Paraganglioma: A Multidisciplinary Approach


Renato Mariani-Costantini, MD (ed)
Center of Aging Science and Translational Medicine (CeSI-MeT) Gabriele d’Annunzio University Chieti, Italy; Mario Sanna Foundation (MSF) Piacenza, Italy


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.


  • Front Matter
    Pages i-v
  • Foreword
    Cecilia Söderberg-Nauclér…..Pages vii-viii
  • Preface
    Renato Mariani-Costantini…..Pages ix-x
  • Contributors
  • Chapter 1: Genetics of Pheochromocytoma and Paraganglioma
    Bernardo Dias Pereira, Henrique Vara Luiz, Ana Gonçalves Ferreira, Jorge Portugal…..Pages 1-22
  • Chapter 2: Biochemical Diagnosis of Pheochromocytoma and Paraganglioma
    Ying Shen, Liming Cheng…..Pages 23-39
  • Chapter 3: Imaging of Pheochromocytoma and Paraganglioma
    Malak Itani, Joyce Mhlanga…..Pages 41-61
  • Chapter 4: Surgical and Pharmacological Management of Functioning Pheochromocytoma and Paraganglioma
    Maria Isabel Del Olmo García, Rosana Palasí, Rosa Cámara Gómez, José Luis Ponce Marco, Juan Francisco Merino-Torres…..Pages 63-80
  • Chapter 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, Mario Sanna…..Pages 81-98
  • Chapter 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, Mario Sanna…..Pages 99-123
  • Index
    Pages 125-127

Author Biography

Renato Mariani-Costantini, MD, Center of Aging Science and Translational Medicine (CeSI-MeT) Gabriele d’Annunzio University Chieti, Italy; Mario Sanna Foundation (MSF) Piacenza, Italy


High_Definition_RMC_photo_250.pngRenato Mariani-Costantini is Full Professor of General Pathology in the Medical School of “G. d’Annunzio University”, Chieti, Italy. He graduated from the University of Rome “La Sapienza” Medical School (1976) and was a resident in anatomic pathology at George Washington University, Washington, D.C., USA (1978–1981). He then served as associate researcher at the Istituto Nazionale per lo Studio e la Cura dei Tumori, Milan, Italy (1981–1983) and as Fogarty Visiting Associate in the Laboratory of Tumor Immunology and Biology, National Cancer Institute, NIH (1984–1987). From 1988 to 1994 he was an Associate Professor of General Pathology in the Faculty of Medicine, University of Chieti, where, since 1994, he is Full Professor of General Pathology and head of the Laboratory of General Pathology at the Center of Research on Ageing and Translational Medicine (CeSI-MeT). Professor Mariani-Costantini’s scientific activity has been mainly focused on the molecular and genetic epidemiology of gastrointestinal and mammary cancer and on the study of the etiology and development of head and neck paragangliomas. With regard to the latter, Prof. Mariani-Costantini proposed a new model for paraganglioma origin, where the tumor arises from mesenchymal stem-like cells with vasculo-neural differentiation potential, in keeping with the neural crest derivation of paraganglia and with the hyperplastic carotid body model.

Cover for Paraganglioma: A Multidisciplinary Approach
June 10, 2019

Details about the available publication format: Front Matter

Front Matter
ISBN-13 (15)