Faculty Bibliography

The combination of cyclophosphamide and topotecan (cyclo/topo) has shown objective responses in relapsed Ewing sarcoma, but the response duration is not well documented. We reviewed characteristics and outcome of 14 patients with Ewing sarcoma, treated uniformly at a single institution and offered cyclo/topo at first relapse. Six patients (43%) had relapse at distant sites. All patients received first-line salvage therapy with cyclophosphamide 250 mg/m and topotecan 0.75 mg/m, daily for 5 days repeated every 21 days. The median number of cycles was 4 (range 1 to 10). All toxicities were manageable, the most common being transient cytopenias. There were also 4 episodes of febrile neutropenia, and 3 episodes of gross hematuria. Response was assessable in 13 patients and showed progressive disease in 6 (46%), stable disease in 4 (31%), and partial response in 3 (23%). Nine patients had local control, consisting of radical surgery in 2, radiation in 3, and a combination in 4 patients. Response, when it occurred, was maintained for a median of 8 months (range, 4 to 28 mo). Four patients (29%) are alive at 3, 7, 9, and 110 months after relapse; 1 is receiving cyclo/topo, 1 is on third-line therapy, and 2 are in second and fourth remission. The low toxicity of this combination, and the lack of sustained responses, warrant its investigation in combination with targeted or novel therapeutic agents in relapsed disease.

OBJECTIVES: The purpose of this study was to determine the importance of nondiagnostic fine-needle aspiration biopsy results for post-thyroidectomy bed lesions in patients with thyroid cancer. METHODS: The Institutional Review Board approved this retrospective Health Insurance Portability and Accountability Act-compliant study with a waiver for informed consent. Twenty-one patients with nondiagnostic fine-needle aspiration biopsy of a thyroid bed lesion after thyroidectomy with at least 1 year of follow-up with neck imaging were retrospectively enrolled in our study. The tumor type, local staging, radiotherapy, serum thyroglobulin levels, and imaging characteristics of the thyroid bed lesions were recorded. All patients underwent sonographically guided fine-needle aspiration biopsy of the thyroid bed lesion. RESULTS: The mean imaging follow-up +/- SD was 46.3 +/- 28.7 months. Lesions in 20 patients (95.2%) were stable on imaging. Most thyroid bed lesions were hypoechoic (80.9%), and none showed calcifications. The mean thyroid bed mass maximum diameter was 1.17 +/- 0.6 cm (range, 0.3-2.9 cm). CONCLUSIONS: Thyroid bed lesions with nondiagnostic fine-needle aspiration biopsy results after thyroidectomy can be managed with imaging follow-up; 1-year imaging is a reasonable interval after biopsy.

To report a rare case of bilateral superior semicircular canal dehiscence (SCCD) in a child. Case report, 11-year-old female patient. Descriptive case report. Audiological findings of bilateral symmetrical low frequency sensorineural hearing loss with ascending curves and bilateral superior semicircular canal dehiscence on a high resolution computed tomography (CAT) scan. The young child presented with bilateral fluctuating hearing loss with no vestibular symptoms. She was found to have bilateral superior semicircular canal dehiscence. We hereby present a rare case of bilateral semicircular canal dehiscence found incidentally on high resolution CAT scan in a young child during work up for bilateral sensorineural hearing loss.

BACKGROUND: Hypercoagulability and venous thromboembolism are common in patients with beta-thalassemia intermedia (TI), especially in the splenectomized adult. Although arterial involvement is not commonly reported, we have recently observed a high prevalence (60%) of silent brain infarction on brain MRI in 30 splenectomized adults with TI. The pathophysiology of these white matter lesions remains unknown. METHODS: In this prospective work, we evaluated magnetic resonance angiography (MRA) scans of the same cohort of 30 patients. Data collected were the presence or absence of vascular lesions, their locations, and severity. Correlations between MRA abnormality and patients/disease characteristics were evaluated. Comparisons between MRA and previous MRI findings were made. RESULTS: Of 29 evaluable patients, 8 (27.6%) had evidence of arterial stenosis on MRA. The majority of lesions had mild narrowing and mostly involved the internal carotid artery. Five patients (17.2%) had evidence of aneurysms. Low total hemoglobin and high non-transferrin-bound iron levels independently characterized patients with evidence of stenosis on MRA. Among the 18 patients with silent brain infarction on MRI, three had evidence of stenosis on MRA with only one patient having lesions that could explain the silent infarcts. CONCLUSIONS: Cerebral vasculopathy is common in splenectomized adults with TI. However, large-vessel disease does not explain the occurrence of silent brain infarction. The combined use of MRA and MRI better identifies splenectomized TI adults with neuroimaging abnormalities.

BACKGROUND: Extracranial venous stenosis (EVS) has recently been implicated as the primary cause of multiple sclerosis (MS). Objective: The aim of this study was to determine the presence of EVS in MS patients. METHODS: We performed selective extracranial venography on 42 patients with early MS (EMS): clinically isolated syndrome (CIS) or relapsing-remitting MS (RRMS) of less than 5 years duration, and late MS (LMS): RRMS of more than 10 years duration. Magnetic resonance imaging (MRI) and clinical relapse data were reviewed for all patients with EVS. RESULTS: EVS was present in 7/29 patients with EMS and 12/13 patients with LMS, a highly significant statistical difference (p< 0.001). Only 3/42 patients (all in the LMS group) had two vessel stenoses, while the rest had only one vessel involved. EVS was seen in 1/11 patients with CIS compared with 6/18 RRMS patients of less than 5 years duration. Disease duration was greater in patients with EVS overall (p < 0.005). LMS remained an independent predictor of EVS following multivariate adjustment for gender, age at disease onset and Expanded Disability Status Scale (EDSS) (Adjusted Odds Ratio = 29 (3-298); p = 0.005]. Within the EMS group, patients with (n = 7) and without (n = 22) EVS had similar EDSS and disease duration, suggesting similar disease severity. No clear correlation could be found between site of EVS and anatomic localization of either clinical relapses or MRI gadolinium-enhancing lesions. CONCLUSIONS: We conclude that EVS is an unlikely cause of MS since it is not present in most patients early in the disease and rarely involves more than one extracranial vein. It is likely to be a late secondary phenomenon.