Nicole J. Freling

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.

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.

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.

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.

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.

Amore protocol in pediatric head and neck rhabdomyosarcoma: Descriptive analysis of failure patterns

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.

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.

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.

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.