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1.
World Neurosurg ; 121: e223-e229, 2019 Jan.
Article in English | MEDLINE | ID: mdl-30261394

ABSTRACT

OBJECTIVE: Readmission and reoperation are risks in the resection of benign cranial nerve tumors (BCNTs). This report analyzes the impact of patient-level and surgical factors on these adverse outcomes. METHODS: This retrospective cohort review comprised patients with a diagnosis of BCNT enrolled in the American College of Surgeons National Surgical Quality Improvement Program registry from 2011 to 2015. Multivariate logistic regression was used to determine the impact of select medical and operative factors on the primary outcomes of readmission and reoperation within 30 days, adjusted for relevant covariates. RESULTS: We identified 996 patients who underwent resection of a BCNT. The most frequent major complications were readmission (11%), reoperation (8%), surgical site infections (2.6%), and venous thromboembolism (1.5%). The most frequent indications for readmission were management of infection (2.5%), cerebrospinal fluid leak (2.3%), and hydrocephalus (0.5%). Repair of cranial and meningeal defects (3.0%), correction of lagophthalmos (1.2%), and repair of middle ear defects (1.0%) were the most common indications for reoperation. Logistic regression revealed that extremes of age were associated with readmission, whereas preoperative steroid usage, long operative time, and postoperative length of stay >3 days were associated with reoperation (P < 0.05). Obesity trended toward an association with readmission and reoperation. CONCLUSIONS: Extremes of age were associated with readmission; preoperative steroid use, long operative time, and postoperative length of stay >3 days were associated with reoperation. Surgeons should consider these factors when assessing risk of postoperative complications for BCNTs.


Subject(s)
Cranial Nerve Neoplasms/surgery , Patient Readmission/statistics & numerical data , Adult , Cranial Nerve Neoplasms/epidemiology , Female , Humans , Length of Stay/statistics & numerical data , Male , Middle Aged , Neuroma, Acoustic/epidemiology , Neuroma, Acoustic/surgery , Operative Time , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Postoperative Complications/surgery , Reoperation/statistics & numerical data , Risk Factors , Steroids/therapeutic use , United States/epidemiology
2.
Article in English | MEDLINE | ID: mdl-28002663

ABSTRACT

OBJECTIVE: Here, we examine rates of intracranial tumor diagnoses in patients with and without comorbid psychiatric diagnoses to better understand how psychiatric disease may alter risk profiles for brain tumor diagnosis. METHODS: We used a longitudinal version of the California Office of Statewide Health Planning and Development (OSHPD) database, which includes all inpatient admissions in California from 1995 to 2010. We examined patients with confirmed hospital admissions from 1997 to 2004. Patients with an intracranial tumor or psychiatric diagnosis on their first hospital admission were excluded. The primary outcome of interest was the diagnosis of intracranial tumor on any subsequent hospitalization within 5 years. Risk of tumor diagnosis was determined via Cox proportional hazard models adjusted for age, gender, race/ethnicity, and comorbidity burden. Subset analyses were performed for various tumor types. RESULTS: The risk for diagnosis of an intracranial tumor within 5 years, as determined by the hazard ratio, was 1.61 (95% CI, 1.28-2.04) for bipolar, 1.59 (95% CI, 1.41-1.72) for anxious, and 1.34 (95% CI, 1.25-1.43) for depressed cohorts relative to controls. More specifically, the risk for diagnosis of a primary benign neoplasm was elevated in depressed patients, while the risk for diagnosis of a meningioma was elevated in depressed, anxious, and bipolar disorder patients. CONCLUSIONS: Patients admitted with certain psychiatric diagnoses appear more likely to be readmitted within 5 years with specific types of intracranial tumor diagnoses. The association between certain psychiatric diagnoses and subsequent brain tumor diagnosis most likely reflects the long-held belief that slow-growing tumors may first present as psychiatric symptoms before being diagnosed. Primary care physicians should consider the possibility of an underlying intracranial tumor in patients with new psychiatric diagnoses.


Subject(s)
Brain Neoplasms/epidemiology , Cranial Nerve Neoplasms/epidemiology , Meningioma/epidemiology , Mental Disorders/epidemiology , Adult , Brain Neoplasms/complications , Brain Neoplasms/diagnosis , California/epidemiology , Comorbidity , Cranial Nerve Neoplasms/complications , Cranial Nerve Neoplasms/diagnosis , Female , Hospitalization/statistics & numerical data , Humans , Inpatients/statistics & numerical data , Longitudinal Studies , Male , Meningioma/complications , Meningioma/diagnosis , Mental Disorders/complications , Mental Disorders/diagnosis , Middle Aged , Proportional Hazards Models , Prospective Studies , Risk , Sex Factors , Time Factors
3.
Clin Neurol Neurosurg ; 148: 105-9, 2016 Sep.
Article in English | MEDLINE | ID: mdl-27434528

ABSTRACT

OBJECTIVE: Obesity has been associated with increased risk for postoperative CSF leak in patients with benign cranial nerve tumors. Other measures of postoperative morbidity associated with obesity have not been well characterized. METHODS: Patients enrolled in the American College of Surgeons' National Surgical Quality Improvement Program (ACS-NSQIP) from 2007 to 2013 with a diagnosis code of a benign neoplasm of a cranial nerve were included. The primary outcome of postoperative morbidity was analyzed as well as secondary outcomes of readmission and reoperation. The main covariate of interest was body mass index (BMI). RESULTS: A total of 561 patients underwent surgery for a benign cranial nerve neoplasm between 2007 and 2013. Readmission data, available for 2012-2013(n=353), revealed hydrocephalus, facial nerve injury, or CSF leak requiring readmission or reoperation occurred in 0.85%, 1.42%, and 3.12%, respectively. Composite morbidity included wound complications, infection, respiratory insufficiency, transfusion requirement, stroke, venous thromboembolism, coma and cardiac arrest. On multivariable analysis patients with class I (BMI 30-34.9) and II (BMI 35-39.9) obesity showed trends towards increasing return to operating room, though not significant, but there was no trend for composite complications in class I and II obesity patients. However, class III obesity, BMI≥40, was associated with increased odds of composite morbidity (OR 4.40, 95% CI 1.24-15.88) and return to the operating room (OR 5.97, 95% CI 1.20-29.6) relative to patients with a normal BMI, 18.5-25. CONCLUSIONS: Obesity is an independent and important risk factor for composite morbidity in resection of benign cranial nerve neoplasms, and as such, merits discussion during preoperative counseling.


Subject(s)
Cranial Nerve Neoplasms/surgery , Obesity, Morbid/complications , Postoperative Complications/etiology , Reoperation/statistics & numerical data , Cranial Nerve Neoplasms/epidemiology , Female , Humans , Male , Middle Aged , Obesity, Morbid/epidemiology , Postoperative Complications/epidemiology , Risk
4.
J Neurosurg ; 125(5): 1120-1129, 2016 11.
Article in English | MEDLINE | ID: mdl-26745487

ABSTRACT

OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) of the eighth cranial nerve (CN) are exceedingly rare. To date the literature has focused on MPNSTs occurring after radiation therapy for presumed benign vestibular schwannomas (VSs), while MPNSTs arising without prior irradiation have received little attention. The objectives of the current study are to characterize the epidemiology, clinical presentation, disease course, and outcome using a large national cancer registry database and a systematic review of the English literature. Additionally, a previously unreported case is presented. METHODS The authors conducted an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, a systematic review of the literature, and present a case report. Data from all patients identified in the SEER database with a diagnosis of MPNST involving the eighth CN, without a history of prior radiation, were analyzed. Additionally, all cases reported in the English literature between January 1980 and March 2015 were reviewed. Finally, 1 previously unreported case is presented. RESULTS The SEER registries identified 30 cases between 1992 and 2012. The average incidence was 0.017 per 1 million persons per year (range 0.000-0.0687 per year). The median age at diagnosis was 55 years, and 16 (53%) were women. Thirteen cases were diagnosed upon autopsy. Of the 17 cases diagnosed while alive, the median follow-up was 118 days, with 3 deaths (18%) observed. When compared with the incidence of benign VS, 1041 VSs present for every 1 MPNST arising from the eighth CN. Including a previously unreported case from the authors' center, a systematic review of the English literature yielded 24 reports. The median age at diagnosis was 44 years, 50% were women, and the median tumor size at diagnosis was 3 cm. Eleven patients (46%) reported isolated audiovestibular complaints typical for VS while 13 (54%) exhibited facial paresis or other signs of a more aggressive process. Treatment included microsurgery alone, microsurgery with adjuvant radiation, or microsurgery with chemoradiation. Sixty-one percent of patients receiving treatment experienced recurrence, 22% of which were diagnosed with drop metastases to the spine. Ultimately, 13 patients (54%) died of progressive disease at a median of 3 months following diagnosis. The ability to achieve gross-total resection was the only feature that was associated with improved disease-specific survival. CONCLUSIONS MPNSTs of the eighth CN are extremely rare and portend a poor prognosis. Nearly half of patients initially present with findings consistent with a benign VS, often making an early diagnosis challenging. In light of these data, early radiological and clinical follow-up should be considered in those who elect nonoperative treatment, particularly in patients with a short duration of symptoms or atypical presentation. These data also provide a baseline rate of malignancy that should be considered when estimating the risk of malignant transformation following stereotactic radiosurgery for VS.


Subject(s)
Cranial Nerve Neoplasms , Nerve Sheath Neoplasms , Vestibulocochlear Nerve Diseases , Vestibulocochlear Nerve , Adult , Cranial Nerve Neoplasms/diagnosis , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/etiology , Female , Humans , Male , Middle Aged , Nerve Sheath Neoplasms/diagnosis , Nerve Sheath Neoplasms/epidemiology , Nerve Sheath Neoplasms/etiology , Vestibulocochlear Nerve Diseases/diagnosis , Vestibulocochlear Nerve Diseases/epidemiology , Vestibulocochlear Nerve Diseases/etiology
5.
Neurosurg Focus ; 34(3): E5, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23451818

ABSTRACT

OBJECT: Malignant peripheral nerve sheath tumors (MPNSTs) are a rare form of malignancy arising from the Schwann cells of peripheral nerves. MPNSTs of the trigeminal nerve are exceptionally rare, with only a handful of reports in the literature. These tumors are typically very aggressive, resulting in significant patient morbidity and a generally grim prognosis. Most current reports suggest that radical resection with radiation therapy offers the best benefit. In this study, the authors systematically reviewed the world English-language literature on MPNSTs of the trigeminal nerve to analyze the presentations, treatment options, and outcomes for patients with this disease. METHODS: A literature search for MPNSTs of the trigeminal nerve confined to nonanimal, English-language articles was conducted utilizing the PubMed database, with additional cases chosen from the references of selected articles. Only cases of confirmed MPNSTs of the trigeminal nerve or its peripheral branches, based upon surgical, pathological, or radiological analysis, were included. RESULTS: From the literature search, 29 articles discussing 35 cases of MPNSTs of the trigeminal nerve were chosen. With the addition of 1 case from their own institution, the authors analyzed 36 cases of trigeminal MPNSTs. The average age of onset was 44.6 years. These tumors were more commonly seen in male patients (77.1%). The gasserian ganglion was involved in 36.1% of the cases. Of the cases in which the nerve distribution was specified (n = 25), the mandibular branch was most commonly involved (72.0%), followed by the maxillary branch (60.0%) and the ophthalmic branch (32.0%), with 44.0% of patients exhibiting involvement of 2 or more branches. Altered facial sensation and facial pain were the 2 most commonly reported symptoms, found in 63.9% and 52.8% of patients, respectively. Mastication difficulty and diplopia were seen in 22.2% of patients, facial weakness was seen in 19.4%, and hearing loss was present in 16.7%. With regard to the primary treatment strategy, 80.6% underwent resection, 16.7% underwent radiation therapy, and 2.9% received chemotherapy alone. Patients treated with complete resection followed by postoperative radiation therapy had the most favorable outcomes, with no patients showing evidence of disease recurrence with a mean follow-up of 34.6 months. Patients treated with incomplete resection followed by postoperative radiation therapy had more favorable outcomes than patients treated with incomplete resection without radiation therapy or radiation therapy alone. CONCLUSIONS: Trigeminal MPNSTs most commonly present as altered facial sensation or facial pain, although they exhibit a number of other clinical manifestations, including the involvement of other cranial nerves. While a variety of treatment options exist, due to their highly infiltrative nature, aggressive resection followed by radiation therapy appears to offer the greatest chance of recurrence-free survival.


Subject(s)
Cranial Nerve Neoplasms/surgery , Nerve Sheath Neoplasms/surgery , Trigeminal Nerve Diseases/surgery , Adolescent , Adult , Age of Onset , Aged , Aged, 80 and over , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Child , Combined Modality Therapy , Cranial Nerve Neoplasms/drug therapy , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/pathology , Cranial Nerve Neoplasms/radiotherapy , Diplopia/etiology , Facial Neuralgia/etiology , Female , Humans , Incidence , Male , Middle Aged , Neoplasm Recurrence, Local , Nerve Sheath Neoplasms/drug therapy , Nerve Sheath Neoplasms/epidemiology , Nerve Sheath Neoplasms/pathology , Nerve Sheath Neoplasms/radiotherapy , Neurosurgery/methods , Prognosis , Radiotherapy, Adjuvant , Treatment Outcome , Trigeminal Nerve Diseases/drug therapy , Trigeminal Nerve Diseases/epidemiology , Trigeminal Nerve Diseases/pathology , Trigeminal Nerve Diseases/radiotherapy , Young Adult
6.
World Neurosurg ; 80(1-2): 142-7, 2013.
Article in English | MEDLINE | ID: mdl-22722042

ABSTRACT

OBJECTIVE: To investigate recent trends in surgical volume and associated patient outcomes in the treatment of acoustic neuromas. METHODS: A retrospective analysis was performed using the Nationwide Inpatient Sample database from 2000-2007; cases from 2005 were excluded because of coding inconsistencies. Univariate and multivariate analyses were used to describe surgical trends and analyze inpatient outcomes. RESULTS: Among 14,928 patients studied, 87.1% were treated at large (based on number of beds) hospitals. Cases at these hospitals declined progressively from 2054 to 1467 cases (a 28.6% decrease) from 2000-2007; a 40.8% (178 cases per year, R(2) = 0.73) reduction in surgeries was observed from 2001-2007. Although mortality remained steady at 0.3%, nonroutine discharge (increase from 10.9% to 19.1%) and complication rates (increase from 21.5% to 23.3%) increased in recent years. Patients without private insurance had an increased risk of nonroutine discharge (odds ratio [OR] 1.7, P = 0.0033; OR 1.5, P = 0.0382), and patients with more comorbidities had an increased risk of complications (OR 1.8, P < 0.0001; OR 1.5, P < 0.0001). High surgical caseload reduced nonroutine discharge by 30% (OR 0.7, P < 0.0001) and complications by 10% (OR 0.9, P < 0.0281). CONCLUSIONS: A 41%, or 178 cases per year, reduction in surgical excision of acoustic neuroma cases was observed during the period 2001-2007. A possible explanation for this trend includes increased use of stereotactic radiosurgery. Nonroutine discharge and complications after surgical excision have increased perhaps because of surgery being used for larger tumors.


Subject(s)
Cranial Nerve Neoplasms/surgery , Neuroma, Acoustic/surgery , Neurosurgical Procedures/methods , Analysis of Variance , Comorbidity , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/mortality , Female , Health Facility Size , Humans , Income , Insurance, Health/statistics & numerical data , International Classification of Diseases , Male , Middle Aged , Neuroma, Acoustic/epidemiology , Neuroma, Acoustic/mortality , Neurosurgical Procedures/mortality , Neurosurgical Procedures/statistics & numerical data , Postoperative Complications/epidemiology , Radiosurgery/statistics & numerical data , Risk , Treatment Outcome
8.
Neurosurg Rev ; 35(2): 203-9; discussion 209-10, 2012 Apr.
Article in English | MEDLINE | ID: mdl-22006092

ABSTRACT

Chronic severe facial pain is a feared sequel of cranial base surgery. This study explores the symptomatology, incidence and impact on the individual of postoperative de novo trigeminal nerve affection as well as the recovery potential. Out of 231 patients operated for cranial base meningiomas at the Karolinska University Hospital during 7 years, 25 complained of de novo trigeminal symptoms at clinical follow-up 3 months after surgery. Six were later lost to follow-up leaving 19 participants in the study, which was conducted using a questionnaire and a structured telephone interview. All patients complained of facial numbness, affecting the V1 branch in 10/19 patients (53%), the V2 branch in 18/19 (95%) and the V3 branch in 9/19 (47%). Surprisingly, only three (16%) suffered from trigeminal pain, which could be adequately managed by pharmacotherapy. However, five patients (26%) demonstrated ocular dysaesthetic problems. Twelve (63%) described their handicap to be mild, while seven (37%) had daily or severe symptoms. Five patients (26%) reported no improvement over time, while nine (47%) showed improvement and four (21%) stated good recovery. Only one patient (5%) claimed complete symptom remission. In the present study, 11% of the patients presented with a de novo postoperative affection of the trigeminal nerve after removal of a cranial base meningioma; 37% of these reported daily/severe symptoms. Only 26% showed good recovery, observed in patients without tumour infiltration of the nerve or intraoperative nerve damage. In spite of frequent complaints of numbness, pain was uncommon (16%) and often manageable by pharmacotherapy, while ocular symptoms turned out to be more frequent and more disabling than expected.


Subject(s)
Facial Pain/epidemiology , Meningeal Neoplasms/epidemiology , Meningeal Neoplasms/surgery , Meningioma/epidemiology , Meningioma/surgery , Postoperative Complications/epidemiology , Trigeminal Nerve Diseases/epidemiology , Adult , Aged , Causality , Comorbidity , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/surgery , Female , Follow-Up Studies , Humans , Male , Meningeal Neoplasms/diagnosis , Meningioma/diagnosis , Middle Aged , Radiosurgery , Sex Factors , Skull Base/surgery , Treatment Outcome , Trigeminal Nerve/pathology , Trigeminal Nerve/surgery , Trigeminal Nerve Diseases/surgery
9.
Neuro Oncol ; 12(12): 1291-9, 2010 Dec.
Article in English | MEDLINE | ID: mdl-21068153

ABSTRACT

Vestibular schwannoma (VS) (or acoustic neuroma) accounts for about 5%-6% of all intracranial tumors; little is known about the etiology. We investigated the association between various sociodemographic indicators and VS in a cohort of 3.26 million Danish residents, with 1087 cases identified in 35 308 974 person-years under risk, with data accrued from 1993 to 2006. Complete ascertainment of cases was ensured by using population-based and clinical cancer registries. Information on sociodemographic indicators was obtained on an annually updated individual level from Statistics Denmark. Log-linear Poisson regression models were used to estimate incidence rate ratios (IRRs). Linear regression models were used to examine the association between sociodemographic indicators and tumor size. We found that IRRs decreased gradually with decreasing level of education, with values of 0.62 (95% CI: 0.49-0.78) for men and 0.62 (95% CI: 0.50-0.77) for women with a basic education compared with a higher education. Similar results were found for disposable income. Marital status was associated with a higher incidence of VS in men but not women; nonmarried men with a basic education had an IRR of 0.34 (95% CI: 0.23-0.50) compared with married men with a higher education. Lower incidence rates were also observed among unemployed or early-retirement pensioners, whereas there were no differences in incidence rates across the broad groups of occupations and across the types of districts. Sociodemographic indicators were not associated with the tumor size. The magnitude of the differences in incidence rates across the groups of different socioeconomic indicators suggests a high potential for earlier diagnosis of VS by improving the awareness of early symptoms.


Subject(s)
Cranial Nerve Neoplasms/epidemiology , Neuroma, Acoustic/epidemiology , Adult , Aged , Cohort Studies , Cranial Nerve Neoplasms/pathology , Denmark/epidemiology , Education , Female , Humans , Incidence , Income , Male , Marital Status , Middle Aged , Neuroma, Acoustic/pathology , Occupations , Prognosis , Socioeconomic Factors , Survival Rate
10.
Ann Epidemiol ; 20(10): 759-65, 2010 Oct.
Article in English | MEDLINE | ID: mdl-20816315

ABSTRACT

PURPOSE: To compare ascertainment of central nervous system (CNS) neoplasms with the use of mortality and incidence data as part of an occupational epidemiology study. METHODS: Deaths were identified by matching the cohort of 223,894 jet engine manufacturing employees to the U.S. Social Security Administration death files and the National Death Index. Incident cancer cases were identified by matching the cohort to 19 state cancer registries. RESULTS: We identified 718 cases overall: 59% by the use of both mortality and cancer incidence tracing; 24% by the use of only mortality tracing, and 17% by the use of only cancer incidence tracing. Compared with state cancer registries, death certificates missed 38% of the malignant, more than six times the benign and nearly 1.5 times the unspecified CNS cases. The positive predictive value of death certificates, with cancer registry as gold standard, was 6% for unspecified, 35% for benign, and 86% for malignant histologies. CONCLUSIONS: Death certificates seriously underascertained benign and unspecified CNS tumors; analyses determined with mortality data would not accurately capture the true extent of disease among the cohort. Most state cancer registries have only collected nonmalignant CNS tumor information since 2004, which currently limits the usefulness of state cancer registries as a source of nonmalignant CNS tumor identification. Underascertainment of CNS deaths could seriously affect interpretation of results, more so if examining nonmalignant CNS.


Subject(s)
Brain Neoplasms/epidemiology , Cranial Nerve Neoplasms/epidemiology , Death Certificates , Occupational Exposure , Registries/statistics & numerical data , Aged , Aged, 80 and over , Brain Neoplasms/diagnosis , Brain Neoplasms/etiology , Brain Neoplasms/mortality , Cause of Death , Cohort Studies , Cranial Nerve Neoplasms/diagnosis , Cranial Nerve Neoplasms/etiology , Cranial Nerve Neoplasms/mortality , Female , Humans , Incidence , Male , Middle Aged , Retrospective Studies , United States/epidemiology
11.
Otol Neurotol ; 31(9): 1493-7, 2010 Dec.
Article in English | MEDLINE | ID: mdl-20729780

ABSTRACT

OBJECTIVE: To analyze surgical treatment and outcome in patients with facial neuromas at a tertiary referral hospital. STUDY DESIGN: A chart review of 26 patients treated between 1971 and 2006, with questionnaire follow-up ranging from 2 to 19 years. All patients except one were operated with radical tumor removal approaches. RESULTS: Approximately 54% of the patients presented with symptoms related to the VIIth cranial nerve (facial palsy and facial spasm), 58% with symptoms related to the VIIIth cranial nerve (hearing deficit, tinnitus, and vertigo), and 8% related to the Vth cranial nerve (facial pain and facial sensory deficit). Approximately 39% presented with no facial symptoms. Twenty-one patients received a facial nerve graft from the greater auricular nerve or the sural nerve; 1 patient had an accessory-facial anastomosis. One patient had a subtotal tumor removal preserving the facial nerve. Three patients were not grafted. Most tumors (88%) affect the geniculate ganglion. Approximately 82% of the grafted patients regained a House-Brackmann facial nerve function (HB) grade III; 14% regained HB grades IV to V. No serious morbidity or mortality was reported. No recurrences have been reported where a total tumor removal was performed. CONCLUSION: Surgical removal of facial neuroma is a safe procedure with a low complication rate and a low recurrence rate. First symptoms are diverse and are predominantly derived from the facial and vestibulocochlear nerve. Facial nerve grafting is reliable, giving the patient an acceptable facial nerve function (HB III).


Subject(s)
Cranial Nerve Neoplasms/surgery , Facial Nerve/surgery , Neuroma/surgery , Adult , Age of Onset , Aged , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/pathology , Cranial Nerves/transplantation , Facial Nerve/pathology , Facial Nerve Diseases/epidemiology , Female , Follow-Up Studies , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Neuroma/epidemiology , Neuroma/pathology , Postoperative Complications/epidemiology , Retrospective Studies , Tomography, X-Ray Computed , Transplantation, Autologous , Young Adult
12.
Otol Neurotol ; 28(8): 1083-90, 2007 Dec.
Article in English | MEDLINE | ID: mdl-18043434

ABSTRACT

OBJECTIVE: To describe the prevalence and location of cranial nerve schwannomas, other than bilateral vestibular schwannoma, in patients with neurofibromatosis 2 (NF2). The NF2 Natural History Consortium prospectively gathered cranial magnetic resonance imaging for 83 patients across 3 annual evaluations. The time between the first and last evaluation was approximately 3 years. RESULTS: Forty-two patients (51%) had nonvestibular cranial nerve schwannomas (NVSs). Of these, 25 (60%) also had cranial meningiomas. Twenty-one of those without NVS (25% of 83) had at least 1 meningioma. The average size of the NVS was 0.4 cubic centimeters. Overall, there was no significant change in NVS size from Year 1 to Year 3 or from Year 1 to Year 2. The most common locations of the NVS were occulomotor and trigeminal. A family history of NF2 did not predict NVS location or growth. CONCLUSION: Nonvestibular cranial nerve schwannoma usually affect cranial nerves III and V, as was the case in our NF2 sample. Fortunately, neuropathies associated with these tumors are rare. In contrast, lower cranial nerve schwannomas, although also rare, are associated with swallowing difficulty, aspiration, and other sequelae.


Subject(s)
Cranial Nerve Neoplasms/etiology , Neurilemmoma/etiology , Neurofibromatosis 2/complications , Adolescent , Adult , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/pathology , Cranial Nerves/pathology , Disease Progression , Female , Genes, Neurofibromatosis 2/physiology , Humans , Magnetic Resonance Imaging , Male , Meningioma/epidemiology , Meningioma/etiology , Meningioma/pathology , Neurilemmoma/epidemiology , Neurilemmoma/pathology , Neurofibromatosis 2/pathology , Oculomotor Nerve/pathology , Trigeminal Nerve/pathology
13.
Pediatr Blood Cancer ; 46(5): 586-96, 2006 May 01.
Article in English | MEDLINE | ID: mdl-16411210

ABSTRACT

Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While these tumors are most frequently low grade astrocytomas, they follow a highly variable clinical course, and accordingly, there is much debate regarding their optimal management. Their propensity to occur in very young children and infants further complicates selection of therapy. Historically, surgery and radiotherapy have played a primary role in management, however, in the last 15 years, chemotherapy has evolved into the first-line treatment of choice. Nonetheless, chemotherapy frequently fails, but serves to delay implementation of radiotherapy or surgery until the child has progressed neuropsychologically. An overall favorable prognosis for this tumor emphasizes the need for careful selection of therapy. Herein, we review the major features of optic pathway glioma, including epidemiology, pathology, therapeutic interventions, outcome, and treatment sequelae.


Subject(s)
Cranial Nerve Neoplasms , Optic Nerve Glioma , Combined Modality Therapy , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/pathology , Cranial Nerve Neoplasms/therapy , Humans , Optic Nerve Glioma/epidemiology , Optic Nerve Glioma/pathology , Optic Nerve Glioma/therapy , Treatment Outcome
14.
Med Wieku Rozwoj ; 10(3 Pt 2): 923-48, 2006.
Article in Polish | MEDLINE | ID: mdl-17401183

ABSTRACT

Among different subtypes of neurofibromatosis (Nf), type 1 (Nf-1) predominates in frequency (approximately 97% of Nfs' patients) with an incidence of approximately 1 in 3500 live births. Nf-2, comprises 2% of the Nf population and is a very rare disease (1:40,000). Both are autosomal dominant disorders with 100% penetration, variable expression and 50% rate of new (de novo) mutations. The protein products of both, NF1 andNF2 genes are best known and the genes serve as tumour suppressors. Mutations result in a predisposition to develop a variety of tumours of the central and peripheral nervous systems, as well as other malignancies. Nf-2 is a multisystem genetic disorder associated with bilateral vestibular schwannomas, spinal cord schwannomas, meningiomas, gliomas, and juvenile cataracts with a paucity of cutaneous features, which are seen more consistently in Nf-1. In contrast to Nf-1, Nf-2 is associated with significant morbidity and decreased life span and a higher incidence of CNS tumours. However, morbidity and mortality rates in Nf-1 are not negligible. The cardinal features of Nf-1 are cafe-au-lait spots, axillary and inguinal freckling, cutaneous neurofibromas, and iris hamartomas (Lisch nodules). Optic gliomas and both malignant and benign peripheral nerve sheet tumours are the most common malignancies arising in Nf-1 patients. Among neurological symptoms epilepsy, intellectual disability and learning difficulty are also observed. Bone dysplasia results in scoliosis. There is no known medical treatment beneficial to both groups of patients. The mainstay of care for Nf patients is anticipatory guidance, and early detection and symptomatic treatment of disease complications.


Subject(s)
Genes, Neurofibromatosis 1 , Genes, Neurofibromatosis 2 , Neurofibromatoses/diagnosis , Neurofibromatoses/genetics , Comorbidity , Cranial Nerve Neoplasms/diagnosis , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/genetics , Genetic Predisposition to Disease , Humans , Neurofibromatoses/epidemiology , Neurofibromatosis 1/diagnosis , Neurofibromatosis 1/genetics , Neurofibromatosis 2/diagnosis , Neurofibromatosis 2/genetics , Optic Nerve Diseases/diagnosis , Optic Nerve Diseases/genetics , Pigmentation Disorders/diagnosis , Pigmentation Disorders/genetics , Scoliosis/diagnosis , Scoliosis/epidemiology , Scoliosis/genetics , Skin Neoplasms/diagnosis , Skin Neoplasms/epidemiology , Skin Neoplasms/genetics , Soft Tissue Neoplasms/diagnosis , Soft Tissue Neoplasms/epidemiology , Soft Tissue Neoplasms/genetics , Spinal Cord Neoplasms/diagnosis , Spinal Cord Neoplasms/epidemiology , Spinal Cord Neoplasms/genetics
15.
Laryngoscope ; 113(4): 663-7, 2003 Apr.
Article in English | MEDLINE | ID: mdl-12671425

ABSTRACT

OBJECTIVES/HYPOTHESIS: Microwave radiation exposure from cellular telephone use has been implicated in the development of intracranial tumors. The intratemporal facial nerve (IFN) is exposed to higher levels of cellular telephone radiation than intracranial tissues. The purpose of the study was to determine whether cellular telephone use is associated with an increased risk of IFN tumors. STUDY DESIGN: Case-control using a structured telephone survey at an academic, tertiary-care referral center. METHODS: Patients with IFN tumors (n = 18) were case-matched with patients treated for acoustic neuroma (n = 51), rhinosinusitis (n = 72), and dysphonia or gastroesophageal reflux disease (n = 69). Risk of facial nerve tumorigenesis was compared by extent of cellular telephone use and other risk factors. RESULTS: The odds ratio of developing an IFN tumor was 0.6 (95% CI, 0.2-1.9) with any handheld cellular telephone use and 0.4 (95% CI, 0.1-2.1) with regular cellular telephone use. No factors were associated with an increased risk for IFN tumor development. CONCLUSIONS: Regular cellular telephone use does not appear to be associated with a higher risk of IFN tumor development. The short duration of widespread cellular telephone use precludes definite exclusion as a risk for IFN tumor development.


Subject(s)
Cell Phone/statistics & numerical data , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/pathology , Electromagnetic Fields/adverse effects , Facial Nerve/pathology , Temporal Muscle/pathology , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Surveys and Questionnaires , United States/epidemiology
16.
Ann Otol Rhinol Laryngol ; 112(3): 287-92, 2003 Mar.
Article in English | MEDLINE | ID: mdl-12656424

ABSTRACT

Facial paraganglioma is an extremely rare tumor that originates from abnormal paraganglionic tissue situated in the intrapetrous facial canal. A review of the English-language literature shows that only 8 cases of facial nerve paraganglioma have been published. In each case the facial glomus presented itself sporadically, completely independent of any other form of paraganglioma. This study reports an intrapetrous facial glomus that occurred in a case of multiple paragangliomas with a hereditary pattern. To our knowledge, this is the first report of such a combination.


Subject(s)
Cranial Nerve Neoplasms/genetics , Facial Nerve , Glomus Tumor/genetics , Neoplasms, Multiple Primary/genetics , Paraganglioma/genetics , Adult , Cranial Nerve Neoplasms/epidemiology , Glomus Tumor/epidemiology , Humans , Male , Neoplasms, Multiple Primary/epidemiology , Paraganglioma/epidemiology , Pedigree
17.
Ned Tijdschr Geneeskd ; 144(44): 2088-93, 2000 Oct 28.
Article in Dutch | MEDLINE | ID: mdl-11103667

ABSTRACT

A vestibular schwannoma (acoustic neurinoma) is a benign tumour localized in the cerebellopontine angle; it can give rise to cranial nerve symptoms. In recent years stereotactic irradiation has become an alternative to radical surgery. Stereotactic irradiation is administered with a gamma knife unit or with an adapted linear accelerator, as a single fraction (radiosurgery) or fractionated (stereotactic radiation therapy). Stereotactic irradiation gives local control rates of over 90%. Post treatment hearing preservation rate is over 60% and treatment related toxicity is low. Comparable treatment results are also found in the Netherlands at the VU-Ziekenhuis in Amsterdam.


Subject(s)
Cranial Nerve Neoplasms/radiotherapy , Cranial Nerve Neoplasms/surgery , Neuroma, Acoustic/radiotherapy , Neuroma, Acoustic/surgery , Radiosurgery/methods , Computer Simulation , Cranial Nerve Neoplasms/epidemiology , Disease-Free Survival , Dose Fractionation, Radiation , Follow-Up Studies , Hearing Loss, Sensorineural/etiology , Hearing Loss, Sensorineural/prevention & control , Humans , Netherlands/epidemiology , Neuroma, Acoustic/epidemiology , Radiosurgery/adverse effects , Survival Analysis
18.
Am J Otol ; 21(2): 173-80, 2000 Mar.
Article in English | MEDLINE | ID: mdl-10733180

ABSTRACT

OBJECTIVE: Evaluation of the ability of screening high-resolution, nonenhanced, fast spin echo (FSE) T2-weighted magnetic resonance imaging (MRI) of the internal auditory canal (IAC) and cerebellopontine angle (CPA) to detect nonacoustic schwannoma causes of unilateral sensorineural hearing loss (SNHL). FSE-MRI is equally sensitive in detecting acoustic (vestibular) schwannoma as gadolinium-enhanced MRI, but sensitivity to other causes of hearing loss is unknown. STUDY DESIGN: Retrospective review of screening FSE-MRI studies. SETTING: Academic otology/neurotology and neuroradiology practices. PATIENTS: There were 1,070 patients with unilateral SNHL who underwent radiologic screening for retrocochlear pathology. RESULTS: Normal findings were found in 944 cases. Typical (acoustic) vestibular schwannoma were found in 56 patients. Seventy additional lesions were identified: 27 CPA lesions, 29 inner ear lesions, and 12 intraaxial lesions including 9 infarctions, 1 multiple sclerosis case, 1 mesial temporal lobe sclerosis, and 1 colloid cyst. CONCLUSIONS: High-resolution T2 FSE-MRI of the IAC and CPA is a highly sensitive screening tool for unilateral SNHL, which can detect a variety of lesions in addition to vestibular schwannomas. To our knowledge in 2 years of follow-up in these patients screened for IAC/CPA lesions, no other lesions causing SNHL have been found. High-resolution FSE screening technique, used in conjunction with appropriate clinical prescreening and referral, can provide an equally sensitive method of evaluating unilateral SNHL compared to gadolinium-enhanced T1 MRI while reducing costs and providing distinct advantages in evaluating nonacoustic schwannoma causes of SNHL.


Subject(s)
Cerebellopontine Angle/pathology , Cochlea/pathology , Echo-Planar Imaging/methods , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/etiology , Adult , Cranial Nerve Neoplasms/complications , Cranial Nerve Neoplasms/diagnosis , Cranial Nerve Neoplasms/epidemiology , Female , Hearing Loss, Sensorineural/epidemiology , Humans , Male , Multiple Sclerosis/complications , Neuroma, Acoustic/complications , Neuroma, Acoustic/diagnosis , Neuroma, Acoustic/epidemiology , Retrospective Studies
19.
Otolaryngol Head Neck Surg ; 122(4): 482-7, 2000 Apr.
Article in English | MEDLINE | ID: mdl-10740165

ABSTRACT

Vagal paraganglioma is a rare tumor of neural crest origin. Although the literature is in agreement with regard to epidemiology, diagnosis, and tumor biology, there is some controversy over treatment modalities for these patients. We performed a nonrandomized retrospective study in a large single-institution series of patients (n = 19) in whom vagal paraganglioma was diagnosed. General statistics included age, male/female ratio, tumor size, and duration of follow-up. Other variables such as signs and symptoms at presentation, family history, multicentricity, metastatic disease, and secretion of catecholamines were included. CT scan, MRI, and angiography were used in combination for diagnostic purposes as well as for treatment planning. Preoperative embolization was performed in 5 of the more recently treated patients. Current issues regarding the use of preoperative embolization and choice of surgical approach were analyzed. In this article the possibility and sequela of vagus nerve-sparing procedures will be presented. Operative complications and postoperative morbidity related to cranial neuropathies will be discussed. The rationale for performing adjunct procedures, including cricopharyngeal myotomy and vocal fold medialization, to facilitate the rehabilitation of patients with postoperative cranial nerve deficits will be given. Our findings and recommendations will be compared with currently accepted treatment protocols in conjunction with a review of the literature.


Subject(s)
Cranial Nerve Neoplasms , Paraganglioma , Vagus Nerve Diseases , Adult , Aged , Cranial Nerve Neoplasms/diagnosis , Cranial Nerve Neoplasms/epidemiology , Cranial Nerve Neoplasms/surgery , Embolization, Therapeutic , Female , Humans , Male , Methods , Middle Aged , Paraganglioma/diagnosis , Paraganglioma/epidemiology , Paraganglioma/surgery , Postoperative Complications , Retrospective Studies , Vagus Nerve Diseases/diagnosis , Vagus Nerve Diseases/epidemiology , Vagus Nerve Diseases/surgery
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