Surgical Treatment Of Craniopharyngiomas Research Articles

Surgical Approaches to Craniopharyngiomas

Surgical treatment of craniopharyngioma still presents several challenges. The tumor recurs at a high rate when its removal is insufficient. However, total resection of the tumor has a high risk of complications owing to its proximity to the hypothalamus and the pituitary stalk. In addition, tumor control by radiation treatment is insufficient for long follow-up periods of over 10 years. Therefore, various treatments have been selected, ranging from partial tumor removal followed by radiation to total tumor removal, even if it involves sacrificing pituitary functions. In this article, we explain the surgical treatment with a focus on endoscopic endonasal surgery for craniopharyngiomas.

Surgical Treatment of Craniopharyngiomas in Adults: Comparison between Primary Surgery and Surgery for Recurrence

ObjectiveFew studies have investigated the differences in outcomes between primary and repeat surgery for a craniopharyngioma in adults. As a result, a treatment concept for adult patients with a craniopharyngioma has not yet been established. The present study aimed to retrospectively analyze adult patients with craniopharyngioma to compare surgical outcomes between primary surgery and surgery for recurrence.MethodsThe demographic and clinical data of 68 adult patients with craniopharyngioma who had primary surgery (n=50) or surgery for recurrence (n=18) were retrospectively analyzed. In addition, the patients were followed up for an average of 38.6 months (range: 1–133 months).ResultsThe cohorts of patients undergoing primary surgery or repeat surgery did not differ preoperatively in terms of demographic data, or radiological tumor features. However, patients with recurrent craniopharyngioma had significantly more pituitary hormone deficits and hypothalamo-pituitary disorders before surgery compared with patients with newly diagnosed craniopharyngioma. The success rate of complete resection in primary surgery was 53.2%. Even after repeat surgery, a satisfactory rate of complete resection of 35.7% was achieved. Operative morbidity was increased neither in patients with repeat surgery compared with those with primary surgery (postoperative bleeding P=0.560; meningitis P=1.000; CSF leak P=0.666; visual disturbance P=0.717) nor in patients with complete resection compared with those with partial resection. We found no difference in recurrence-free survival between initial surgery and repeat surgery (P=0.733). The recurrence rate was significantly lower after complete resection (6.9%) than after partial resection (47.8%; P<0.001).ConclusionAttempting complete resection is justified for not only those with newly diagnosed craniopharyngioma but also for those with recurrent craniopharyngioma. However, the surgeon must settle for less than total resection if postoperative morbidity is anticipated.

NCOG-31. ROLE OF AGE IN CSF LEAK FOLLOWING TRANSSPHENOIDAL RESECTION OF CRANIOPHARYNGIOMA: ANALYSIS OF OUTCOMES IN PEDIATRIC VERSUS ADULT POPULATION

Abstract Postoperative cerebrospinal fluid (CSF) leak is a serious complication after surgical treatment of craniopharyngioma. The objective of this study is to analyze postoperative CSF leak rates following transsphenoidal resection of craniopharyngioma in adult versus pediatric patient populations. PRISMA guidelines were used to query the Pubmed, Web of Science, and Cochrane databases for studies of craniopharyngioma resection in adult or pediatric patients from the past three decades. The following search terms, ("craniopharyngioma") AND ("cerebrospinal fluid" OR "CSF") AND ("leak" OR "rhinorrhea") AND ("resection" OR "surgery") AND ("transsphenoidal" OR "endonasal"), were used. The primary outcome variable collected was presence of postoperative CSF leak. Pooled analyses were performed to determine the rates of CSF leak in adult and pediatric cases. In addition, a meta-analysis was performed to compare the rates of CSF leak in the adult versus pediatric cohort. Of the 21 included studies, 8 studies reported adult cases, 9 studies reported pediatric cases, and 4 studies reported both adult and pediatric cases, for a total of 468 cases. The majority of studies were level 4 evidence (90.5%, 19/21), while two studies were level 3 evidence (9.5%, 2/21). Analysis of postoperative CSF leak in adult and pediatric cases revealed rates of 29% (95% CI: 21%-39%) and 13% (95% CI: 9%-19%), respectively. Using a random-effects meta-analysis model, we found that there was no significant difference between the two cohorts (RR: 1.56; 95% CI = 0.52-4.71; P = 0.43). The present study is the first to analyze postoperative CSF leak rates in adult and pediatric patients undergoing transsphenoidal resection of craniopharyngiomas. While we found that there is no significant difference in CSF leak rates between adult and pediatric patients, further studies of higher methodological quality are warranted to provide a better understanding of complication profiles following resection of adult and pediatric craniopharyngiomas.

Pathogenesis and Surgical Treatment of Craniopharyngiomas

Pathogenesis and Surgical Treatment of Craniopharyngiomas

Comparative assessment of energy metabolism, body composition and metabolic features in children with hypothalamic and simple obesity

Background: Hypothalamic obesity often develops after surgical treatment of craniopharyngioma and is characterized by rapid weight gain, high frequency of metabolic disorders, body composition specificity and resistance to standard lifestyle modification approaches and medication therapy of obesity. Recent studies show that one of the mechanisms, explaining weight gain in these children is decrease in resting energy expenditure (REE).Aims: To compare REE, body composition parameters, and the frequency of metabolic disorders in children with hypothalamic and simple obesity.Materials and methods: The study included 60 obese children aged 7 to 17 years, divided into two groups. The study group included 20 children with hypothalamic obesity, developed after craniopharyngioma treatment. The control group consisted of 40 children with simple obesity. Body composition, REE, and metabolic disorders were associated in all children.Results: Children with hypothalamic obesity showed a significant decrease of resting energy expenditure. The average decrease in REE was 13.1%, but in single patients it reached 33.4%. The percentage of fat mass in hypothalamic and simple obesity does not differ significantly (39.7% [36.2; 42.6] vs 38.8 % [35.9; 43.2]; p=0.69). Screening for metabolic disorders revealed a high prevalence of metabolic disorders in hypothalamic obesity: impaired glucose tolerance - in 10%; dyslipidemia - 55%, insulin resistance-50%, non-alcoholic fatty liver disease - 60 %.Conclusions: Children with hypothalamic obesity showed a significant decrease of resting energy expenditure. When planning a diet in this group of patients it is preferable to use indirect calorimetry. Hypothalamic obesity even at an early age is associated with a high frequency of metabolic disturbance. Hypothalamic obesity in children is not associated with more pronounced hyperinsulinemia and insulin resistance compared to the simple obesity.

Comparative analysis on microsurgical removal of craniopharyngioma via lateral supraorbital approach and standard pterional approach

BackgroundCraniopharyngioma is a kind of intracranial benign tumor that is primarily treated with surgery. At present, a variety of surgical approaches are used for tumor resection. We have conducted a comparative analysis of the two approaches most used in our department.MethodsThe study retrospectively analyzed the clinical data from 65 patients with craniopharyngioma surgically treated by the two approaches mentioned above. Among these patients, 24 were treated by lateral supraorbital (LSO) approach and 41 by standard pterional approach. Indicators including, but not limited to, length of incision, operation time, postoperative pituitary function, urine volume, visual function improvement, and hospitalization were used to compare these two groups of patients.ResultsThe data shows that there was no significant difference in total tumor resection rate (P = 0.54), postoperative visual field improvement (P = 0.68) and postoperative function of endocrine. However, the LSO approach significantly reduced the operative incision (P = 0.001), shortened the operation time (P = 0.001) and operative complexity, while reducing the incidence of postoperative complications (P = 0.04).ConclusionsIn surgical treatment of craniopharyngioma, LSO approach has similar surgical effect with standard pterional approach, but it can significantly shorten the operation time, reduce surgical trauma and the incidence of complications. Therefore, LSO provides another alternative to surgical approach for microsurgical removal of craniopharyngioma.

Endoscopic endonasal transplanum transtuberculum approach for retrochiasmatic craniopharyngiomas: Operative nuances.

The surgical treatment of craniopharyngiomas is challenging. An optimal surgical approach is extremely important to achieve complete removal of the tumor, which is often the goal of treatment. Conventionally, the endoscopic transsphenoidal approach is used for resection of craniopharyngiomas that are essentially confined to the sellar cavity, or have smaller suprasellar extension. However, the tumors located in the retrochiasmatic space are difficult to remove surgically due to a poor access. Traditionally, various transcranial microsurgical routes have been employed with limited success for resection of retrochiasmatic craniopharyngiomas. The transcranial approaches generally do not provide adequate exposure of the tumors originating in the space under the optic chiasm and nerves. Recently, the extended endonasal endoscopic surgical route, obtained by removal of the tuberculum sellae and planum sphenoidale, has been used with great success in the surgical management of tumors lying ventral to the optic chiasm, including craniopharyngiomas. It offers a direct midline access to the retrochiasmatic space and provides excellent visualization of the undersurface of the optic chiasm. It also allows extracapsular dissection using binostril-bimanual technique and facilitates complete removal of these formidable tumors. In this report, we describe step-by-step, the technical details of the endonasal endoscopic transplanum transtuberculum approach with emphasis on the operative nuances for removal of retrochiasmatic craniopharyngiomas.

Minirin for the sublingual application to manage diabetes insipidus in the patients treated for brain tumours

The objective of the present study was to compare the effectiveness of desmopressin (standard oral and sublingual minirin tablets) and patient compliance with these two therapeutic modalities. The study included 51 patients at the age from 1 to 23 years presenting with diabetes insipidus (DI) at least 6 months in duration who had undergone the surgical treatment of craniopharyngioma (n=25), germinoma (n=21, glioma (n=3), and pituitary adenoma (n=2). The patients were transferred from the oral minirin formulation to the sublingual one (the dose of 100 mcg of oral minirin is equivalent to a 60 mcg sublingual tablet). The patients were interviewed to estimate their preference as regards either therapeutic modality within one week after the onset of the treatment. It turned out after the completion of the study that 30 of the 51 patients (59%) adhered to the new sublingual formulation of minirin. Their preferences were unrelated to the age of the patients, diagnosis, and duration of the disease but significantly correlated with a single dose of the preparation. Specifically, the single dose was lower than 1 tablet in 71% of the patients who chose to use oral minirin. No adverse reactions in response to the treatment with sublingual minirin were documented.

MELATONIN SECRETION AND HYPERSOMNIA IN CHILDREN AND ADOLESCENTS AFTER CRANIOPHARYNGIOMA RESECTION

Craniopharyngioma is a rare embryonic tumor of the chiasm-sellar region with low malignancy rate but high frequency of recurrence. In spite of the favorable outcome and satisfactory results of treatment the patients quality of life worsens due to complications associated with anatomic localization of craniopharyngioma close to the optic nerve, optic chiasm, hypophysis and hypothalamus. The development of hypothalamic obesity is observed in 25–60% of children and adolescents after surgical treatment of craniopharyngioma, which is usually accompanied with significant disturbances of circadian rhythm and has the most important influence on patients quality of life.

Benefit of 1.5-T intraoperative MR imaging in the surgical treatment of craniopharyngiomas

As low-field magnetic resonance imaging (MRI) has very limited significance for intraoperative control of total tumor removal (TTR), we examined the influence of 1.5-T MRI, incorporating higher resolution into the intraoperative strategy of craniopharyngioma surgery. Surgery with intraoperative imaging was performed in 25 selected patients in whom tumor resection was anticipated to be difficult according to pre-operative findings. Intraoperative MRI confirmed the intended extent of tumor removal in 15 patients (14 TTRs, one intended incomplete removal, while a second procedure was scheduled due to complex shape). Misinterpretation was false positive or negative in one patient each. The extent of removal was not achieved as expected in eight patients (expectation: seven TTRs, one incomplete removal). In three patients, the expected TTR was achieved by resuming surgery. In another case, that goal was accomplished by performing an unscheduled second procedure. In total, by using intraoperative imaging, the rate of TTR was increased by 16% (four patients), leading to 80% in the entire series. Compared with the literature, the rate of new ophthalmologic and endocrine deficits is acceptable; the rate of other surgical complication is slightly higher but not directly caused by intraoperative imaging. Intraoperative 1.5-T MRI provides benefits because of good early prediction of TTR (sensitivity, positive predictive value: 93.8%; specificity, negative predictive value: 88.9%) and a low rate of false-positive results. Moreover, extended resection of remnants visualized is enabled and helps to increase the rate of TTR but does not exclude recurrence.

Update on the Surgical Management of Craniopharyngiomas

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-ophthamologic evaluation as well as imaging, and the approach, whether trans-sphenoidal or transcranial, must take into account the nature of the tumor and its location. In addition, optimal management of craniopharyngiomas must consist of individualized and multidisciplinary therapy including an array of subspecialty fields such as endocrinology, neuro-ophthalmology, neuropsychology, and radiation oncology.

Surgical management of craniopharyngiomas

Surgical treatment of craniopharyngiomas has been historically challenging and, despite advancements in microsurgical and skull base techniques, continues to pose a challenge to modern day surgeons. In particular, proponents of subtotal resection in conjunction with radiotherapy argue that this less aggressive approach can yield equivalent control rates with lower morbidity, while others argue that gross total resection is superior. Regardless of whether gross total or subtotal resection is the goal, surgical planning must include a thorough endocrine and neuro-ophthalmologic evaluation as well as imaging, and the approach, whether transsphenoidal or transcranial, must take into account the nature of the tumor and its location. In addition, optimal management of craniopharyngiomas must consist of an individualized and multidisciplinary approach not only including neurological surgery, but also including endocrinology, neuro-ophthalmology, neuropsychology, and, often, radiation-oncology.

Craniopharyngiomas: our experience in Lyon

We reviewed our experience in surgical treatment of craniopharyngiomas. Surgical treatment of craniopharyngiomas in children represents a challenge for neurosurgeons because it presents a different set of surgical problems. Results are controversial and debates concerning strategies to ameliorate the rate of success and to decrease the rate of morbidity and mortality are ongoing. Post-surgical quality of life is related to the anatomical relationship between the hypothalamic region, vascular structures and optic pathways. From 1985 to 2004, 60 children (age range, 4 months to 18 years) were treated in our institution. Since 1987, all patients were studied with pre- and postoperative MRI. Two groups of patients were distinguished: a group of 36 patients treated with surgical direct surgery; a second group of 24 patients treated only with intracystic chemotherapy with bleomycin (18 patients) or associated with surgery (six patients). In the first group, the removal of lesion was total in 74% of cases. Two patients died in the early postoperative period and two more died later (early mortality of 5% and late mortality of 11%). All patients presented ante-pituitary insufficiency and diabetes insipidus, which required substitutive treatment. Twenty-two patients presented with visual problems, including amaurosis in two cases. In the group treated with bleomycin, 18 patients presented a primary cystic or a mixed form of craniopharyngioma and six patients showed a cystic recurrence. Eighteen patients were treated with bleomycin only. The dose used varied from 30 mg to a maximal dose of 150 mg, with a middle dose of 60 mg in the large majority of cases. In this group, the cyst disappeared in 12 patients and reduced to 30% of its initial volume, and stabilization of the lesion was achieved in the other six patients. Only 11 patients presented partial endocrine insufficiency. Of the 18 patients, 16 were of school age, 14 of whom were following a normal educational program and two an assisted program. No mortality was reported. Our experience showed that in the group treated with intracystic chemotherapy alone, results were better with a low rate of morbidity and mortality. Endocrinological, visual and neuropsychological evaluations were also correlated with better outcome. In cases of cystic craniopharyngiomas, we considered bleomycin as the treatment of choice. For solid forms or for cases resistant to intracystic chemotherapy with bleomycin, direct surgery has to be proposed.

Serum leptin levels in obese children operated on for craniopharyngioma

The study was undertaken to investigate the concentrations of leptin after surgical treatment of craniopharyngioma. Patients with obesity were studied. There were the following 3 groups: 1) 8 boys and 2 girls aged 15.6+0.8 years after surgical treatment of craniopharyngioma; 2) 10 boys and 10 girls aged 8.48±0.45 years who had constitutional obesity and stage i puberty (after tanner);and3) loboysand logirlsaged 12.31+0.44years who had constitutional obesity and tanner stages ii-v puberty. There were no significant changes in the levels of leptin in pre­pubertal children. There was an age-related change in the con­centration of leptin in girls, i.e. A significant increase in puberty. After surgical treatment of craniopharyngioma, the obese boys had lower levels of leptin than the comparison group boys with the same degree of sexual development and body weight values. There was a positive correlation of the levels of leptin and the body mass index in all age groups of patients with exogenous con­stitutional obesity.

Memory deficits among children with craniopharyngiomas.

To describe neuropsychological functioning (with a specific focus on cognition and memory) after surgical treatment of craniopharyngiomas. Sixteen patients who were between 6 and 15 years of age at the time of surgery comprised the sample. Each child had been treated for a craniopharyngioma with surgery only, on Dana-Farber Cancer Institute Protocol 92-077. The overall level of cognitive functioning was well within the average range, with both language and visuospatial functioning being generally intact; however, specific memory problems, in both the language and visuospatial domains, were evident. Although general cognitive functioning was intact after the surgical treatment of craniopharyngiomas, difficulties in the retrieval of learned information were observed. Neuropsychological assessments, with a focus on memory recall, should be a component of the medical management plan for each child.

Surgical treatment of craniopharyngiomas: endocrinological results.

This study aimed to elucidate the endocrinological outcome of craniopharyngioma surgery. In particular, endocrinological results were analyzed in relation to the surgical approach. The study includes 143 patients who underwent pre- and postoperative endocrinological assessment and who had not previously undergone surgery. Diabetes insipidus was the most common postoperative deficiency in both the transcranial and transsphenoidal groups. The overall percentage of patients with diabetes insipidus increased from 16.1% preoperatively to 59.4% postoperatively. After transcranial surgery, the rate of anterior pituitary deficiency also increased. However, normal preoperative anterior pituitary function was maintained in more than 50% of patients for each endocrine axis. Similar results were attained in the group of patients undergoing complete tumor removal. The best result was achieved for gonadal function: the incidence of hypogonadism increased only slightly from 77.4 to 79.8%. The rate of anterior pituitary failure at presentation was much higher in the transsphenoidal than in the transcranial group. During transsphenoidal surgery, intact anterior pituitary functions were generally preserved. The rate of panhypopituitarism increased only slightly, from 40% before surgery to 42.9% after surgery. Endocrinological results were not inferior in patients with a ventrally displaced pituitary. This variant required midline incision of the gland for exposure of the craniopharyngioma. In the entire series of 143 patients the pituitary stalk was generally preserved. Postoperative panhypopituitarism was encountered in only one of eight patients in whom the pituitary stalk was partially resected because of tumor infiltration. None of the 88 patients who remained recurrence-free demonstrated endocrinological deterioration during follow-up review, compared with the early postoperative assessment 3 months postsurgery. On the other hand, complete recovery of one endocrine axis was observed in nine of these patients during later follow-up evaluations. In five of them, diabetes insipidus had regressed. It is worth preserving the pituitary stalk and gland at surgery because of the definite chance that intact anterior pituitary functions can be maintained. Postoperative diabetes insipidus must be accepted as a common sequela following attempts at complete removal of the craniopharyngioma.

Surgical treatment of craniopharyngiomas: experience with 168 patients.

The goal of this study was to assess the outcome of surgical management in 168 consecutive patients harboring craniopharyngiomas treated between January 1983 and April 1997. In 148 patients undergoing initial (primary) surgery, the pterional approach was most frequently used (39.2%), followed by the transsphenoidal approach (23.6%). For large retrochiasmatic craniopharyngiomas, the bifrontal interhemispheric approach was used increasingly over the pterional approach and led to improved surgical results. Total tumor removal was accomplished in 45.7% of transcranial and 85.7% of transsphenoidal procedures. The main reasons for incomplete removal were attachment to and/or infiltration of the hypothalamus, major calcifications, and attachment to vascular structures. The success rate in total tumor removal was inferior in the cases of tumor recurrence. The operative mortality rate in transcranial surgery was 1.1% in primary cases and 10.5% in cases of tumor recurrence. No patient died in the group that underwent transsphenoidal surgery. The rate of recurrence-free survival after total removal was 86.9% at 5 years and 81.3% at 10 years. In contrast, the 5-year recurrence-free survival rate was only 48.8% after subtotal removal and 41.5% after partial removal. Following primary surgery, the actuarial survival rate was 92.7% at 10 years, with the best results after complete tumor removal. At last follow up, 117 (79%) of 148 patients who underwent primary surgery were independent and without impairment. Total tumor removal while avoiding hazardous intraoperative manipulation provides favorable early results and a high rate of long-term control in craniopharyngiomas.

Status of vision following surgical treatment of craniopharyngiomas.

The authors report visual results in a series of 42 patients with craniopharyngiomas. Visual abnormalities were present in 91% of the children and in 83% of the adults. Total removal was achieved in 16 patients with 3 postoperative deaths and subtotal removal in 26 patients with only one postoperative death. Of the 38 survivors, 63% improved, 26% remained the same and 10% worsened. When analyzing the influence of factors such as age, nature and location of the tumour, extent of removal, surgical approach, use of CT scan facilities and duration and degree of the deficit in the 31 survivors with preoperative visual impairment, it became apparent that only the duration and degree of preoperative visual deficit had a significant influence the outcome. The authors discuss also the suggestion that a conservative attitude may be associated with better visual results.

What can be expected from the surgical treatment of craniopharyngiomas in children. Report of 92 cases.

92 cases of craniopharyngiomas have been operated, 63 with a follow-up extending from 30 months to 20 years. The possibilities of the surgical treatment are mainly discussed on the basis of the anatomical location of the tumors. Simple intrasellar craniopharyngiomas can be operated by a transphenoidal approach with, sometimes, spectacular improvement (case of spontaneous normal pregnancy). The tumors most frequently seen are those with predominantly prechiasmatic expansion. A subfrontal approach is always required, aiming at radical excision. Microscopy can offer interesting possibilities for selective surgery. However, if the vital prognosis is excellent and recurrences rare, functional results remain very poor. In retrochiasmatic tumors involving the posterior hypothalamus, the principle of radical excision must be seriously discussed and sometimes challenged. The various combined approaches are described with their own limits and risks. Other therapeutic techniques are suggested. In many cases, selective neurosurgical dissection may raise some hope for better improvement of functional results.

Diagnosis and surgical treatment of childhood brain tumors.

As the most frequent solid tumor occurring in childhood, brain tumors constitute an important segment of pediatric oncology. Neurologic manifestations may be deceptively mild and easily overlooked or misinterpreted, particularly in the very young, because of the remarkable resiliency of the immature central nervous system and the skull's ability to expand throughout the pre-adolescent years. The majority of childhood tumors produce increased intracranial pressure, usually the consequence of obstructive hydrocephalus. Specific neurologic deficits correspond to the tumor's location. The posterior fossa harbors two-thirds of childhood tumors, and each of the four common tumors in this location produces a characteristic syndrome. Supratentorial tumors occupy the cerebral hemisphere, the suprasellar area, and the pineal gland. Diagnostic studies have reached a state of great sophistication and precise anatomical localization. Surgery, either alone or with adjuvant radiotherapy, cures no more than one-third of all tumors; for the remainder, it has a diagnostic and palliative role. The introduction of operative microsurgery has advanced the art, particularly in the surgical treatment of craniopharyngiomas and pinealomas, but any significant improvement in the treatment of brain tumors as a group seems unlikely to be achieved by surgery alone.