Patterns of Hearing Loss in Irradiated Survivors of Head and Neck Rhabdomyosarcoma.

PURPOSE: The frequency and patterns of HL in a HNRMS survivor cohort were investigated. A dose-effect relationship between the dose to the cochlea and HL was explored. METHODS: Dutch survivors treated for HNRMS between 1993 and 2017 with no relapse and at least two years after the end of treatment were eligible for inclusion. The survivors were evaluated for HL with pure-tone audiometry. HL was graded according to the Muenster, Common Terminology Criteria for Adverse Events (CTCAE) v4.03 and International Society for Paediatric Oncology (SIOP) classification. We defined deleterious HL as Muenster ≥ 2b, CTCAE ≥ 2, and SIOP ≥ 2. Mixed-effects logistic regression was used to search for the dose-effect relationship between the irradiation dose to the cochlea and the occurrence of HL. RESULTS: Forty-two HNRMS survivors underwent pure-tone audiometry. The Muenster, CTCAE and SIOP classification showed that 19.0% (n = 8), 14.2% (n = 6) and 11.9% (n = 5) of survivors suffered from HL, respectively. A low-frequency HL pattern with normal hearing or milder hearing loss in the higher frequencies was seen in four survivors. The maximum cochlear irradiation dose was significantly associated with HL (≥Muenster 2b) (p = 0.047). In our series, HL (≥Muenster 2b) was especially observed when the maximum dose to the cochlea exceeded 19 Gy. CONCLUSION: HL occurred in up to 19% of survivors of HNRMS. More research is needed on HL patterns in HNRMS survivors and on radiotherapy dose-effect relationships.

Prevalence of chronic rhinosinusitis in the general population based on sinus radiology and symptomatology.

BACKGROUND: The prevalence of chronic rhinosinusitis (CRS) measured in epidemiologic studies is 5% to 12%. This might be an overestimation because of overlap with other diseases, such as allergic rhinitis. OBJECTIVE: We aimed to calculate the prevalence of CRS using a combination of epidemiologically based CRS according to the European Position Paper on Rhinosinusitis and Nasal Polyps (EPOS) together with sinonasal opacification on imaging. METHODS: Subjects who underwent a computed tomographic or magnetic resonance imaging scan of the head for any nonrhinologic indication were asked to fill in the Global Allergy and Asthma European Network survey containing EPOS symptom criteria. The scans were evaluated according to the Lund-Mackay (LM) scoring system. Epidemiologically based CRS is based on nasal symptoms according to EPOS; clinically based CRS also encompasses endoscopy and/or CT scanning. RESULTS: Eight hundred thirty-four subjects were included. One hundred seven (12.8%) had epidemiologically based CRS according to EPOS. Of these subjects, 50% had an LM score of 0, 26% had an LM score of 1 to 3, and 23% had an LM score of 4 or greater. Twenty-five (3.0%) subjects had clinically based CRS (based on LM score ≥4), and 53 (6.4%) subjects had clinically based CRS (based on LM score >0). Allergic rhinitis was reported by 167 (20%) subjects. In subjects who did not report upper airway symptoms, 57% had an LM score of 0, 30% had an LM score of 1 to 3, and 12% had an LM score of 4 or greater. CONCLUSION: We found a prevalence of 3.0% to 6.4% of clinically based CRS (depending on an LM cutoff point; ie, LM ≥ 4 or LM > 0, respectively) in a relatively randomly selected group of subjects.

Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review.

Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and limitation of abduction in the affected eye. Magnetic resonance imaging findings included an orbital mass in combination with absent nasal septum and partial destruction of the paranasal sinuses. Biopsies and histopathologic examination of the nasal cavity and the orbital mass revealed chronic inflammation. Both patients were treated with oral corticosteroids, ocular movements completely normalized but no improvement of visual acuity was noted. Intranasal cocaine abuse can cause orbital complications from chronic sinonasal inflammatory disease and these patients are at risk to develop optic neuropathy. Optic neuropathy may be caused by compression, infiltration, or ischaemia.

Staging and follow-up of high-grade malignant salivary gland tumours: The role of traditional versus functional imaging approaches - A review.

Following up on the Consensus Conference on high-grade malignant salivary gland tumours (HGMSGT) in adult patients in Brescia, Italia, in October 2014, we review the current imaging modalities for diagnosing and staging these rare tumours. The advantages of functional MR imaging techniques such as diffusion weighted imaging (DWI) and dynamic contrast-enhanced MR imaging (DCEMRI) in comparison with traditional MRI will be outlined. The limited role of (18)FDG-PET CT and fMRI will be addressed. Finally, in view of new experimental treatment options, we will discuss the role of imaging during follow-up.

Local Resection and Brachytherapy for Primary Orbital Rhabdomyosarcoma: Outcome and Failure Pattern Analysis.

PURPOSE: Survival in patients with orbital rhabdomyosarcoma (RMS) is excellent. Therefore, new local treatment modalities, such as brachytherapy, have been developed to minimize adverse events. Since 1990, patients with orbital RMS and a residual tumor after induction chemotherapy were eligible for resection and brachytherapy. Otherwise patients received external beam radiotherapy. In this study, the authors describe the outcome for 20 patients with primary orbital RMS. The aim was to assess risk factors for treatment failure in this single center cohort. METHODS: In this retrospective cohort study, the authors reviewed imaging studies, surgery reports, histology reports, and radiotherapy plans in a multidisciplinary setting. The authors included 20 consecutive patients with orbital RMS, treated between 1990 and 2007, (median age: 7.4 years, range: 0.7-16.1; median follow up: 11.5 years). RESULTS: After induction chemotherapy, 12 patients were treated with surgery and brachytherapy, 2 with external beam radiotherapy, and in 5 patients who achieved complete remission, local treatment was withheld. In 1 patient, brachytherapy was incorrectly withheld after delayed surgery. Seven patients relapsed (no local treatment, N = 2; surgery and brachytherapy, N = 2; external beam radiotherapy, N = 2; surgery only, N = 1). The authors found no patient, tumor, or treatment characteristics that predisposed for treatment failure. Ten-year-overall survival and event-free survival were 89% and 65%, respectively. CONCLUSIONS: Overall survival in this cohort of orbital RMS patients was good, including surgery and brachytherapy as treatment modality for orbital RMS resulted in an effective local treatment approach with fewer adverse events than external beam radiotherapy. The authors could not identify factors predisposing for treatment failure.

Predictors of endoscopic transsphenoidal surgery outcome in acromegaly: patient and tumor characteristics evaluated by magnetic resonance imaging.

The availability of various first-line treatment modalities for acromegaly and evolving surgical techniques emphasize the need for accurately defined predictors of surgical outcome. We retrospectively analysed the outcome of 30 patients with acromegaly after initial endoscopic transsphenoidal surgery in two university hospitals from 2001 until 2009, and reviewed comparable literature investigating predictive tumor characteristics. Medical records were monitored for patient characteristics. Each pituitary magnetic resonance imaging (MRI) scan was revised independently by two neuroradiologists using a standardised analysis form to record distinctive predefined tumor characteristics. All characteristics were independently analysed as predictors for persistent disease, and a multivariable predictive model was created. Literature from 2000 onwards was searched for studies describing tumor characteristics predictive for surgical outcome. The cohort consisted of 27 macroadenomas with 90 % demonstrating signs of parasellar extension. The surgical cure rate overall was 30 %. Independently, next to male sex and increasing tumor size, infrasellar and parasellar extension based on MRI staging tended to increase the risk of persistent disease. In a multivariable analysis, sex and parasellar extension of the tumor were demonstrated to be the variables allowing for the best fitted predictive model for persistent disease. Earlier studies on preoperative tumor characteristics showed comparable results, although these were based on several different tumor classification systems. This retrospective study demonstrates that accurately defined tumor characteristics based on imaging, especially for cavernous sinus invasion, can be helpful in predicting surgical outcome. Comparative studies on different treatment modalities are essential for clinical practice within the scope of re-evaluation of the role of surgery in GH-secreting adenomas.

Endoscopic management of Ewing's sarcoma of ethmoid sinus within the AMORE framework: a new paradigm.

The Ablative surgery, MOulage brachytherapy and REconstruction) (AMORE) protocol developed in the Academic Medical Center of Amsterdam has been used successfully to treat sarcomas. The use of endoscopic surgery fits well within this framework. A 6-year-old boy presented with Ewing Sarcoma of left ethmoid sinus closest to orbit. The patient underwent neoadjuvant chemotherapy followed by complete endoscopic resection, brachytherapy and reconstruction. Brachytherapy was administered by iridium catheters through limited Lynch-Howarth incision. Skull base defect was reconstructed with a galea flap. The use of endoscopic surgery complemented by neoadjuvant chemotherapy and brachytherapy might maximize tumor control while reducing morbidity.

Computer-aided detection of small pulmonary nodules in chest radiographs: an observer study.

RATIONALE AND OBJECTIVES: To evaluate the impact of computer-aided detection (CAD, IQQA-Chest; EDDA Technology, Princeton Junction, NJ) used as second reader on the detection of small pulmonary nodules in chest radiography (CXR). MATERIALS AND METHODS: A total of 113 patients (mean age 62 years) with CT and CXR within 6 weeks were selected. Fifty-nine patients showed 101 pulmonary nodules (diameter 5-15mm); the remaining 54 patients served as negative controls. Six readers of varying experience individually evaluated the CXR without and with CAD as second reader in two separate reading sessions. The sensitivity per lesion, figure of merit (FOM), and mean false positive per image (mFP) were calculated. Institutional review board approval was waived. RESULTS: With CAD, the sensitivity increased for inexperienced readers (39% vs. 45%, P < .05) and remained unchanged for experienced readers (50% vs. 51%). The mFP nonsignificantly increased for both inexperienced and experienced readers (0.27 vs. 0.34 and 0.16 vs. 0.21). The mean FOM did not significantly differ for readings without and with CAD irrespective of reader experience (0.71 vs. 0.71 and 0.84 vs. 0.87). All readers together dismissed 33% of true-positive CAD candidates. False-positive candidates by CAD provoked 40% of all false-positive marks made by the readers. CONCLUSION: CAD improves the sensitivity of inexperienced readers for the detection of small nodules at the expense of loss of specificity. Overall performance by means of FOM was therefore not affected. To use CAD more beneficial, readers need to improve their ability to differentiate true from false-positive CAD candidates.

Surgical treatment of sphenoorbital meningiomas.

AIM: To evaluate the outcome of surgery and radiotherapy in the treatment of sphenoorbital meningioma (SOM). METHOD: A retrospective study of 66 consecutive cases treated with surgery for SOM with a minimum follow-up of 4 years. Clinical and radiological information were compared before and after the following surgical approaches: frontotemporal craniotomy, frontotemporal craniotomy combined with orbitozygomatic resection and extended lateral orbitotomy alone. RESULTS: The median age at presentation was 46 years (range, 26-68 years) and the median follow-up after surgery was 102 months (range, 48-288 months). In total, 48 (73%) patients showed preoperative visual deterioration, with visual field defects. All patients had proptosis at presentation (mean ± SD=6.4 ± 3.0 mm). Surgery for patients with SOM arrested visual deterioration in 61% and improved vision in 30% of cases. Furthermore, a substantial reduction of proptosis was achieved in 85% of patients. The proptosis in this group was reduced by 2.6 ± 2.6 mm. There was no correlation between surgical approach and proptosis reduction (p = 0.125). The recurrence rate was 17%. Only 1 out of 15 patients who underwent radiotherapy showed signs of recurrence. CONCLUSIONS: The surgical aims in the treatment of SOM should be the restoration of visual acuity and reduction of proptosis, rather than complete tumour removal. The surgical approach can be tailored to individual cases. The authors recommend radiotherapy in cases of subtotally removed SOM.

Natural history of spheno-orbital meningiomas.

BACKGROUND: To investigate the natural history and the growth rate of spheno-orbital meningiomas (SOMs). METHODS: Ninety patients with a diagnosis of SOM were included, and patient charts and imaging were evaluated. In a subset of 32 patients, volumetric studies were performed. RESULTS: The median follow-up for the entire group was 4 years (range, 1-15); the mean age was 47.8 (range, 26-93) years; 94% of the patients were female. The most common clinical signs and symptoms were proptosis (93%), visual deterioration (65%), retro-bulbar pain (23%) and diplopia (6%). In 35% of patients in this series, no visual deterioration occurred, and in 30% only mild proptosis was present. The median annual growth rate of the SOMs in the subset of 32 patients was 0.3 cm³/year (range, 0.03-1.8 cm³/year). We assessed a trend for more rapid tumour growth in younger patients and found the initial volume of the tumour (rho = 0.63) and of the soft tissue component (rho = 074) to be significantly related to the growth rate. CONCLUSION: SOMs are slow-growing tumours that cause primarily proptosis and visual deterioration. In a significant number of patients, these tumours cause minimal discomfort and symptomatology. Therefore, in the absence of risk factors, we advocate a "wait and see" policy. For patients with large SOMs or with a large soft tissue component at first visit or with fast growing SOMs (>1cm³/year), a follow-up examination every 6 months is indicated.

Imaging findings in craniofacial childhood rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the commonest paediatric soft-tissue sarcoma constituting 3-5% of all malignancies in childhood. RMS has a predilection for the head and neck area and tumours in this location account for 40% of all childhood RMS cases. In this review we address the clinical and imaging presentations of craniofacial RMS, discuss the most appropriate imaging techniques, present characteristic imaging features and offer an overview of differential diagnostic considerations. Post-treatment changes will be briefly addressed.

Brachytherapy as part of the multidisciplinary treatment of childhood rhabdomyosarcomas of the orbit.

INTRODUCTION: Rhabdomyosarcomas in the orbit form a major challenge in terms of cure without severe side effects in childhood cancer. Our specifically developed approach consists of applying brachytherapy to the tumor area using a mold. Analysis of its results for 20 patients was performed. METHODS AND MATERIALS: Thirteen patients were referred for brachytherapy if complete remission was not reached after chemotherapy (Group I) and 7 in case of relapse (Group II). In total, 20 patients were treated between 1991 and 2007. Four were female and 16 male; their ages varied from 1.1 to 16.5 years, with an average of 8.5 years. After macroscopically radical tumor resection, molds with holes drilled to hold flexible catheters were placed into the orbit. The dose to the clinical target volume was 40-50 Gy. RESULTS: Three patients of Group I and 1 patient of Group II developed local recurrence and underwent exenteration. The progression-free survival in Group I is 71.9% (95% CI 0.44-1.0), in Group II 85.7% (95% CI 0.60-1.0), the overall 5-year survival rate of the entire group is 92% (95% CI 0.76-1.0). During treatment, no serious side effects were observed. The late complications encountered in this series were cataract in 2 patients, 1 of whom also developed mild retinopathy. Two patients with ptosis needed surgical correction. No facial asymmetries or bone growth anomalies were observed. CONCLUSIONS: This entire procedure of brachytherapy with a mold offers a tailor-made treatment for orbital rhabdomyosarcomas with only few signs of late toxicity.

Somatostatin receptor scintigraphy for optic nerve sheath meningiomas.

OBJECTIVE: To investigate the value of somatostatin receptor scintigraphy (SSRS) in the diagnosis of optic nerve sheath meningiomas (ONSMs). DESIGN: Prospective, comparative case series. PARTICIPANTS: SSRS was used to investigate 68 orbits in 61 patients diagnosed with an orbital tumor. METHODS: Patients were injected intravenously with 200 MBq of indium-111 ((111)In)-octreotide. SSRS with single photon emission computed tomography (SPECT) was performed 24 hours after injection. MAIN OUTCOME MEASURES: The (111)In-octreotide uptake in orbital lesions was determined by semiquantitative uptake-ratio analysis (lesion/brain) on attenuation-corrected transverse SPECT slices. RESULTS: All orbital meningiomas, including 14 ONSMs and 12 spheno-orbital meningiomas, showed high median (111)In-octreotide uptake ratios of 7.2 (range, 4.6-15.4) and 16.3 (range, 4.6-15.4), respectively. In 7 patients with a diagnosis of ONSM, the SSRS uptake ratio was significantly decreased 2.7 (1-6.8) after treatment with 54 Gy of radiotherapy. Median uptake ratios of other tumors were vascular anomalies/vascular tumors, 1.2 (range, 0.5-3.3); non-Hodgkin lymphomas, 2.9 (range, 0.9-4.2); optic nerve gliomas, 1.5 (range, 0.2-3.3); and idiopathic orbital inflammation, 1.6 (range, 1.4-1.9). By using a threshold uptake ratio of 5.9, the sensitivity of SSRS with (111)In-octreotide for ONSM was 100%, with a specificity of 97.2%. CONCLUSIONS: SSRS is a useful additional tool in diagnosing ONSM and has 100% sensitivity and 97% specificity at a threshold uptake ratio of 5.9.

Prediction of deep neck abscesses by contrast-enhanced computerized tomography in 76 clinically suspect consecutive patients.

OBJECTIVES/HYPOTHESIS: Contrast-enhanced computerized tomography (CECT) has become the imaging method of choice in patients with clinical suspicion of a deep neck abscess. The purpose of this retrospective study was to assess the predictive value of the diagnosis of deep neck abscess using CECT. STUDY DESIGN: Retrospective chart review study over a 5-year period from 2002 to 2007 by a blinded observer. METHODS: CECT examinations of patients with clinical suspicion of a deep neck abscess were reviewed for the presence of fluid collections, rim enhancement, and abnormal air collections, which we considered as indicative for an abscess. Additionally, the location and extension of the abnormalities were documented. RESULTS: In this series of 76 patients, 61 adults and 15 children, fluid collections with complete or partial rim enhancement were seen in 65 patients. In two patients large abnormal air collections were seen with nonenhancing fluid accumulation in all deep spaces. Final diagnosis was confirmed by surgery in 57 patients. In 36 patients, two or more adjacent compartments were involved. Nine patients with an abscess were treated successfully by intravenous antibiotics only. The positive predictive value (PPV) for the presence of an abscess was 82% (53/65) if all radiological signs were included. The PPV was 67% (8/12) for fluid collections without rim enhancement. Air within or adjacent to a fluid collection or excessive free air between the fascias indicated an abscess in all cases. Negative predictive value (prediction of no abscess) was 100%. CONCLUSIONS: The predictive value of the diagnosis deep neck abscess by CECT is high, with a PPV of 82%. The presence of air indicates abscess in all cases.

The AMORE protocol for advanced-stage and recurrent nonorbital rhabdomyosarcoma in the head-and-neck region of children: a radiation oncology view.

PURPOSE: A multidisciplinary approach, consisting of consecutive Ablative Surgery, MOld technique with afterloading brachytherapy and immediate surgical REconstruction (AMORE) applied after chemotherapy, was designed for children with rhabdomyosarcoma in the head-and-neck region. Analysis of the first 42 patients was performed. METHODS AND MATERIALS: After macroscopically radical tumor resection, molds were constructed for each individual to fit into the surgical defect. The molds, made of 5-mm-thick layers of thermoplastic rubber, consisted of different parts. Flexible catheters were positioned between layers. After brachytherapy, the molds were removed. Surgical reconstruction was performed during the same procedure. RESULTS: Dose to the clinical target volume varied from 40 to 50 Gy for the primary treatment (31 patients) and salvage treatment groups (11 patients). There were 18 females and 24 males treated from 1993 until 2007. Twenty-nine tumors were located in the parameningeal region, and 13 were located in the nonparameningeal region. Patient age at the time of AMORE was 1.2-16.9 years (average, 6.5 years). Follow-up was 0.2-14.5 years (average, >5.5 years). Eleven patients died, 3 with local recurrence only, 6 with local and distant disease, 1 died of distant metastases only, and 1 patient died of a second primary tumor. Overall 5-year survival rates were 70% for the primary treatment group and 82% for the salvage group. Treatment was well tolerated, and acute and late toxicity were mild. CONCLUSIONS: The AMORE protocol yields good local control and overall survival rates, and side effects are acceptable.

Spread of excitation measurements for the detection of electrode array foldovers: a prospective study comparing 3-dimensional rotational x-ray and intraoperative spread of excitation measurements.

OBJECTIVE: The optimal positioning of electrode arrays in the cochlea is extremely important. Our standard approach is to use a 3-dimensional rotational x-ray for the intraoperative determination of the position of the electrode array. We wanted to see if spread of excitation (SOE) is useful for determining the electrode array position within the cochlea. STUDY DESIGN: Prospective blind study design. SETTING: Tertiary University Referral Center (Cochlear Implantation Center Amsterdam-Academic Medical Center, University of Amsterdam). PATIENTS: Seventy-two implanted ears with a Cochlear Freedom device. INTERVENTION: After cochlear implantation, we compared the 3-dimensional rotational x-ray imaging and SOE measurements. The investigators were blinded for the intraoperative surgeon findings and also for the imaging findings. OUTCOME MEASURE(S): Electrode array foldovers within the cochlea and the reliability of the SOE measurements. RESULTS: We placed implants in 72 ears in this study, and all procedures seemed to be surgically uneventful. To our surprise, we discovered 4 electrode foldovers in this group. Of the 4 foldovers, 3 were corrected intraoperatively. CONCLUSION: We found that intraoperative imaging and/or electrophysiologic measurements such as the SOE provide very useful information regarding electrode position within the cochlea. Spread of excitation is effective in detecting electrode array foldovers if the audiologist is experienced. Some software modifications are suggested.

AMORE protocol in pediatric head and neck rhabdomyosarcoma: descriptive analysis of failure patterns.

BACKGROUND: The AMORE protocol is a local treatment for patients with nonorbital pediatric head and neck rhabdomyosarcoma (HNRMS). The objectives of this study were: (1) to assess the adequacy of the concept, and (2) to identify factors associated with relapse. METHODS: We performed a retrospective multidisciplinary review of 22 children primarily treated according to the AMORE protocol, excluding two children with inadequate imaging data. RESULTS: Seven patients had a local relapse, six within and one outside the residual tumor area. Five of the six patients with relapse in the residual area had gross total or debulking (incomplete) surgery, suboptimal position of the mold for brachytherapy, or both. In the 15 nonrecurrent cases, four patients had either incomplete surgery or suboptimal mold position. Both surgical and brachytherapeutic factors seem to be associated with relapse. CONCLUSIONS: AMORE is an adequate concept. More rigid preoperative imaging and intraoperative verification of the brachytherapy mold position might lead to a reduction in the number of local failures.