Paragangliomas and pheochromocytomas are rare tumors of the autonomic nervous system that represent a diagnostic and therapeutic challenge. While the classic sites of origin are the adrenal medulla and the autonomic branches of the lower cranial nerves, they may arise from any site in connection with the autonomic neural branches in the thoracic, abdominal, and head and neck regions. The uncontrolled release of catecholamines by these tumors can be life-threatening. Paragangliomas and pheochromocytomas are the tumor types in which the impact of genetic predisposition factors is the highest. The treatment of paraganglioma and pheochromocytoma requires specialized knowledge and solid experience, which, given the rarity of these diseases, is available only in highly specialized centers. This book includes specific chapters written by experts from several countries that deal with relevant aspects of paraganglioma and pheochromocytoma diagnosis and therapy. Chapter 1 addresses the genetic findings that are most relevant to diagnosis and management, chapter 2 deals with the biochemical diagnosis, chapter 3 with imaging, chapter 4 with the surgical and pharmacological management, mainly of trunk paraganglioma and pheochromocytoma, chapters 5 and 6 with the histopathological, genetic, and clinical characteristics of carotid body, and vagal and tympano-jugular paragangliomas.

Contents

• Foreword
• Preface
• List of Contributors
• 1. Genetics of Pheochromocytoma and Paraganglioma
  Bernardo Dias Pereira, Henrique Vara Luiz, Ana Gonçalves Ferreira, and Jorge Portugal.
  • INTRODUCTION
  • MOLECULAR PATHWAYS ASSOCIATED WITH PPGL
  • SYNDROMIC PRESENTATIONS
  • MULTIFOCAL TUMORS
  • METASTATIC DISEASE
  • BILATERAL PHEOCHROMOCYTOMA
  • PEDIATRIC PPGL
  • BIOCHEMICAL PHENOTYPE
  • IMMUNOHISTOCHEMICAL PHENOTYPE
  • FUNCTIONAL IMAGING
  • NEXT-GENERATION SEQUENCING
  • CONCLUSIONS
  • REFERENCES
• 2. Biochemical Diagnosis of Pheochromocytoma and Paraganglioma
  Ying Shen and Liming Cheng.
  • INTRODUCTION
  • CATECHOLAMINES BIOLOGY
  • BIOCHEMICAL PHENOTYPES
  • CHOICE OF TESTS
  • ANALYTICAL METHODS
  • SAMPLING AND STORAGE CONDITIONS
  • INTERPRETATION OF TEST RESULTS
  • CONCLUSION
  • REFERENCES
• 3. Imaging of Pheochromocytoma and Paraganglioma
  Malak Itani and Joyce Mhlanga.
  • INTRODUCTION
  • IMAGING INDICATIONS
  • ANATOMIC IMAGING
  • PHYSIOLOGIC IMAGING
  • POSITRON EMISSION TOMOGRAPHY
  • RECENT ADVANCES IN IMAGING
  • CONCLUSION
  • REFERENCES
• 4. Surgical and Pharmacological Management of Functioning Pheochromocytoma and Paraganglioma
  Maria Isabel Del Olmo García, Rosana Palasí, Rosa Cámara Gómez, José Luis Ponce Marco, and Juan Francisco Merino-Torres.
  • INTRODUCTION
  • MEDICAL PREPARATION FOR SURGERY
  • COMBINED ALPHA- AND BETA-ADRENERGIC BLOCKADE
  • RECOMMENDED ALGORITHMS
  • HOSPITAL ADMISSION
  • SURGERY
  • POSTOPERATIVE MANAGEMENT
  • SURGICAL COMPLICATIONS
  • CONCLUSION
  • REFERENCES
• 5. Carotid Body and Vagal Paragangliomas: Epidemiology, Genetics, Clinicopathological Features, Imaging, and Surgical Management
  Sampath Chandra Prasad, Carlo Terenzio Paties, Mattia Russel Pantalone, Renato Mariani-Costantini, and Mario Sanna.
  • INTRODUCTION
  • EPIDEMIOLOGY AND GENETICS
  • GROSS PATHOLOGY
  • HISTOPATHOLOGY
  • DIFFERENTIAL HISTOPATHOLOGICAL DIAGNOSIS
  • METASTATIC BEHAVIOR
  • CORRELATIONS BETWEEN GENETIC AND PATHOLOGICAL FEATURES
  • CLINICAL FEATURES
  • DIAGNOSTIC INVESTIGATIONS
  • CLINICAL CLASSIFICATION OF CBPS
  • EMBOLIZATION
  • SURGICAL TREATMENT
  • CONCLUSION
  • REFERENCES
• 6. Tympanojugular Paragangliomas: Surgical Management and Clinicopathological Features
  Sampath Chandra Prasad, Carlo Terenzio Paties, Francesca Schiavi, Diana Liberata Esposito, Lavinia Vittoria Lotti, Renato Mariani-Costantini, and Mario Sanna.
  • INTRODUCTION
  • CLINICAL PRESENTATIONS
  • DIAGNOSTIC WORKUP
  • CHOOSING THE RIGHT APPROACH FOR THE JUGULAR FOSSA
  • SURGICAL STEPS
  • EXTENSIONS OF THE INFRATEMPORAL FOSSA TYPE A APPROACH (ITFA-A)
  • TRANSCONDYLAR-TRANSTUBERCULAR EXTENSION OF THE ITFA-A
  • STEPS OF ITFA-A WITH TRANSTUBERCULAR-TRANSCONDYLAR EXTENSION
  • CONSIDERATIONS IN THE MANAGEMENT OF COMPLEX TJPS
  • GENETICS OF TJP
  • DEVELOPMENTAL ORIGIN
  • HISTOPATHOLOGY
  • CONCLUSION
  • REFERENCES

Paraganglioma: A Multidisciplinary Approach

ISBN: 978-0-9944381-7-1

DOI: http://dx.doi.org/10.15586/paraganglioma.2019

Edited by

Renato Mariani-Costantini, MD, Department of Medicine, Dentistry and Biotechnology, Unit of General Pathology, Center for Aging Science and Translational Medicine (CeSI-MeT), “G. d’Annunzio” University, Italy

Published by

Codon Publications

Brisbane, Australia

Copyright© 2019 Codon Publications

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First Published in June 2019

Printed in Australia