Springer-Verlag Italia SrI. Giovanni Broggi (Ed.) Craniopharyngioma Surgical Treatment Springer GIOVANNI BROGG!, M.D. Istituto Nazionale Neurologico "C. Besta" Via Celoria, 11 1-20133 Milan ISBN 978-3-540-75001-7 ISBN 978-88-470-2291-1 (eBook) DOI 10.1007/978-88-470-2291-1 This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplications of this publication or parts thereof is permitted only under the provisions of the German Law of September 9, 1965, in its current version, and permission for use must always be obtained from Springer-Verlag. Violations are liable for prosecution under the German Copyright Law. © Springer-Verlag ltalia 1995 Originally published by Springer-Verlag Italia, Milano in 1995. The use of general descriptive names, registered 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. Foreword The Paolo Zorzi Association for Neurosciences was founded in 1978 to promote scientific and clinical research in the field of neuroscience, with particular attention to neurooncology, epileptology, and those diseases that cause movement disorders in infancy and childhood. During these past years the activity of the Association has increasingly developed, making important contributions to the study of these diseases and to their treatment by integrating the basic sciences and clinical research. Within the Association, a Scientific Commitee formed by Giovanni Broggi, the promoter of the workshop whose proceedings are published in this book, Giuliano Avanzini, Lucia Angelini and Roberto Spreafico, who all work at the Istituto Nazionale Neurologico "C. Besta" in Milan, guarantees that the sponsored initiatives correspond to the goals of the Association. The many papers published in reputable international journals and in books of great scientific relevance - the latest of which is this one - testify to the activity of the Association. Moreover, the Association directly supports some research programs by awarding grants to promising young neurologists in Italy and abroad and by promoting scientific congresses on neurosurgery, neuropsychiatry, neuroanatomy and neurophysiology. An example of this was the organization of the Workshop: "Craniopharyngioma: Surgical Treatment" held in Milano, Italy, in May 1993. We thank Giovanni Broggi and all those who made this Workshop a success and the publication of this book possible, and the Association whishes to be able to promote congresses of the same scientific level in the near future. Dr. Franco Santamato, President Paolo Zorzi Association for Neurosciences Table of Contents Neuropathology of Craniopharyngioma ................................................ 1 A. ALLEGRANZA Neuroradiology of Craniopharyngiomas ............................................... 7 M. SAVOIARDO AND E. CICER! Craniopharyngioma in Children: Surgical Considerations .................... 20 M. CHOUX, G. LENA, AND L. GENITOR! Radical Removal of Craniopharyngiomas 1971-1991 ....................... ... 31 P. W. CARMEL Direct Microsurgery of Craniopharyngiomas: A Review of the Series of the Istituto Neurologico of Milan, Italy....................... 52 S. GIOMBINI AND F. PLUCHINO Surgical Craniopharyngioma Treatment .................. , ............... ........ ...... 64 V. V. DOLENC, G. MARINIELLO, A. HORVAT, J. SUSTERSIC, B. P. PRESTOR, AND R. PREGEU Surgical Management of Craniopharyngiomas from 1976 to 1992: Problems and Results ............................... ............... .............................. 73 R. M. VILLANI, E. P. SGANZERLA, S. M. GAINI, L. BELLO, AND M. GIOVANELLI Some Problems of Craniopharyngioma Treatment ............................... 88 A. N. KONOVALOV Role of the Transsphenoidal Approach in the Surgical Treatment of Craniopharyngiomas .. ........................ ........................ ...... 97 G. NICOLA, G. LASlO, L. VALENTINI, S. VISINTINI, AND C. L. SOLERO Surgical Treatment of Craniopharyngiomas .......................................... 104 G. CANTORE, V. ESPOSITO, AND B. FRAIOLI Therapeutic Role of Intracavitary Bleomycin Administration in Cystic Craniopharyngioma................................................................ 113 G. BROGGI, C. GIORGI, A. FRANZINI, F. LEOCATA, AND D. RrVA VIII Gamma Knife Radiosurgery and Intracystic Colloidal Isotope Treatment of Craniopharyngiomas ........................................................ 120 T. RAHN Three-Dimensional Dosimetry for LINAC-Based Radiosurgery and Fractionated Stereotactic Radiotherapy of Craniopharyngiomas ......................................................................... 126 C. GIORGI, M. LUZZARA, U. CERCHIARI, AND A. GRAMAGLIA Endocrine Tests and Hormonal Therapy in Craniopharyngioma ........................................................................... 132 R. COZZI, G. OPPIZZI, P. ORLANDI, D. DALLABONZANA, AND I. CHIODINI Concluding Remarks ...... .......... .......... ........ ....... .................................... 144 G. BROGGI, EDITOR Subject Index ......................................................................................... 154 Neuropathology of Craniopharyngioma A. ALLEGRANZA Craniopharyngiomas are benign epithelial tumors of congenital malforma tive origin. They are thought to originate, though this is still under debate, from remnants of Rathke's pouch, which is a protrusion of the roof of the stomodeum or primitive oral cavity (Russell and Rubinstein 1977). These remnants, called Erdheim's remnants (hence the name Erdheim's tumor) are often found in the pituitary stalk, in the sellar, parasellar location or even in the sphenoid bone. Tachibana et al. (1994) have recently demonstrated by immunohistochemical study that in the cells of the adenohypophysis and in craniopharyngiomas glycoprotein P (PGP) present in the cytotrophoblast and human chorionic gonadotropin (HCG) are coexpressed. These data suggest that craniopharyngiomas produce HCG-like peptides; therefore they can be considered the only squamocellular tumors that, in the sellar region, origi nate from neuroendocrine precursors. Craniopharyngiomas are usually located over the sellar diaphragm and less frequently in the sella. Their rupture in the third ventricle is very rare (Fig. 1). The sphenoid bone and exceptionally the paranasal sinuses, nasal pharynx, and the pineal region and the pontocerebellar angle have been re ported as ectopic localizations. Two types are classified: the classic type, called adamantinomatosus, and the papillary type (Burger and Vogel 1982). Transitional forms have also been described (Petito et al. 1976; Szeifert et al. 1993). The adamantinomatous craniopharyngioma is found in young subjects during the first two decades. It is commonly found as a suprasellar lesion involving the pituitary stalk, chiasm, optic nerve, and contiguous vessels up to the ventricle (Figs. 2,3). Macroscopically, it has solid and cystic areas: the cavities of various sizes contain fluid material, sometimes dense, yellow, dark, similar to "motor oil." Histologically, in the solid areas, it is formed by clusters, cords, and sheets of epithelial cells that, at the periphery, are ar ranged like a palisade, while in the center, for a process of differentiation, they are loosely arranged and assume a starry form, creating patterns which Consultant pathologist, Dept. of Neurosurgery, Istituto Nazionale Neurologico "c. Besta", Via Celoria 11,20133 Milan, Italy 2 A. Allegranza Fig.I. Craniopharyngioma located in the interpeduncular-chiasmatic fossa, spread ing to the right sylvian fissure. In a frontal section, it shows a solid cystic hemorrhag ic aspect. In the progression toward the third ventricle it caused a sudden and fatal hemorrhage of the white matter of the left hemisphere are reminiscent of the "stellate reticulum" of the pulp of the enamel organ and of the mandibular adamantinomas (thus it is called adamantinomatous craniopharyngioma) (Fig. 4). Clusters of squamous epithelial cells, keratin ized pearls, and calcifications are observed. No keratohyalin granules are present in the epithelial squamous elements. In the cystic areas the cavities are filled with glycoproteic material mixed to cholesterol crystals, squamous dying cells, and cellular debris. This mate rial can reach the adjacent stroma and cause formation of granulomas with foreign body giant cells surrounding negative images of cholesterol crystals. Papillary craniopharyngioma is found mainly in adults. It is rarely supra sellar and more frequently it develops in the third ventricle. Macroscopical ly, it appears as a solid mass with small cavities well delimited and without the presence of "motor oil" fluid. Microscopically, it is formed by cords of well differentiated squamous epithelial cells that are sustained by fibrovas cular septa, thus giving the aspect of papillary structures (Fig. 5). Adamanti noid aspects, keratinized whorls, calcifications, cholesterol deposit, and gran ulomatous reactions with giant cells are lacking. Szeifert et al. (1991), on the basis of histochemical and ultrastructural studies, think that the elements that delimit the cavities of craniopharyngi- Neuropathology of Craniopharyngioma 3 Fig. 2 A, B. Behind the optic chiasm (A) presence of a nodule, well delimited, that in a frontal section (B) is connected to a microcystic mass separating the optic tracts, raising the floor of the third ventricle, where it assumes the form of a wide cystic cavity occupied by homogeneous dark material. Female, 60 years old Fig. 3. Frontal section at the level of the basal nuclei. The lesion is mainly cystic and well delimited and appears to nest in the third ventricle. Male, 32 years old