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MEDICAL RADIOLOGY

Radiation Oncology

Editors:

L. W. Brady, Philadelphia

H.-P. Heilmann, Hamburg

M. Molls, Munich

C. Nieder, Bodø

B. Jeremić ∙ S. Pitz (Eds.)

Primary Optic

Nerve Sheath

Meningioma

With Contributions by

G. Bednarz ∙ M. Buchgeister ∙ E. B. Butler ∙ S. T. Chao ∙ J. O. Hernandez ∙ J. Hinojosa

B.Jeremić ∙ J. S. Kennerdell ∙ D. Linero ∙ S. S. Lo ∙ M. F. Mafee ∙ J. H. Naheedy

A. C. Paulino ∙ F. Paulsen ∙ S. Pitz ∙ G. Reifenberger ∙ M. J. Riemenschneider ∙ J. H. Suh B. S. Teh ∙ R. E. Turbin ∙ S. Villà ∙ M. Werner-Wasik ∙ H. Wilhelm

Foreword by

L.W. Brady, H.-P. Heilmann, M. Molls, and C. Nieder

With 102 Figures in 134 Separate Illustrations

123

Branislav Jeremić, MD, PhD

Susanne Pitz, MD, PhD

International Atomic Energy Agency

University Eye Hospital

Wagramer Strasse 5

Langenbeckstrasse 1

P.O. Box 100

55101 Mainz

1400 Vienna

Germany

Austria

 

MEDICAL RADIOLOGY ∙ Diagnostic Imaging and Radiation Oncology Series Editors:

A.L. Baert ∙ L.W. Brady ∙ H.-P. Heilmann ∙ M. Knauth ∙ M. Molls ∙ C. Nieder ∙ K. Sartor

Continuation of Handbuch der medizinischen Radiologie

Encyclopedia of Medical Radiology

ISBN 978-3-540-77557-7

e-ISBN 978-3-540-77558-4

DOI 10.1007/978-3-540-77558-4

Library of Congress Control Number: 2008925011

© 2008 Springer-Verlag Berlin Heidelberg

This work is subject to copyright. All rights are reserved, wether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broad-casting, reproduction on microfilm or any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in it current version, and permission for use must always be

obtained from Springer. Violations are liable to prosecution under the German Copyright Law.

The use of general descriptive names, registed names, trademarks etc. in this publication 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.

Product liability: the publishers cannot guarantee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such information by consulting the relevant literature.

Cover design: Frido Steinen-Broo, eStudio Calamar, Spain

Production, reproduction and typesetting: le-tex publishing services oHG, Leipzig, Germany

Printed on acid-free paper

9 8 7 6 5 4 3 2 1

springer.com

DEDICAtION

To my wife Aleksandra and my daughter Marta, for their endless encouragement and support during this project and throughout my career.

B. Jeremić

 

 

 

 

D

PROOFS

 

 

 

 

 

 

 

 

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u

 

 

 

 

 

Für Andreas

S. Pitz

978-3-540-77557-7_FRO NtM At tE R_2008-08-06_1

Foreword

Optic nerve sheath meningiomas represent a rare tumor about which very little has been published in the literature over the last 50 years. Therefore, there remains a major lack of information relating to the disease process and how best to treat such tumors. These tumors can arise intraorbitally, intracanalicularly, or intracranially. In general, they proceed by local continuous extension along the optic canal into the orbit or into the base of the brain.

This book — put together by Professor Branislav Jeremić and Professor Susanne Pitz — covers all the important aspects of the diagnosis and treatment of this disease process. The authors represent experts in the field relative to this disease process and their recommendations relative to general considerations, clinical presentation, the necessary clinical examinations, and tech-

patients can be treated adequately and appropriatelyPROOFSwith the newest technologies in radiation

niques for diagnosis with imaging and histology are very appropriately and properly presented.

From these data, treatment options can be developed that allow for surgical intervention or external beam radiation therapy or stereotactic fractionated radiation therapy using the newest of

technologies available in radiation oncology.

Aggressive intervention and appropriate proper treatment are associated with good results relative to management, and emerging from these discussions is the fact that the majority of

oncology.

 

 

D

 

The work presented by Professors JeremićE and Pitz represent a significant and important doc-

umentation of the approach to treatmentt in this disease process.

 

Philadelphia

 

 

C

Luther W. Brady

 

E

Hamburg

 

R

 

Hans-Peter Heilmann

Munich

 

R

 

Michael Molls

Bodø

 

CO

 

Carsten Nieder

 

 

 

 

 

N

 

 

u

 

 

 

978-3-540-77557-7_FRO NtM At tE R_2008-08-06_1

Preface

As an extremely rare and benign disease with virtually no lethal outcomes, optic nerve sheath meningioma has traditionally been treated worldwide with different approaches, and without a consensus on both how to diagnose and treat it. With only occasional reports in the literature, and with those reports being predominantly single-institutional experiences with few anecdotal cases discussed, optic nerve sheath meningioma remains on the fringes of oncological “happenings” and is rarely covered by the media.

Why then write a book about it?

Because time has come to begin a new chapter in this story. Many years have been spent improving our clinical understanding of the disease, our ability to diagnose it better and the treatments available to patients. These developments have led to a shift in treatment towards non-surgical options, primarily radiotherapy. With the safer and more precise use of CT and/or MRI, and the development of computer-driven treatment planning and execution, radiotherapy has become capable of delivering adequate doses to the tumor with minimal surrounding/safety margins, which

 

D

has led to lower toxicity. The most recent resultsPROOFSobtained with stereotactic fractionated radiation

 

E

therapy indicates that a change in the existing practice in a majority of patients is needed.

This book is a summary of what wethave learned and what we know today. It also represents a

comprehensive guide to the mostCimportant aspects of the diagnosis and treatment of the dis-

ease.

R

E

A distinguished group ofRoptic nerve sheath meningioma experts have contributed to this book.

 

CO

These colleagues have spent their professional lives dedicated to better understanding and treat-

 

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ing this disease, and have made substantial contributions to the field in recent decades. With their work and dedication, we have been able to create a book that presents the current state of the art in the fielduof diagnosis and treatment of optic nerve sheath meningioma, and the effort should lead to a more optimized and individualized approach in patients with this disease.

We would like to thank all of our former and current colleagues with whom we have collaborated during the past decade. Their tireless efforts and dedication to the cause has made our professional lives more interesting and rewarding.

We would also like to express our thanks to the Alexander von Humboldt Foundation, Bonn, for its continuous support of B. Jeremić since 1998. Special thanks to Ms. Ursula Davis and Ms. Anne Strohbach for their kind, patient, flexible and effective management during the preparation of this book. Without them, the book would not have been possible.

Vienna

Branislav Jeremić

Mainz

Susanne Pitz

978-3-540-77557-7_FRO NtM At tE R_2008-08-06_1

Contents

1

Overall Introduction, Problem Definition, Incidence . . . . . . . . . . . . . . . . . . . . .

 

Branislav Jeremić and Susanne Pitz

 

2

Clinical Presentation . .

. . . . . .

. . .

. . . .

. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

 

Susanne Pitz and Helmut Wilhelm

 

3

Clinical Evaluation of Primary Optic Nerve Sheath Meningioma . . . . . . . . . .

 

Roger E. Turbin and John S. Kennerdell

4

 

 

 

 

PROOFS

Imaging Diagnosis of the Optic Nerve Sheath Meningioma . . . . . . . . . . . . . . .

 

Mahmood F. Mafee and John H. Naheedy

5

Histology and Molecular Genetics . . .

. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

 

 

 

 

D

 

 

Markus J. Riemenschneider and Guido Reifenberger

 

 

 

E

 

 

 

t

 

 

6

 

C

 

 

 

Surgery in Primary Optic Nerve Sheath Meningioma . . . . . . . . . . . . . . . . . . . . .

 

E

 

 

 

 

Roger E. Turbin and John S. Kennerdell

 

R

 

 

 

 

7

Conventional RadiationR

Therapy in Primary Optic Nerve Sheath

 

Meningioma . . . . . . . . . .

. . . . . .

. . .

. . . . .

. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

 

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Branislav JeremićCO

 

 

 

 

8

u

 

 

 

 

3D Conformal RT . . . . .

. . . . . .

. . .

. . . . .

. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

 

Simon S. Lo, Samuel T. Chao, and John H. Suh

9

Intensity Modulated Radiotherapy for Optic Nerve Sheath Meningioma . . .

 

Jose Hinojosa, Bin S. Teh, Arnold C. Paulino,

 

Jorge Omar Hernandez, and E. Brian Butler

1

7

17

39

55

67

77

85

95

10 Stereotactic Radiation Therapy in Primary Optic Nerve Sheath

 

Meningioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

105

Branislav Jeremić, Maria Werner Wasik, Salvador Villà,

 

Frank Paulsen, Greg Bednarz, Dolors Linero, and Markus Buchgeister

 

978-3-540-77557-7_FRO NtM At tE R_2008-08-06_1

XII Contents

Subject Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

129

List of Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

133

 

 

 

 

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PROOFS

 

 

 

 

 

 

 

 

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978-3-540-77557-7_FRO NtM At tE R_2008-08-06_1

Overall Introduction,

1

Problem Definition, Incidence

Branislav Jeremić and Susanne Pitz

C o n t e n t s

 

 

 

 

 

 

K E Y P O I N t S

 

 

 

 

 

 

 

1.1

Introduction 1

 

 

 

 

 

 

 

 

ONSM is a rare tumour. It is usually separated

1.2

Incidence

4

 

 

 

 

into the primary ONSM (pONSM) (intraorbital or

 

 

 

 

 

 

 

 

References

5

 

 

intracanalicular) and secondary (sONSM) (intra-

 

 

 

 

 

 

cranial). The tumour can also originate within the

 

 

 

 

 

 

optic nerve sheath in the orbit or optic canal and

 

 

 

 

 

 

may grow intracranially to involve various struc-

 

 

 

 

 

 

tures there. ONSM can also present in a bilateral

 

 

 

 

 

 

form. pONSM represent approximately 96% of

 

 

 

 

 

 

all intraorbital and approximately 1%–2% of all

 

 

 

 

 

 

D

 

 

 

 

 

 

intracranialPROOFSmeningiomas. It is the second most

 

 

 

 

 

 

common orbital tumour after optic nerve gliomas,

 

 

 

 

 

 

Ebut represents only 10% of all ONSM, all the oth-

 

 

 

 

 

t ers (90%) being secondary ONSM. Of the pONSM,

 

 

 

 

 

C

approximately 96% are true pONSM and only 4%

 

 

 

 

E

were considered ectopic; pONSM typically de-

 

 

 

 

R

 

velop in middle aged women with the proportion

 

 

 

 

R

 

of females ranging from 70 to 80, although they do

 

 

 

 

CO

 

occur in children as well.

 

 

 

N

 

 

 

 

 

 

 

 

 

1.1

 

 

u

 

 

 

 

Introduction

Optic nerve sheath meningioma (ONSM) is a rare tumour. It is usually separated into the primary ONSM (pONSM) which arise from the cap cells of the arachnoid surrounding the intraorbital (Figs. 1.1–1.3) or, less commonly, the intracanalicular portions of the optic nerve (Figs. 1.4–1.7), and secondary (sONSM), which

B. Jeremić, MD, PhD

International Atomic Energy Agency, Wagramer Strasse 5, P.O.

Box 100, 1400 Vienna, Austria

S. Pitz, MD, PhD

University Eye Hospital, Langenbeckstrasse 1, 55101 Mainz,

Germany

arise intracranially, usually from the sphenoid ridge or tuberculum sellae and subsequently invade the optic canal and orbit by extending between the dura and arachnoid of the optic nerve in these regions. The term ONSM thus does not imply a definite site of origin. Nevertheless, once this type of tumour gains access to the subdural space of the intracanalicular or intraorbital optic nerve, the tumour grows up and down the sheath, invading the dura and obliterating the pial blood supply. In most instances, the tumour encircles the optic nerve without invading it. In other cases, the tumour may invade the nerve by growing along the fibrovascular septa (Samples et al. 1983; Probst et al. 1985). Such a tumour may eventually surround and obstruct the central retinal vein, central retinal artery, or both

978-3-540-77557-7_1_2008-08-06_1