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Surgery Update on the Surgical Management of Craniopharyngiomas Ricardo J Komotar,1 Christopher P Kellner2 and Jeffrey N Bruce3


1. Chief Resident; 2. Junior Resident; 3. Professor of Neurological Surgery, Department of Neurological Surgery, The Neurological Institute of New York, Columbia University Medical Center


Abstract


Despite advances in microsurgical and skull base techniques, the surgical treatment of craniopharyngiomas continues to pose a challenge to surgeons. Controversy continues to exist between proponents of subtotal resection in conjunction with radiotherapy, who argue that this less aggressive approach can yield equivalent control rates with lower morbidity, and others who argue for the superiority of gross total resection. Regardless of whether gross total or subtotal resection is the goal, surgical planning must include a thorough endocrine and neuro- ophthamological evaluation as well as imaging, and the approach, whether trans-sphenoidal or transcranial, must take into account the nature of the tumour and its location. In addition, optimal management of craniopharyngiomas must consist of individualised and multidisciplinary therapy including an array of subspeciality fields such as endocrinology, neuro-ophthalmology, neuropsychology and radiation oncology.


Keywords Neurosurgery, craniopharyngioma, craniotomy


Disclosure: The authors have no conflicts of interest to declare. Received: 25 May 2010 Accepted: 29 June 2010 Citation: European Neurological Review, 2010;5(1):107–12 Correspondence: Jeffrey N Bruce, Department of Neurological Surgery, The Neurological Institute of New York, Columbia University Medical Center, 710 West 168th Street, New York, NY 10032, US. E: jnb2@columbia.edu


Craniopharyngiomas are epithelial tumours that arise from embryonic epithelial cells of the craniopharyngeal duct. They account for 1.2–4% of all primary intracranial neoplasms and 5–10% of intracranial tumours in children.1


Although these tumours are rarely malignant,


they often grow adjacent to and distort critical neurovascular structures, particularly the visual pathways and hypothalamus, leading to significant morbidity and mortality. In addition, their sellar or parasellar location poses a challenge to surgical access. However, recent advances in microsurgical and skull base techniques have made resection possible in many patients.2


Historical Perspective


Early attempts at resecting skull base tumours, among them craniopharyngiomas, were limited by a lack of exogenously administered steroids, inadequate visualisation secondary to poor illumination and magnification techniques, poor diagnostic imaging modalities and mortality associated with hypothalamic injury. Despite these extraordinary limitations, early neurosurgeons made significant contributions to the management of craniopharyngiomas. As early as 1912, McArthur addressed the surgical section of the American Medical Association (AMA) regarding surgery of the hypophysis and famously stated: “long considered safe from invasion by the most daring surgeon, both because of its almost inaccessible location and because of its intimate relation to life, the question of its feasibility to surgical attack has been answered in the affirmative.”3


Due to the proximity of craniopharyngiomas to pituitary tumours, the original surgical approaches were very similarly described as either intracranial or extracranial/transphenoidal. Early intracranial


© TOUCH BRIEFINGS 2010


approaches for the resection of pituitary tumours were met with significant failure and high mortality. Following consistent failure of the intracranial approach and given the anatomical belief that these tumours grew downward from the floor of the sella, surgeons often preferred to work through the trans-sphenoidal approach. By contrast, early trans-sphenoidal approaches were more successful. In November 1909, Kanavel devised an infranasal technique that contrasted with other trans-sphenoidal approaches because it passed through the upper part of the nasal cavity. Albert Edward Halstead became the first surgeon to use this technique for the resection of a sellar tumour, notably a craniopharyngioma. Despite success with the trans-sphenoidal approach, not all surgeons were satisfied with the technique. For example, Harvey Cushing initially used the trans-sphenoidal approach, but abandoned it later in his career for the transfrontal approach, likely for better access to suprasellar lesions. Despite improvements in surgical techniques and the occasional success story, the prognosis of hypophyseal tumours in the early 1900s remained dismal.


As knowledge and experience accumulated throughout the early 1900s, intracranial approaches from the side of worse vision improved enough to supplant the trans-sphenoidal approach. By the end of the first half of the 20th century, radical resection was thought to give the best chance of cure, but the high morbidity and mortality associated with radical surgery prevented total resection in the majority of cases. The use of hormonal preparations and ‘Roentgen therapy’ laid the foundation for future craniopharyngioma treatment.4


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