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.