Quality of life considerations for patients with anterior and central skull base malignancies.

INTRODUCTION: The extirpation of skull base malignancies may be associated with significant morbidity and can profoundly impact health related quality of life (HRQOL). In this review, we sought to provide an overview of HRQOL and the factors that influence it for patients with skull base malignancies. MATERIALS AND METHODS: Narrative review. RESULTS: HRQOL tends to improve in the months to years following treatment and is profoundly impacted by both tumor location and treatment approach. Wherever possible, HRQOL should always be elicited from the patient directly. Several HRQOL instruments have been used in this population including generic, site-specific and disease specific measures. CONCLUSION: Given that HRQOL is a multidimensional concept with several important facets, validated disease-specific instruments are generally preferred.

The clinical importance of quality-of-life scores in patients with skull base tumors: a meta-analysis and review of the literature.

Health-related quality of life (QOL) outcomes are frequently used by clinicians, patients, and researchers for assessing the effectiveness of an intervention. Small differences in QOL may be statistically significant but their clinical relevance remains undefined. The smallest changes in QOL scores of the anterior skull base surgery questionnaire (ASBS-Q) which could be considered clinically significant have not been delineated. Here we present a meta analysis and review of the literature of 273 patients undergoing skull base tumor resection. The minimal clinically important difference (MCID), defined as "the smallest change in QOL which patients perceive as beneficial", was calculated using several statistical approaches. The MCID of the ASBS-Q was 0.4 (8%, score range 1-5). Various other instruments for QOL estimations revealed a larger range of MCID score (between 6.2%-17.5%) for the different QOL domains. The statistical analyses reveal that histology (benign vs malignant), time elapsed from surgery (< or ≥6 months), and surgical approach (open vs endoscopic) have significant clinical impact on different QOL domains. This paper brings level 1b evidence which demonstrates the importance of MCID as an adjunct for estimation of QOL in patients undergoing skull base surgery.

Quality of life in extended endonasal approaches for skull base tumours.

OBJECTIVE: To assess the quality of life (QOL) impact of extended endonasal approaches and nasoseptal flap reconstruction for benign skull base tumours METHODS: A random sample of 110 patients undergoing either limited endonasal transphenoidal hypophysectomy or extended endonasal approaches (trans-cribriform, trans-sellar, trans-tuberculum, trans-pterygoid) for the removal of benign skull base tumours were asked to complete Rhinosinusitis Outcome Measure (RSOM-31) questionnaire. RESULTS: A total of 91 patients returned the completed questionnaire. All patients in the limited approach group had pituitary adenomas, while patients in the extended group had a variety of tumours including adenomas with suprasellar or cavernous extension, chordomas, meningiomas, craniopharyngiomas and dermoids. Median time to completion of questionnaire was 1104 days in the limited group and 142 days in the extended approaches group. Although smell and headache were significantly worse in the group undergoing reconstruction with Haddad flap, there was no significant difference in overall, nasal, general, emotional or sleep quality of life between the two groups. Both smell and headache showed significant improvement with time. In linear regression, the single most important factor independently associated with overall worse RSOM-31 total scores was the presence of secreting adenomas. CONCLUSION: The use of nasoseptal flap appears to have a limited negative impact in nasal quality of life, mainly related to heada- che and reduced smell, both of which tend to improve with time. Hormone-secreting tumours have the most important adverse effect in quality of life extending in general, emotional, sleep and overall wellbeing, as reflected in RSOM 31 subscales.

Preoperative and postoperative neurological, neuropsychological and behavioral impairment in children with posterior cranial fossa astrocytomas and medulloblastomas: the role of the tumor and the impact of the surgical treatment.

INTRODUCTION: The aim of the present study was to prospectively investigate if a correlation might exist between preoperative and postoperative neurological conditions, neuroradiological/intraoperative findings and results of a complete neuropsychological evaluation in children with posterior fossa medulloblastomas and astrocytomas. MATERIALS AND METHODS: Of the 65 children admitted at the Pediatric Neurosurgery of the UCSC of Rome between January 2005 and October 2009, 41 were selected; the only two exclusion criteria were represented by age under 24 months and severe neurological conditions, seen that in both cases it would not have been a possible reliable evaluation. All children underwent a preoperative and immediate postoperative complete MR study. Hydrocephalus was graded on the Evans score; brainstem infiltration was defined on intraoperative findings. Neuropsychological assessment consisted of a battery of tests tailored on the patient's age, cognitive level, and level of cooperation. Post operative neuropsychological evaluation was performed at a mean time of 2.5 min (2 mos, max 4.5 mos) from the operation, before any eventually needed adjuvant treatment (i.e., chemotherapy, radiotherapy). RESULTS: Concerning neurological status, we found a statistically significant relation between the presence of oculomotor impairment and both verbal fluency deficits (p = 0.044) and imagery disorders (p = 0.03); also, the presence of ataxia/dysmetria was significantly correlated to attention dysfunction (p = 0.01) and, more tightly, to planning dysfunction (p = 0.006). For neuroradiological/intraoperative features, Intelligence Quotient (IQ) impairment was significantly correlated to the intraoperative evidence of tumor infiltration of the brainstem (p = 0.003), a severe hydrocephalus at diagnosis (p = 0.001) and the histological diagnosis of medulloblastoma (MB) (p = 0.002). For selective skills, a significant correlation was found between linguistic processing deficits and the evidence of dentate nuclei infiltration (blindly defined on MR); procedural memory defects and imagery disorders related to the severity of the hydrocephalus (p = 0.02), infiltration of the brain stem (p = 0.01) and a histological diagnosis of MB (p = 0.01). After surgery no patient showed a worsening of his/her cognitive profile; the relationships between clinical, intraoperative, and radiological findings were substantially confirmed. DISCUSSION: Our results support the hypothesis that when present, neuropsychological impairment is already present at diagnosis and that the most statistically significant factors, which might be related with cognitive deficits in the preoperative as well as in the postoperative period, are tumor infiltration of the brainstem, the severity of hydrocephalus, and a histological diagnosis of MB.

Psychological intervention in young brain tumor survivors: the efficacy of the cognitive behavioural approach.

PURPOSE: Cognitive and behavioural therapy (CBT) is often used to treat behavioural and emotional disorders in children, and its efficacy has been described in several studies. As behavioural and emotional disorders are frequent sequelae in brain tumor survivors, the goal of this work is to describe the efficacy of a CBT intervention in the treatment of young brain tumor survivors. METHODS: Forty young patients, aged 4-18 years, were included in the study. The treatment group, composed of 17 patients, received sessions of CBT. The Child Behaviour Checklist 4-18 (CBCL/4-18) and the Vineland Adaptive Behavioural Scales (VABS) were administered to parents at the beginning and at the end of the hospitalisation. The statistical significance of changes for clinical subjects during the CBT administration was estimated. RESULTS: With regard to the CBCL/4-18, the clinical group showed a significant advantage on the withdrawn, somatic complaints, social problems, attention problems, internalising and total problem scales. On the VABS, the treatment group improved to a significantly greater extent in the social skills domain. CONCLUSIONS: These results substantiate our assumption that CBT is an effective intervention for young patients surviving brain tumors and may be particularly helpful to younger individuals in managing cancer-related limitations.

Quality of life after extended versus transsellar endoscopic skull base surgery from 767 patients.

OBJECTIVES/HYPOTHESIS: The aim of this study was to compare sinonasal-related quality of life (QOL) in patients treated by extended or transsellar endoscopic skull base surgery. STUDY DESIGN: Prospective data analysis. METHODS: Prospectively collected data from patients who underwent endoscopic skull base surgery between 2012 and 2017 were analyzed. Primary outcomes were preoperative Sino-Nasal Outcome Test-20 (SNOT-20) scores and then 1-month, 3-month, and 6-month follow-up. Comparative analysis was performed between the endoscopic transsellar approach (ETA) group (n = 647) and an extended endoscopic endonasal approach (EEEA) group (n = 120). In ETA group, the SNOT-20 score was compared between patients with a nasoseptal flap (NSF) (ETA-NSF) and without an NSF (ETA-no NSF). RESULTS: The mean total SNOT-20 score was significantly worse in the EEEA than ETA group at 1, 3, and 6 months postoperatively (P < .05). Although there was no significant difference in total SNOT-20 score between the ETA-NSF and ETA-no NSF group at 3 and 6 months after surgery, the percentage of patients with significant change (≥0.8) in the SNOT-20 score was higher in the NSF used group at 1, 3, and 6 months postoperatively (22.92% vs. 13.51%, P = .029; 20.59% vs. 5.59%, P = .039; and 24.00% vs. 4.03%, P = .003, respectively). According to multivariate analysis conducted regarding factors that deteriorate sinonasal QOL at 6 months following surgery, only NSF usage is significantly associated with poor outcome (odds ratio: 4.371, P = .011) CONCLUSIONS: Sinonasal-related QOL was significantly worse in patients treated by the EEEA versus ETA. Use of an NSF is the only poor prognostic factor in sinonasal QOL after endoscopic skull base surgery. LEVEL OF EVIDENCE: 2b Laryngoscope, 129:1318-1324, 2019.

EQ-5D Quality-of-Life Analysis and Cost-Effectiveness After Skull Base Meningioma Resection.

BACKGROUND: Skull base meningioma management is complicated by their proximity to intracranial neurovascular structures because complete resection may pose a risk of worsening morbidity. OBJECTIVE: To assess the influence of clinical outcomes and surgical management on patient-perceived quality-of-life outcomes, value, and cost-effectiveness. METHODS: Patients who underwent resection of a skull base meningioma, had adequate clinical follow-up, and completed EQ-5D-3L questionnaires preoperatively and at 1 mo and 1 yr postoperatively were identified in a retrospective review. Cost data from the Value Driven Outcomes database were analyzed. RESULTS: A total of 52 patients (83.0% women, mean age 51.9 yr) were categorized by worsened (n = 7), unchanged (n = 24), or improved (n = 21) EQ-5D-3L index scores at 1-mo follow-up. No difference in subcategory cost contribution or total cost was seen in the 3 groups. Patients with improved scores showed a steady improvement through each follow-up period, whereas those with unchanged or worsened scores did not. Mean quality-adjusted life years (QALYs) and cost per QALY improved for all groups but at a higher rate for patients with better outcomes at 30-d follow-up. Female sex, absence of proptosis, nonfrontotemporal approaches, no optic nerve decompression, and absence of surgical complications demonstrated improved EQ-5D-3L scores at 1-yr follow-up. A mean cost per QALY of $27 731.06 ± 22 050.58 was observed for the whole group and did not significantly differ among patient groups (P = .1). CONCLUSION: Patients undergoing resection of skull base meningiomas and who experience an immediate improvement in EQ-5D are likely to show continued improvement at 1 yr, with improved QALY and reduced cost per QALY.

Sinonasal quality of life after endoscopic resection of malignant sinonasal and skull base tumors.

OBJECTIVES: Improvement in sinonasal quality of life (QoL) following sinus surgery has been well-documented across the literature. To our knowledge, only one series has evaluated long-term QoL in patients undergoing tumor resection, and that study demonstrated no improvement in rhinologic QoL following malignant tumor resection at 2-year follow-up. The objective of the present study was to evaluate QoL in the 2 years following endoscopic malignant tumor resection. METHODS: A prospective cohort study was performed, including patients with both malignant and benign sinonasal tumors in a tertiary academic medical center. Patients undergoing endoscopic tumor resection who had completed Sinonasal Outcome Test 22 (SNOT-22) questionnaires were included in the cohort. SNOT-22 questionnaires were administered preoperatively and over a 2-year follow-up period at clinic visits. Longitudinal linear mixed-effects regression was used to compare preoperative QoL to QoL over the 2 years following surgery. RESULTS: Among 145 patients included in this study, 64 had malignant tumors. There was a statistically significant improvement in SNOT-22 score from baseline to 2 years for patients with both malignant tumors (37.0, 95% confidence Interval [CI] 32.0-42.1 at baseline; 26.5 95% CI 20.8-32.2 at 2 years; P < 0.001) and benign tumors (26.5, 95% CI 21.4-30.4 at baseline; 12.9 95% CI 7.6-18.2 at 2 years; P < 0.001). CONCLUSION: In contrast to previously reported series, in this cohort endoscopic resection of sinonasal tumors appears to be followed by an improvement in QoL, which is sustained over a 2-year period. LEVEL OF EVIDENCE: 2b. Laryngoscope, 128:789-793, 2018.

Factors of influence upon the SF-36-based health related quality of life of patients following surgery for petroclival and lateral posterior surface of pyramid meningiomas.

OBJECTIVE: To describe the patient's self assessed health related quality of life (saHRQoL) based upon the medical outcome study 36-item short form health survey (SF-36) as well as the factors of influence upon the saHRQoL following surgery for petroclival (PCM) and lateral posterior surface of the pyramid (LPPM) meningiomas. PATIENTS AND METHODS: In a series of 78 patients operated consecutively for PCM (n = 46) or LPPM (n = 32) the preoperative, intraoperative and postoperative data were collected retrospectively. The saHRQoL was obtained by mailing the SF-36 questionnaire to the patients. The SF-36 data of the whole patients group was compared with a healthy population. The SF-36 data of the PCM- and LPPM were compared to each other. The influence of pre-, intra- and postoperative findings upon the SF-36 was assessed by uni- and multifactorial analysis. RESULTS: 58 (69%) out of the 78 patients answered the SF-36 questionnaire at a median postoperative follow-up of 59 months. The patients, who answered the SF-36 questionnaire, had a significant lower perioperative complication rate than those who did not (46% vs. 75%, p = 0.019). The saHRQoL of the LPPM and PCM was reduced on several sub-scales, when compared to the German reference population. The outcome of PCM is, assessed by saHRQoL as well as by conventional neurosurgical grading scales, inferior to that of LPPM. The saHRQoL of LPPM correlated in the uni- and multivariate analysis with the early postoperative KPI on the sub-scales SF1 (physical functioning) and SF5 (vitality). Accordingly, the sub-scale SF2 (role-physical) of PCM correlated with the change of the KPI from preoperative to the last follow up. CONCLUSIONS: The saHRQoL of the evaluable patients was lower than that of the normal population. The saHRQoL score of PCM-patients was lower than that of LPPM-patients. For the future the saHRQol should be assessed routinely; It reflects the patients' perspective upon postoperative outcome and enables the comparison with other treatment modalities of these difficult to treat tumors.

Combined subcranial approaches for excision of complex anterior skull base tumors.

OBJECTIVE: To present our method for excision of complex anterior skull base tumors via combinations of the subcranial approach. PATIENTS: Of 120 anterior skull base tumor resections, 41 that included 27 (66%) malignant and 14 (34%) benign lesions were performed via combinations of the subcranial approach. Unilateral or bilateral medial maxillectomy was performed using the subcranial approach alone for 13 tumors infiltrating the anterior skull base, ethmoid bones, and medial maxillary wall. A combined subcranial-transfacial approach in 2 lesions or a combined subcranial-midfacial degloving approach in 14 lesions was performed for tumors involving the skull base and the lower or lateral segments of the maxilla. A combined subcranial-transorbital or transfacial-transorbital approach was used for 5 tumors invading the orbit. An extended subcranial-orbitozygomatic approach was used for 6 tumors invading the middle cranial fossa or involving the cavernous sinus. A combined subcranial-Le Fort I down-fracture approach was used for 1 dedifferentiated chordoma invading the anterior skull base and lower clivus. The surgical results, patient quality of life, survival, and complications were measured. RESULTS: Thirty-seven of 41 tumors (90%) were completely resected. Fifteen patients (35.5%) had perioperative complications. There were no postoperative deaths. Two-year overall and disease-free survival in patients with malignant tumors who underwent combined approaches was 66% and 60%, respectively. There was no significant difference in the quality of life between patients operated on via combined or classic subcranial approaches. CONCLUSION: Combinations and modifications of the subcranial approach for excision of complex anterior skull base tumors yield surgical results, survival, quality of life, and complications similar to those found with the classic subcranial technique.

Neuropsychological function in adults after high dose fractionated radiation therapy of skull base tumors.

PURPOSE: To evaluate the long term effects of high dose fractionated radiation therapy on brain functioning prospectively in adults without primary brain tumors. METHODS AND MATERIALS: Seventeen patients with histologically confirmed chordomas and low grade chondrosarcomas of the skull base were evaluated with neuropsychological measures of intelligence, language, memory, attention, motor function and mood following surgical resection/biopsy of the tumor prior to irradiation, and then at about 6 months, 2 years and 4 years following completion of treatment. None received chemotherapy. RESULTS: In the patients without tumor recurrence or radiation necrosis, there were no indications of adverse effects on cognitive functioning in the post-acute through the late stages after brain irradiation. Even in patients who received doses of radiation up to 66 Cobalt Gy equivalent through nondiseased (temporal lobe) brain tissue, memory and cognitive functioning remained stable for up to 5 years after treatment. A mild decline in psychomotor speed was seen in more than half of the patients, and motor slowing was related to higher radiation doses in midline and temporal lobe brain structures. CONCLUSION: Results suggest that in adults, tolerance for focused radiation is relatively high in cortical brain structures.

Development of a cancer-specific anterior skull base quality-of-life questionnaire.

OBJECT: The goal of this study was to develop a disease-specific, multidimensional quality of life (QOL) assessment instrument for patients undergoing surgical extirpation of anterior skull base tumors. METHODS: This investigation included 35 patients who had been surgically treated for more than 3 months before the study was begun. Relevant QOL questions were generated from a review of the literature and interviews with health professionals, patients, and their caregivers. The initial multidimensional, 80-item questionnaire was reduced to a 35-item questionnaire by using standard psychometric criteria. Six relevant domains were identified using factor analysis: performance, physical function, vitality, pain, specific symptoms, and influence on emotions. The internal consistency of the instrument had a correlation coefficient of 0.8 and a reliability coefficient (test-retest reliability) of 0.9. The validity of the construct was assessed by testing whether the clinical variable of the patient influenced his QOL domain score as hypothesized. Patients older than 60 years of age had significantly poorer scores in the domains of performance and physical function than younger patients. Patients with malignant tumors had significantly poorer scores in the domains of specific symptoms, influence on emotions, physical function, and performance compared with patients with benign tumors. Radiotherapy was associated with poorer scores in the domains of specific symptoms and influence on emotions. Comorbidity was associated with poor physical function scores. Using the final questionnaire, we prospectively evaluated the QOL of 12 additional patients before they underwent surgery and again between 5 and 6 months postoperatively to test the utility and validity of the instrument further. Again, significantly poorer QOL scores were recorded for patients with malignancy. CONCLUSIONS: The proposed questionnaire appears to be sufficiently reliable and valid in estimating a patient's QOL after extirpation of anterior skull base tumors. The instrument can be used in face-to-face interviews and via electronic or regular mail.

Evaluation of outcome and QOL in patients with craniofacial resection for malignant tumors involving the anterior skull base.

The purpose of this study was to retrospectively evaluate the effectiveness of anterior craniofacial resection in the treatment of nasal and paranasal malignant tumors involving anterior skull base. Between 1992 and 1998, 13 patients with nasal or paranasal malignant tumors underwent this surgical procedure. The site and time of metastasis or recurrence, and survival outcome were retrospectively surveyed. Current status of long-surviving patients and their subjective assessment of the surgical treatment were also evaluated through questionnaires. Median follow-up period was 52 months. Nine patients (69%) were alive with no evidence of disease. Of these patients, eight had survived for more than three years. Recurrence or metastasis occurred in four patients (31%). The mean time interval between surgery and recurrence or metastasis was 11 months. According to the results of questionnaires to long-surviving patients, 89% patients had some complaints. In particular, complaints of unsightly appearance were manifested by all these patients. When the patients themselves evaluated their current conditions resulting from this surgical treatment, 63% were dissatisfied. These results suggest that this surgical treatment is valid for selected patients in regard to survival outcome. When the effectiveness of this treatment is evaluated, however, psychological and functional issues should not be taken lightly.

Quality of life following surgery for anterior skull base tumors.

OBJECTIVES: To evaluate patients' quality of life (QOL) after surgical extirpation of anterior skull base tumors, to elucidate different QOL domains, and to define possible predictors of functional outcome postoperatively. DESIGN: Retrospective survey. SETTING: University-affiliated medical center. PARTICIPANTS: Sixty-nine patients (76 consecutive cases) who underwent subcranial surgery between 1994 and 2002 for extirpation of anterior skull base tumors. MAIN OUTCOME MEASURE: A multidimensional, disease-specific questionnaire with 39 items was used. Six relevant domains of QOL were assessed: role of performance, physical functioning, vitality, pain, specific symptoms, and impact on emotions. RESULTS: The response rate for completing the questionnaire was 98% (40/41) after excluding patients who died (n = 13), were lost to follow-up (n = 10), and were operated on within 3 months of commencement of the study (n = 5). Thirty patients (74%) reported a significant improvement or no change in overall QOL within 6 months after surgery. The worst impact of surgery on the patients' QOL was on their financial status and emotional state. The most influential factor on QOL was malignancy leading to a significant decrease in the overall score. Radiotherapy, old age, comorbidity, and wide resection also significantly worsened QOL scores of specific domains. CONCLUSION: After subcranial extirpation of anterior skull base tumors, the overall outcome of the patients is good. Old age, malignancy, comorbidity, wide resection, and radiotherapy are negative prognostic factors for these patients' QOL.

[Gamma knife radiosurgery for skull base meningiomas: the treatment results and patient satisfaction expressed in answers to a questionnaire].

Skull base meningiomas can be treated by radiosurgery with low morbidity. We evaluated the effectiveness of gamma knife radiosurgery for skull base meningiomas. We also evaluated patient satisfaction through answers to a questionnaire. We treated 77 patients of skull base meningiomas using gamma knife radiosurgery from January 1994 to June 1998. Among these patients, 73 patients were able to be followed up from 6 to 60 months (average 25 months). The diagnosis was made by operations on 39 patients (53%), and on magnetic resonance imagings in 34 patients (47%). 21 patients (28.7%) had residual tumors after operations and 18 patients (24.3%) had recurrence after operations. 40% of neurological deficits occurred after previous operations and 20% of the patients were asymptomatic. The locations of the tumors were mainly the cavernous sinus region in 35 patients and the petroclival region in 20 patients. The tumor sizes were 8.6 to 58.3 mm (average 24.7 mm) and five cases of tumors with a mean diameter above 40 mm were treated by two-staged radiosurgery. Treatment volume was 0.3 to 31 ml (average 9.8 ml). The treatment dose was 8 to 16 Gy (average 11.2 Gy) at the tumor margin. The tumor decreased in size in 50 patients (68%) and was unchanged in 20 patients (28%) and we were able to achieve 96% tumor growth control. The clinical symptoms improved in 22 patients (30.1%) and were unchanged in 47 patients (64.4%) and only 4 patients (5.5%) showed deterioration. 50 patients (68%) felt clinical improvement and only 3 patients (4%) sensed deterioration. 58 patients (79%) were able to continue their previous work. Gamma knife radiosurgery has a very low morbidity rate and enables good tumor control, and patients with skull base meningiomas express satisfaction with the treatment results.

Education and information needs identified by patients and key family members prior to surgery for a skull base neoplasm: implications for practice.

Many patients with extensive skull base neoplasms poorly understand the nature of their problems. Therefore, education of patients and family members is an important component of care. Research has established the benefits of pre-operative education for other types of surgery and information seeking as an important method of coping. Yet in 184 references on skull base surgery (1996-1999), no case-based or research data that examined education and information needs prior to hospitalization for surgery was found. The investigators present findings from Phase 1 of a descriptive research study designed to determine the education and information needs perceived by patients and family members at their initial visit to the neurosurgeon and on admission to hospital. Data was collected, using interviews and a questionnaire, from 18 patients with skull base neoplasms and 15 key family members. The study findings provide insight into the experience of patients and families during a time period (prehospitalization) that has not been explored. Results indicate that key education needs of participants are related to the brain tumour and surgery. Findings reveal patient participants in contrast to family members had little in the way of information needs. Underlying and impacting the education and information needs is the theme of 'Hearing the News'. Relevance of the results to nursing practice in the pre-operative phase is addressed.

Narratives of patients with skull base tumors and their family members: lessons for nursing practice.

The meaning of the experience of being told you or a family member have/has a serious brain tumor was revealed during a descriptive research study conducted to determine preoperative education needs. Eighteen patients and 15 family members participated immediately following the office visit with the neurosurgeon and/or on admission to hospital, for a total of 29 interviews and 13 questionnaires. Twenty-four interviews were conducted with 13 patients and 11 family members in the pre-discharge phase of the research. The key themes of hearing the news, education needs and information needs were identified through content analysis of transcribed interviews and questionnaire data. Patients and family members portrayed experiences, revealed emotions related to hearing the news, and shared ways of coping. Interviews with patients and their family members that contained clear narratives were identified. From these interviews, three sets of narratives were selected. Each of the narratives was analyzed to determine how the patients and their key family members articulated and made sense of the diagnosis and surgical treatment of a skull base tumor. The themes of a sense of comfort, the known is better than the unknown, waiting for news of the surgical outcome, and quality of recovery emerged from the analysis. Lessons learned about the individuality of patients' and family members' needs and approaches to support effective coping were identified.

Determinants of quality of life in patients with skull base chordoma.

OBJECT: Skull base chordomas can be managed by surgical intervention and adjuvant radiotherapy. As survival for this disease increases, identification of determinants of quality of life becomes an important focus for guiding comprehensive patient care. In this study the authors sought to measure functional outcome and quality of life in patients with skull base chordomas and to identify determinants of quality of life in these patients. METHODS: The authors carried out an internet-based cross-sectional survey, collecting detailed data for 83 individual patients. Demographic and clinical variables were evaluated. Functional outcomes were determined by Karnofsky Performance Scale (KPS) and Glasgow Outcome Scale Extended (GOSE), quality of life was measured using the 36-Item Short Form Health Survey (SF-36), and depression was assessed using Patient Health Questions-9 (PHQ-9) instrument. Caregiver burden was assessed using the Zarit Burden Interview (ZBI). Univariate and multivariate analysis was performed to identify determinants of the physical and mental components of the SF-36. RESULTS: Patients with skull base chordomas who have undergone surgery and/or radiation treatment had a median KPS score of 90 (range 10-100, IQR 10) and a median GOSE score of 8 (range 2-8, IQR 3). The mean SF-36 Physical Component Summary score (± SD) was 43.6 ± 11.8, the mean Mental Component Summary score was 44.2 ± 12.6, and both were significantly lower than norms for the general US population (p < 0.001). The median PHQ-9 score was 5 (range 0-27, IQR 8). A PHQ-9 score of 10 or greater, indicating moderate to severe depression, was observed in 29% of patients. The median ZBI score was 12 (range 0-27, IQR 11), indicating a low burden. Neurological deficit, use of pain medication, and requirement for corticosteroids were found to be associated with worse SF-36 Physical Component Summary score, while higher levels of depression (higher PHQ-9 score) correlated with worse SF-36 Mental Component Summary score. CONCLUSIONS: Patients with skull base chordomas have a lower quality of life than the general US population. The most significant determinants of quality of life in the posttreatment phase in this patient population were neurological deficits (sensory deficit and bowel/bladder dysfunction), pain medication use, corticosteroid use, and levels of depression as scored by PHQ-9.

Quality of life instruments for skull base pathology: systematic review and methodologic appraisal.

BACKGROUND: Several quality of life (QOL) instruments exist for skull base pathology, however, there have been no attempts to appraise and systematically review these instruments. METHODS: We systematically reviewed MEDLINE, EMBASE, Central, AMED, Health and Psychosocial Instruments, and PsychoInfo for anterior or central skull base QOL instruments to January 2010. We queried experts, bibliographies, and meeting proceedings from the North American Skull Base Society from 2005 to 2009. Included instruments were evaluated for instrument characteristics, item generation and reduction, field testing, and measurement properties using predefined criteria. RESULTS: We identified 9 QOL instruments: 7 measuring QOL for pituitary pathology, 1 for midface pathology, and 1 for anterior skull base pathology. Eight of the 9 instruments have had some psychometric testing. None demonstrated all of the predefined psychometric properties. CONCLUSIONS: There are several QOL instruments for patients with skull base pathology. None of these instruments met all predefined requirements, and further instrument development is needed.

Skull base surgery for tumors in children: long-term clinical and functional outcome.

OBJECT: Skull base tumors in children are rare but require complex approaches with potential morbidity to the developing craniofacial skeleton, in addition to tumor-related morbidity. Reports of long-term clinical and functional outcome following skull base approaches in children are scarce. The authors report long-term outcome in children with tumors undergoing multidisciplinary skull base surgery. METHODS: A retrospective analysis was undertaken of children undergoing surgery at a single institution between 1998 and 2008 for benign and malignant lesions of the anterior, middle, or posterior cranial base. Patients with craniopharyngioma, pituitary tumors, and optic glioma were excluded. Histology, surgical morbidity, length of hospital stay, progression-free survival, and adjuvant therapy were recorded. Functional and cognitive outcome was assessed prospectively using the Late Effects Severity Score (LESS). RESULTS: Twenty-three children ranging in age from 13 months to 15 years underwent skull base approaches for resection of tumors during the study period. The median follow-up duration was 60 months. Tumor types included meningioma, schwannoma, rhabdomyosarcoma, neuroblastoma, angiofibroma, and chordoma. Complete resection was achieved in 12 patients (52%). Thirteen patients (57%) had benign histology. The median hospital stay was 7 days. There were 3 deaths, 1 perioperative and 2 from tumor progression. Two patients had CSF leakage (9%) and 2 developed meningitis. Two children (9%) had residual neurological deficit at last follow-up evaluation. Thirteen (59%) of 22 surviving patients received adjuvant therapy. The majority of the patients remain in mainstream education and 19 of the 20 surviving children have an LESS of 3 or lower. CONCLUSIONS: Children tolerate complex skull base procedures well, with minimal surgical-related morbidity as well as good long-term tumor control rates and functional outcomes from maximal safe resection combined with adjuvant treatment when required.