Outcomes following proton therapy for Ewing sarcoma of the cranium and skull base.

PURPOSE: Despite the dosimetric advantages of proton therapy, little data exist on patients who receive proton therapy for Ewing sarcoma of the cranium and skull base. This study reports local disease control and toxicity in such patients. MATERIALS/METHODS: We reviewed 25 patients (≤21 years old) with nonmetastatic Ewing sarcoma of the cranium and skull base treated between 2008 and 2018. Treatment toxicity was graded per the Common Terminology Criteria for Adverse Events v4.0. The Kaplan-Meier product limit method provided estimates of disease control and survival. RESULTS: Median patient age was 5.9 years (range, 1-21.7). Tumor subsites included the skull base (48%), non-skull-base calvarial bones (28%), paranasal sinuses (20%), and nasal cavity (4%). All patients underwent multiagent alkylator- and anthracycline-based chemotherapy; 16% underwent gross total resection (GTR) before radiation. Clinical target volume (CTV) 1 received 45 GyRBE and CTV2 received 50.4 GyRBE following GTR or 54-55.8 GyRBE following biopsy or subtotal resection. Median follow-up was 3.7 years (range, 0.26-8.3); no patients were lost. The 4-year local control, disease-free survival, and overall survival rates were 96%, 86%, and 92%, respectively. Two patients experienced in-field recurrences. One patient experienced bilateral conductive hearing loss requiring aids, two patients developed intracranial vasculopathy, and 6 patients required hormone replacement therapy for neuroendocrine deficits. None developed a secondary malignancy. CONCLUSION: Proton therapy is associated with a favorable therapeutic ratio in children with large Ewing tumors of the cranium and skull base. Despite its high conformality, we observed excellent local control and no marginal recurrences. Treatment dosimetry predicts limited long-term neurocognitive and neuroendocrine side effects.

Clinical perineural invasion of the trigeminal and facial nerves in cutaneous head and neck squamous cell carcinoma: Outcomes and prognostic implications of multimodality and salvage treatment.

BACKGROUND: Clinical perineural invasion (PNI) of the trigeminal and facial nerves from cutaneous head and neck squamous cell carcinoma (cutaneous HNSCC) is a rare clinical entity that poses unique therapeutic challenges. METHODS: A retrospective chart review of a skull base oncology database was performed of patients with the diagnosis of clinical PNI from a cutaneous HNSCC. Patients who were previously untreated underwent multimodal treatment entailing surgical resection and postoperative radiotherapy (PORT) and patients who were previously treated with radiotherapy underwent salvage surgical resection between the years 2006 and 2012. Survival outcomes, such as disease-free survival (DFS) and overall survival (OS), were analyzed and correlated with surgical factors, such as margin status, previous treatment, zone involvement, and trigeminal involvement (branch-specific), as well as the pretreatment and posttreatment pain scores. RESULTS: Of 21 patients with clinical PNI from cutaneous HNSCC, 7 patients (33%) were previously treated for their disease with primary radiotherapy. Negative tumor margins were achieved in 18 patients (86%). Three of the 7 patients (43%) undergoing salvage surgery had positive margins. One-year and 3-year DFS for previously untreated patients was 91% and 67%, respectively, whereas 1-year and 3-year DFS was 72% and 28%, respectively, for the previously treated patients. Previous radiotherapy, ophthalmic nerve involvement, and positive margins portended poorer survival outcomes in this study. CONCLUSION: The retrospective study of this rare clinical entity demonstrates that multimodal treatment can achieve favorable survival outcomes. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1280-1286, 2017.

Stereotactic radiosurgery for non-vestibular cranial nerve schwanommas.

Non-vestibular cranial nerve schwannomas (NVCNS) are rare lesions, representing <10 % of cranial nerve schwannomas. The optimal treatment for NVCNS is often derived from vestibular schwannomas experience. Surgical resection has been referred to as the first line treatment for those benign tumors, but significant complication rates are reported. Stereotactic radiosurgery (SRS) has arisen as a mainstay of treatment for many benign tumors, including schwanommas. We retrospectively reviewed the outcomes of NVCNS treated by SRS to characterize tumor control, symptom relief, toxicity, and the role of hypo-fractionation of SRS dose. Eighty-eight (88) patients, with ninety-five (95) NVCNS were treated with either single or multi-session SRS from 2001 to 2014. Local control was achieved in 94 % of patients treated (median follow-up of 33 months, range 1-155). Complications were seen in 7.4 % of cases treated with SRS. At 1-year, 57 % of patients had improvement or resolution of their symptoms, while 35 % were stable and 8 % had worsening or increased symptoms. While 42 % received only one session, results on local control were similar for one or multiple sessions (p = 0.424). SRS for NVCNS is a treatment modality that provides excellent local control with minimal complication risk compared to traditional neurosurgical techniques. Tumor control obtained with a multi-session treatment was not significantly different from single session treatment. Safety profile was also comparable for uni or multi-session treatments. We concluded that, as seen in VS treated with CK SRS, radiosurgery treatment can be safely delivered in cases of NVCNS.

Combined approaches to the skull base for intracranial extension of tumors via perineural spread can improve patient outcomes.

Many neoplasms of the head and neck extend centripetally, gaining access to the central nervous system via nerves through the skull base foramina. Often patients with perineural spread have been excluded from aggressive interventions given the overall poor prognosis and technical difficulty when addressing the perineural components. However, in carefully selected patients combined surgical approaches can provide the greatest potential for disease control as well as neural decompression for symptom relief. We performed a retrospective chart review of 20 consecutive patients who underwent skull base approaches for resection of tumors with intracranial extension via perineural spread from 2011 to 2014. Patients were evaluated for symptom change, surgical approaches, histopathology, adjuvant therapy, outcome, and prognosis. The most common presenting symptoms were pain or cranial nerve palsies. 55% of patients underwent endoscopic endonasal approaches, 50% transcranial approaches, and 15% underwent transfacial approaches. Overall 85% of patients reported symptom improvement in the post-operative period while 40% were completely asymptomatic following surgical resection. Ultimately, we observed a 45% mortality rate with an average survival of 8 months after diagnosis. In carefully selected patients, an aggressive multidisciplinary approach using a combination of surgical avenues to the skull base for the treatment of intracranial tumor via perineural extension can improve patient quality of life.

Is Patient Age Associated with Perioperative Outcomes After Surgical Resection of Benign Cranial Nerve Neoplasms?

OBJECTIVE: Previous studies have demonstrated that increasing age is associated with decreased rates of operative management and gross total resection in patients with vestibular schwannomas. METHODS: The American College of Surgeons National Surgical Quality Improvement Program data registry was used for this retrospective cohort analysis. Patients enrolled in the registry from 2007 to 2013 with a diagnosis of a benign neoplasm of a cranial nerve were included. The association between age and postoperative morbidity and mortality was evaluated by the use of multivariable logistic regression analyses. RESULTS: Of 565 patients included for analysis, the mean (median) age was 51 (53) years. Three clusters were evaluated: <50, 50-69, and ≥70 years. Mortality (0% vs. 1.03% vs. 4.55%, P = 0.012), stroke (0% vs. 0.69% vs. 6.82%, P < 0.001), and ventilator weaning failure (0.43% vs. 2.41% vs. 6.82%, P = 0.014) increased with age. Mean age was significantly greater among patients who died (70.60 vs. 50.87 years, P = 0.002) or had a stroke (68.00 vs. 50.89 years, P = 0.006), cardiac arrest (71.50 vs. 50.89 years, P = 0.038), or coma (74.00 vs. 50.96 years, P = 0.020) and those who failed ventilator weaning (62.55 vs. 50.82, P = 0.006). Age as both a continuous (odds ratio 1.10, 95% confidence interval 1.03-1.18) and categorical variable (age 70+ years odds ratio 78.88, 95% confidence interval 3.41-1825.57) was associated with an increased odds of composite morbidity. CONCLUSIONS: In patients undergoing surgery for benign cranial nerve neoplasms, increasing age is associated with increased mortality, stroke, coma, and ventilator weaning failure. Composite complication rates are significantly greater in patients ≥70 years, with a near 80-fold increased risk of complications.

Frameless stereotactic radiosurgery for the treatment of primary intracranial tumours in dogs.

Stereotactic radiosurgery (SRS) is a procedure that delivers a single large radiation dose to a well-defined target. Here, we describe a frameless SRS technique suitable for intracranial targets in canines. Medical records of dogs diagnosed with a primary intracranial tumour by imaging or histopathology that underwent SRS were retrospectively reviewed. Frameless SRS was used successfully to treat tumours in 51 dogs with a variety of head sizes and shapes. Tumours diagnosed included 38 meningiomas, 4 pituitary tumours, 4 trigeminal nerve tumours, 3 gliomas, 1 histiocytic sarcoma and 1 choroid plexus tumour. Median survival time was 399 days for all tumours and for dogs with meningiomas; cause-specific survival was 493 days for both cohorts. Acute grade III central nervous system toxicity (altered mentation) occurred in two dogs. Frameless SRS resulted in survival times comparable to conventional radiation therapy, but with fewer acute adverse effects and only a single anaesthetic episode required for therapy.

Subtotal resection for management of large jugular paragangliomas with functional lower cranial nerves.

OBJECTIVES: To evaluate tumor control following subtotal resection of advanced jugular paragangliomas in patients with functional lower cranial nerves and to investigate the utility of salvage radiotherapy for residual progressive disease. STUDY DESIGN: Case series with planned chart review. SETTING: Tertiary academic referral center. SUBJECTS AND METHODS: Patients who presented with advanced jugular paragangliomas and functional lower cranial nerves were analyzed. Primary outcome measures included extent of resection, long-term tumor control, need for additional treatment, and postoperative lower cranial nerve function. RESULTS: Twelve patients (mean age, 46.2 years; 7 women, 58.3%) who met inclusion criteria were evaluated between 1999 and 2013. The mean postoperative residual tumor volume was 27.7% (range, 3.5%-75.0%) of the preoperative volume. When the residual tumor volume was less than 20% of the preoperative volume, no tumor growth occurred over an average of 44.6 months of follow-up (P < .01). Four tumors (33.3%) demonstrated serial growth at a mean of 23.5 months following resection, 2 of which were treated with salvage stereotactic radiotherapy providing control through the last recorded follow-up. No patient experienced permanent postoperative lower cranial neuropathy as a result of surgery. CONCLUSION: Subtotal resection of jugular paragangliomas with preservation of the lower cranial nerves is a viable management strategy. If more than 80% of the preoperative tumor volume is resected, the residual tumor seems less likely to grow.

Trends in surgical use and associated patient outcomes in the treatment of acoustic neuroma.

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.

Intraoperative biopsy of the major cranial nerves in the surgical strategy for adenoid cystic carcinoma close to the skull base.

OBJECTIVE: Adenoid cystic carcinoma of the salivary glands has a propensity for perineural invasion, which could favor spread along the major cranial nerves, sometimes to the skull base and through the foramina to the brain parenchyma. This study evaluated the relationship between neural spread and relapse in the skull base. STUDY DESIGN: During surgery, we performed multiple biopsies with extemporaneous examination of the major nerves close to the tumor to guide the surgical resection. RESULTS: The percentage of actuarial local control at 5 years for patients with a positive named nerve and skull base infiltration was 12.5%, compared with 90.0% in patients who were named nerve-negative and without infiltration of the skull base (P = .001). CONCLUSIONS: Our study shows that local control of disease for patients who are named nerve-positive with skull base infiltration is significantly more complex compared with patients who are named nerve-negative without infiltration of the skull base.

A novel definition of extrathyroidal invasion for patients with papillary thyroid carcinoma for predicting prognosis.

OBJECTIVES: Extrathyroidal invasion is one of the most significant risk factors for patients with papillary thyroid carcinoma (PTC). The purpose of this study was to evaluate a novel definition of extrathyroidal invasion for patients with PTC as a method for predicting a patient's prognosis. METHODS: The prospective study was conducted for consecutive 930 patients with primary PTC who received surgery during 1993-2009. We defined only patients who had preoperative recurrent laryngeal nerve palsy or patients in whom the tumor had invaded to the mucosa of the trachea and/or esophagus as Ex3. Patients with minimal invasion were classified as Ex1, and patients with massive invasion, when we could shave off the tumors, were classified as Ex2. Patients without extrathyroidal invasion were classified as Ex0. RESULTS: Patients classified Ex3 showed significantly shorter disease-free survival (p = 0.03) and disease-specific survival (p = 0.007) than patients classified Ex2. The time to recurrence at resection sites was shorter in patients classified Ex3 than in patients classified Ex2 (p = 0.02). The time to death due to distant metastasis of patients classified Ex3 was significantly shorter than that of patients classified Ex2 (p = 0.02). Within the patients classified Ex3, disease-specific survival of patients with invasion to other nearby structures was shorter than that of patients with invasion to only recurrent laryngeal nerve (p = 0.008). CONCLUSIONS: The degree and site of invasion is an important prognostic factor for PTC. Our novel classification of extrathyroidal invasion is valuable in predicting the prognosis of PTC.

Long-term health experience of jet engine manufacturing workers: IV. A comparison of central nervous system cancer ascertainment using mortality and incidence data.

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.

Stereotactic radiosurgery for trigeminal schwannoma: tumor control and functional preservation Clinical article.

OBJECT: To evaluate outcome predictors after stereotactic radiosurgery (SRS) in patients with trigeminal schwannomas, the authors compared tumor control, functional preservation, and complications with tumor grade, tumor volume, patient age, and tumor imaging characteristics. METHODS: The records of 33 consecutive patients with trigeminal schwannoma treated via Gamma Knife surgery were retrospectively reviewed. The median patient age was 49.5 years (range 15.1-82.5 years). Eleven patients had undergone prior tumor resection. Two patients had neurofibromatosis Type 2. Lesions were classified as root type (6 tumors), ganglion type (17 tumors), and dumbbell type (10 tumors) based on their location. The median radiosurgery target volume was 4.2 cm3 (range 0.5-18.0 cm3), and the median dose to the tumor margin was 15.0 Gy (range 12-20 Gy). RESULTS: At an average of 6 years (range 7.2-147.9 months), the rate of progression-free survival (PFS) at 1, 5, and 10 years after SRS was 97.0, 82.0, and 82.0%, respectively. Factors associated with improved PFS included female sex, smaller tumor volume, and a root or ganglion tumor type. Neurological symptoms or signs improved in 11 (33.3%) of 33 patients and were unchanged in 19 (57.6%). Three patients (9.1%) had symptomatic disease progression. Patients who had not undergone a prior tumor resection were significantly more likely to show improvement in neurological symptoms or signs. CONCLUSIONS: Stereotactic radiosurgery is an effective and minimally invasive management option in patients with residual or newly diagnosed trigeminal schwannomas. Predictors of a better treatment response included female sex, smaller tumor volume, root or ganglion tumor type, and the application of SRS as the primary treatment.

Prognostic impact of magnetic resonance imaging-detected cranial nerve involvement in nasopharyngeal carcinoma.

BACKGROUND: : The purpose of this study was to evaluate the prognostic value of magnetic resonance imaging (MRI)-detected cranial nerve (CN) involvement in nasopharyngeal carcinoma (NPC). METHODS: : Retrospective analysis was made of the magnetic resonance images and medical records of 924 consecutive patients with newly diagnosed NPC. RESULTS: : Of 924 patients, 82 (8.9%) initially presented with CN palsy. CN involvement was seen on MRI in 333 (36%) patients. In T3-4 disease, MRI-evidenced CN involvement was associated with poor 3-year overall survival (OS) (35.7% vs 89.2%, P = .001) and distant metastasis-free survival (DMFS) (77.1% vs 87.8%, P = .002) rates. The survival curves of OS and DMFS for T3 disease with MRI-detected CN involvement approximated those of T4 disease (P = .322 and P = .809, respectively). In patients with MRI-detected CN involvement, no significant differences were observed in 3-year OS (78.3% vs 72.9%, P = .120), local relapse-free survival (LRFS) (89.7% vs 84.1%, P = .154), or DMFS (79.6% vs 74.8%, P = .466) rates between those with and without intracranial or orbital CN involvement. Furthermore, in patients with clinical and/or MRI-detected CN involvement, there were no significant differences in the 3-year OS (74.2% vs 80.1%, P = .067), LRFS (86.7% vs 87.9%, P = .899), or DMFS (74.6% vs 84.6%, P = .094) rates between symptomatic and asymptomatic patients. CONCLUSIONS: : The incidence of MRI-detected CN involvement was higher than CN palsy. MRI-detected CN involvement has a negative impact on the prognosis independent of lesion localization and symptoms. Cancer 2009. (c) 2009 American Cancer Society.

Cranial nerve deficits in patients with metastatic prostate carcinoma: clinical features and treatment outcomes.

BACKGROUND: Cranial nerve lesions due to metastases from prostate carcinoma to the skull base are an uncommon yet clinically significant finding. METHODS: The authors report the clinical features, treatment, and outcomes for 15 patients who presented with cranial nerve palsies complicating metastatic prostate carcinoma. Patient charts identified from a Fox Chase Cancer Center treatment data base were reviewed. RESULTS: All patients had hormone-refractory disease at the time of symptom onset. Twelve of 15 patients had received prior chemotherapy, and 13 of 15 patients had received prior radiation therapy to areas of bony pain. Symptoms varied from recognized clinical syndromes involving multiple cranial nerves to isolated cranial nerve lesions. All patients had lesions at the skull base that were visualized on computed tomography scans or magnetic resonance images. All patients were treated with palliative radiation therapy to either the whole brain or the skull base. Fourteen of 15 patients had a clinical (either partial or complete) response to radiation therapy. All responding patients subsequently died of prostate carcinoma without worsening of residual or development of new cranial nerve symptoms. Ten of 15 patients (67%) died within 3 months of developing symptoms, and the remaining 5 patients lived between 9 months and 31 months from onset of symptoms. CONCLUSIONS: The authors concluded that palliative radiation therapy should be considered in this heterogeneous group of patients given the potential for significant symptom improvement.

[Brain tumors mortality in Brazil, 1980-1998]. / Mortalidade por tumores de cérebro no Brasil, 1980-1998.

Brain tumors are rare, but their incidence and mortality have increased in different countries, including Brazil, especially among the elderly. This paper presents the mortality pattern of brain tumors in Brazil, including distribution of mortality rates by gender, age, topography, and tumor staging, from 1980 to 1998. Age-adjusted (by world population) brain cancer mortality rates increased from 2.24/100,000 to 3.35/100,000 inhabitants among the overall population during this period (an increase of 50%). Observed rates were higher during childhood than adolescence and increased with age, reaching the highest levels among the elderly. Among individuals 70 years of age and older, an average annual increase of 6% was observed in the series. Brain and meningeal tumor rates are presented for the entire country and selected State capitals. Further study is needed to elucidate the role of both new diagnostic technologies and environmental exposures potentially associated with the observed changes in brain cancer mortality rates.

Trigeminal neuroma: analysis of surgical experience with 73 cases.

OBJECTIVE: We reviewed the clinical and radiological features of 73 cases of trigeminal neuromas treated with radical surgery. METHODS: The records for 73 patients with trigeminal neuromas who were surgically treated in the neurosurgery department of King Edward VII Memorial Hospital and Seth Gordhandas Sunderdas Medical College (Mumbai, India), between 1989 and 2001, were retrospectively analyzed. The appropriateness of the selected surgical route was studied. The postoperative and follow-up data for the patients were analyzed, to determine the outcomes of radical surgery. RESULTS: In addition to the other presenting features of trigeminal neuromas, nine patients presented with the rarely reported symptom of pathological laughter. Three approaches were observed to be appropriate for treatment of these tumors, i.e., the infratemporal fossa interdural approach, the lateral basal subtemporal approach, and the retrosigmoid approach. In 51 cases (70%), total tumor excision was achieved. Two patients died during the postoperative period. With an average follow-up period of 38 months, there has been a recurrence in 1 case and 71 patients are leading independent and active lives. CONCLUSION: Radical surgery is associated with excellent clinical outcomes and long-term tumor control. A majority of tumors, even those that are large and multicompartmental, can be removed in a single surgical stage and exposure.

Tumors and tumorous masses presenting as temporomandibular joint syndrome.

OBJECTIVE: Neoplasms of the temporomandibular joint (TMJ) usually mimic common causes of TMJ syndrome, leading to delay in diagnosis. To increase awareness of TMJ neoplasms and establish guidelines for early intervention, we performed a retrospective analysis of a series of patients with neoplasms of the TMJ. STUDY DESIGN AND SETTING: A retrospective review of the records of patients with neoplasms of the TMJ from 1990 to 1997 was done. RESULTS: Six patients were identified. The neoplasms included benign and malignant neoplasms. The time from initial presentation to final diagnosis was in most cases prolonged, ranging from 3 months to 8 years. Patients typically showed advanced lesions radiographically. All patients required surgical extirpation. Postoperative radiation therapy was used for malignant lesions. Patient outcomes were delineated. CONCLUSIONS AND SIGNIFICANCE: Neoplasms, both benign and malignant, of the TMJ are rare but represent a challenging diagnostic problem. In patients with 1 month or more of recalcitrant TMJ swelling or pain, radiographic imaging may be considered to rule out these rare neoplasms. This may lead to earlier intervention and improved outcome.

Carboplatin for the treatment of children with newly diagnosed optic chiasm gliomas: a phase II study.

Management of low grade optic glioma in children and adolescents remains controversial. Treatment with chemotherapy may delay or eliminate the need for radiation therapy. Children with newly diagnosed optic chiasm glioma were eligible for enrollment in this phase II trial and received intravenous carboplatin (CBDCA) (560 mg/m2) every four weeks. Patients were monitored closely for toxicity and tumor status. Twelve children were enrolled. Six patients had stable disease, four a partial response and two progressed on therapy. Overall progression free survival was 83 +/- 11%. The median duration of follow-up was 38.6 months (range 18-63 months). No deaths were noted in our series. Thrombocytopenia was the major toxicity, and two patients required platelet transfusions. One child developed an urticarial reaction requiring discontinuation of therapy. Another child developed unilateral high frequency hearing loss. No renal toxicity was encountered. We have demonstrated that carboplatin can eliminate or delay radiation therapy in children and adolescents with low grade optic glioma. CBDCA deserves further investigation in larger clinical trials as a treatment for children with optic chiasm glioma.

Long-term follow up of 69 patients treated for optic pathway tumours before the chemotherapy era.

AIM: To analyse the long-term results of conservative management with radiotherapy in patients with optic pathway tumours. DESIGN: All 69 patients were symptomatic at diagnosis and most neoplasms involved the optic chiasm and hypothalamus. RESULTS: At 10 years, overall survival and progression free survival were 83% and 65.5%, respectively. After radiotherapy, vision improved in 18 patients and remained stable in 29 other patients. Cerebrovascular complications occurred in nine of 53 patients treated with radiotherapy after a median interval of two and a half years. These complications were five times more frequent in patients with neurofibromatosis type 1 (NF1). Severe intellectual disabilities were present in 18 children, most of whom underwent irradiation at a very young age (median age, 4 years). IMPLICATIONS: Radiotherapy is a valuable treatment in terms of tumour response, visual outcome, and progression free survival. However, in young children and in patients with NF1, major sequelae are encountered and new treatment strategies should be proposed for these patients.