Frontiers in Neurosurgery

In this chapter, we firstly briefed reviewed the history and the terminology of craniopharyngioma (CP). Then with summarizing our own data based on 10 top hospitals in China and previous publications, we focused on description of the Epidemiology of craniopharyngioma (CP). And correspondingly, clinical manifestation of CP patients was depicted as well.

As histological benign tumor with malignant cellular characteristic, craniophayrngyioma (CP) caused big trouble not only for surgery but also for the postsurgical management. So, it is important to emphase the understanding of the pituitary gland and also it’s connected structures, such as pituitary stalk, hypothalamus, and so on. In this chapter, we described the anatomical and cytological constructors of pituitary gland. As with combined the etiology of CP, we depicted the embryonic development of gland and its accessory structures. On the other hand, several hypotheses about the origin of CP were also analysed and described.

Craniopharyngioma is a type of epithelial neoplasm that develops in the sellar region, a feature that distinguishes these tumors from other glial or neuronal lesions in the same location. Although the etiology of craniopharyngioma has not yet been confirmed, this type of tumor is generally thought to derive from the epithelial remnants of the craniopharyngeal duct that eventually forms part of the adenohypophysis. In this chapter, we address the pathological classification of craniopharyngioma and its molecular aspects. craniopharyngiomas can be divided into two subtypes: adamantinomatous craniopharyngioma (ACP) and squamous papillary craniopharyngioma (SPCP). ACP is more common (90% of all craniopharyngiomas), particularly among children. ACP features various types of epithelial characteristics such as peripheral palisades, stellate reticulum, whorl-like array cells, wet keratin, and cholesterol clefts. SPCP tumors are found almost exclusively in adults and generally present as solid masses comprised histologically of sheets of well-differentiated squamous epithelial cells, with prominent papillae composed of squamous epithelial cells that grow around fibrovascular cores. ACp contain mutations in CTNNB1, encoding β-catenin: a component of the adherens junction and mediator of Wnt signalling. reported frequency of CTNNB1 mutations varies widely (16–100%). Recently, it was reported that pCps contain BRAF p.V600e mutations in 95% of cases and that CTNNB1 and BRAF mutations are mutually exclusive and specific to tumour subtype.

Craniopharyngiomas are benign midline tumors that have a propensity for local recurrence and are ideally curable via total surgical resection. Many survivors suffer from behavioral, cognitive, endocrine, hypothalamic, and visual disturbances. Optimal management remains highly controversial. In this chapter, we reviewed the therapy strategies for Craniopharyngiomas including surgery, radiotherapy and chemotherapy. Complete resection is even more important in children, especially those younger than 3 years, because of the additional morbidity associated with radiotherapy during early childhood. The aim of radiotherapy is to achieve long-term disease control in patients lacking complete removal or with recurrent tumors. Systemic chemotherapy has rarely been reported in terms of craniopharyngioma management. Local intratumoral chemotherapy for craniopharyngioma was used to treat difficult, recurrent cystic tumors and has subsequently been employed as a strategy to avoid late-term effects of surgery or radiotherapy. The roles of all therapies should be balanced according to factors such as the patient’s age, the tumor size and location, and prior treatment.

Craniopharyngioma, an intracranial, extra-axial, epithelial tumor of the sellar or parasellar region that grows along the craniopharyngeal duct, was established in 1904 as a distinct tumor type by the Austrian neuropathologist Jakob Erdheim. Though classified as a Grade I tumor by the World Health Organization, craniopharyngioma can cause significant morbidity through its intimate involvement with and mass effects on surrounding critical structures, including the hypothalamus, pituitary gland, and optic chiasm, and its tendency to recur even after successful therapy. Many extensive and in-depth studies of craniopharyngioma have been conducted; however, during the past century, studies of the genetic and molecular basis of the two major craniopharyngioma subtypes (adamantinomatous and squamous papillary) have been limited because of the benign histology, epidemiological rarity (relative to malignant tumors), and lack of a tumor research platform and technical conditions associated with these lesions. Therefore, consensus remains lacking with respect to the etiology, pathological features, pathogenesis, and biological characteristics of these formidable neoplasms. In this chapter, we reviewed experimental craniopharyngioma research.

As the surrounding vital constructures, such as pituitary stalk, hypothalamus, third verntricule, et al, the surgery of craniopharyngioma (CP) is one of the most difficult in the field of neurosurgery. Better understanding the anatomy of sellar region, especially the related constructures to the possible origin site of CP are particularly important. In this chapter, we focus depicted the anatomy of sellar region based on the pterional approach, which is the most common surgical approach used in resection of CP. Within the description, we added the understandings about the surgical skills when removal the CP. Moreover, the morphology of tumor was analyzed and the reason of choosing pterional and its extension approach were also discussed.

In this chapter, based on interhemispheric approaches including the corridor through fronto-basal, anterior part of interhemispheric space and also through interfonix corridor, we illustrated the advantage of different approaches. And the related structures such as, pituitary gland, pituitary stalk, and hypothalamus were depicted under such surgical view. The other accessory structures, for example, optic pathway, sphenoid sinus and also the cavernous sinus were described as well. Moreover, the morphological features of craniopharyngioma were analyzed and then how to choose the most appropriate approaches was discussed.

As the sellar region location, the trans-nasal transsphenoidal corridor was thought to be the most effective approach to reach the surgical area. In recent decade, the endoscopic surgery developed extremely rapidly. As the result, more and more scholars tried to use transsphenoidal and it extended approach to dissect the craniopharyngioma (CP). In this chapter, we provided the basic anatomical details concerning the endonasal transsphenoid approach and its expanded application for surgical removal of CP. Moreover, several clinical cases and the morphological features of CP were analyzed. Base on the different classification of CP, the appropriate application of this approach was discussed.

The growth pattern of craniopharyngiomas (CP) is yet to be understood due to challenges arising from the diversity of morphological features that exist. This in turn has had implications on the development of safe surgical strategies for management of these lesions. In this chapter, we proposed a morphological classification of CP based on their tumor–membrane relationship, which will contribute to better understanding of CP morphology and prediction of the intraoperative classification. Moreover, based on our studies, the arachnoidal sleeve around the pituitary stalk (ASPS) was noted, and divided the pituitary stalk into four segments. Correspondingly, the growth of CPs was divided into four basic patterns—infradiaphragmatic (ID), extra-arachnoidal (EA), intra-arachnoidal (IA) and sub-arachnoidal (SA) growth. After analysis of 195 clinical cases of CP, we finally proposed the clinical classification “QST” classification, which detailed the relationship of the surrounding structures to CPs and purports to predict and identify the intraoperative anatomical stratification. It also attempts to help predict the growth patterns of these tumors. Finally, the surgical principle of approach selection for CP was discussed and proposed.

Craniopharyngiomas (CPs) could occurred in anywhere along craniopharyngeal canal. The diverse original points of tumor lead to significant differences in expansion [1]. Although the majority of CPs have a supradiaphragmatic origin (infundibular stalk/pars tuberalis), a number of CPs arise primarily from the subdiaphragmatic space and usually cause marked sellar enlargement before extending intracranially. Several terms, such as “sub- or infradiaphragmatic CP” and “sellar CP” have been coined to describe this type of tumor [2-6]. However, details of the clinical and morphological features of this tumor type were missing from previous literature reports. In this chapter, we focus on CPs that originate from the infradiaphragmatic space (Id-CP) and illustrate the tumor growth patterns, preoperative clinical and radiological features, intraoperative findings, and long-term outcomes.

In this chapter, we focus on a subgroup of craniopharyngioma (CP) in which the main tumor body is located exclusively in the suprasellar subarachnoid cisternal space around the pituitary stalk (PS) and there is no third ventricle involvement. Notably, the cases of CP reported in this chapter were not clearly defined in previous literature. The main reason for the confusion regarding this type of CP might be the lack of uniform criteria for evaluating the relationship between the tumor and third ventricle walls. The terminology “suprasellar extraventricular CP,” which is used in previous literature, might be another term for the cases described in this chapter; however, this term does not truly reflect the tumor origin and location.

Suprasellar infundibulo-tuberal craniopharyngiomas “Type T” were the most important and difficult tumors with all types of CP, which also the most common type. In this chapter, we focused on the clinical manifestation, growth pattern and prognosis of this type of CP. With summarizing our data and previous literatures review, the advantage of frontobasal interhemispheric approach was depicted emphasizedly. Moreover, by analysis of the relationship between tumor and the third ventricular walls, the defect and merit of transnasal and transcranial approach were discussed as well. Finally, the injury of hypothalamic structures and the psychological evaluation were depicted.

As we known, adamantinomatous craniopharyngioma (ACP) is thought to originate from residual Rathke’s pouch cells along the long axis from nasopharynx to infundibulum [1, 2]. However, the squamous papillary subtype of CP (SPCP) is believed to occur throught squamous metaplasia in the pars tuberalis [2, 3]. In consideration of these inferences, the neural parenchymal layer of third ventricular floor (3rd VF) should locate above the CP, separate both the tumor and third ventricular chamber. However, the first study conducted by Dubos et al. in 1953 extensively reported the tumors completely located inside the third ventricle cavity by autopsy or intraoperative finding [4-13]. Because of this topographical location, the theory of CP origin has been challenged and much more neurosurgeons are interested in pursuing the true morphological characteristics and diagnostic criteria of these tumors [9, 14]. In this chapter, we firstly reviewed the definition of the intraventricular CP in several publications. Then based on our histological and clinical study, the true morphology of intraventricular CP was proposed. The related approach selection and surgical skills was also depicted.

The infra-sellar and nasopharyngeal craniopharyngiomas (CPs) were the special type, which predominantly presented in the children. In this chapter, we briefly depicted the clinical features of this type of tumor. Patients suffered with this type of CP were rarely more than 16 years old. The pathology was all adamantous tumor. As concerned to the clinical manifestation, except the pituitarism, growth retardation, and optical pathway injury, the pediatric patients always suffered with rhinocleisis, homorrhinia and airway obstruction. As extensive involvement, it was quite difficult to radical remove the tumor. As the result, tumor recurrent caused poor quality of life. Until now, the best treatment strategy was still missing.

Any neurosurgeon can’t avoid the tumor recurrence within the treatment of craniopharyngiomas. In this chapter, we focused on the description of the clinical manifestation, surgical strategy and prognosis of recurrent CP. Especially, the growth pattern of recurrent tumor as compared to the primary morphological features was emphasized and discussed. The influences of the first surgery or treatment strategy (pallilate or radical) were proposed based on the clinical casese analysis. As our opinion, the recurrent CP can be divided into 1) Intrasellar recurrence of Infradiaphgragmatic tumor, and 2) Suprasellar infundibulo-tuberal recurrence. Correspondingly, the different treatment strategies were also summarized. We thought for intrasellar recurrent, more radical surgery should be taken. While for the infundibulo-tuberal recurrence casued by palliative first surgery, more radical surgery was still the first choise. However, if radical total removal was selected in the first surgery, the repeated operation must be paid more attention.

Endocrine dysfunction is a common complication of craniopharyngiomas due to tumor location and the growth pattern. How to diagnose hypopituitarism and implement reasonable hormone replacement therapy, promote the recovery of endocrine function after operation is still a puzzle need to be solved urgently. The purpose of the present chapter is to summarize data on endocrinological evaluation in these patients, discuss clinical characters, and elaborate the growth features of childhood craniopharyngiomas. To understand the hypopituitarism patterns and its influencing factors of patients with craniopharyngioma has important effect on improving the prognosis and long-term quality of life.

Hypothalamic obesity is a condition with extremely high morbidity. Mechanisms leading to the profoundly disturbed energy homeostasis are complex. Craniopharyngioma is one of the most common pathogenesis of hypothalamic obesity, which leads to the damage of hypothalamic nucleis due to the tumor compression or/and cytokines stimulation. The purpose of the present chapter is to summarize data on the prevalence of hypothalamic obesity, discuss the clinical features and review the diagnosis and treatment of these patients. Differences tumor growth patterns and locations should be considered when comparing outcomes and survival across different treatment paradigms in patients with CP. Further studies should be conducted to better understand the mechanisms of rapid weight gain and metabolic syndrome in patients with CP and could be used to enhance effective treatments and establish prevention strategy.

In this chapter, we focused on the description of the conception, clinical manifestation, surgical treatment and prognosis of pediatric craniopharyngiomas (CP), which were extremely different from the adult patients. As most of the tumors grew with development of the pituitary gland, stalk and hypothalamic structures in pediatric patients, surgery always caused severe endocrinological and hypothalamic dysfunction. And the postrugical irradiation was also restriced for younger children, with result as big problem for the quality of life in childhooh CP. In this chapter, we proposed two types of pediatric CP: infradiaphragmatic CP and intra-third ventricular floor CP. Those two types of CP presented totally variant characteristics with aspect in clinical manifestation, surgical strategy, recurrence, and prognosis, which will also be described and discussed in details.

In this section, we have provided clinical data from 15 cases of craniopharyngioma (CP) in which the details differ with respect to growth pattern. Emphasis has been placed on performance of the presurgical analysis and determination of the surgical aims, as well as potential surgical difficulties and the selection of a proper approach to achieve satisfactory exposure for tumor removal. Along with depictions of the tumors’ morphological features and schematic Fig. (1), we have described the surgical techniques and arachnoidal interfaces used for safe tumor removal while protecting vital neurovascular structures. Long-term follow up data have also been provided to demonstrate the patients’ prognoses. These cases have been subdivided into three types: infradiaphragmatic CP (Id-CP or “Q” type), suprasellar extra-ventricular CP with expansion in the subarachnoid cistern (SeV-CP or “S” type), and suprasellar subarachnoid CP with invagination to the third ventricular walls (SiV-CP or “T” type). Because of tumor growth along the longitudinal axis of the pituitary stalk (PS), each case might display different variations. We will illustrate this in special cases.