Primary Adrenal Malignancies.

• Superior document: Updates in Surgery Series
• Author: Tiberio, Guido A. M.
• TeilnehmendeR: Tiberio
• Place / Publishing House: Cham : : Springer International Publishing AG,, 2024.; ©2025.
• Year of Publication: 2024
• Edition: 1st ed.
• Language: English
• Series: Updates in Surgery Series
• Physical Description: 1 online resource (177 pages)

Table of Contents

• Intro
• Foreword
• Preface
• Contents
• 1: Epidemiology, Presentation, Staging, and Prognostic Factors in Adrenocortical Carcinoma
• 1.1 Epidemiology
• 1.2 Clinical Presentation
• 1.3 Staging and Risk Assessment
• References
• 2: Epidemiology, Presentation, Staging, and Prognostic Factors in Malignant Pheochromocytoma
• 2.1 Introduction
• 2.2 Presentation
• 2.3 Biochemical Diagnosis
• 2.4 Perioperative Management
• 2.5 Staging
• 2.6 Prognostic Factors
• 2.7 Postoperative Follow-Up
• References
• 3: Genetics and Molecular Biology of Adrenocortical Carcinoma
• 3.1 Introduction
• 3.2 Germline DNA Mutations and the Hereditary Component of Adrenocortical Carcinoma
• 3.3 Chromosomal Number Alteration (Aneuploidy)
• 3.4 Somatic DNA Mutations and Tumor Mutation Burden
• 3.5 Epigenetic (Post-Translational) Changes
• 3.6 An Integrative View of Molecular Biology of Adrenocortical Carcinoma
• 3.7 Spatial/Temporal Molecular Heterogeneity in Adrenocortical Carcinoma
• References
• 4: Genetics and Molecular Biology of Pheochromocytoma and Paraganglioma
• 4.1 Introduction
• 4.2 NF1 Gene
• 4.3 RET Gene
• 4.4 VHL Gene
• 4.5 SDHx Genes and SDHAF2 Gene
• 4.5.1 SDHD Gene (PGL1 Syndrome)
• 4.5.2 SDHAF2 Gene (PGL2 Syndrome)
• 4.5.3 SDHC Gene (PGL3 Syndrome)
• 4.5.4 SDHB Gene (PGL4 Syndrome)
• 4.5.5 SDHA Gene (PGL5 Syndrome)
• 4.6 TMEM127 Gene
• 4.7 MAX Gene
• 4.8 FH Gene
• 4.9 Other Genes
• 4.10 Molecular Biology
• References
• 5: Imaging in Adrenocortical Carcinoma and Malignant Pheochromocytoma
• 5.1 Introduction
• 5.2 Washout CT of Adrenal Lesions
• 5.3 MRI of Adrenal Lesions
• 5.4 Adrenocortical Carcinoma
• 5.5 Malignant Pheochromocytoma
• 5.6 Nuclear Medicine and Pheochromocytoma
• 5.7 Comparison Between Nuclear Medicine Modalities
• 5.8 Conclusions
• References.
• 6: Management of Endocrine Syndromes Associated with Adrenocortical Carcinoma
• 6.1 How Often Will I Find an Adrenocortical Carcinoma with Associated Endocrine Syndromes in My Practice?
• 6.2 What Is the Clinical Presentation of Adrenocortical Carcinoma with Associated Endocrine Syndromes?
• 6.3 How to Diagnose the Endocrine Syndrome Associated with Adrenocortical Carcinoma?
• 6.4 Does Hypercortisolism Affect Patient Outcome?
• 6.5 Which Treatment for Endocrine Syndromes?
• References
• 7: Management of Hereditary Syndromes Associated with Pheochromocytoma/Paraganglioma
• 7.1 Introduction
• 7.2 Neurofibromatosis Type 1
• 7.3 Multiple Endocrine Neoplasia 2
• 7.4 Von Hippel Lindau Syndrome
• 7.5 SDHx-associated Hereditary PPGL
• 7.6 TMEM127- and MAX-associated PPGL
• 7.7 FH-associated PPGL
• References
• 8: Adrenal Incidentaloma
• 8.1 What Does "Adrenal Incidentaloma" Mean?
• 8.2 How Often Will I Find an Adrenal Incidentaloma in My Practice?
• 8.3 What Type of Adrenal Tumor Can Be Found Incidentally?
• 8.4 What Should I Do Next After Discovering an Adrenal Incidentaloma?
• 8.4.1 Risk of Malignancy
• 8.4.2 Hormonal Activity
• 8.5 Which Treatment?
• 8.6 Which Patients Deserve Particular Consideration?
• 8.6.1 Mild Autonomous Cortisol Secretion
• 8.6.2 Bilateral Adrenal Incidentalomas
• 8.6.3 Younger People (&lt
• 40 Years)
• References
• 9: Surgery for Adrenocortical Carcinoma
• 9.1 Introduction
• 9.2 Upfront Adrenalectomy: The Guidelines
• 9.2.1 The Literature
• 9.2.1.1 R.3.1
• 9.2.1.2 R.3.2 and R.3.4
• 9.2.1.3 R.3.7
• 9.2.2 Reasoning to Establish a Sound Surgical Strategy
• 9.3 Surgery for Recurrent Disease: The Guidelines
• 9.3.1 The Literature
• 9.3.2 Reasoning to Establish a Sound Surgical Strategy
• References
• 10: Adrenocortical Carcinoma with Vena Cava Involvement.
• 10.1 The Guidelines
• 10.2 The Multidisciplinary Team
• 10.3 The Cancer and the Patient
• 10.4 Technical Aspects
• 10.5 Results
• 10.6 Final Considerations
• References
• 11: Adrenocortical Carcinoma: The Posterior Minimally Invasive Approach
• 11.1 Introduction
• 11.2 Surgical Technique
• 11.3 Discussion
• 11.4 Conclusions
• References
• 12: Surgery for Malignant Pheochromocytoma
• 12.1 The Guidelines
• 12.2 Indication for Surgery
• 12.3 Surgical Strategies and Techniques
• 12.4 Case Report
• References
• 13: Open or Laparoscopic Surgery in the Management of Adrenocortical Carcinoma?
• 13.1 Introduction
• 13.2 Studies in Favor of Minimally Invasive Adrenalectomy
• 13.3 Studies in Favor of Open Adrenalectomy
• 13.4 Discussion and Guideline Recommendations
• References
• 14: Pathology of Adrenocortical Carcinoma and Malignant Pheochromocytoma
• 14.1 Introduction
• 14.2 Adrenocortical Carcinoma
• 14.2.1 Gross Pathology
• 14.2.2 Cytological and Histological Findings
• 14.2.3 Scoring Systems
• 14.2.4 Histological Subtypes
• 14.2.5 Pediatric Adrenocortical Tumors
• 14.2.6 Grading
• 14.2.7 Staging
• 14.2.8 Immunohistochemical Profile
• 14.3 Malignant Pheochromocytoma
• 14.3.1 Gross Pathology
• 14.3.2 Cytological and Histological Findings
• 14.3.3 Pathological Prediction of a Clinically Aggressive Course
• 14.3.4 Staging
• 14.3.5 Immunohistochemical Profile
• References
• 15: Medical Treatment in Advanced Adrenocortical Carcinoma
• 15.1 Introduction
• 15.2 Standard Systemic Therapy: Mitotane
• 15.3 Combination Therapy: Chemotherapy plus Mitotane (EDP-M)
• 15.4 Beyond EDP-M
• References
• 16: Integrated Approach in Locally Advanced, Oligometastatic or Recurrent Adrenocortical Carcinoma
• 16.1 Introduction
• 16.2 Neoadjuvant Chemotherapy Followed by Surgery.
• 16.3 Adrenalectomy: Upfront or After Primary Chemotherapy in the Metastatic Setting?
• 16.4 Cytoreduction and Hyperthermic Intra-peritoneal Chemotherapy
• 16.4.1 Alternative Locoregional Treatments
• References
• 17: Medical Treatment of Malignant Pheochromocytoma
• 17.1 Chemotherapy
• 17.2 Targeted Therapy
• 17.3 Immunotherapy
• References
• 18: Role of Radiotherapy in Adrenocortical Carcinoma and Pheochromocytoma
• 18.1 Role of Radiotherapy in Adrenocortical Carcinoma
• 18.1.1 Radiobiology of Adrenocortical Carcinoma
• 18.1.2 Adjuvant Radiotherapy
• 18.1.2.1 Aim of Radiotherapy
• 18.1.2.2 Patient Selection
• 18.1.2.3 Efficacy and Timing
• 18.1.2.4 Acute and Late Toxicity
• 18.1.2.5 Radiotherapy Technique, Dose and Volumes
• 18.1.2.6 Concurrent Systemic Therapy to Radiotherapy
• 18.1.3 Definitive Radiotherapy for Unresectable Disease or Local Recurrence
• 18.1.4 Radiotherapy for Metastatic Disease and Palliation
• 18.2 Role of Radiotherapy in Pheochromocytoma
• 18.2.1 Radiobiology of Pheochromocytoma
• 18.2.2 Radiotherapy for Metastatic Disease and Palliation
• 18.2.3 Adjuvant Radiotherapy and Definitive Radiotherapy (Unresectable Disease)
• References
• 19: Radionuclide Treatment in Malignant Pheochromocytoma
• 19.1 Introduction
• 19.2 131I-MIBG Therapy
• 19.2.1 HSA 131I-MIBG
• 19.2.2 LSA 131I-MIBG
• 19.2.3 Contraindications and Adverse Effects
• 19.3 Peptide Receptor Radionuclide Therapy
• 19.3.1 90Y- and 177Lu-Labeled DOTA Compounds
• 19.3.2 Contraindications and Adverse Effects
• 19.4 Comparison Between 131I-MIBG Therapy and Peptide Receptor Radionuclide Therapy
• References
• 20: Preclinic and Translational Research in Adrenal Malignancies
• 20.1 Introduction
• 20.2 Adrenocortical Carcinoma: Preclinical and Translational Models
• 20.2.1 Cell Lines
• 20.2.2 3D Cell Models.
• 20.2.3 Cell Xenograft in Mouse
• 20.2.4 Genetically Engineered Mouse Models
• 20.3 Pheochromocytoma/Paraganglioma Preclinical and Translational Models
• 20.3.1 Cell Lines
• 20.3.2 Rat Pheochromocytoma (PC12)
• 20.3.3 Mouse Pheochromocytoma Cell Line and Mouse Tumor Tissue Cells
• 20.3.4 Immortalized Chromaffin Cells
• 20.3.5 Rat SDH-Deficient RS0 Cells
• 20.3.6 Progenitor Cells Derived from a Human Pheochromocytoma
• 20.3.7 Animal Models
• 20.4 Complex 3D Adrenal In Vitro Preclinical Models
• References.