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Updates in Surgery Guido Gasparri Michele Camandona Nicola Palestini Editors Primary, Secondary and Tertiary Hyperparathyroidism Diagnostic and Therapeutic Updates
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Primary, Secondary and Tertiary Hyperparathyroidism

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Primary, Secondary and Tertiary Hyperparathyroidism Diagnostic and Therapeutic Updates
Updates in Surgery
Primary, Secondary and Tertiary Hyperparathyroidism
Diagnostic and Therapeutic Updates
Foreword by Francesco Corcione
Editors Guido Gasparri Michele Camandona Department of Surgical Sciences Department of Surgical Sciences University of Turin, Turin, Italy University of Turin, Turin, Italy
Nicola Palestini Department of Surgery Città della Salute e della Scienza University Hospital, Turin, Italy
The publication and the distribution of this volume have been supported by the Italian Society of Surgery
ISSN 2280-9848 ISBN 978-88-470-5757-9 ISBN 978-88-470-5758-6 (eBook)
DOI 10.1007/978-88-470-5758-6
Library of Congress Control Number: 2015948222
© Springer-Verlag Italia 2016
This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recita- tion, broadcasting, reproduction on microfilms or in any other physical way, and transmission or infor- mation storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifically for the pur- pose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher’s location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this publica- tion does not imply , even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use.
While the advice and information in this book are believed to be true and accurate at the date of publi- cation, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein.
Cover design: eStudio Calamar S.L. External publishing product development: Scienzaperta, Novate Milanese (Milan), Italy Typesetting: Graphostudio, Milan, Italy
Springer-Verlag Italia S.r.l. – Via Decembrio 28 – I-20137 Milan Springer is a part of Springer Science+Business Media (www.springer.com)
Endocrine surgery certainly represents a highly specialized field, within which parathyroid surgery has long been considered especially specialized and mul- tidisciplinary.
In this very interesting volume, which is also enjoyable to read, my friend Guido Gasparri has succeeded in addressing all the important aspects of parathyroid diseases. Given the nature of the task, this success, while desir- able, was not inevitable.
Based on cooperation among surgeons, endocrinologists and other special- ists, the volume covers in detail all aspects from the origins of parathyroid sur- gery through to the modern treatment techniques. The embryological, anatom- ic, diagnostic and therapeutic characteristics of the different parathyroid dis- eases are clearly presented with the aid of excellent illustrations and up-to- date references.
This book highlights the remarkable advances made during recent years in the field of endocrine surgery, from the pioneering work through to the stan- dardization of very difficult technical procedures. Furthermore, everyone who is looking for a reliable update in the treatment of parathyroid diseases will find accurate messages and guidelines.
The most remarkable feature to emerge from this volume is the importance of the organizational set-up and the technological advances that offer the sur- geon support when performing such difficult and noble surgery. These aspects are crucial to the successful treatment of parathyroid diseases.
Thanks are due to Professor Gasparri and to all the eminent authors who have collaborated brilliantly to produce this book, which offers an easy-to- read and detailed update that will be of value for every surgeon.
Rome, September 2015 Francesco Corcione President, Italian Society of Surgery
Foreword
v
The discovery of the parathyroids dates back to the nineteenth century, but their function and the associated diseases remained poorly understood for a long time thereafter. In 1907, referring to the four tiny glands posterior to the thyroid, William Halsted observed that it was “hardly credible that the loss of bodies so tiny should be followed by a result so disastrous”. It was not until 1925 that the first surgical treatment of primary hyperparathyroidism was performed, by Felix Mandl in Vienna. Three years later, Isaac Olch of Barnes Hospital performed the first suc- cessful parathyroidectomy in the USA. After these initial attempts to treat primary hyperparathyroidism surgically, the disease was well described and was associated not only with solitary adenoma, but also with double adenomas and multiglandular hyperplasia. Conventional bilateral cervical exploration has remained the proce- dure of choice until the late 1990s, when minimally invasive procedures began to gain acceptance.
Today, hyperparathyroidism is not uncommon: it is the third most common endocrine disease, after diabetes and thyroid pathology. It seems certain that in the future biomolecular studies and genetic testing will open new horizons in the treat- ment of primary hyperparathyroidism. Studies on estrogen receptor gene polymor- phism are also very important, because they might influence both the peripheral action of parathyroid hormone and regulation of calcium secretion. An important issue is the role of the calcium sensing receptor mRNA in normal and abnormal parathyroid tissues, and this requires further investigation.
Another controversial point is the real incidence of asymptomatic hyperparathy- roidism. Analysis of outcomes after parathyroidectomy for asymptomatic primary hyperparathyroidism has demonstrated improvements in all aspects of health status 6 months postoperatively, the benefits being particularly evident with regard to body pain, energy levels, and emotional and physical function.
The exact role of autotransplantation and cryopreservation in parathyroid sur- gery for correction of postoperative hypocalcemia remains a matter of debate. Autotransplantation is a good standardized technique but the indications for cryop- reservation are rare and they should be refined.
vii
Preface
The aim of this book on primary, secondary, and tertiary hyperparathyroidism is to provide the reader with a complete understanding of the history, surgical anatomy, etiology, pathogenesis, clinical aspects, and medical and surgical thera- pies, including new avenues. The book is intended primarily for endocrine sur- geons, but also for endocrinologists, specialists in internal medicine, nephrologists, urologists, gynecologists, and radiologists.
We hope that readers will find subjects of relevance to daily practice and new technical points regarding minimally invasive surgery, intraoperative parathyroid hormone assay, and reoperations.
Turin, September 2015 Guido Gasparri Michele Camandona
Nicola Palestini
Prefaceviii
1 History of Parathyroids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Guido Gasparri, Michele Camandona, and Nicola Palestini
2 Surgical Anatomy of the Parathyroid Glands . . . . . . . . . . . . . . . . . . . 9 Nicola Palestini
3 Parathyroid Hormone Secretion and Action . . . . . . . . . . . . . . . . . . . . 21 Giancarlo Isaia, Lorenzo Marchese, Margherita Marchetti, Patrizia D’Amelio, and Giorgia Fornelli
4 Clinical Use of the Parathyroid Hormone Assay . . . . . . . . . . . . . . . . . 29 Giulio Mengozzi
5 Etiology and Pathogenesis of Primary Hyperparathyroidism and Hypercalcemias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41 Paolo P. Limone, Maurilio Deandrea, Elena Gamarra, Francesca Garino, Aurora Grassi, Gabriella Magliona, Alberto Mormile, Federico Ragazzoni, Maria Josefina Ramunni, and Paola Razzore
6 Clinical Manifestations of Primary Hyperparathyroidism . . . . . . . . . 57 Massimo Procopio, Marco Barale, Ruth Rossetto Giaccherino, Alessandro Piovesan, and Ezio Ghigo
7 Diagnosis and Differential Diagnosis of Primary Hyperparathyroidism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73 Fabio Orlandi, Federico Arecco, Stefania Corvisieri, and Ilaria Messuti
8 Management of Primary Hyperparathyroidism . . . . . . . . . . . . . . . . . 85 Michele Camandona
ix
Contents
9 Preoperative Localization for Parathyroid Surgery in Primary and Secondary Hyperparathyroidism . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Giovanni Gandini, Filippo Marchisio, Giuseppe Isolato, Antonella Sargiotto, Michela Zotta, and Gianni Bisi
10 Pathology of the Parathyroid Glands . . . . . . . . . . . . . . . . . . . . . . . . . . 111 Eleonora Duregon, Marco Volante, and Mauro Papotti
11 Parathyroid Exploration for Primary Hyperparathyroidism . . . . . . . 117 Guido Gasparri, Nicola Palestini, Milena Freddi, Gabriella Sisto, and Michele Camandona
12 Minimally Invasive Video-Assisted Parathyroidectomy . . . . . . . . . . . 133 Paolo Miccoli and Gabriele Materazzi
13 Surgical Technique in Reoperations . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 Rocco Bellantone, Emanuela Traini, Marco Raffaelli, and Celestino P. Lombardi
14 Genetic Syndromes Associated with Primary Hyperparathyroidism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 Maria L. Brandi and Francesco Tonelli
15 Parathyroid Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183 Gennaro Favia and Maurizio Iacobone
16 Hypocalcemia After Parathyroidectomy . . . . . . . . . . . . . . . . . . . . . . . . 193 Matteo A. Cannizzaro and Massimiliano Veroux
17 Secondary Hyperparathyrodism in Adult Predialysis and Dialysis Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 201 Luigi Biancone, Gianluca Leonardi, Massimo Gai, and Giuseppe P. Segoloni
18 Indications for Parathyroidectomy in End-Stage Renal Disease and After Renal Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 215 Guido Gasparri, Michele Camandona, Michele Giaccone, Mario Pio Capozzi, and Nicola Palestini
19 Patient Information for Surgery in Primary and Secondary Hyperparathyroidism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231 Pier Giorgio Nasi and Lodovico Rosato
Contentsx
Federico Arecco Endocrinology Unit, Gradenigo Hospital, Turin, Italy
Marco Barale Department of Medical Sciences, University of Turin, Turin, Italy
Rocco Bellantone Endocrine and Metabolic Surgery Unit, Agostino Gemelli Hospital, Università Cattolica del Sacro Cuore, Rome, Italy
Luigi Biancone Department of Medical Sciences, University of Turin, Turin, Italy
Gianni Bisi Department of Medical Sciences, University of Turin, Turin, Italy
Maria L. Brandi Department of Surgery and Translational Medicine, University of Florence, Florence, Italy
Michele Camandona Department of Surgical Sciences, University of Turin, Turin, Italy
Matteo A. Cannizzaro Department of Medical and Surgical Sciences and Advanced Technologies, University of Catania, Catania, Italy
Mario Pio Capozzi Department of Surgery, Città della Salute e della Scienza University Hospital, Turin, Italy
Stefania Corvisieri Department of Oncology, University of Turin, Turin, Italy
Patrizia D’Amelio Department of Medical Sciences, University of Turin, Turin, Italy
Maurilio Deandrea Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Eleonora Duregon Department of Oncology, University of Turin, Turin, Italy
Gennaro Favia Endocrine Surgery Unit, 3rd Surgical Clinic, University Hospital of Padua, Padua, Italy
Contributors
xi
Giorgia Fornelli Department of Medical Sciences, University of Turin, Turin, Italy
Milena Freddi Department of Surgery, Città della Salute e della Scienza University Hospital, Turin, Italy
Massimo Gai Division of Nephrology, Dialysis and Transplantation, Città della Salute e della Scienza University Hospital, Turin, Italy
Elena Gamarra Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Giovanni Gandini Department of Surgical Sciences, University of Turin, Turin, Italy
Francesca Garino Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Guido Gasparri Department of Surgical Sciences, University of Turin, Turin, Italy
Ezio Ghigo Department of Medical Sciences, University of Turin. Turin, Italy
Michele Giaccone Department of Surgery, Città della Salute e della Scienza University Hospital, Turin, Italy
Aurora Grassi Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Maurizio Iacobone Endocrine Surgery Unit, 3rd Surgical Clinic, University Hospital of Padua, Padua, Italy
Giancarlo Isaia Department of Medical Sciences, University of Turin, Turin, Italy
Giuseppe Isolato Department of Diagnostic Imaging and Radiotherapy, Città della Salute e della Scienza University Hospital, Turin, Italy
Gianluca Leonardi Division of Nephrology, Dialysis and Transplantation, Città della Salute e della Scienza University Hospital, Turin, Italy
Paolo P. Limone Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Celestino P. Lombardi Endocrine and Metabolic Surgery Unit, Agostino Gemelli Hospital, Università Cattolica del Sacro Cuore, Rome, Italy
Gabriella Magliona Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Lorenzo Marchese Department of Medical Sciences, University of Turin, Turin, Italy
Margherita Marchetti Department of Medical Sciences, University of Turin, Turin, Italy
Filippo Marchisio Department of Diagnostic Imaging and Radiotherapy, Città della Salute e della Scienza University Hospital, Turin, Italy
Gabriele Materazzi Department of Surgical, Medical, Molecular and Critical Area Pathology, University of Pisa, Pisa, Italy
Giulio Mengozzi Department of Laboratory Medicine, Città della Salute e della Scienza University Hospital, Turin, Italy
Ilaria Messuti Department of Oncology, University of Turin, Turin, Italy
Paolo Miccoli Department of Surgical, Medical, Molecular and Critical Area Pathology, University of Pisa, Pisa, Italy
Alberto Mormile Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Pier Giorgio Nasi Thyroid Disease and Endocrine Surgery, Sedes Sapientiae Private Hospital, Turin, Italy
Fabio Orlandi Department of Oncology, University of Turin, Turin, Italy
Nicola Palestini Department of Surgery, Città della Salute e della Scienza University Hospital, Turin, Italy
Mauro Papotti Department of Oncology, University of Turin, Turin, Italy
Alessandro Piovesan Division of Oncological Endocrinology, Città della Salute e della Scienza University Hospital, Turin, Italy
Massimo Procopio Division of Endocrinology, Diabetes and Metabolism, Città della Salute e della Scienza University Hospital, Turin, Italy
Marco Raffaelli Endocrine and Metabolic Surgery Unit, Agostino Gemelli Hospital, Università Cattolica del Sacro Cuore, Rome, Italy
Federico Ragazzoni Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Maria Josefina Ramunni Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Paola Razzore Division of Endocrinology, Diabetes and Metabolism, A.O. Ordine Mauriziano, Turin, Italy
Lodovico Rosato Department of Surgery, ASL4, Ivrea Hospital, Ivrea, Italy
Ruth Rossetto Giaccherino Division of Endocrinology, Diabetes and Metabolism, Città della Salute e della Scienza University Hospital, Turin, Italy
Contributors xiii
Antonella Sargiotto Department of Diagnostic Imaging and Radiotherapy, Città della Salute e della Scienza University Hospital, Turin, Italy
Giuseppe P. Segoloni Department of Medical Sciences, University of Turin, Turin, Italy
Gabriella Sisto Department of Surgery, Città della Salute e della Scienza University Hospital, Turin, Italy
Francesco Tonelli Department of Surgery and Translational Medicine, University of Florence, Florence, Italy
Emanuela Traini Endocrine and Metabolic Surgery Unit, Agostino Gemelli Hospital, Università Cattolica del Sacro Cuore, Rome, Italy
Massimiliano Veroux Department of Medical and Surgical Sciences and Advanced Technologies, University of Catania, Catania, Italy
Marco Volante Department of Oncology, University of Turin, Turin, Italy
Michela Zotta Department of Medical Sciences, University of Turin, Turin, Italy
Contributorsxiv
G. Gasparri et al. (Eds), Primary, Secondary and Tertiary Hyperparathyroidism, Updates in Surgery DOI: 10.1007/978-88-470-5758-6_1, © Springer-Verlag Italia 2016
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1
G. Gasparri () Department of Surgical Sciences, University of Turin Turin, Italy e-mail: [email protected]
1.1 Owen’s discovery
The history of the parathyroids — the small glands of 40 to 50 mg, about which William Halsted declared: “It seems hardly credible that the loss of bodies so tiny should be followed by a result so disastrous” [1] — began in 1849 when Sir Richard Owen, Professor and Conservator of the Museum at the Royal College of Surgeons of England, discovered, while doing an autopsy on an Indian rhinoceros which had died after a scuffle with an elephant, “a small, compact, yellow glandular body attached to the thyroid at the point where the veins emerge” [2]. This great Indian rhinoceros, Rhinoceros unicornis (the African one has two horns), was purchased by the Zoological Society of London in 1834. Cave [3], in 1953, pointed out that Owen was the first to dis- cover these glands because “although Remak of Berlin described what may have been parathyroid glands in 1855, Owen’s paper was published in 1852”. So the parathyroid eponym began with the rhinoceros.
1.2 Sandström’s discovery
The anatomical description of the parathyroid glands in humans is attributed to two anatomists, Swedish Ivar Sandström, in 1880 [4], born by a strange coin- cidence in the year when Owen published his observations (1852), and Baber, an Englishman, who, one year later, in 1881, described them. They differenti- ated histologically these glands from the thyroid tissue and from the lymph nodes. Baber was attributed with having identified the parafollicular or C-cells
of the thyroid, the description of which would only be completed by Pearse in 1966. The story of Sandström is interesting [5]; the fifth of seven children, he lost his father while he was in preschool. He began his medical studies in the fall of 1872 and finished them after 15 years when at that time studies were usu- ally completed in ten years.
He had a summer job in the Anatomy department in Uppsala, where he was paid a salary, which was not very high, but was sufficient to allow him to con- tinue studying. He was melancholy by nature and his job in the department was to dissect animals. In 1880, he wrote in a paper: “Almost three years ago I found on the thyroid of a little dog a tiny growth, barely the size of a hemp seed, which lay enclosed within the same capsule of tissues as that gland, though it was dissimilar in its lighter color”. He named these structures “glan- dulae parathyroidea”. He continued with his research on cats, oxen, horses and rabbits and finally he performed 50 dissections on humans finding four glands in the neck of 43 of them. It is very exciting to read his conclusions: “Although the glands were generally united with the thyroid by means of soft connective tissue, they were often movable against its capsule. Many of the glands are well-defined fat lobules separated from the thyroid gland capsule. To each gland there are one or more small arteriole branches from the inferior thyroid artery, and in the interstitial tissue there are often considerable fat cells and may be so numerous that the parenchyma of the gland…