Intraventricular Craniopharyngiomas Research Articles

Impact of three surgical approaches on the therapeutic efficacy of intraventricular craniopharyngiomas: a single-center retrospective analysis.

This study aimed to investigate the therapeutic efficacy of three different surgical approaches for the treatment of intraventricular craniopharyngiomas (IVCs). The three surgical approaches investigated in this study were the endoscopic endonasal approach (EEA), pterional trans-lamina terminalis approach (PTA), and interhemispheric trans-lamina terminalis approach (ITA). Patient demographics, preoperative symptoms, endocrine and hypothalamic status, tumor characteristics, and surgical outcomes were analyzed and compared among the different surgical groups. A total of 31 patients with IVCs were included in the analysis, with 12 patients in the EEA group, 8 patients in the ITA group, and 11 patients in the PTA group. The mean follow-up time was 39 ± 23months. Statistical analysis of the data revealed significant differences in the gross total resection (GTR) rate among the three surgical groups (P = 0.033). The GTR rate for the EEA group was 100%, that for the ITA group was 88%, and that for the PTA group was 64%, which was the lowest rate observed. After surgery, only 8.3% of the patients in the EEA group did not experience new postoperative hypopituitarism, while the percentages in the ITA and PTA groups were 75% and 73%, respectively (P = 0.012). Finally, we found that postoperative hypopituitarism may be related to the transection of the pituitary stalk during the operation (P = 0.020). Based on the results of this study, we recommend using the EEA and the ITA instead of the PTA for the surgical resection of IVCs. Furthermore, the appropriate surgical approach should be selected based on the tumor's growth pattern.

Intraventricular Craniopharyngiomas-Overcoming Their Relative Inaccessibility: Institutional Experience With a Review of Literature.

Introduction: Craniopharyngiomas constitute 2–4% of intracranial neoplasms. Intraventricular craniopharyngiomas (IVCrs) are the rarely encountered varieties of these lesions.Objective: The objective of the study was to study the special features in clinical presentation, imaging, management, and surgical outcome of IVCrs.Materials and Methods: This retrospective analysis included the combined experience from two tertiary care institutions. Medical records of histopathologically proven cases of IVCrs from January 1994 to June 2021 were assessed, and images were analyzed based on the criteria by Migliore et al. for inclusion of solely intraventricular lesion with the third ventricular ependyma demarcating it from the suprasellar cistern.Results: Among the 25 patients included (mean age: 35.4 years), the most common presentation included headache (n = 21, 84%), vomiting and other features of raised ICP (n = 18, 72%), visual complaints (n = 12, 48%), and endocrinopathies (n = 11, 44%). Fifteen had predominantly cystic tumors, two were purely solid, and eight were of mixed consistency. Primary open microsurgical procedures were performed in 18 (72%) patients, of which four (16%) were endoscope-assisted. Seven (28%) underwent a purely endoscopic procedure. One underwent a staged surgery with endoscopic cyst fenestration and intracystic interferon (IFN)-alpha therapy, followed by microsurgical excision. Complete excision was achieved in 10 patients, near-total in nine, and partial excision in six. Four patients underwent a ventriculoperitoneal shunt (one before the definitive procedure). At a median follow-up of 36 months (range:11–147 months), five patients developed a recurrence, and one had a stable small residue. This patient and two others with small cystic recurrences were observed. One patient was managed with radiotherapy alone. Another underwent re-surgery after a trial of radiotherapy, and the last patient developed a local recurrence, which was managed with radiotherapy; he then later developed an intraparenchymal recurrence, which was operated.Conclusion: Purely IVCrs present with raised intracranial pressure, and visual disturbances are less common. Their deep-seated location and limited surgical field-of-view makes minimally invasive endoscopic-assisted surgery most suitable for their excision. The thin-walled cystic lesions may be occasionally adherent to the ependymal wall in close vicinity to the thalamus–hypothalamus complex, making complete excision difficult. Their responsiveness to radiotherapy, often leads to a gratifying long-term outcome.

The Anterior Interhemispheric Transcallosal Approach in Craniopharyngiomas

Objective: Craniopharyngioma is a type of embryo-epithelium tumor which originates from the remnant tissue of Rathke’s pouch, and the tumor mostly grows in saddle. These tumors are associated with significant morbidity and mortality, primarily from their anatomic proximity to many critical neurological and vascular structures. This study aimed to summarize the clinical experiences and postoperative effects of the transcallosal approach for craniopharyngiomas. Methods: A retrospective analysis was performed on 14 consecutive patients with craniopharyngioma who underwent surgical resection via anterior transcallosal route at the department of Neurosurgery of Cherchell Hospital between January 2015 and June 2020. All patients had thorough preoperative and postoperative ophthalmological and endocrine evaluations. The follow-up period ranged from 10 to 36 months. Results: Gross total resection was achieved in eight patients (57%) in this series. Six patients (43%) with preoperative visual impairment experienced significant visual improvement. The overall recurrence rate was 36%. Conclusion: The interhemispheric transcallosal approach can be used alone for purely intraventricular craniopharyngiomas, or they can be used in combination with other anterolateral and midline transcranial approaches to respect the intra and extra ventricular portions of the tumor.

Mental disorders in the clinical picture of craniopharyngiomas in adults

The clinical picture of craniopharyngiomas (CPs) is diverse and polymorphic in its manifestations, including endocrine, neurological disorders, visual and mental impairment. Mental disorders in the clinical picture of CPs are detected from 6 to 100 %, according to the literature. But more often one can find a description of individual syndromes only — memory impairment, disorders of the emotional sphere and/or behavior. There is not enough information about mental disorders in different topographic and anatomical variants of craniopharyngioma, possible pathognomonic psychopathological symptoms and/or syndromes. Objective of the article is to describe mental disorders in the clinical picture of craniopharyngioma with different topographic and anatomical variants in adults. Materials and methods. The study included 120 adult patients (18–68 years old, mean age 39 ± 2; 59 women and 61 men) who were first admitted for treatment to the National Medical Research Center for Neurosurgery named after academician N. N. Burdenko with the diagnosis of craniopharyngioma (2007–2015). The clinical-psychopathological method was the main in the research. Results. Mental disorders in the clinical picture of CPs were detected in 103 (85.8 %) patients before surgery in the form of the main syndromes: asthenic syndrome was found in 83 (69 %) patients, emotional-volitional disorders in 79 (65,8 %), amnestic disorder in 71 (59,1 %), personality changes in 71 (59,1 %), paroxysmal conditions in 55 (45,8 %), sleep and dream disorders in 54 (45 %), consciousness disorders in 24 (20 %) patients. Mental disorders did not appear separately, but in combination with each other, prevailing in the clinical picture to one degree or another in various topographic and anatomical variants of craniopharyngioma, respectively. Conclusion. Mental disorders in the clinical picture of CPs were detected in 85,8 % of patients before surgery, they were determined by the location of the tumor lesion — topographic and anatomical variant of CP, the spread and influence of the tumor on the structures of the diencephalic region, limbic system, and third ventricle: in 65 % of cases with endosuprasellar craniopharyngioma, in 86 % of cases with stem craniopharyngioma, in 100 % of cases with extra- and intraventricular CPs.

Complications of endoscopic endonasal transsphenoidal approach for management of craniopharyngiomas

Abstract Introduction: Craniopharyngiomas have been classically removed by various transcranial approaches (craniotomy). Nowadays with the advance in endoscope transsphenoidal surgery, the extended transsphenoidal approach is the best choice for removal of suprasellar tumors and even intra-ventricular craniopharyngiomas. However, this is still challenging to surgeons in removing the tumors by this approach and the surgical complications could be occurred. Material and Methods: From 7/2013 – 7/2017, 50 patients (39 adults and 11 children) underwent surgery for craniopharyngioma by nasal transsphenoidal approaches. The complications regarding the surgery was recorded. Results: The early postoperative mortality was 2/50 (4%) caused by meningitis, intra-ventricular hemorrhage. The epidural hematoma was 1/50 (2%); Meningitis 6/50 (12%); Cerebrospinal fluid (CSF) leakage occurred in 3/50 (6%); Hypothalamus damage was 1/50 (2%), Visual deterioration was 3/50 (6%) Conclusion: Almost Craniopharyngioma have been removed successfully by endoscope transsphenoidal surgery. However, still the surgical complication rate was related high such as meningitis, visual deterioration, CFS leakage and postoperative mortality rate recorded.

Craniopharyngiomas Primarily Involving the Hypothalamus: A Model of Neurosurgical Lesions to Elucidate the Neurobiological Basis of Psychiatric Disorders

This study provides a systematic review and meta-analysis of psychiatric disorders caused by craniopharyngiomas and the hypothalamic alterations underlying these symptoms. We investigated a collection of 210 craniopharyngiomas reported from 1823 to 2017 providing detailed clinical and pathologic information about psychiatric disturbances, including 10 of our own series, and compared the hypothalamic damage in this cohort with the present in a control cohort of 105 cases without psychiatric symptoms. Psychiatric disorders occurred predominantly in patients with craniopharyngiomas developing primarily at the infundibulotuberal region (45%) or entirely within the third ventricle (30%), mostly affecting adult patients (61%; P < 0.001). Most tumors without psychic symptoms developed beneath the third ventricle floor (53.5%; P < 0.001), in young patients (57%; P < 0.001). Psychiatric disturbances were classified in 6 major categories: 1) Korsakoff-like memory deficits, 66%; 2) behavior/personality changes, 48.5%; 3) impaired emotional expression/control, 42%; 4) cognitive impairments, 40%; 5) mood alterations, 32%; and 6) psychotic symptoms, 22%. None of these categories was associated with hydrocephalus. Severe memory deficits occurred with damage of the mammillary bodies (P < 0.001). Mood disorders occurred with compression/invasion of the third ventricle floor and/or walls (P < 0.012). Coexistence of other hypothalamic symptoms such as temperature/metabolic dysregulation or sleepiness favored the emergence of psychotic disorders (P < 0.008). Postoperative psychiatric outcome was better in strictly intraventricular craniopharyngiomas than in other topographies (P < 0.001). A multivariate model including the hypothalamic structures involved, age, hydrocephalus, and hypothalamic symptoms predicts the appearance of psychiatric disorders in 81% of patients. Craniopharyngiomas primarily involving the hypothalamus represent a neurobiological model of psychiatric and behavioral disorders.

Progress and Achievements of Endoscopic Endonasal Transsphenoidal Surgery

For the past 11 years we gained a great experience in treating over 5,000 patients by endoscopic endonasal transsphenoidal approach. The majority of patients had pituitary adenomas (p.a.) (85%). The other tumors were represented by different pathologies: craniopharyngiomas (more 500 pts), chordomas, meningiomas, gliomas, cancers, arachnoid cysts etc. Use of endoscopic technique permitted us to considerably broaden indications for transsphenoidal surgery. In particular, this approach allowed removal of p.a. with a small-size sella, adenomas with different secondary nodes, p.a. with a narrow neck between their superior and basal parts, and giant pituitary tumors (over 60 mm). Introduction of endoscopic technologies into daily practice permitted to evacuate tumors via anterior extended transsphenoidal approach which earlier could be hardly accessed by a transcranial approach: suprasellar and intraventricular craniopharyngiomas, anterior scull base meningiomas, chiasmal and III ventricular gliomas and other suprasellar tumors. Lateral extended transsphenoidal approach allows safety removal tumors invading cavernous sinus (especially when medial displacement internal carotid artery)—p.a., chordomas, trigeminal and oculomotors nerves schwannomas. Via posterior extended transsphenoidal approach is available the removal clivus chordomas invading in posterior fossae, clivus meningiomas, some ventral brainstem tumors, clipping aneurism of Basilar artery. A modified method was used for a multilayer hermetic closure using auto- and allotransplant of major postoperative skull base defects and prevention of postoperative CSF leakage.

Strictly Intraventricular Craniopharyngioma: Case Report and Literature Review

AbstractStrictly intraventricular craniopharyngiomas are a rare topographical variety of craniopharyngiomas. The correct diagnosis is important in order to define the surgical planning, as the surgical access is different for suprasellar tumors with secondary invasion of the third ventricle. An image diagnosis may be difficult, though suggestive patterns exist. The aim of the present case report and literature review is to add to the scarce literature on strictly intraventricular craniopharyngiomas, as well as to remind the neurosurgeon of this rare diagnosis so that the proper treatment is provided.

The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part I: historical review of the topographical diagnosis and classification schemes of craniopharyngiomas

This study reviews the historical evolution of pathological, neuroradiological and surgical evidence that influenced the topographical concepts and classification schemes of craniopharyngiomas. An extensive, systematic analysis of the surgical series of craniopharyngiomas reported in the literature was performed (n=145 series, 4,588 tumours) to describe the fundamental anatomical findings guiding the topographical classification schemes used for this tumour throughout history. These findings were compared with topographical relationships reported for well-described operated craniopharyngiomas (n=224 cases) as well as for non-operated cases studied in autopsies (n=201 cases). Two major variables define the topography of a craniopharyngioma: its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. Suprasellar lesions displacing the third ventricle floor upwards (pseudointraventricular craniopharyngiomas) are amenable to safe, radical resection and must be differentiated from lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas). The latter group typically shows tight, circumferential adhesion to the third ventricle floor and represents approximately 40% of all cases. A triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning surgical approach and degree of resection. The group of infundibulo-tuberal craniopharyngiomas associates the highest risk of hypothalamic injury (50%). The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the topography of the craniopharyngioma and its degree of adherence to the hypothalamus.

Premio Sixto Obrador 2013. Modelo topográfico de 3 ejes para el tratamiento quirúrgico de los craneofaringiomas. Parte II: Evidencias anatómicas y neurorradiológicas que definen el modelo de clasificación de 3 ejes y su utilidad para predecir el riesgo quirúrgico individual

This study evaluates the pathological and magnetic resonance imaging evidence to define the precise topographical relationships of craniopharyngiomas and to classify these lesions according to the risks of hypothalamic injury associated with their removal. An extensive, systematic analysis of the topographical classification models used in the surgical series of craniopharyngiomas reported in the literature (n=145 series, 4,588 craniopharyngiomas) was performed. Topographical relationships of well-described operated craniopharyngiomas (n=224 cases) and of non-operated cases reported in autopsies (n=201 cases) were also analysed. Finally, preoperative and postoperative magnetic resonance imaging studies displayed in craniopharyngiomas reports (n=130) were compared to develop a triple-axis model for the topographical classification of these lesions with qualitative information regarding the associated risk of hypothalamic injury. The 2 major variables with prognostic value to define the topography of a craniopharyngioma are its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. A multivariate diagnostic model including 5 variables -patient age, presence of hydrocephalus and/or psychiatric symptoms, the relative position of the hypothalamus and the mammillary body angle- makes it possible to differentiate suprasellar craniopharyngiomas displacing the third ventricle upwards (pseudointraventricular craniopharyngiomas) from either strictly intraventricular craniopharyngiomas or lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas). A triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning the surgical approach and degree of resection. Infundibulo-tuberal craniopharyngiomas represent 42% of all cases. These lesions typically show tight, circumferential adhesion to the third ventricle floor, with their removal being associated with a 50% risk of hypothalamic injury. The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the degree and extension of craniopharyngioma adherence to the hypothalamus.

Transcortical–transforaminal microscopic approach for purely intraventricular craniopharyngioma

Purely intraventricular craniopharyngiomas are rare and pose particular surgical challenges. The two main surgical approaches to these lesions based in the anterior third ventricle are the frontal transventricular approach (through a transcortical or transcallosal approach) and the trans-lamina terminalis approach. The authors note that the pituitary stalk in many of these cases is located in a normal position, which suggests that the third ventricular floor is intact. In such cases, the senior author chooses an approach to avoid disruption of the floor of the third ventricle. Specifically, a traditional frontotemporal approach is not used; we have found that in such cases, a frontal transventricular approach through the usually dilated foramen of Monro provides an optimal visualization of the tumor while minimizing the risks of injury to the hypothalamus and pituitary stalk. The endoscope can be very helpful in exploring blind angles, hidden from the microscopic view. Recognition of this rare location variant of craniopharyngioma is helpful in preoperative planning in an effort to reduce hypothalamic pituitary axis damage. Two patients presenting with craniopharyngiomas that were entirely intraventricular are shown in the video. The patients underwent removal of their tumors without incurring new long-term endocrine deficits. The video can be found here: http://youtu.be/VFlhm_lsrGY. (http://thejns.org/doi/abs/10.3171/2013.V1.FOCUS12347)

Intraventricular craniopharyngioma: morphological analysis and outcome evaluation of 17 cases

There is still some confusion with regard to the tumor-third ventricle floor (3rd VF) relationship of craniopharyngiomas located exclusively within the third ventricle. This study aims to provide some evidence to clarify the growth pattern of intraventricular craniopharyngiomas (IVC), and to summarize the surgical strategy and outcome. Seventeen cases of IVC were reviewed retrospectively in relation to preoperative imaging, clinical presentation, intraoperative findings, tumor pathology, and surgical outcome. The tumor-3rd VF relationship and the tumor's stratification were analyzed based on intraoperative inspection and histology. Variable adherence patterns of IVC to the 3rd VF were found, which were classified as (a) purely IVC with pedicle attachment to 3rd VF (two cases), (b) intra-3rd VF tumors with wide-based attachment but a dissectible tumor boundary (seven cases), and (c) intra-3rd VF tumors with an undissectible wide, tight attachment (eight cases). Histological analysis revealed that both of the two cases with growth pattern "a" intruded into the third ventricular cavity without a covering layer of neural tissue (which only exists in the squamous-papillary subtype). Tumors with growth pattern "b" and "c," in contrast, were noted to have a thin layer of neural tissue. This occurred in both subtypes (11 adamantinomatous, 4 papillary). Total removal was accomplished in all tumors demonstrating growth pattern "a" and "b." There was also better preservation of the 3rd VF and consequently a better outcome. On the other hand, total removal was only achieved in 50% of tumors showing growth pattern "c" including one mortality. No recurrence has been encountered in patients whose tumors were totally removed. Variable adherence patterns and tumor subtypes were observed in IVCs, which were correlated to the tumor pathology, resectability, and subsequent prognosis.

EXTENDED ENDOSCOPIC TRANSSPHENOIDAL APPROACH FOR EXTRASELLAR CRANIOPHARYNGIOMAS

Suprasellar craniopharyngiomas have been classically removed using a variety of transcranial approaches. Historically, the transsphenoidal route was reserved for intrasellar-infradiaphragmatic, and preferably cystic, lesions. With the advent of the endoscope in transsphenoidal surgery, its obvious advantages combined with neurosurgeons' increasing interest in extended transsphenoidal approaches made suprasellar and even intraventricular craniopharyngiomas accessible for removal via such a low route. Between January of 2004 and April of 2006, six men and four women (mean age, 57.2 yr; range, 26-70 yr) underwent surgery for craniopharyngioma, including two intrasuprasellar, one suprasellar, six suprasellar-intraventricular, and one that was purely intraventricular. Three patients had undergone a previous transcranial surgery via the pterional approach, whereas one patient had undergone a transsphenoidal microsurgical approach. The surgical method consisted of an extended endoscopic transsphenoidal approach with removal of the upper half of the sella, the tuberculum sellae, and the posterior part of the planum sphenoidale, using a "three-four hands" technique. Total craniopharyngioma removal was achieved for seven patients, subtotal removal was possible for two patients, and one patient had a partial removal. Two patients developed a postoperative cerebrospinal fluid leak that required a successful endoscopic revision of the cranial base reconstruction. One patient died 5 weeks later because of hypothalamic dysfunction. All patients with visual field and/or visual acuity defect improved except one patient, in which we observed a slight worsening of visual acuity in one eye. Preoperative pituitary dysfunction did not improve in any patient. In three patients, we observed the new occurrence of permanent diabetes insipidus. One patient developed a sphenoid sinus mycosis, which was treated with antimycotic therapy. We did not observe carotid injury, epistaxis from the sphenopalatine artery, or airway difficulties. For selected patients, the extended endoscopic endonasal approach for removal of suprasellar craniopharyngioma seems to provide a valid alternative to transcranial approaches.

Accessibility and Resectability of Craniopharyngiomas

Background: The degree of adherence of craniopharyngiomas to surrounding structures can not be fully assessed by means of preoperative neuroradiological examination. Reasonable extent of the radicality of tumor removal may be established only after its sufficient exposure during the operation. Aim and Methods: The results of morphological studies of 30 sectional cases of craniopharyngiomas of different topographical groups and the results of magnetic resonance imaging and operative findings in 102 consecutive patients operated during the years 1991 to 2006 were analyzed in order to find the principles of the choice of the most convenient surgical approach to craniopharyngiomas. Results: The trans-sphenoidal approach to primarily infradiaphragmatic (that is, intrasellar or intrasellar and suprasellar) craniopharyngiomas allows identification of the remnants of the pituitary at the bottom of a large and deep sella more readily than does craniotomy. The transcranial approach is necessary in intrasellar and suprasellar tumors of dumbbell shape, with parasellar growth, or of considerable size and relatively small sella. Partial prechiasmatic extension of the great majority of extraventricular suprasellar craniopharyngiomas allows tumor removal between the optic nerves, through the opticocarotid triangle, or laterally to the carotid via subfrontal or pterional approach. Removal of the extraventricular tumor through the lamina terminalis or the foramina of Monro may jeopardize hypothalamic structures of the floor of the third ventricle located on the upper surface of the tumor. The most common location of supradiaphragmatic craniopharyngiomas is partially inside and partially outside the cavity of the third ventricle. These intraventricular and extraventricular craniopharyngiomas are located behind the optic chiasm. Opening of the lamina terminalis allows good exposure and removal of the majority of these tumors as well as of rare purely intraventricular craniopharyngiomas. However, direct visualization of the superior part of a large or giant tumor reaching the roof of the third ventricle can be reached only after extending the exposure above the anterior communicating artery or by using a transcallosal or transfrontal approach. According to our experience, a suitable approach to giant intraventricular and extraventricular craniopharyngiomas is combined: one-stage subfrontal and transcallosal approach through one large unilateral frontal craniotomy. Conclusions: There is no universal surgical approach to either intrasellar and suprasellar or to suprasellar craniopharyngiomas. The relationships of the tumor with the surrounding structures (sella and the pituitary, optic chiasm, floor of the third ventricle) are variable and at the same time characteristic for different topographical groups of craniopharyngiomas which necessitate the choice of an appropriate surgical approach.

Intraventricular craniopharyngiomas: topographical classification and surgical approach selection based on an extensive overview.

This retrospective study analyzes the clinical, neuroradiological, pathological and surgical characteristics of well-described intraventricular craniopharyngiomas with the aims of: (i) critically to review the criteria used to affirm the diagnosis of an intraventricular location (ii) defining more accurately this topographical diagnosis preoperatively, and (iii) to investigate factors influencing the surgical outcome. Clinical, neuroradiological, pathological and surgical objective data of 104 well-described intraventricular craniopharyngiomas (IVC) reported in the literature, in addition to a new case, were analyzed. On the basis of the proofs provided for third ventricle intactness, a new topographical classification for IVC was developed, distinguishing between: (i) strict IVC, with a proved third ventricle floor integrity and (ii) non-strict IVC, without any reliable proof confirming the intactness of the third ventricle floor. Following this classification, clinical features, pathology and surgical outcome for strictly and non-strictly IVC were compared. For 105 IVC compiled, 36 belonged to the strictly group and 69 to the non-strictly group. Two pathological features were associated with the non-strictly IVC group: a preferentially adamantinomatous pattern (p=0.106) and wider and tighter adherences to third ventricle margins (p=0.01). The non-strict topography was also associated with a worse postoperative outcome (p=0.046). There was a significant relationship between the surgical approach and the final outcome (p=0.05), being the translamina terminalis approach associated with the best outcome. Two different topographies might be considered among IVC: strict and non-strict intraventricular location. Non-strictly IVC have wider and tighter adhesions to third ventricle boundaries and this subtype is associated with a worse outcome.

Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature

Background Craniopharyngiomas constitute 2 to 4% of intracranial neoplasms. However, the purely intraventricular variety are rare. Their magnetic resonance imaging (MRI) characteristically shows an intact third ventricular floor, a patent suprasellar cistern, an intact pituitary stalk, and the absence of sellar abnormalities. Methods Between 1994 and June 2002, 6 patients with purely intraventricular craniopharyngioma were surgically managed. There were 4 cystic and 2 solid lesions. The surgical approaches utilized included a frontal, parasagittal, transcallosal approach with the third ventricle being accessed using either the transforaminal or subchoroidal approach ( n = 3); pterional, transsylvian ( n = 1), and bifrontal interhemispheric ( n = 2) approaches in which the third ventricle was accessed via the lamina terminalis. A ventriculoperitoneal shunt was required for one of the 3 patients with hydrocephalus. Results Total excision was performed in 3 patients with cystic craniopharyngioma, while a small residual lesion was left adherent to the third ventricular floor in the others. There was one perioperative mortality because of septicemia. Two patients required thyroxine supplementation. Two patients developed transient and one other patient a sustained diabetes insipidus. The 2 patients with solid tumors received radiotherapy for the residual lesions. At follow up ranging from 8 to 36 months, neither tumor recurrence nor regrowth was observed in any of the patients. The symptoms of raised intracranial pressure, hypothalamic dysfunction or visual field defects had resolved. Conclusions Intraventricular craniopharyngiomas occur in an older population and present mainly with raised intracranial pressure. Visual and endocrinologic imbalances are much less in these lesions compared to the suprasellar craniopharyngiomas. They mainly attach to the third ventricular floor. The surgical approaches to the third ventricle, along with radiotherapy and hormone supplementation, were successful in the management of these rare tumors.

Craniopharyngiomas of the third ventricle: trans-lamina terminalis approach.

Craniopharyngiomas usually grow on the cisternal surface of the hypothalamic region; these tumors can also grow from the infundibulum or tuber cinereum on the floor of the third ventricle, developing exclusively into the third ventricle. The aim of the present work was to establish the usefulness of the pterional trans-lamina terminalis approach for the removal of these tumors. Eight patients who were surgically treated for craniopharyngiomas located exclusively within the third ventricle were considered. The initial symptoms were acute hydrocephalus in two cases, psychological disturbances in two, amenorrhea in two, headaches in one, and hypopituitarism in one. The diagnoses were established, in all cases except one, with magnetic resonance imaging. In all cases, the tumor completely filled the third ventricle. Total removal of the lesion was achieved in seven cases. One patient underwent partial removal. In the immediate postoperative period, no major complications were observed. Five patients required replacement hormonal therapy. All patients returned to a normal life. Many months after surgery, two patients exhibited psychological disturbances and died, the first because of voluntary withdrawal of replacement therapy (12 mo after surgery) and the second because of a severe imbalance in body fluids and electrolytes, with a subsequent hyperosmolar coma (27 mo after surgery). Only one patient who underwent initial total removal experienced a small recurrence of the lesion (30 mo after surgery); after 3 years, the lesion exhibited unchanged size. In our experience, the trans-lamina terminalis approach is a valid choice for the removal of purely intraventricular craniopharyngiomas. These tumors can be removed without significant sequelae related to the surgical approach. The proximity to the hypothalamus requires accurate neuroendocrine and electrolyte control in the postoperative period, in some cases even years after surgery.

Intraventricular craniopharyngioma: a long-term follow-up of six cases

Intraventricular craniopharyngiomas are rare tumours. They are wholly within the third ventricle and can be distinguished from suprasellar lesions which extend into the third ventricle by the presence of an intact floor of the third ventricle. They are attached to the wall of the third ventricle to a variable extent, most commonly in the region of the tuber cinereum. The long-term follow-up on six cases treated by one of the authors is presented. Headache and visual disturbance were the most common presenting features but, unlike the more common suprasellar lesions, symptomatic endocrine disturbances were not a common presenting feature. Total surgical removal can cause hypothalamic damage and the resulting morbidity can be serious and sometimes life-threatening. Subtotal removal followed by radiotherapy is probably the treatment of choice for these lesions.