JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM

 

CRANIOPHARYNGIOMA: ACHIEVEMENTS AND CHALLENGES

 

Genoa, Italy, November, 2004

 

Guest Editor: Maria Luisa Garrè

Co-editors: Frank Saran, Christian Saint-Rose, Renata Lorini and Hermann Müller

Foreword

     Craniopharyngioma represents approximately 10% of intracranial tumours in the paediatric age range. Craniopharyngiomas are slow growing midline epithelial tumours that arise embryonically from remnants of Rathke’s pouch in the suprasellar area. A macroscopic complete resection is curative in more than 80% of cases where this can be achieved. Yet due to its frequent large size at presentation and common involvement and inva­sion of adjacent structures, such as the hypo­thalamus, optic chiasm and brain, the attempt at complete macroscopic resection is potentially fraught with unacceptable morbidity. Surgically, two main approaches are currently pursued: either aiming to achieve macroscopic complete excision as the primary objective, or a primarily conservative approach combining a limited surgical procedure such as partial resection and/or cyst drainage with other adjuvant treatments, e.g. fractionated radio­therapy, radiosurgery or intracystic instillation of bleomycin or P32 colloids.

     Paediatric patients diagnosed with a malignancy have traditionally experienced a high focus and concerted efforts through both national and inter­national groups to improve outcome and to reduce late morbidity. Such an approach has led to significant improvements in outcome in paediatric malignancies such as acute lymphoblastic leuk­aemia, Wilms’ tumours, Hodgkin’s disease and medulloblastomas. As craniopharyngiomas are rare, classified as benign, and only exceptionally referred to paediatric oncology units, they have so far not benefited from such an approach. Young children and adolescents with craniopharyngiomas still suffer disproportionately severely from tumour and/ or treatment related morbidity. The frequent combination of hypothalamic, behavioural and/or visual deficits poses a special challenge to co-ordinate a successful multidisciplinary approach to the management of these patients. This is particu­larly important as most of these patients will become long-term survivors.

     In the absence of prospective clinical studies the management of these tumours has been predomi­nantly based on individual clinicians’ preference. Yet over the last decades the optimal management approach remains a topic of significant controversy leading to heterogeneous treatment policies within centres and countries.

     In November 2004, an international conference, the first ever held in this field, was dedicated to craniopharyngioma. Acknowledged experts of different specialities, from Europe and the USA, met together in Genoa and promoted a multi­disciplinary active discussion on key issues of the management of children with craniopharyngiomas.

     The number of participants far exceeded expectations. Registered participants, many of them neurosurgeons, came from all over Europe (UK, Belgium, Germany, France, Scandinavia, The Netherlands) and a few also from outside Europe (Egypt, Turkey). Within the faculty and registered participants active debate went on during the 1.5 days of the meeting. At the end, even the experts present came to the conclusion that what they thought as well established in craniopharyngioma treatment could be modified or seen under another light, and that a greater consensus in the manage­ment of craniopharyngiomas could be achieved. The possibility of a standard of care to be prospectively evaluated in a multinational treatment protocol creating an evidence based body of data to guide future practice was then considered an achievable goal.

     The conference was part of the educational programme of the International School of Pediatrics of Giannina Gaslini Research Hospital and the result of a cooperative project with the Radiation Department of Royal Marsden Hospital in London. Scientific sponsors of the event were the Inter­national Society for Pediatric Oncology (SIOP), the European Society for Pediatric Neurosurgery (ESPN), the Italian Society for Pediatrics (SIP) and the Italian Society for Pediatric Endocrinology (SIEDP). Special support was given by the Associ­azione per la Ricerca sui Tumori Cerebrali del Bambino, founded by a group of families whose children, treated at Giannina Gaslini Hospital, suffered brain tumours.

     To leave a sign of this fruitful meeting, the faculty accepted unanimously to contribute to this issue which is the first stone in a path that we hope in future will continue with new up-to-date progress in craniopharyngioma management. Our warmest thanks to Dr. Stanhope who was the first to believe in this project and who made it possible by his encouragement.

     We are conscious that not all the main topics concerning craniopharyngiomas have been covered in this issue of JPEM; in fact, during the con­ference, the main focus was on neurosurgical, radiation therapy and endocrine aspects and treatment decisions around the key issue debated:
Is complete resection the primary management option versus a conservative surgical approach followed by radiation therapy? Neurological, neuropsychological and ophthalmological aspects deserve more attention in future meetings. We all hope that the message resulting from this conference and published in this issue of JPEM will be the basis for promoting cooperative international studies in Europe and USA in order to improve the quality of life of children treated for cranio­pharyngioma.

 

 

 

The Editors

 

CONTENTS

Preface               287

 

Epidemiological Aspects of Craniopharyngioma

R. Haupt, C. Magnani, M. Pavanello, S. Caruso, E. Dama and M.L. Garrè, Italy                   289

 

Paediatric Craniopharyngioma: A Pathological Review

P. Nozza and C. Gambini, Italy                  295

 

Neuroimaging of Pediatric Craniopharyngiomas: A Pictorial Essay

A. Rossi, A. Cama, A. Consales, C. Gandolfo, M.L. Garrè, C. Milanaccio, M. Pavanello, G. Piatelli, M. Ravegnani and P. Tortori-Donati, Italy                 299

 

Craniopharyngioma: Ophthalmological Aspects at Diagnosis

S. Defoort-Dhellemmes, F. Moritz, I. Bouacha and M. Vinchon, France                   321

 

The Pre-operative Endocrine Assessment of Craniopharyngiomas

N. Hopper, A. Albanese, S. Ghirardello and M. Maghnie, UK and Italy                      325

 

Radical Resection for Craniopharyngiomas in Children: Surgical Technique and Clinical Results

P. Dhellemmes and M. Vinchon, France                    329

 

Craniopharyngioma: Experience at a Single Institution in Italy

A. Cama, M. Ravegnani, G. Piatelli, A. Rossi, C. Gandolfo and M.L. Garrè, Italy                  337

 

The Role of Transventricular Neuroendoscopy in the Management of Craniopharyngiomas: Three Patient Reports and Review of the Literature

G. Cinalli, P. Spennato, E. Cianciulli, A. Fiorillo, S. Di Maio and G. Maggi, Italy                  341

 

Surgical Management of Craniopharyngiomas - Experience with a Pediatric Series

C. Di Rocco, M. Caldarelli, G. Tamburrini and L. Massimi, Italy                          355

 

Multimodal Treatment of Craniopharyngioma: Defining a Risk-adapted Strategy

S. Puget, J. Grill, J.-L. Habrand and C. Sainte-Rose, France                 367

 

Pediatric Craniopharyngioma - Rationale for Multimodal Management: The Egyptian Experience

M.A. Hafez, S. ElMekkawy, H. AbdelBadie, M. Mohy and M. Omar, Egypt                371

 

Management of Cystic Craniopharyngiomas in Childhood by a Transsphenoidal Approach

G. Zona and R. Spaziante, Italy                 381

 

Radiation Therapy in the Management of Craniopharyngioma: Current Concepts and Future Developments

J.-L. Habrand, F. Saran, C. Alapetite, G. Noel, R. El Boustany and J. Grill, France and UK             389

 

Review of the Role of Radiotherapy in Craniopharyngiomas: How Does Patient Age Influence Management Decisions?

P. Franzone, L. Berretta and S. Barra, Italy                    395

 

Acute and Late Morbidity After Limited Resection and Focal Radiation Therapy in Craniopharyngiomas

G. Scarzello, M.S. Buzzaccarini, G. Perilongo, E. Viscardi, R. Faggin, C. Carollo, M. Calderone, A. Franchi and G. Sotti, Italy             399

 

Does Chemotherapy Have a Role in the Management of Craniopharyngioma?

D.R. Hargrave, UK             407

 

Diabetes Insipidus in Craniopharyngioma: Postoperative Management of Water and Electrolyte Disorders

S. Ghirardello, N. Hopper, A. Albanese and M. Maghnie, Italy and UK               413

 

Secondary Narcolepsy may be a Causative Factor of Increased Daytime Sleepiness in Obese Childhood Craniopharyngioma Patients

H.L. Müller, S. Müller-Stöver, U. Gebhardt, R. Kolb, N. Sörensen and G. Handwerker, Germany                      423

 

Endocrine and Growth Features in Childhood Craniopharyngioma: A Mono-institutional Study

E. Di Battista, A. Naselli, S. Queirolo, F. Gallarotti, M.L. Garré, C. Milanaccio and A. Cama, Italy               431

 

Craniopharyngioma Radiotherapy: Endocrine and Cognitive Effects

T.E. Merchant, USA             439

 

A Multimodality Approach to the Treatment of Craniopharyngiomas Avoiding Hypothalamic Morbidity: A UK Perspective

H.A. Spoudeas, F. Saran and B. Pizer, UK                  447

 

Consensus and Perspectives on Treatment Strategies in Childhood Craniopharyngioma: Results of a Meeting of the Craniopharyngioma Study Group (SIOP), Genova, 2004

H.L. Müller, A. Albanese, G. Calaminus, D. Hargrave, M.L. Garré, U. Gebhardt, F. Saran, N. Sörensen and H.A. Spoudeas, Germany, UK and Italy             453

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Journal of Pediatric Endocrinology & Metabolism

Vol. 19, Supplement 1, 2006

170 pp, $50.00

ISSN 0334-018X/19-S1

© 2006 Freund Publishing House