Endocrine Surgery Comprehensive Board Exam Guide

✍ Scribed by Alexander L. Shifrin; Marco Raffaelli; Gregory W. Randolph; Oliver Gimm

• Publisher: Springer International Publishing
• Year: 2022
• Tongue: English
• Leaves: 1117
• Category: Library

✦ Table of Contents

Preface
Acknowledgments
Contents
Contributors
Abbreviations
I: Thyroid
1: Thyroid Gland: Anatomy, Physiology, Pathophysiology, and Ultrasonography
Case Presentation:
1.1 Thyroid Physiology
1.1.1 Thyroid Cellular Physiology
1.2 Hypothalamic-Pituitary-Thyroid Axis
1.3 Thyroid Function Testing
1.3.1 Serum TSH
1.3.2 Total Thyroid Hormone
1.3.3 Serum Free T4 and Free T3
1.3.4 Serum Thyroid Autoantibodies
1.3.5 Serum Thyroglobulin
1.3.6 Hypothyroidism
1.3.7 Hyperthyroidism
1.4 Thyroid Anatomy
1.4.1 Blood Supply and Lymphatics
1.5 Surgical Thyroid Anatomy
1.5.1 External Branch of the Superior Laryngeal Nerve
1.5.2 Recurrent Laryngeal Nerve
1.5.3 Parathyroid Glands
1.6 Ultrasonography of the Thyroid Gland
References
2: Non-toxic Thyroid Nodules and Multinodular Goitre
Case Presentation
2.1 Introduction
2.2 Clinical Presentation
2.3 Natural History
2.4 Aetiology
2.5 Pathogenesis
2.6 Diagnosis
2.7 Clinical Examination
2.8 Thyroid Function Tests
2.8.1 Thyroid Ultrasound
2.8.2 Fine-Needle Aspiration Biopsy (FNAB)
2.8.3 Precision Medicine
2.8.4 Cross-Sectional Scans
2.8.5 Laryngeal Examination
2.9 Management
2.9.1 Conservative
2.10 Non-surgical (Means to Debulk the Thyroid)
2.10.1 Radio-iodine Therapy
2.10.2 Thermal Ablation
2.11 Surgical
2.11.1 Thyroid Surgery
2.11.2 Hemithyroidectomy
2.11.3 Subtotal Thyroidectomy or Total Thyroidectomy
2.11.4 Retrosternal Goitre
2.11.5 Video-Assisted Thoracoscopic Thyroidectomy
2.11.6 Transoral Thyroidectomy
References
3: Graves’ Disease and Toxic Nodular Goiter (Plummer’s Disease)
Case Presentation
3.1 Introduction
3.1.1 Clinical Presentation of Hyperthyroidism
Box 3.1 Symptoms and Manifestations of Hyperthyroidism
Box 3.2 Signs of Hyperthyroidism
3.1.2 Natural History of Hyperthyroidism
3.1.3 Diagnosis of Hyperthyroidism
3.1.4 Treatment of Hyperthyroidism
3.1.4.1 Antithyroid Drugs
3.1.4.2 Radioactive Iodine (RAI)
3.1.5 Indications for Surgery in Hyperthyroidism
3.1.6 Prognosis in Hyperthyroidism
3.2 Graves’ Disease
3.2.1 Pathogenesis
3.2.2 Epidemiology
3.2.3 Clinical Presentation
3.2.4 Diagnosis
3.2.5 Treatment
3.2.5.1 Antithyroid Drugs
Box 3.3 Manifestation of the Ineffectiveness of  Pharmacotherapy in GD
3.2.5.2 Radioactive Iodine (RAI)
3.2.6 Indications for Surgery and Surgical Details
3.2.7 Prognosis
3.3 Toxic Multinodular Goiter (Plummer’s Disease)
3.3.1 Pathogenesis
3.3.2 Epidemiology
3.3.3 Clinical Presentation
3.3.4 Diagnosis
3.3.5 Treatment
3.3.5.1 Radioactive Iodine (RAI)
3.3.6 Indications for Surgery and Surgical Details
3.3.7 Prognosis
References
4: Diagnosis and Management of Thyroiditis: Hashimoto, de Quervain, Riedel
Case Presentation
4.1 Introduction
4.2 Clinical Presentation and Diagnosis
4.2.1 Hashimoto’s Thyroiditis
4.2.2 de Quervain’s Thyroiditis
4.2.3 Riedel’s Thyroiditis
4.3 Treatment
4.3.1 Hashimoto’s Thyroiditis
4.3.2 de Quervain’s Thyroiditis
4.3.3 Riedel’s Thyroiditis
4.4 Surgical Indications and Outcomes
4.4.1 Hashimoto’s Thyroiditis
4.4.2 de Quervain’s Thyroiditis
4.4.3 Riedel’s Thyroiditis
4.5 Association with Malignancy
References
5: Papillary Thyroid Carcinoma
Case Presentation
5.1 Introduction
5.2 Clinical Presentation
5.3 Natural History
5.4 Diagnosis
5.5 Risk Stratification and Treatment
5.6 Follow-Up
5.7 Outcomes
References
6: Follicular Thyroid Cancer
Case
6.1 Introduction
6.2 Definition
6.3 Epidemiology
6.4 Clinical Presentation
6.5 Molecular Pathogenesis
6.6 Diagnosis
6.7 Staging and Prognostic Features
6.8 Treatment
6.9 Recurrence
6.10 Summary and Conclusion
References
7: Hürthle Cell Carcinoma
Case Presentation
7.1 Introduction
7.2 Clinical Presentation
7.3 Natural History
7.4 Diagnosis
7.5 Treatment
7.6 Surgical Details
7.7 Outcomes or Prognosis
References
8: Medullary Thyroid Carcinoma: Diagnosis and Treatment of Sporadic and Hereditary Tumors
Cases
Case 1
Case 2
Case 3
Case 4
Case 5
8.1 Introduction
8.2 Clinical Presentation
8.3 Ultrasonography
8.4 FNAB
8.5 FNAB and Evaluation of Ct in the Fine-Needle Aspirate
8.6 Carcinoembryonic Antigen
8.7 Procalcitonin
8.8 Ct
8.9 Interpretation of bCt Levels
8.10 Surgery
8.11 Clinically Occult MTC – “Mildly” Elevated bCt Levels
8.12 Biochemically and Clinically Apparent MTC – “Highly” Elevated bCt Levels
8.13 Surgical Strategy – Central Neck
8.14 Surgical Strategy – Lateral Neck
8.15 Locally Advanced MTC and Distant Metastasis
8.16 Special Situations
8.17 TNM Classification and Staging
8.18 Hereditary MTC
8.19 MEN 2A
8.20 MEN 2B
8.21 Prognosis and Postoperative Follow-Up
8.22 Unresectable, Progressive, and Symptomatic Sporadic and Hereditary MTC
References
9: Anaplastic Thyroid Carcinoma, Thyroid Lymphoma, and Metastases
Case Presentation
9.1 Introduction
9.2 Clinical Presentation
9.3 Diagnosis
9.4 Treatment
9.5 Indications for Surgery and Surgical Details
9.6 Prognosis
References
10: Surgical Procedures. Thyroidectomy: Indications, Flexible Laryngoscopy, Operative Techniques, Recurrent Laryngeal Nerve Monitoring, and Management of Complications
10.1 Introduction
10.2 Indications for Thyroidectomy
10.3 Flexible Laryngoscopy
10.4 Operative Description and Technique
10.4.1 Airway and Anesthesia
10.4.2 Positioning (. Fig. 10.4)
10.4.3 Incision (. Fig. 10.5)
10.4.4 Creation of a Working Space to Remove the Thyroid Gland (. Fig. 10.6)
10.4.5 Exposure of the Thyroid Lobe
10.4.6 Exposure, Ligation, and Division of the Superior Pole Vessels
10.4.7 Identification and Preservation of the Recurrent Laryngeal Nerve (. Fig. 10.7)
10.4.8 Division of the Inferior Pole Vessels
10.4.9 Preservation of the Parathyroid Glands
10.4.10 Separation of the Thyroid Lobe from the Recurrent Laryngeal Nerve
10.4.11 Removal of the Thyroid Lobe and Isthmus
10.4.12 Closure
10.5 Intraoperative Neuromonitoring (IONM)
10.6 Postoperative Care
10.6.1 General Instructions
10.6.2 Duration of Observation
10.6.3 Pain Management
10.6.4 Calcium Monitoring and Supplementation
10.6.5 Thyroid Hormone Replacement
10.6.5.1 After Total Thyroidectomy
10.6.5.2 After Thyroid Lobectomy
10.7 Management of Complications
10.7.1 Neck Hematoma
10.7.2 Recurrent Laryngeal Nerve Injury
10.7.3 Hypocalcemia and Hypoparathyroidism
References
11: Neck Dissection: Indications, Extension, Operative Technique
Case Presentation
11.1 Introduction
11.2 Applied Anatomy
11.2.1 Standardized Terminology in Neck Dissection
11.2.2 Levels of Cervical Lymph Nodes
11.3 Classification of Neck Dissections
11.4 Central Neck Dissection: Indications and Extension
11.4.1 Lateral Neck Dissection: Indications and Extension
11.5 Operative Procedure
11.5.1 Planning the Surgical Procedure
11.5.2 Intraoperative Surgical Adjuncts
11.5.3 Surgical Technique
11.5.3.1 Patient’s Preparation and Positioning
11.5.3.2 Skin Incision and Flap Elevation
11.5.3.3 Selective Lateral Neck Dissection: Levels II–Vb
11.5.3.4 Central Neck Dissection (Level VI–VII)
11.5.4 Alternate Operative Approaches (Minimally Invasive, Remote Access, and Radio-Guided Procedures)
11.5.5 Knowing and Avoiding Complications of ND
References
II: Parathyroid
12: Parathyroid Glands: Anatomy, Physiology, Pathophysiology, and Ultrasound
12.1 Introduction
12.2 Parathyroid Anatomy
12.2.1 Embryologic Development
12.2.2 Normal Parathyroid Gland Anatomy
12.2.3 Ectopic Parathyroid Gland Anatomy
12.2.4 Supernumerary Glands
12.3 Parathyroid Physiology
12.4 Parathyroid Pathophysiology
12.4.1 Hyperparathyroidism
12.4.1.1 Primary Hyperparathyroidism
12.4.1.2 Secondary and Tertiary Hyperparathyroidism
12.4.2 Hypoparathyroidism
12.5 Parathyroid Ultrasound
References
13: Diagnosis and Surgical Management of Primary Hyperparathyroidism
Case Presentation
13.1 Introduction
13.2 Diagnosis of PHPT
13.3 Indications for Surgical Treatment of PHPT
13.4 Preoperative Radiological Studies and Surgical Treatment of PHPT
13.5 Preoperative Management
13.6 Intraoperative PTH Monitoring
13.7 Surgical Approach
13.8 PHPT During Pregnancy
13.9 Location of Ectopic Parathyroid Glands
13.10 Prediction and Treatment of Postoperative Hypocalcemia
13.11 Postoperative Follow-Up
13.12 Long-Term Effect of Parathyroidectomy in Patients with PHPT
References
14: Renal Hyperparathyroidism
Case
14.1 Introduction
14.2 Clinical Presentation
Box 14.1 Some signs and symptoms in rHPT (Adapted from Pasieka et al. [118])
14.3 Natural History
14.3.1 Chronic Kidney Disease (CKD)
14.3.2 FGF23, Klotho
14.3.3 Phosphate Retention
14.3.4 Vitamin D and Calcium
14.3.5 Parathyroid Gland Hyperplasia
14.3.6 Tertiary Hyperparathyroidism
14.4 Diagnosis
14.5 Non-surgical Treatment
14.5.1 Medical
14.5.1.1 Vitamin D
14.5.1.2 Control of Calcium and Phosphate
Restriction of Dietary Intake
Treatment with Phosphate Binders
Active Vitamin D Analogs
Calcimimetics
14.5.2 Renal Transplantation
14.6 Surgical Treatment: Parathyroidectomy
14.6.1 Indications
14.6.1.1 Is There an Absolute Threshold of PTH When PTX Is Indicated?
14.6.1.2 Are There Other Specific, Absolute Indications for PTX, Apart from PTH Levels?
14.6.1.3 Should PTX Be Performed Before or After Renal Transplantation?
14.6.1.4 What, Exactly, Constitutes “Medical Failure”?
Box 14.2 Indications for operation in renal hyperparathyroidism
14.6.2 Surgical Technique
14.6.2.1 Intraoperative Measurement of Parathyroid Hormone (ioPTH)
14.6.2.2 Preoperative Localization
14.6.2.3 Intraoperative Angiography
14.6.2.4 Surgical Complications
14.6.2.5 Postoperative Management
14.7 Outcomes and Prognosis
References
15: Diagnosis and Surgical Management of Parathyroid Carcinoma
Case Presentation
15.1 Introduction
15.2 Clinical Presentation
15.3 Non Functional PC
15.4 Natural History
15.5 Diagnosis
15.6 Treatment
15.6.1 Medical Treatment
15.6.2 Surgical Treatment
15.6.3 Chemotherapy
15.6.4 Radiotherapy
15.7 Prognostic Factors
15.8 Clinical Follow-Up
References
16: Surgical Procedures. Parathyroidectomy: Indications, Operative Techniques, Management of Complications, Intraoperative PTH Monitoring, Role of Parathyroid Autofluorescence and ICG
Case Presentation
16.1 Introduction
16.2 Indications for Surgery
16.3 Preoperative Evaluation
16.4 Operative Techniques
16.4.1 Bilateral Exploration Parathyroidectomy
16.4.2 Focused Parathyroidectomy
16.5 Intraoperative PTH Monitoring and Gamma-Probe Localization
16.6 Indocyanine Green and Autofluorescence
16.7 Cryopreservation and Autotransplantation
16.8 Management of Complications
16.9 Outcomes and Prognosis
References
III: Adrenals
17: Adrenal Glands: Anatomy, Physiology, and Pathophysiology
17.1 Anatomy
17.1.1 Embryology
17.1.2 Macroscopic Aspect
17.1.2.1 Relationship to Surrounding Structures
17.1.3 Arterial Supply
17.1.4 Venous Drainage
17.1.5 Lymphatic Drainage
17.1.6 Nerve Supply
17.1.7 Histology
17.2 Physiology
17.2.1 The Adrenocortical Hormones
17.2.1.1 Glucocorticoids: Corticosterone and Cortisol
Effects of Glucocorticoids
Regulation of Glucocorticoid Release
17.2.1.2 Mineralocorticoids: Aldosterone
Effects of Mineralocorticoids
Regulation of Mineralocorticoid Release
17.2.1.3 Adrenal Androgens
Effects of Adrenal Androgens and Estrogens
Regulation of Adrenal Androgen Release
17.2.2 The Adrenomedullary Hormones
17.2.2.1 Effects of Epinephrine and Norepinephrine
17.2.2.2 Effects of Dopamine
17.2.2.3 Regulation of Adrenal Medullary Secretion
17.3 Pathophysiology
17.3.1 Introduction
17.3.2 Cushing’s Syndrome
17.3.3 Conn’s Syndrome
17.3.4 Pheochromocytoma
17.3.5 Adrenocortical Carcinoma
References
18: Adrenal Incidentaloma
Case Presentation
18.1 Introduction
18.2 Anatomical Imaging
18.2.1 CT
18.2.2 MRI
18.3 Functional Imaging
18.3.1 FDG PET CT
18.4 Conclusions
References
19: Cushing’s Syndrome
Case Presentation
19.1 Introduction
19.2 Clinical Presentation
19.3 Natural History
19.4 Diagnosis
19.5 Treatment of CS and Indications for Surgery
19.6 ACTH-Dependent Hypercortisolism
19.6.1 Pituitary-Dependent CD
19.6.2 Ectopic ACTH-Dependent CS
19.7 ACTH-Independent Hypercortisolism
19.7.1 Adrenal Adenoma
19.7.2 Adrenal Carcinoma
19.7.3 Primary Adrenal Hyperplasia
References
20: Primary Aldosteronism
Case Presentation
20.1 Introduction
20.2 Clinical Presentation
20.3 Natural History
20.4 Diagnosis
20.5 Treatment
20.5.1 Pharmacologic Treatment
20.6 Indications for Surgery and Surgical Details
20.6.1 Adrenalectomy by the Laparoscopic Transabdominal Approach
20.6.2 Adrenalectomy with the Retroperitoneoscopic Approach
20.6.3 Complications
20.6.4 Postoperative Care (Correction of Potassium and Antihypertensive Medications)
20.7 Outcomes and Prognosis
20.7.1 Histopathology
20.7.2 Potassium and Hypertension
20.7.3 Long-Term Prognosis of Cardiovascular Disease and Mortality
Box 20.1 Types of Primary Aldosteronism
20.7.4 Renal Function After Surgery
References
21: Pheochromocytoma and Paraganglioma
Case Presentation
Box 21.1 Laboratory Tests
21.1 Introduction
21.2 Clinical Presentation
Box 21.2 Signs and Symptoms Associated with Catecholamine-Secreting Tumors
21.3 Natural History
21.3.1 Genetic Forms of Pheochromocytoma and Paraganglioma
21.4 Diagnosis
21.4.1 Differential Diagnosis
Box 21.3 Medications that May Increase Measured Levels of Fractionated Catecholamines and Metanephrines
21.4.2 When to Do Case Detection Testing
21.4.3 Measurement of Fractionated Metanephrines and Catecholamines in Urine and Plasma
21.4.4 Genetic Testing
21.4.5 Localization
21.5 Treatment
21.5.1 Preoperative Management
21.5.2 Acute Hypertensive Crises
21.6 Indications for Surgery and Details
21.6.1 Adrenalectomy for Pheochromocytoma: Surgical Approach
21.6.2 Perioperative Concerns
21.6.3 Special Cases
21.6.4 Abdominal Paraganglioma
21.6.5 Subtotal (Partial, Cortex-Sparing) Adrenalectomy
21.7 Outcomes and Recurrences
References
22: Adrenocortical Carcinoma, Adrenal Lymphoma and Metastases to Adrenal Gland
Case Presentation
22.1 Introduction
22.2 Adrenocortical Carcinoma
22.2.1 Clinical Presentation
22.2.2 Natural History
22.2.3 Diagnosis
22.2.4 Treatment
22.2.4.1 Indications for Surgery and Surgical Details
22.2.5 Outcomes or Prognosis
22.3 Metastases to the Adrenal Gland
22.3.1 Clinical Presentation
22.3.2 Natural History
22.3.3 Diagnosis
22.3.4 Treatment
22.3.4.1 Indications for Surgery and Surgical Details
22.3.5 Outcomes or Prognosis
22.4 Adrenal Lymphoma
22.4.1 Clinical Presentation
22.4.2 Natural History
22.4.3 Diagnosis
22.4.4 Treatment
22.4.5 Indications for Surgery and Surgical Details
22.4.6 Outcomes or Prognosis
References
23: Surgical Procedure: Adrenalectomy – Indications, Operative Techniques and Management of Complications
Case Presentation
23.1 Introduction
23.2 Minimally Invasive Adrenalectomy
23.2.1 Laparoscopic Lateral Transabdominal Adrenalectomy
23.2.1.1 Right Adrenalectomy
23.2.1.2 Left Adrenalectomy
23.2.1.3 Pitfalls and Management of Complications
23.2.2 Posterior Retroperitoneoscopic Adrenalectomy
23.2.2.1 Pitfalls and Management of Complications
23.2.3 Robot-Assisted Adrenalectomy
23.2.4 Operative Technique
23.3 Open Approaches for Adrenalectomy
23.3.1 Operative Technique
23.3.1.1 Pitfalls and Management of Complications
References
IV: Neuroendocrine Tumors (Thymic and Gastroenteropancreatic)
24: Neuroendocrine Tumors (Thymic and Gastroenteropancreatic): Anatomy, Endocrine Physiology, and Pathophysiology
Case Presentation
24.1 Introduction
24.1.1 Incidence and Prevalence
24.1.2 Heredity in NET
24.2 Clinical Presentation
24.2.1 Gastric NET
24.2.2 Endocrine Pancreas
24.2.3 Duodenal NET
24.2.4 Small Intestinal NET
24.2.5 Appendix NET
24.2.6 NETs of the Colon and Rectum
24.2.7 Neuroendocrine Cancer (NEC)
24.2.8 Thymic NET
24.3 Natural History
24.4 Diagnosis and Common Syndromes
24.4.1 Classic Carcinoid Syndrome
24.4.2 Atypical Carcinoid Syndrome
24.4.3 Diarrhea
24.4.4 Small Bowel Obstruction
24.4.5 Gastrointestinal Bleeding
24.4.6 Epigastric Pain and Dyspepsia
24.4.7 Flushing
24.4.8 Jaundice
24.4.9 Effects on Blood Pressure
24.4.10 Heart Failure
24.4.11 Pulmonary Obstructive Disorders
24.4.12 Abdominal Angina
24.4.13 Pallor
24.4.14 Hypoglycemia
24.4.15 Hyperglycemia
24.4.16 Tachycardia
24.4.17 Necrolytic Migratory Exanthema
24.5 Diagnostics
24.5.1 Biochemical Markers
24.5.1.1 Chromogranin A
24.5.1.2 Serotonin and 5-Hydroxyindoleacetic Acid (5-HIAA)
24.5.1.3 Gastrin
24.5.1.4 Insulin, C-Peptide, and Pro-insulin
24.5.1.5 Glucagon, Vasoactive Intestinal Peptide (VIP), Pancreatic Polypeptide (PP), and Others
24.5.2 Endoscopy
24.5.3 Radiological Diagnostics
24.5.3.1 Nuclear Medicine Diagnostics
24.5.3.2 Pathology
24.5.3.3 TNM Staging
24.5.3.4 Multidisciplinary Conference
24.6 Treatment
24.6.1 Surgery
24.6.2 Oncological and Medical Treatment
24.7 Indications for Surgery and Surgical Strategy
24.7.1 Pancreatic NET
24.7.2 Gastric NET
24.7.3 Duodenal NET
24.7.4 Small Intestinal NET
24.7.5 Appendix NET
24.7.6 Thymic NET
24.7.7 Other NETs
24.7.8 Emergency Surgery in NET
24.8 Prognosis
24.8.1 Follow-Up
References
25: Neuroendocrine Tumors of the Thymus
Case Presentation
25.1 Introduction
25.2 Clinical Presentation
25.3 Prognosis
25.4 Treatment of TNET
25.5 Surgical Details
25.6 Prophylactic Transcervical Thymectomy
25.7 Medical Treatment and Radiotherapy
References
26: Neuroendocrine Tumors: Stomach
Case Presentation
26.1 Introduction
26.2 Classification
26.3 Clinical and Pathological Characteristics
26.4 Diagnosis and Staging
26.4.1 Upper Endoscopy
26.4.2 Imaging Studies
26.4.2.1 CT Scans
26.4.2.2 Nuclear Medicine
26.4.2.3 Magnetic Resonance Imaging (MRI)
26.4.3 Biochemical Tests
26.4.3.1 Gastrin
26.4.3.2 Chromogranin A (CgA)
26.5 Clinical and Pathologic Evaluation and Staging
26.6 Local Treatment Modalities for gNETs
26.7 Management of gNETs
26.7.1 Management of Type I gNETs
26.7.1.1 Medical Treatment
26.7.1.2 Type I gNETs’ Treatment Summary
26.7.2 Management of Type II gNETs
26.7.2.1 Type II gNETs’ Treatment Summary
26.7.3 Management of Type III gNETs
26.7.3.1 Type III gNETs’ Treatment Summary
References
27: Diagnosis and Management of Functional Pancreatic Neuroendocrine Tumors
Case Presentation (Adapted from Ref. [1])
27.1 Introduction
27.2 Insulinoma
27.2.1 Clinical Presentation
27.2.2 Diagnosis
27.2.3 Localization
27.2.4 Treatment
27.3 Gastrinoma
27.3.1 Clinical Presentation
27.3.2 Diagnosis
27.3.3 Localization
27.3.4 Treatment
27.4 Other F-pNETs
27.4.1 Glucagonoma
27.4.2 VIPoma
27.4.3 SSomas
References
28: Non-functional Pancreatic Neuroendocrine Tumors
Case Presentation
28.1 Introduction
28.2 Hereditary Tumor Syndromes
28.3 Clinical Presentation
28.4 Diagnosis
28.5 Treatment
28.5.1 Locoregional Ablative Therapy
28.5.2 Liver Transplantation
28.5.3 Medical Therapy in Advanced Disease
28.6 Indications for Surgery and Surgical Details
28.6.1 Surgery in Metastatic Patients
28.6.2 Palliative Surgery
28.6.3 Surgery in High-Grade NF-pNET
28.6.4 Surgery in MEN 1 Patients
28.7 Outcomes or Prognosis
28.7.1 Follow-Up
References
29: Small Intestine NETs
Case Presentation and Case-Specific Questions
29.1 Introduction
29.2 Natural History
29.3 Incidence
29.4 Risk Factors
29.5 Prognosis
29.6 Clinical Presentation
29.6.1 Non-specific Symptoms and Intestinal Obstruction
29.6.2 Desmoplastic Reaction
29.6.3 Carcinoid Syndrome
29.6.4 Carcinoid Heart Disease (Hedinger Syndrome)
29.6.5 Carcinoid Crisis
29.7 Diagnostic Workup
29.7.1 Biochemistry
29.7.1.1 Chromogranin A
29.7.1.2 5-Hydroxy Indole Acetic Acid (5-HIAA)
Box 29.1: Foods and Medications That Could Affect the Result of 24 h Urinary 5-HIAA
29.7.2 Imaging
29.7.2.1 Anatomical Cross-Sectional Imaging
29.7.2.2 Functional Imaging
29.7.3 Endoscopy
29.7.4 Biopsy and Histopathology
29.7.5 Cardiac Workup
29.8 Treatment
29.8.1 Surgery for siNETs
29.8.1.1 Indication
29.8.1.2 Pre- and Peri-operative Care
29.8.1.3 Surgery for Locoregional Disease (Stage I– III)
29.8.1.4 Systematic Intraoperative Exploration and Primary Tumor Resection
29.8.1.5 Lymphadenectomy
29.8.1.6 Prophylactic Cholecystectomy
29.8.1.7 Surgery in Metastatic Disease (Stage IV)
29.8.1.8 Surgery in Metastatic Liver Disease (M1a)
29.8.1.9 Surgery for the Primary Tumor in Inoperable Liver Disease (M1a)
29.8.1.10 Surgery in Cases with Peritoneal Spread
29.8.2 Non-surgical Treatment Options
29.8.2.1 Somatostatin Analogs (SSAs)
29.8.2.2 Interferon Alpha (IFN)
29.8.2.3 Everolimus
29.8.2.4 Peptide Receptor Radionuclide Therapy (PRRT)
29.8.2.5 Liver-Directed Therapy
29.8.2.6 Chemotherapy
29.8.2.7 Symptomatic Therapy for Serotonin-Induced Diarrhea
29.8.2.8 Treatment of Carcinoid Heart Disease (CHD, Hedinger Syndrome)
29.9 Follow-Up
29.9.1 Frequency and Duration of the Follow-Up
29.9.2 Investigations
References
30: Large Intestine NETs
Case Presentation
30.1 Incidence and Presentation
30.2 Diagnosis
30.3 Treatment
30.3.1 Preoperative Management
30.3.2 Prophylactic Cholecystectomy
30.3.3 Endoscopic Management
30.3.4 Carcinoid Syndrome Management
30.4 Non-surgical Management of Metastatic Disease
30.4.1 Systemic Treatment
30.4.2 Liver-Directed Therapy
30.5 Surgical Treatment for Appendix NETs
30.6 Surgical Treatment for Colonic NETs
30.7 Surgical Treatment for Rectal NETs
30.8 Surgical Treatment for Liver Metastases
30.9 Follow-Up
30.10 Prognosis
References
31: Surgical Procedure Steps and Key Points: Whipple Procedure, Enucleations, and Management of Complications
31.1 Introduction
31.2 Clinical Presentation
31.3 Pre-operative Evaluation: Diagnostic Imaging and Staging
31.4 Consideration for Method of Surgical Treatment
31.5 Surgical Technique
31.6 Post-operative Care and Complications
31.7 Summary
References
V: Familial Endocrine Syndromes
32: Familial Non-medullary Thyroid Carcinoma Syndrome (FNMTC) and Familial Syndromes Associated with Thyroid Cancer
Case Presentation
32.1 Introduction
32.2 Definition, Screening, and Clinical Management of FNMTC
32.2.1 Syndromic FNMTC
32.2.1.1 Familial Adenomatous Polyposis
32.2.1.2 Cowden Syndrome
32.2.1.3 Carney Complex
32.2.1.4 Werner Syndrome
32.2.1.5 Pendred Syndrome
32.2.1.6 Ataxia-Telangiectasia
32.2.1.7 Bannayan-Ruvalcaba-Riley Syndrome
32.2.1.8 PTEN Hamartoma Tumor Syndrome
32.2.1.9 Peutz-Jeghers Syndrome
32.2.1.10 DICER1 Syndrome
32.2.1.11 McCune-Albright Syndrome
32.2.1.12 Papillary Renal Neoplasia (Non-syndromic FNMTC)
32.2.2 Non-syndromic FNMTC
32.3 Prognosis and Aggressiveness in FNMTC Compared to Sporadic Disease
32.4 Susceptibility Genes in Non-syndromic FNMTC
References
33: Familial Primary Hyperparathyroidism
Case Presentation
33.1 Introduction
33.2 Preoperative Localization Studies
33.3 Role of Intraoperative PTH Measurement in Familial HPT
33.4 Cryopreservation
33.5 The Syndromes
33.5.1 MEN 1
33.5.1.1 Clinical Presentation
33.5.1.2 Natural History
33.5.1.3 Diagnosis
33.5.1.4 Indications for Surgery and Surgical Details
33.5.1.5 Outcomes/Prognosis
33.5.2 MEN 2A
33.5.2.1 Clinical Presentation
33.5.2.2 Natural History
33.5.2.3 Diagnosis
33.5.2.4 Indications for Surgery and Surgical Details
33.5.2.5 Outcomes/Prognosis
33.5.3 MEN 4
33.5.3.1 Clinical Presentation
33.5.3.2 Natural History
33.5.3.3 Diagnosis
33.5.3.4 Indications for Surgery and Surgical Details
33.5.4 Hereditary Hyperparathyroidism-Jaw Tumor Syndrome (HPT-JT)
33.5.4.1 Clinical Presentation
33.5.4.2 Natural History
33.5.4.3 Diagnosis
33.5.4.4 Surgery and Surgical Details
33.5.4.5 Outcomes/Prognosis
33.6 Non-syndromic PHPT
33.6.1 Familial Isolated Hyperparathyroidism (FIHPT)
33.6.1.1 Clinical Presentation and Natural History
33.6.1.2 Diagnosis
33.6.1.3 Surgery and Surgical Details
33.6.2 Familial Hypocalciuric Hypercalcemia (FHH)
33.6.2.1 Clinical Presentation and Natural History
33.6.2.2 Diagnosis
33.6.2.3 Treatment
33.6.2.4 Outcomes/Prognosis
33.6.3 Neonatal Severe Hyperparathyroidism (NSHPT)
33.6.3.1 Clinical Presentation and Natural History
33.6.3.2 Diagnosis
33.6.3.3 Medical Treatment, Surgery and Surgical Details
33.6.3.4 Outcomes/Prognosis
33.6.4 Autosomal Dominant Moderate Hyperparathyroidism (ADMH)
33.6.4.1 Clinical Presentation and Natural History
33.6.4.2 Diagnosis
33.6.4.3 Surgery and Surgical Details
33.6.4.4 Outcome
References
34: Familial Hypocalciuric Hypercalcemia (FHH)
Case Presentation
34.1 Introduction
34.2 Molecular Genetics
34.3 Clinical Presentation
34.4 Natural History
34.5 Diagnosis
34.6 Treatment
34.7 Indications for Surgery and Surgical Details
34.8 Prognosis
References
35: Hyperparathyroidism-Jaw Tumor Syndrome
Case Presentation
35.1 Introduction
35.2 Etiology
35.3 Clinical Presentation
35.3.1 pHPT
35.3.2 Jaw Tumors
35.3.3 Renal Involvement
35.3.4 Uterine Involvement
35.3.5 Other Features
35.4 Diagnosis
Box 35.1 Indication for CDC73 genetic testing
35.5 Treatment
35.6 Surveillance
References
36: Multiple Endocrine Neoplasia Type 1
Case
36.1 Introduction
36.2 Molecular Genetics of MEN1
36.3 Clinical Presentation and Diagnosis
36.3.1 Primary Hyperparathyroidism
36.3.2 Gastroduodenopancreatic Neuroendocrine Tumours
36.3.3 Pituitary Tumours
36.3.4 Adrenal Tumours
36.3.5 Bronchial and Thymic Neuroendocrine Tumours
36.3.6 Non-endocrine Tumours
36.3.7 Diagnosis
36.4 Surgical Management
36.4.1 Primary Hyperparathyroidism
36.4.2 Gastroduodenopancreatic Neuroendocrine Tumours
36.5 Genetic Counselling
36.5.1 Family Planning
36.5.2 Prenatal Testing
36.5.3 Preimplantation Genetic Testing
36.6 Surveillance
36.6.1 Primary Hyperparathyroidism
36.6.2 Gastroduodenopancreatic Neuroendocrine Tumours
36.6.3 Pituitary Adenoma
36.6.4 Adrenal Tumours
36.6.5 Bronchial and Thymic Carcinoids
36.7 Summary
References
37: Multiple Endocrine Neoplasia Type 2 (MEN 2)
Case Presentation
37.1 Introduction
37.1.1 RET Proto-Oncogene
37.1.2 Medullary Thyroid Cancer
37.1.3 Medullary Thyroid Carcinoma and Biomarkers
37.1.4 MTC and Ectopic ACTH Secretion
37.2 Clinical Presentation
37.2.1 MEN 2A
37.2.1.1 Classical Type of MEN 2A
37.2.1.2 MEN 2A with CLA
37.2.1.3 MEN 2A with HD
37.2.1.4 FMTC
37.2.2 MEN 2B
37.3 Natural History
37.3.1 Classical MEN 2A Syndrome
37.3.2 MEN 2A with CLA
37.3.3 MEN 2A and HD
37.3.4 MEN 2B
37.4 Diagnosis
37.5 Treatment
37.5.1 Risk Categories for Hereditary MTC
37.5.2 Extent of the Initial Surgery for MTC
37.5.3 Systematic Treatment
37.6 Surgical Considerations in MEN2 Patients
37.6.1 Complications Related to Surgery for MTC
37.6.2 What About Parathyroid Transplantation During Thyroidectomy in MEN 2 Patients?
37.6.3 Excluding Pheochromocytoma Before Neck Surgery
37.6.4 Prophylactic Thyroidectomy
37.7 Prognosis and Follow-Up
37.7.1 The Importance of the Initial Operation
37.7.2 What About the Impact of HD in the Prognosis of MEN 2A Patients?
37.7.3 Follow-Up
References
38: Multiple Endocrine Neoplasia Type 4 (MEN 4)
Case
Genetic Analyses
38.1 Introduction
38.2 The MENX Syndrome
38.3 Molecular Genetics of MEN 4
38.4 Clinical Presentation of MEN 4
38.4.1 Primary Hyperparathyroidism
38.4.2 Pituitary Adenomas
38.4.3 Neuroendocrine Neoplasms
38.4.4 Other Endocrine Tumors Associated with Mutation of p27KIP1
38.4.5 Non-endocrine Tumors and Other Conditions Associated with Mutation of p27KIP1
38.5 Diagnosis
38.6 Genetic Counseling
38.7 Surgical Management
38.8 Surveillance and Follow-Up
38.9 Summary
References
39: Non-MEN Familial Endocrine Syndromes: Von Recklinghausen Disease, Von Hippel-Lindau Syndrome, Pheochromocytoma/Paraganglioma
Case Presentation
39.1 Hereditary Pheochromocytoma and Paraganglioma
39.1.1 Introduction
39.1.2 Epidemiology and Genetics
39.1.3 Clinical Presentation
39.1.4 Diagnosis and Indications for Surgery
39.1.5 Preoperative Management and Radiological Studies
39.1.6 Surgical Techniques
39.1.7 Postoperative Follow-Up and Prognosis
39.2 Von Hippel-Lindau Syndrome
39.2.1 Introduction
39.2.2 Epidemiology and Genetics
39.2.3 Clinical Presentation
39.2.3.1 Visceral Lesions
39.2.3.2 CNS Lesions
39.2.3.3 Retina
39.2.3.4 Inner Ear
39.2.4 Diagnosis and Indications for Surgery
39.2.5 Preoperative Management and Radiological Studies
39.2.6 Surgical Techniques
39.2.7 Postoperative Follow-Up and Prognosis
39.3 Neurofibromatosis Type 1 (Von Recklinghausen Disease)
39.3.1 Introduction
39.3.2 Epidemiology and Genetics
39.3.3 Clinical Presentation
39.3.4 Diagnosis and Indications for Surgery
39.3.5 Preoperative Management and Radiological Studies
39.3.6 Surgical Techniques
39.3.7 Postoperative Follow-Up and Prognosis
References
VI: Endocrine Emergencies
40: Thyroid Storm
Case Presentation
40.1 Introduction
40.2 Clinical Presentation
40.3 Natural History
Box 40.1: Factors Precipitating the Thyroid Storm
40.4 Diagnosis
40.5 Treatment
Box 40.2: Treatment of Thyroid Storm
40.6 Indications for Surgery and Surgical Details
40.7 Outcomes and Prognosis
References
41: Hypercalcemic Crisis
Case
41.1 Introduction
41.2 Clinical Presentation
41.3 HC Due to PHPT in Pregnancy
41.4 HC in Young Patients
41.5 Natural History
Box 41.1: Important Milestones in the Management of Hypercalcemia Due to Hyperparathyroidism in the Last Century
41.6 Histopathological Characteristics in HC
41.7 Assessment of the Risk of Developing HC
41.8 Diagnosis of HC
41.9 Differential Diagnosis of HC (7 Box 41.2)
Box 41.2: Differential Diagnosis of HC
41.10 Medical Management of HC Due to PHPT
41.11 Surgical Management
41.12 Outcome for Parathyroidectomy for HC
References
42: Endocrine Hypertensive Emergencies
Case Presentation
42.1 Introduction
42.2 Pheochromocytoma
42.2.1 Diagnosis
42.2.2 Treatment
42.3 Primary Hyperaldosteronism
42.3.1 Diagnosis
42.4 Cushing’s Disease and Syndrome
42.4.1 Diagnosis
42.4.2 Treatment
42.5 Thyroid Disease
42.5.1 Diagnosis
42.5.2 Treatment
References
43: Carcinoid Crisis
43.1 Introduction
43.2 Diagnosis and Management of CS
43.3 Carcinoid Crisis
43.4 Perioperative Delivery and Management of Octreotide
43.5 Use of Octreotide Prophylaxis in Procedures
43.6 Recommendations Concerning Anesthesia
43.7 Conclusion
References
VII: Miscellaneous
44: Ethics in Endocrine Surgery
44.1 Introduction
44.2 Autonomy
44.2.1 Informed Consent
44.2.2 Operative Volume and the Role of Trainees
44.3 Beneficence
44.3.1 Surgical Decision-Making
44.4 Non-maleficence
44.4.1 Complications
44.4.2 Disclosure
44.4.3 Innovation in Surgery
44.5 Justice
44.6 Conclusions
References
45: How to Read and Interpret a Scientific Paper
45.1 Introduction
45.2 What Scientific Report to Read?
45.3 How to Structure the Reading?
45.4 What About Evidence and Its Grading in Medicine?
45.5 Checkbox Basic Questions on Study Quality and Credibility
References
46: Training and Board Certification in Endocrine Surgery
46.1 Introduction
46.2 Training and Volume
46.2.1 Thyroid Gland Surgery and Neck Dissections
46.2.2 Parathyroid Gland Surgery
46.3 Adrenal Gland Surgery
46.4 Surgery of Gastro-Entero-Pancreatic Neuroendocrine Neoplasms
46.4.1 Summary
46.5 Board Certification
46.6 Conclusion
References
47: Center Accreditation for Endocrine Surgery
47.1 Introduction
47.2 Accreditation Process: Aspects to Be Certified – Hierarchy of Units
47.3 Requirements
47.3.1 Minimum Number of Interventions
47.3.1.1 Thyroid Surgery
47.3.1.2 Parathyroid Surgery
47.3.1.3 Adrenal or Paraganglioma Surgery
47.3.1.4 Gastroenteropancreatic Neuroendocrine Neoplasms (GEP-NEN)
47.3.2 Quality Indicators
47.3.2.1 Thyroid Surgery
47.3.2.2 Parathyroid Surgery
47.3.3 Requirements: Equipment, Staff, and Multidisciplinary Collaboration
47.3.3.1 Structure
47.3.3.2 Staff
47.3.3.3 Equipment and Diagnostic Procedures
47.3.3.4 Multidisciplinary Collaboration
47.3.4 Training and Research
47.3.4.1 The Trainee and Training Center
47.3.4.2 Research
47.4 Quality Control and Auditing
47.5 Conclusions
References
Index