Table Of ContentTumours of the
Central Nervous System
in Infancy and Childhood
Edited by
D. Voth P. C. Langmaid
Gu~ahr
With l36 Figures and 116 Tables
Springer-Verlag Berlin Heidelberg New York 1982
Professor Dr. med. DIETER V OTH, N eurochirurgische Klinik und Poliklinik,
Klinikum der Johannes Gutenberg-Universitat, LangenbeckstraBe I, D-6500 Mainz
Professor Dr. med. PETER GUTJAHR, Kinderklinik und Kinder-Poliklinik, Klinikum
der Johannes Gutenberg-Universitat, LangenbeckstraBe 1, D-6500 Mainz
Mr. CHARLES LANGMAID, 174, Lake Road East, Roath Park, Cardiff CF2 5NR,
South Glam. Wales, Great Britain
ISBN-13: 978-3-642-95415-3 e-ISBN-13: 978-3-642-95413-9
DOT: 10.1007/978-3-642-95413-9
LIbrary ot Congress Cataloglllg III PublIcatIOn Data
Tumours of the central nervous system in infancy and childhood. Lectures given at a congress. held in
Mainz. between 22 and 24 October 1981. I. Tumours in children-Congresses. 2. Youth-Disease~ongresses.
I. Voth, D. (Dieter), 1935- . II. Gutjahr, P. (Peter), 1945- . III. Langmaid, C. (Charles), 1913- .
[DNLM: I. Brain neoplasms-In infancy and childhood. 2. Spinal cord neoplasms-In infancy and child
hood. WL 358 T926] RC281.C4 T857 1982 618.92'9928 82-10745
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© by Springer-Verlag Berlin Heidelberg 1982.
Softcover reprint of the hardcover 1s t edition 1982
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specific statement, that such names are exempt from the relevant protective laws and regulations and there
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Preface
Tumours of the central nervous system in infancy and childhood show so many di
verse pathomorphological characteristics and present so many diagnostic problems
that a congress dealing specifically with the subject and thus bringing together a
wide range of experts in the field seemed called for. The programme of the congress,
. held in Mainz between 22 and 24 October 1981, was designed to provide compre
hensive coverage of diagnosis and the various therapeutic procedures, as well as of
basic research in the field. The various lectures given are contained in this book,
which thus reflects the complete spectrum of topics discussed.
The interest generated by the congress amply justified our decision to organize
it. Representatives of various specialities, such as neuropathology, paediatrics, on
cology, radiology, neurosurgery, paediatric surgery and neurology, and, last but
not least, basic research, provided lively and interesting lectures which admittedly
raised more problems than they solved. In addition to the actual papers presented,
we attached considerable importance to the different opinions voiced during the
congress, as reflected in the discussions included at the end of each chapter.
In organizing the congress we were supported by Prof. Dr. J. M. Schroder,
Aachen, Prof Dr. J. Kutzner, Mainz, and Dr. K. Kretzschmar, Mainz; we are very
grateful for their help. Our thanks are also due to the publisher, who helped us to
edit the congress reports in the form of a monograph. Weare extremely hopeful that
the book will present those interested with a concise account of "tumours of the cen
tral nervous system in infancy and childhood" and provide them with a summary of
the present state of knowledge which will form a basis for further investigations.
Mainz - Cardiff THE EDITORS
Contents
Chapter 1 Basic Research and Special Pathology
Intracranial Tumours ofInfancy and Childhood. By K. J. Zulch.
With I Figure and 2 Tables . . . . . . . . . . . . . . . .
Remarks on the WHO Classification of Tumours of the Central Nervous
System. By O. Stochdorph. With 2 Figures ......... 17
Developmental Neuro-Oncogenesis in Experimental Animals.
By P. Kleihues, S. Bamborschke, M. Kiessling, and O. Wiestler.
With 4 Figures and 1 Table . . . . . . . . . . . . . . . 19
Brain Tumours and Genetics. By J. Spranger. With 4 Figures and 4 Tables 32
Interferon and Antitumour Effects. By H.-J. Rothig. With 1 Table . . 42
Rare Intracranial Tumours in Infancy and Childhood. By K. Jellinger
and E. Machacek. With 3 Figures and 4 Tables ......... 44
Intracranial and Spinal Tumours in Newborns and Infants. By H. Gerlach,
W. Janisch, and D. Schreiber. With I Figure and 4 Tables . . . . . . 53
The Usefulness ofImmunocytological Demonstration of Glial Fibrillary
Acidic Protein for Diagnostic Problems of Cerebral Tumours.
By K. H. H. Merkel, M. Zimmer, W. Wahlen, and E. Schwarz.
With 1 Figure and 1 Table . . . . . . . . . . . . . . . . . . . . 58
CNS Tumours in Infancy, Childhood, and Adolescence. By D. Schreiber,
W. Janisch, and H. Gerlach. With I Figure and 5 Tables ....... 62
Congenital Intracranial Teratoma in a Newborn. By H. Klein and J. Klein.
With 2 Figures 69
Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . 72
Chapter 2 Clinical Presentation, Diagnosis, Follow-Up Studies
Clinical Presentation of Space-Occupying Lesions of the Central Nervous
System. By G. Jacobi. With 2 Figures and 10 Tables ...... 74
Cytology of Cerebrospinal Fluid in Children with Brain Tumours.
By W. Dorffel. With 1 Figure and 2 Tables .......... 85
VIII Contents
Electrophysiological Diagnosis and Follow-Up Controls in Cases of
Tumours of the Optic Chiasm. By D. Wenzel, D. Harms, U. Brandl,
M. Klinger, and F. Albert. With I Figure and 2 Tables ...... 90
Electroencephalograms in Long-Term Survivors of Primary Intracranial
Tumours of Childhood. By E. Dieterich, B. Goebel, and P. Gutjahr.
With 2 Tables . . -. . . . . . . . . . . . . . . . . . . . . . . 95
Early Auditory Evoked Potentials (EAEP) in Children with Neoplastic
Lesions in the Brain Stem. By K. Maurer and M. Rochel. With 4 Figures
and 1 Table. . . . . . . . . . . . . . . . . . . . . . . . . . 99
Conventional Investigations ofCNS Tumours in Children. By C. R. Fitz.
With 9 Figures and 1 Table . . . . . . . . . . . . . . . . . 105
Computer Tomographic Diagnosis ofCNS Tumours in Childhood.
By E. Kazner and K. Kretzschmar. With 4 Figures . . . . . . . 115
Radionuclide Imaging of Cerebral Tumours in Children. By K. Hahn.
With 2 Figures and 3 Tables .............. 122
Follow-Up Studies of Brain Tumours in Infants and Children.
By K. Kretzschmar. With 5 Figures and 3 Tables ..... 130
Computed Tomography and/or Ventriculography? By S. Wende,
T. Kishikawa, N. Huwel, E. Kazner, Th. Grumme, and W. R. Lanksch.
With 1 Table . . . . . . . . . . . . . . . . . . . . . . . . . 142
Ventriculography as a Diagnostic Procedure in CNS Tumours in Childhood:
Its Indications and Interpretation. By H. Altenburg, M. Brandt,
and H.-J. Konig. With 2 Figures . . . . . . . . . . . . . . . . .. 145
The Diffuse Brain Stem Astrocytoma in Childhood: Morphology, Course,
and Prognosis. By H. P. Schmitt and W. Zeisner. With 1 Figure
and 4 Tables . . . . . . . . . . . . . . . . . . . . . . . 149
Astrocytoma with Extracranial Metastases. By R. Bubl, H. R. Hirt,
H. Ohnacker, and A. Probst ................ 165
Diktyoma (Medulloepithelioma). A Rare Neuro-Epithe1ial Tumour
of the Eye. By G. Knoepfle, U. Bode, R. Hoischen, and H. J. Foedisch.
With 2 Figures and 1 Table . . . . . . . . . . . . . . . . . 169
Intracranial Teratoma ofa Newborn. Computer Tomographic and
Morphological Findings. By G. Ebhardt, H. Traupe, J. Kanner,
and L. Goerke. With 3 Figures ............... 174
The Isolated Fourth Ventricle: Review of Current Concepts and Report
on Three Cases in Children. By E. Mahlmann, D. Voth, and M. Schwarz.
With 2 Figures . . . . . . . . . . . . . . . . . . . . . . . .. 180
Follow-Up Findings with CT Scan and Ventriculography After
Radiotherapy ofInoperable Midline Tumours in Childhood.
By H.-J. Konig and H. Altenburg. With 1 Figure 186
Discussion . . . . . . . . . . . . . . . . . . . . . 188
Contents IX
Chapter 3 Surgical Treatment
Evolutional, Developmental, and Clinical Aspects of Brain Stem
Organisation. By P. Glees. With 9 Figures and 2 Tables . . . . 190
Standard Techniques and Microsurgical Procedures in the Removal
of Supratentorial and Infratentorial Tumours. By D. Voth. With 9 Figures 199
Additional Operative Procedures (Shunt Systems, Leksell and Torkildsen
Drainage). By M. Schwarz and D. Voth . . . . . . . . . . . . 214
The Significance of Continuous Recording ofIntracranial Pressure
in Children with Brain Tumours. By M. Brock and H.-J. Schmitz.
With 2 Figures . . . . . . . . . . . . . . . . . . . . . . 218
Preoperative and Postoperative Intensive Care and Oedema Therapy.
By J. Otte, P. Emmrich, and G. Meinig. With 1 Figure and 3 Tables 222
Therapy ofPeritumoural Brain Oedema. By G. Meinig. With 3 Figures
and 1 Table. . . . . . . . . . . . . . . . . . . . . . . . . . 229
CT-Stereotactic Biopsy of Brain Tumours. By F. Mundinger.
With 3 Figures and 3 Tables ............ . 234
Diagnostic Potential of Stereotactic Biopsy of Brain Tumours. A Report
of400 Cases. By M. Kiessling, J. Anagnostopoulos, G. Lombeck,
and P. Kleihues. With 4 Figures and 5 Tables . . . . . . . . . . . 247
Internal Shunt or Perioperative Pressure-Controlled Ventricular Fluid
Drainage (C-VFD) in Children and Juveniles with Infratentorial Tumours.
By K. E. Richard, R. Heller, and R. A. Frowein. With 2 Figures
and 3 Tables . . . . . . . . . . . . . . . . . . . . . . . . .. 257
Indications and Experiences with the Ommaya-Reservoir for Treatment
ofCNS Neoplasms in Childhood. By K. Roosen, W. Havers, and J. Schaaf.
With 3 Figures and 2 Tables .................. 265
Indications for the Trans-Sphenoidal Approach to Craniopharyngioma
Operations in Youth and Childhood. By E. Halves and N. Sorensen.
With 2 Figures . . . . . . . . . . . . . . . . . . . . . . . . 270
Which Considerations Determine the Choice Between the Subfrontal and
Trans-Sphenoidal Approach to Tumours of the Sellar Region in Youth
and Childhood? By W. Ischebeck, S. Miiller, and W. Braun 276
The Influence of Different Antihypertensive Drugs on Intracranial
Pressure. By J. Hidding, C. Puchstein, and H. van Aken. With 2 Figures
and 1 Table. . . . . . . . . . . . . . . . . . . . . . . 278
Ventriculocisternostomy by the Method of Leks ell-Indications,
Technique, and Results. By D. Voth, K. Schiirmann, and M. Schwarz.
With 2 Figures 283
Discussion . . . . . . . . . . . . . . . . . . . . . . . . . 289
x
Contents
Chapter 4 Radiotherapy
The Radiosensitivity ofthe Infant Brain. By W~ Hinkelbein
and M. Wannenmacher. With 4 Figures ....... . 291
Radiation Technique and the Planning of Treatment in Brain Tumours
in Children. By J. Kutzner. With 3 Figures and 1 Table . . . . . 301
Experiences with the Treatment of Cystic Craniopharyngiomas by
Stereotactically Injected Radioisotopes. By V. Sturm, P. Georgi,
H. Netzeband, K. E. Scheer, L. Strauss, W. Schlegel, H. Scharfenberg,
H. Sinn, H. Gahbauer, and H. Penzholz. With 2 Figures ..... 310
Radiosurgery in Intracranial Tumours and Arteriovenous Malformations
in Children. By L. Steiner. With 3 Figures and 4 Tables . . . . . . 315
Radiation Therapy in the Treatment of the Astrocytomas and Midline
Tumours of Childhood. By M. Herbst. With 1 Figure and 4 Tables 325
Radiotherapeutic Principles in the Combined Treatment ofCNS Tumours
in Children. By M. Bamberg and E. Scherer. With 1 Figure and 3 Tables . 333
Postoperative Chemotherapy Prior to Radiotherapy in the Combined
Treatment of Medulloblastoma, Ependymoma and Tumours of the Pineal
Region: Early Results. By H. J. Habermalz, U. Stephani, H. Riehm,
and F. Hanefeld. With 3 Figures and 4 Tables . . . . . . . . . . . 338
Radiotherapy of Medulloblastoma: Radiation Technique, Results, and
Complications. By E. A. Bleher, P. G. Poretti, and P. C. Veraguth.
With 2 Figures . . . . . . . . . . . . . . . . . . . . . . 344
Report on the Medulloblastoma Study of the Society of Paediatric
Oncology - Present Position. By M. Neidhardt . . . . . . . . . 349
Data on the Biology of Medulloblastomas. By W. Muller, D. Afra,
O. Wi1cke, F. Slowik, R. Schroder, and M. Kordas. With 1 Figure
and 5 Tables . . . . . . . . . . . . . . . . . . . . . . . 350
Treatment of Meningeal Malignancy with Intrathecal 198Au-Colloid and
Methotrexate. The Example of Acute Lymphoblastic Leukaemia (ALL).
By O. Metz. With 2 Figures and 2 Tables . . . . . . . . . . 354
Infratentorial Tumours - Treatment and Results in 109 Children.
By P. Gutjahr, D. Voth, J. Heidfeld, B. Walther, J. Kutzner,
and K. Kretzschmar. With 5 Figures 358
Discussion . . . . . . . . . . . . . . . . . . . . . 365
Chapter 5 Chemotherapy, Late Effects, and After-Treatment
Cytostatic Monotherapy of Tumours of the Central Nervous System
in Children. By B. Kornhuber. With 1 Table ........ . 367
Contents XI
Rationale of Combined Chemotherapy of Brain Tumours. By J. Hildebrand
and D. Zegers de Beyl . . . . . . . . . . . . . . . . . . . . 374
Acute Toxicity of Chemotherapy of Tumours of the Central Nervous
System in Children. By W. Havers. With 1 Table . . . . . . . . . 378
Late Effects After Treatment of Brain Tumours in Childhood. By P. Gutjahr
and B. Walther. With 3 Tables ................... 383
Development After Treatment of Cerebellar Medulloblastoma in Childhood.
By B. Walther and P. Gutjahr. With 1 Figure and 4 Tables . . . . 389
Spongioblastoma in Childhood. Remarks on Problems in Diagnosis
and Treatment. By W. Entzian, L. Diaz, and H.-W. Schumacher.
With 1 Figure and 3 Tables . . . . . . . . . . . . . . . . . 399
Medulloblastoma. Relationship of Histologic Type to Survival.
By H. Arnold, H. D. Franke, G. Langendorf, R. Olotu, and J. Grosch-Womer.
With 4 Figures . . . . . . . . . . . . . . . . . . . . . . . .. 404
Ophthalmological Findings in Long-Term Survivors After Primary Brain
Tumours in Childhood. By R. Rochels and Th. Neuhann. With 1 Table 410
Endocrinological Investigations in 68 Children with Brain Tumours.
By W. Andler, K. Roosen, and H.-E. Clar. With 6 Tables ..... 415
Supratentorial Tumours in Infants and Children. By S. Tiyaworabun,
S. Kazkaz, N. Nicola, and M. Schirmer. With 3 Figures and 2 Tables 420
Discussion . . . . . . . . . . . . . . . . . . . . . . . 426
Final Comments and Summing-Up. By P. Gutjahr and D. Voth 427
Subject Index . . . . . . . . . . . . . . . . . . . . . . . . . .. 433
List of Contributors
Afra, D., Dr. med., National Institute of Neurosurgery, Amerikai lit 57,
H-1145 Budapest
Aken, H. van, Dr. med., Klinik fur Anasthesiologie und Intensivpflege, Universitat
Munster, Jungeblodtplatz 1, 0-4400 Munster
Albert, F., Dr. med., Neurochirurgische Klinik der Universitat Erlangen-Nurnberg,
Schwabachanlage 6 (Koptklinikum), 0-8520 Erlangen
Altenburg, H., Dr. med., Neurochirurgische Klinik und Poliklinik der Westfalischen
Wilhelms-Universitat, Jungeblodtplatz 1,0-4400 Munster
Anagnostopoulos, J., Dr. med., Abteilung fur Neuropathologie, Ludwig-Aschoff
Haus, Universitat Freiburg, Albertstra13e 19,0-7800 Freiburg
Andler, W., Dr. med., Kinderklinik, Universitats-Klinikum der Gesamthochschule
Essen, Hufelandstra13e 55, 0-4300 Essen
Arnold, H., Dr. med., Neurochirurgische Universitatsklinik Hamburg, Martini
straBe 52. 0-2000 Hamburg 20
Bamberg, M., PO Dr. med., Strahlenklinik am Klinikum der Gesamthochschule
Essen, Hufelandstra13e 55. 0-4300 Essen
Bamborschke, S., Dr. med., Abteilung fur Neuropathologie, Ludwig-Aschoff-Haus,
Universitat Freiburg, Albertstra13e 19,0-7800 Freiburg
Bleher. E. A., Dr. med .. Institut fur Strahlentherapie, Inselspital Bern, CH-3007 Bern
Bode, 0.. Dr. med., Kinderklinik der Universitat Bonn, Sigmund-Freud-Stra13e 35,
0-5300 Bonn-Venusberg
Brandl, 0., Dr. med., Kinderklinik der Universitat Erlangen-Nurnberg, Kranken
hausstraBe 12. 0-8520 Erlangen
Brandt, M .. Dr. med., Neurochirurgische Klinik und Poliklinik der Westfalischen
Wilhelms-Universitat. Jungeblodtplatz 1,0-4400 Munster
Braun, W., Prof, Dr. med .. Abteilung fur Neurochirurgie, Krankenhaus Bethesda,
HainstraBe 35, 0-5600 Wuppertall
Brock, M., Prof. Dr. med., Abteilung fur Neurochirurgie, Neurochirurgischel
Neurologische Klinik und Poliklinik, Universitatsklinikum Steglitz, Freie Univer
sitat Berlin, Hindenburgdamm 30, 0-1000 Berlin 45
XIV List of Contributors
Bubl, R., Dr. med., Kinderklinik, UniversiHit Basel, Romergasse 8, CH-4005 Basel
Clar, H.-E., Prof. Dr. med., Neurochirurgische UniversiUitsklinik der
Gesamthochschule Essen, HufelandstraBe 55, D-4300 Essen
Diaz, L., Dr. med., Neurochirurgische Universitatsklinik, Sigmund-Freud-StraBe 25,
D-5300 Bonn-Venusberg
Dieterich, E., PD Dr. med., Abteilung flir Neuropadiatrie, Universitatskinderklinik,
Schwanenweg, D-2300 Kiel
Dorffel, W., Dr. med., II. Kinderklinik, Stadtische Klinik Berlin-Buch, Wiltberg
straBe 50, DDR-1115 Berlin-Buch
Ebhardt, G., PD Dr. med., Max-Planck-Institut flir Hirnforschung Koln-Merheim,
Ostmerheimer StraBe 200, D-5000 Koln 91
Emmrich, P., Prof. Dr. med., Kinderklinik der Universitat Mainz, Langenbeck
straBe 1, D-6500 Mainz
Entzian, W., Prof. Dr. med., Neurochirurgische Universitatsklinik, Sigmund-Freud
StraBe 25, D-5300 Bonn-Venusberg
Fitz, C. R., Dr. med., Associate Professor of Radiology, Division of Special
Procedures, Department of Radiology, Hospital for Sick Children, University
of Toronto, 555 University Avenue, Toronto, Ontario, Canada
Foedisch, H. J., Prof. Dr. med., Institut flir Kinderpathologie, Universitat Bonn,
D-5300 Bonn-Venusberg
Franke, H. D., Prof. Dr. med., Klinik flir Strahlentherapie, Universitatskrankenhaus
Eppendorf, MartinistraBe 52, D-2000 Hamburg 20
Frowein, R. A., Prof. Dr. med., Neurochirurgische Universitatsklinik, Joseph
Stelzmann-StraBe 9, D-5000 Koln 41
Gahbauer, H., Dr. med., Abteilung flir Neuroradiologie, Universitatskliniken,
KirschnerstraBe 1, D-6900 Heidelberg
Georgi, P., Prof. Dr. med., Nuklearmedizinische Abteilung, Deutsches Krebs
forschungszentrum, 1m Neuenheimer Fe1d 280, D-6900 Heidelberg
Gerlach, H., Dr. med., Institut flir Pathologie, Martin-Luther-Universitat Halle
Wittenberg, Leninallee 14, DDR-4020 Halle (Saale)
Glees, P., Prof. Dr. med. Dr. phil., Department of Anatomy, University of
Cambridge, Downing Street, Cambridge CB 2 DY, Great Britain
Goebel, B., Kinderkrankenschwester, 'Universitatskinderklinik, Langenbeck
straBe 1, D-6500 Mainz
Goerke, L., Dr. med., Max-Planck-Institut flir Hirnforschung Koln-Merheim,
Ostmerheimer StraBe 200, D-5000 Koln 91
Grosch-Worner, J., Dr. med., Abteilung flir Padiatrische Onkologie, Universitats
kliniken Hamburg, MartinistraBe 52, D-2000 Hamburg 20
