Combined use of maxillomandibular swing approach and neurosurgical ultrasonic aspirator in the management of extensive clival chordoma: A case report

<p>Abstract</p> <p>Introduction</p> <p>Chordoma is a rare malignant tumour with an incidence of metastasis of less than 10 percent. Usually arising from clivus its posterior extension may involve the brainstem before presenting as nasal mass and obstruction. Surgery is the main mode of treatment with adjuvant radiotherapy. However surgery is rarely possible for a large intracranial lesion.</p> <p>Case presentation</p> <p>We report the case of an adolescent patient with a chordoma extending posteriorly to the brainstem and anteriorly to the nasopharynx and managed by the combination of resection using a maxillomandibular swing approach and the use of a neurosurgical ultrasonic aspirator.</p> <p>Conclusion</p> <p>Maxillomandibular swing approach provides good access for large nasopharyngeal tumour extending brainstem area.</p

Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients

<p>Abstract</p> <p>Background</p> <p>Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported.</p> <p>Methods</p> <p>We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008.</p> <p>Results</p> <p>Twenty-five consecutive patients with sacral (11 patients), spine (13 patients), and skull base (1 patient) chordoma went to our observation. Six patients (24%) had primary disease, 14(56%) a recurrent disease, and 5(20%) a metastatic spreading. Surgery was the primary option for treatment in 22 out of 25 patients. Surgical margins were wide in 5 (23%) and intralesional in 17(77%) patients; 3 out of 4 in-house treated patients obtained wide margins. After first surgery, radiotherapy (protons or high-energy photons) were delivered to 3 patients. One out of the 5 patients with wide margins is still without evidence of disease at 20 months from surgery; 2 patients died without evidence of disease after 3 and 36 months from surgery. Sixteen out of 17 (94%) patients with intralesional margins underwent local progression at a median time of 18 months with a 2-year local progression-free survival of 47%. The 5-year metastasis-free survival rate was 78.3%. Seventeen patients with locally advanced and/or metastatic disease expressing platelet-derived growth factor receptor (PDGFR) β were treated with imatinib mesylate. A RECIST stabilization of the disease was the best response observed in all treated cases. Pain relief with reduction in analgesics use was obtained in 6 out of 11 (54%) symptomatic patients. The 5- and 10-year survival rates of the entire series of patients were 76.7 and 59.7%, respectively.</p> <p>Conclusions</p> <p>Despite progress of surgical techniques and the results obtained with targeted therapy, more effort is needed for better disease control. Specific experience of the multidisciplinar therapeutic team is, however, essential to succeed in improving patients' outcome.</p

ICAR: endoscopic skull‐base surgery

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Brain perfusion SPECT with 99mTC-ECD and serum neuron-specific enolase in patients with spontaneous subarachnoid haemorrhage and clinical vasospasm

Aim: To estimate the association between single photon emission computed tomography (SPECT) imaging and neuro-specific enolase (NSE) serum level measurement in patients with subarachnoid haemorrhage (SAH) and also to present our experience in using these data for patient evaluation. Material and method: Thirty-five patients (18 female and 17 male, mean age 57.5 ± 11.9 y) with clinical findings of vasospasm after SAH underwent 99mTC-ECD SPECT imaging. In 23 out of the 35 patients, serum levels of NSE were assessed on admission. Brain perfusion was measured semi-quantitatively using symmetrical regions of interest, automatically drawn over cortical and subcortical structures on consecutive transverse slices. A summed perfusion defect score (SPDS) was used to' quantify the brain perfusion. Statistical analysis was carried out using the Kruskal-Wallis test, Mann-Whitney U test and ROC analysis, as appropriate. Results: Eighteen patients had abnormal SPECT studies. The patients were divided into three groups according to NSE levels. Group I comprised 13 patients with NSE values ≤ 15 ng/ml, group II comprised 7 patients with 15 ng/ml &lt;NSE &lt; 19 ng/ml, and group III comprised 3 patients with NSE ≥ 19 ng/ml. SPECT/SPDS data and NSE levels of groups II and I were positively linearly correlated (Spearman's coeff = 0.71, p&lt;0.05), while group III clearly defines a separate population. The linear correlation between SPECT/SPDS and NSE was statistically significant (p&lt;0.05). Very high NSE values were noticed in three patients who eventually died. Conclusion: Both SPECT abnormalities and high serum NSE concentration are potentially helpful in the evaluation of patients with SAH. ©Borgis