical Techniques of Lymph Node Dissection 194
Sentinel Node Technique 194
Focused Approach 194
Regional Lymph Node Dissection Including Excision of Single Nodes (‘‘Berry Picking’’) 194
Compartment-Oriented Microdissection 194
Central Neck Compartment 195
Lateral Neck Compartment 195
Mediastinal Compartment 195
Surgical Concept 196
Papillary Carcinoma 197
Follicular Carcinoma 197
Medullary Thyroid Carcinoma 197
Conclusion 199
References 200
Management of the Laryngeal Nerves and Voice 207
Introduction 207
Anatomy of the Larynx and Laryngeal Nerves 207
Voice Production 208
Importance of Preoperative Laryngoscopy 209
Nerve Monitoring in Thyroid and Parathyroid Surgery 210
Troubleshooting 211
Advantages of Nerve Monitoring 212
Improved RLN Identification and Dissection 212
Prognostic Function 212
Monitoring the External Branch 213
Surgical Maneuvers to Avoid Injury to EBSLN 214
Intraoperative Injury to the SLN 214
Surgical Maneuvers to Avoid Injury to RLN 214
Routine Identification 215
Nerve Injury 215
Traction Injury 217
Crush Injury 217
Suture Impingement 217
Cautery Injury 217
Intraoperative Resection/Transection 217
Recovery 218
Voice Changes with Thyroid and Parathyroid Surgery 219
Neural Injury 219
Non-Neural Laryngeal Injury 219
Voice Evaluation 220
Laryngoscopy 220
Stroboscopy 220
Laryngeal Electromyography 220
References 221
Section II Parathyroid 224
Embryology, Anatomy, and Physiology of the Parathyroid Glands 225
Introduction 225
Embryology 225
Inferior Parathyroid 225
The Superior Parathyroid 225
Surgical Aspects 226
Calcium Physiology 226
Parathyroid Hormone 228
Vitamin D 229
The Calcium Sensing Receptor 229
Calcitonin 229
Drug Modulators in Parathyroid Disease 230
References 230
Presentation and Diagnosis of Primary Hyperparathyroidism 231
Introduction 231
Clinical Features 232
‘‘Asymptomatic’’ Primary Hyperparathyroidism 232
Symptomatic Primary Hyperparathyroidism 234
Hypercalcemic Crisis 235
Normocalcemic Hyperparathyroidism 237
Differential Diagnosis of Hypercalcemia 238
Familial Hypocalciuric Hypercalcemia 239
Investigation of Severity Primary Hyperparathyroidism 239
Conclusions 242
References 242
Parathyroid Localization and Imaging 245
Introduction 245
Preoperative Localization Tests 245
Noninvasive Tests 245
Ultrasonography 245
Parathyroid Scintigraphy 247
Single-Photon Emission Tomography 249
Computed Tomography 252
Magnetic Resonance Imaging 254
Positron Emission Tomography 254
Invasive Tests 254
Selective Venous Sampling 254
Selective Angiography 255
Fine Needle Aspiration 255
Intraoperative Tests 255
The Parathyroid Surgeon 255
Methylene Blue Staining 255
Intraoperative US 255
Radio-Guided Parathyroid Surgery 256
Intraoperative SVS for QPTH Measurement 256
Indications for Localization Tests 256
Initial Bilateral Cervical Exploration 256
Secondary/Tertiary HPT 257
Minimally Invasive Parathyroidectomy 257
Persistent or Recurrent HPT 258
Conclusion 259
References 260
Intraoperative PTH Monitoring 263
Introduction 263
History 263
Which Patients Benefit from Intraoperative PTH Monitoring? 264
Sporadic Primary Hyperparathyroidism 264
How does Intraoperative PTH Monitoring Guide Parathyroidectomy? 265
Protocol for Blood Sampling 265
Calculation of IPM Accuracy Using the ‘‘> 50% PTH Drop’’ Criterion
Which IPM Criteria Should be Used? 267
Additional Uses and Advantages of the Intraoperative PTH Assay 269
Differential Internal Jugular Venous Sampling 269
Biochemical Fine-Needle Aspiration 270
Decreases in Operative Time and Costs 270
Improves Operative Success 270
Recognizes Abnormal Glands More Accurately than Histopathology 270
Limitations of Intraoperative PTH Monitoring 270
IPM and ‘‘> 50% PTH Drop’’ Criterion Does not Predict the Size of the Remaining Normally Secreting Parathyroid Glands
IPM Does Not Predict PTH Levels in Postoperative Eucalcemic Patients 271
IPM Does Not Assure Hypersecretion of the First Gland Excised in MGD 271
IPM Does Not Predict Late Recurrence 271
IPM Does Not Guarantee Operative Success 271
The Cost of Intraoperative PTH Assays 271
Isolated Familial Hyperparathyroidism or Non-MEN Familial Hyperparathyroidism 271
Secondary Hyperparathyroidism 272
Tertiary Hyperparathyroidism 272
Parathyroid Cancer 273
Multiple Endocrine Neoplasia 273
Summary 273
References 274
Focused Parathyroidectomy 276
Background 276
Evolution of Surgery for pHPT 276
Open Unilateral Neck Exploration (Original approach) 276
Preoperative Localization 277
Intraoperative Measurement of PTH 278
Methylene Blue Dye 279
Radioguidance 280
Local/Regional Anesthesia 280
Focused/Minimal Invasive Parathyroidectomy 280
Overview 280
Open-Focused/Minimal Invasive procedures 281
Endoscopic/Video-Assisted Procedures 281
Evidence-Based Medicine in Parathyroid Surgery 282
Conclusion and Future Aspects 284
References 284
Parathyroid: Bilateral Neck Exploration 288
Introduction 288
Bilateral Neck Exploration: What are we Talking About? 288
Bilateral Versus Unilateral Neck Exploration: The Evidence 290
Systematic Review 290
Prospective, Quasi-Experimental Studies 290
Experimental Studies (Randomized Controlled Trials) 293
Bilateral Versus Unilateral Neck Exploration: In Practice 295
Possibility of Multiglandular Disease - Prevalence 295
Possibility of Multiglandular Disease - Clinical Characteristics 296
Conc lusions 297
References 297
Reoperative Parathyroid Surgery 299
Introduction 299
History 299
Definition 299
The Extent of the Problem 300
Why Does an Initial Operation Fail? (Table 21.1) 300
Reasons for Failure 301
Care of the Patient with Failed Initial Surgery 303
Parathyroid Gland Localization in Recurrent/Persistent HPT 305
Preoperative 305
Intraoperative Localization 308
Reoperative Surgery 308
Operative Strategy 308
Positive Localization 308
Negative preoperative Localization 309
Hyperfunctioning Parathyroid Autograft 309
Parathyromatosis 309
Angiographic Ablation 309
Summary 310
References 310
Management of Secondary and Tertiary Hyperparathyroidism 315
Introduction 315
Pathogenesis 315
Before Kidney Transplantation 315
After Kidney Transplantation 316
Clinical Manifestations 316
Skeletal Disease 316
Diagnosis of Uremic Osteodystrophy 316
Extraskeletal Disease (Tissue Calcification) 316
Cardiovascular Problems 317
Metastatic Pulmonary Calcification 318
Pruritus 318
Calciphylaxis 318
Sexual Dysfunction 319
Anemia 319
After Kidney Transplantation 319
Management 320
Medical Treatment 320
Surgical Treatment 320
Prerenal Transplantation 320
Posttransplantation 321
Preoperative Care 321
Medication and Preoperative Image Studies 321
Localization 321
Surgical Management 322
Type of Surgical Procedure 322
Subtotal Parathyroidectomy (SPTX) 322
Total Parathyroidectomy and Autotransplantation (TPTX+AT) 322
Surgical Intervention for tHPT 323
Applications of the Intraoperative PTH Assay 323
Other Invasive Treatment 323
Intraparathyroid Injection of Alcohol or Vitamin D 323
Complications of Parathyroidectomy 323
Transient Hypocalcemia 324
Permanent Hypocalcemia (hypoparathyroidism) 324
Persistent and Recurrence Hyperparathyroidism 324
Clinical Course after Successful Parathyroidectomy 324
Bone Disease (Osteoporosis) 324
Anemia 325
Cardiovascular Condition 325
Summary 325
References 325
Parathyroid Carcinoma 329
Introduction 329
Incidence 329
Etiology 329
Molecular Pathogenesis 330
Clinical and Laboratories Features 332
Pathologic Features 333
Natural History 335
Management and Prognosis 335
Surgery 335
Chemotherapy 336
Radiotherapy 337
Management of Hypercalcemia 337
Prognosis 338
References 338
Section III Adrenal 342
Adrenal Embryology, Anatomy, and Physiology 343
Embryology 343
Anatomy 344
Adrenal Physiology 346
Aldosterone 346
Cortisol 346
Sex Steroids 347
The Adrenal Medulla 348
References 348
Adrenal Imaging 349
Introduction 349
Adrenal Anatomy 349
Computed Tomography 349
CT Overview 349
CT Characteristics of Benign Adenomas 350
CT Characteristics of ACCs 350
CT Characteristics of Functioning Tumors 351
Pheochromocytoma 351
Retroperitoneal Brown Fat 352
Aldosteronoma 352
Cushing’s Syndrome 352
CT Characteristics of Nonfunctioning Tumors 353
Cysts 353
Myelolipoma 353
Magnetic Resonance Imaging 354
MRI Overview 354
Technique of MRI 354
T1- Versus T2-Weighted Images 354
Gadolinium Enhancement 355
MRI Characteristics of Benign Tumors 355
MRI Characteristics of ACC 355
MRI Characteristics of Pheochromocytoma 356
Nuclear Medicine 356
Nuclear Medicine Overview 356
Adrenocortical Scintigraphy 357
Adrenomedullary Scintigraphy 358
Positron Emission Tomography 360
Role of Nuclear Medicine in Incidentalomas 362
References 362
Adrenal Venous Sampling 364
Introduction 364
Anatomy of Adrenal Venous Drainage 364
Indications for AVS 364
Primary Hyperaldosteronism 365
Incidence of Hyperaldosteronism in Unselected Hypertensive Patients 365
Diagnostic Tests Performed Before Proceeding to AVS 366
Adrenal Venous Sampling 366
Protocol for AVS 367
Criteria for Positive Localization 367
Complications 368
References 368
Primary Hyperaldosteronism 370
Introduction 370
Physiology 370
General Considerations 370
Epidemiology 372
Pathologic Features 372
Clinical Characteristics 373
Diagnosis 373
Biochemical Diagnosis 373
Differentiating Unilateral and Bilateral Disease 374
Localization 374
Treatment 376
Surgical 376
Medical 377
Postoperative Outcomes 377
Conclusion 377
References 378
Cushing’s Disease and Syndrome 383
Introduction 383
Epidemiology and Etiology 383
Clinical Features 384
Investigations 385
Biochemical Evaluations 385
Evaluation of the Etiological Causes 386
Localization 388
Management 388
Medications 388
Surgery 389
Cushing’s Disease 389
Ectopic ACTH-Producing Tumors 391
Adrenal Causes 391
Conclusions 392
References 392
Pheochromocytoma and Paraganglioma 395
Pearls 395
Background 395
Clinical Presentation 396
Diagnosis 396
Localization 397
Preoperative Management 399
Intraoperative Management 399
Operative Techniques 400
Anterior Transperitoneal Laparoscopic Approach 400
Anterior Open Approach 400
Posterior Retroperitoneoscopic Approach 401
Postoperative Management 401
Mayo Clinic Rochester Experience 402
Pathology 402
Pheochromocytoma in Pregnancy 403
Pheochromocytoma in Children 405
Genetic Syndromes 405
MEN Type 2 Syndrome 405
Von Hippel Lindau Disease 405
Succinated Dehydrogenase Mutations (SDHB, SDHC, SDHD) 406
Associated Conditions 406
Summary 406
References 407
Adrenocortical Carcinoma 409
Introduction 409
Molecular Pathogenesis 409
Clinical and Hormonal Presentation 409
Staging 410
Imaging 410
Adrenal Biopsy 412
Pathological Assessment 412
Treatment and Outcome 412
Surgery 412
Laparoscopic surgery 413
Recurrence 414
Adjuvant Therapy 414
Mitotane 414
Future Prospects 416
Conclusion 416
References 417
Incidentaloma 418
Introduction 418
Frequency 418
Pathology 419
The Goal of Evaluation 419
Screening for Subclinical Cushing’s Syndrome 421
Screening for ‘‘Subclinical Pheochromocytoma’’ 421
Screening for ‘‘Subclinical Primary Aldosteronism’’ 422
Screening for Adrenal Carcinoma 422
Size of Tumor 422
Imaging 423
Fine-Needle Aspiration 423
Genetic and Molecular Biology Studies 424
Management of Adrenal Incidentalomas: Surgery Versus Follow-Up 425
What is the Best Surgical Approach in the Management of Adrenal Incidentalomas? 426
References 427
Adrenal Metastases and Rare Adrenal Tumors 430
Introduction 430
Biochemical Evaluation 431
Imaging Adrenal Metastases 432
Computerized Tomography Scan 432
Magnetic Resonance Imaging 433
Positron Emission Tomography 433
Percutaneous Biopsy 434
Management of Adrenal Metastases 434
Chap32Sec9 436
Technique of Adrenalectomy 436
Ablation of Adrenal Metastases 436
Rare Adrenal Tumors 437
Adrenal Hemorrhage 437
Adrenal Cysts 437
Lymphangioma 438
Pseudocysts 438
Parasitic Infections 438
Myolipomas 438
References 438
Technique of Open and Laparoscopic Adrenalectomy 442
Introduction 442
Preoperative Preparation 442
Pheochromocytoma 442
Aldosteronoma 442
Cushing’s Syndrome 443
Operative Approach 443
Anatomic and Technical Considerations 443
Intraoperative Considerations 444
Technique of Laparoscopic Adrenalectomy 444
Laparoscopic Transabdominal (Transperitoneal) Adrenalectomy 444
Patient, Equipment, and Surgeon Position 444
Equipment 445
Right Adrenalectomy 446
Left Adrenalectomy 447
Laparoscopic Posterior (Retroperitoneal) Adrenalectomy 447
Patient, Equipment, and Surgeon Position 448
Equipment 448
Adrenalectomy 448
Technique of Open Adrenalectomy 449
Anterior (Transabdominal) and Thoracoabdominal Approaches 449
Patient and Surgeon Position 449
Equipment 449
Incision 449
Right Adrenalectomy 449
Left Adrenalectomy 450
Closure 450
Flank and Posterior (Retroperitoneal) Approach 451
Postoperative Care 451
Pheochromocytoma 451
Aldosteronoma 451
Cushing’s Syndrome 451
Summary 451
References 452
Laparoscopic Retroperitoneal Adrenalectomy 453
Introduction 453
Benefits of Retroperitoneal Adrenalectomy (Compared to Transabdominal Adrenalectomy) 453
Reduced Operating Times 453
Avoidance of Intraabdominal Adhesions and Irradiated Fields 454
Potential Decrease in Postoperative Pain and Incisional Hernia 454
Improved Hemostasis 454
Disadvantages of Retroperitoneal Adrenalectomy (Compared with Transabdominal Adrenalectomy) 454
Indications and Contraindications 455
Technique 455
Patient Positioning 455
Port Placement 456
Dissection of the Retroperitoneal Space 456
Identification and Ligation of the Adrenal Vein 457
Right Adrenalectomy (Fig. 34.2) 457
Left Adrenalectomy (Fig. 34.3) 457
Removal of the Adrenal Gland 457
Postoperative Care 457
Outcomes 457
Conclusion 458
References 458
Section IV Pancreas 459
Pancreas: Embryology, Anatomy, and Physiology 460
Embryology of the Pancreas 460
Developmental Anomalies of the Pancreas 462
Anatomy of the Pancreas 462
Peritoneal Attachments 463
Pancreatic Ducts 463
Arterial Supply of the Pancreas 464
Venous Supply 465
Lymphatic Drainage 465
Innervation of the Pancreas 465
Physiology of the Pancreas 466
Beta Cells 466
Alpha Cells 467
Delta Cells 468
PP Cells 468
Autonomic Innervation of Islet Cells 468
Parasympathetic Innervation 468
Sympathetic Innervation 469
Neuropeptides Involved in Autonomic Regulation of Islet Cells 469
References 469
Pancreatic Imaging: The Value for Surgery of Neuroendocrine Pancreatic Tumors 471
Introduction 471
Size and Malignancy 472
Radiological Imaging Techniques 472
Transabdominal Ultrasonography 472
Endoscopic US 472
Tumor Characteristics at EUS 474
Performance of EUS in Different Tumor Types 475
Biopsy Using EUS 476
Intraoperative US 476
Intraoperative Endoscopic US 477
Computed Tomography 477
Performance of CT in Different Tumor Types 477
Magnetic Resonance Imaging 481
Digital Subtraction Angiography 483
Arterial Stimulation with Venous Sampling 483
Transhepatic Peripancreatic Venous Sampling 484
Radionuclide Imaging 484
SST Receptor Scintigraphy 484
Positron Emission Tomography 485
The Value of Imaging of PNETs for the Endocrine Surgeon 486
References 488
Diagnosis and Management of Hyperinsulinemic Hypoglycemia 492
Introduction 492
Whipple’s Triad 492
Hypoglycemic Disorders - Classification and Causes 493
Causes of Endogenous Hyperinsulinemic Hypoglycemia 493
Insulinoma 493
Noninsulinoma Pancreatogenous Hypoglycemia Syndrome 493
Insulin Autoantibody Hypoglycemia 495
Diagnostic Tests Used in the Evaluation of Suspected Hypoglycemic Disorders 495
The 72-H Fast 495
Mixed Meal Test 496
Insulin Antibodies 496
Localization Procedures 496
Transabdominal Ultrasonography 497
Endoscopic Ultrasonography 497
Intraoperative Ultrasonography 497
Computed Tomography 497
Arteriography 497
Selective Arterial Calcium Injection 497
Management 498
Management of Endogenous Hyperinsulinism - A Surgeon’s Perspective 500
Results of Pancreatic Surgery for NIPHS 502
Insulinomas and the MEN1 Pancreas 502
Malignant Insulinomas 502
Management of Intraoperative Complications 503
Conclusion 503
References 503
Gastrinoma 506
Introduction 506
Diagnosis 507
Clinical Symptoms 507
Differential Diagnosis 507
Localization 508
Conventional Imaging Techniques 508
Specific Localization Tests 508
SASI Test 508
Somatostatin Receptor Scintigraphy 510
Intraoperative Techniques 510
Surgical Treatment 511
Surgery and Pathological Characteristics of Gastrinoma 511
Surgery for Sporadic Gastrinoma 515
Pancreatic Gastrinoma 515
Duodenal Gastrinoma 515
Surgery for Gastrinoma in MEN 1 515
Duodenal Gastrinoma in MEN 1 515
Pancreatic Neuroendocrine Tumors in MEN 1 517
Surgery for Ectopic Gastrinoma 517
Laparoscopic Surgery for Gastrinoma 517
Chemotherapy and Somatostatin Analogue 517
References 518
Rare Functioning Pancreatic Endocrine Tumors 521
Introduction 521
Glucagonoma 521
Epidemiology 521
Clinical Features and Investigation 521
Vasoactive Intestinal Polypeptide-Secreting Tumor 523
Epidemiology 523
Clinical Features and Investigation 523
Somatostatinoma 524
Epidemiology 524
Clinical Features and Investigation 524
Other Rare Pancreatic Endocrine Tumors 524
ACTHoma 524
PTHrPoma 524
GRFoma 525
Neurotensinoma 525
Association with Multiple Endocrine Neoplasia-1 Syndrome 525
Management 525
Imaging 525
Computed Tomography or Magnetic Resonance Imaging 525
Nuclear Medicine 526
Other Imaging Modalities 526
Clinicopathologic Classification 526
Treatment 526
Surgical Intervention 526
Hepatic Artery Embolization and Chemoembolization 527
Radiofrequency Ablation 528
Radionuclide Therapy 528
Somatostatin Analogues 528
Alpha-Interferon 528
Circulating Tumor Markers 528
Summary 529
References 529
Laparoscopic Radiofrequency Ablation of Metastatic Neuroendocrine Tumors in the Liver 531
Introduction 531
Surgical Anatomy 531
Preoperative Considerations 532
Imaging 532
Tumor Volume 532
Location of Lesions 532
Patient Preparation 532
Operating Room Setup 532
Positioning 532
Thermo pad Placement 533
Operative Technique 533
Trocar Placement 533
Diagnostic Laparoscopy 534
Ultrasound Examination 534
Biopsy 534
Radiofrequency Ablation 535
Postoperative Care 535
Pearls and Pitfalls 536
Lesions in the Dome of the Liver 536
Lesions Near the Gallbladder 537
Lesions Near Main Portal Vein Branches 538
References 538
Pancreatic Incidentaloma 539
Introduction 539
Solid Tumors 541
Pancreatic Cancer 541
Islet Cell Tumors 542
Pancreatic Metastases 543
Chronic Pancreatitis 543
Cystic Tumors 543
Unilocular Cysts 545
Microcystic Lesions 545
Macrocystic Lesions 545
Cysts with a Solid Component 545
Endoscopic US 546
Surgical Treatment 546
References 548
Technique of Pancreatic Resection 551
Introduction 551
Techniques of Laparoscopic Distal Pancreatectomy Resection 551
Laparoscopic Surgery 552
Spleen-Preserving Distal Pancreatectomy with Splenic Vessels Preservation 552
Spleen-Preserving Distal Pancreatectomy Without Splenic Vessels Preservation 552
En-Bloc Laparoscopic Distal Pancreatectomy with Splenectomy 553
Outcome of Laparoscopic Distal Pancreatic Resection 554
Laparoscopic Surgery in Patients with Nonfunctioning PNT 555
Laparoscopic Surgery in Patients with Gastrinoma 556
Laparoscopic Surgery in Patients with Vipoma 557
Laparoscopic Surgery in Patients with Glucagonoma 557
Laparoscopic Surgery in Patients with Carcinoid Tumors 558
Laparoscopic Surgery in MEN-1 Patients with Insulinomas 558
Laparoscopic Surgery in MEN-1 Patients with Zollinger-Ellison Syndrome 558
Laparoscopic Surgery in Patients with Sporadic Insulinoma 559
Conversion to Open Surgery 559
Conclusions 559
References 560
Section V Familial Endocrine Conditions 562
Familial Endocrine Conditions 563
Introduction 563
Multiple Endocrine Neoplasia Type 1 563
Genetic Aspects 563
Genotype-Phenotype Correlation 563
MEN 1 Kindred Without MEN1 Mutation 563
Screening 564
Clinical Aspects 564
Hyperparathyroidism 565
Gastroenteropancreatic Tumors 565
Gastrinoma 565
Insulinoma 566
Adrenal Lesion 566
Gastric ECLoma 566
Thymic Lesions 566
Diagnosis and Imaging 566
Hyperparathyroidism 566
Gastroenteropancreatic Tumors 566
Gastrinoma 566
Insulinoma 567
Adrenal Lesion 567
Gastric ECLoma 567
Thymic Carcinoids 567
Therapy and Prognosis 567
Hyperparathyroidism 567
Gastroenteropancreatic Tumors 567
Gastrinoma 568
Insulinoma 568
Adrenal Lesion 568
Gastric ECLoma 568
Thymic Lesions 569
Follow-Up 569
Hyperparathyroidism 569
Gastrinoma 569
Insulinoma 569
Adrenal Tumors 569
Thymic Lesions 569
Multiple Endocrine Neoplasia Type 2 569
Genetic Aspects 569
Genotype-Phenotype Correlation 569
MEN 2 Kindred Without RET Mutation 571
Screening 571
Clinical Aspects 571
MEN 2A 571
MEN 2B 571
Familial MTC 572
Diagnosis and Imaging 572
Medullary Thyroid Carcinoma 572
Pheochromocytoma 572
Hyperparathyroidism 573
Therapy and Prognosis 574
Medullary Thyroid Carcinoma 574
Pheochromocytoma 574
Hyperparathyroidism 574
Follow-Up 574
Medullary Thyroid Carcinoma 574
Pheochromocytoma 575
Hyperparathyroidism 575
References 575
Section VI Carcinoid 578
Carcinoid: Presentation and Diagnosis, Surgical Management 579
Introduction 579
Gastric Carcinoids 579
Type 1 Gastric Carcinoids 579
Type 2 Gastric Carcinoids 581
Type 3 (Sporadic) Gastric Carcinoids 582
Non-ECL-Cell Gastric Carcinoids 583
Poorly Differentiated Gastric Carcinoids 583
Duodenal Carcinoids 583
Gastrinoma 583
Somatostatin-Rich Carcinoids 584
Gangliocytic Paraganglioma 585
Other Duodenal Carcinoids 585
Poorly Differentiated Duodenal Carcinoids 585
Midgut Carcinoids 585
Appendiceal Carcinoids 588
Goblet Carcinoid (Adenocarcinoid) 588
Colon Carcinoids 588
Poorly Differentiated Colon Carcinoids 589
Rectal Carcinoids 589
Liver Metastases 589
Liver Surgery 590
Radiofrequency Ablation 590
Liver Embolization 590
Liver Transplantation 591
References 591
Index 593