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      A rare case of surgical pathway implantation of clival chordoma presenting as a neck mass

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          Abstract

          Chordomas are rare, locally-aggressive tumours with a high rate of local recurrence. Recurrence along the route of surgical entry is an uncommon form of treatment failure. We report a case of a 59-year-old female who presented with a 3 cm neck mass in the left mid-sternocleidomastoid region. She had a history of a large clival chordoma resected via a transcervical, transparotid and transoral approach along with endoscopic intranasal exposure and a palatal split 4.5 years previously, followed by radiation to the primary site. Biopsy of the neck mass confirmed the diagnosis of chordoma recurrence following implantation in the surgical pathway. This case illustrates that while surgical pathway recurrence is a rare entity, it requires a high index of suspicion and should be considered in the differential diagnosis of a patient with a history of chordoma resection presenting with a mass more than two years after undergoing initial treatment.

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          Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure.

          Prospective analysis of local tumor control, survival and treatment complications in 67 consecutive patients treated with fractionated photon and proton radiation for chordoma or chondrosarcoma of the base of the skull and the cervical spine. Between December 1995 and January 2000, 67 patients with a median age of 52 years (range: 14-85 years), were treated at the Centre de Protonthérapie d'Orsay (CPO), France, using the 201-MeV proton beam, 49 for chordoma and 18 for chondrosarcoma. Irradiation combined high-energy photons and protons. Photons represented two thirds of the total dose and protons one third. The median total dose delivered within gross tumor volume (GTV) was 67 Cobalt Gray Equivalents (CGE; range: 60-70 CGE). Within a median follow-up of 29 months (range: 4-71 months), the 3-year local control rates were 71% and 85% for chordomas and chondrosarcomas, respectively, and the 3-year overall survival rates 88% and 75%, respectively. 14 tumors (21.5%) failed locally (eight within the GTV, four within the clinical target volume [CTV], and two without further assessment). Seven patients died from their tumor and another one from a nonrelated condition (pulmonary embolism). The maximum tumor diameter and, similarly, the GTV were larger in relapsing patients, compared with the rest of the population: 56 mm vs 44 mm (p = 0.024) and 50 ml vs 22 ml (p = 0.0083), respectively. In univariate analysis, age < or = 52 years at the time of radiotherapy (p = 0.002), maximum diameter < 45 mm (p = 0.02), and GTV < 28 ml (p = 0.02) impacted positively on local control. On multivariate analysis, only age was an independent prognostic factor of local control. In chordomas and chondrosarcomas of the skull base and cervical spine, combined photon and proton radiation therapy offers excellent chances of cure. In two thirds of the cases, relapses are located in the GTV. Maximum diameter, GTV, and age are prognostic indicators of local control. These results should be confirmed during a longer follow-up.
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            Skull base chordoma.

            Our purpose was to discuss the optimal treatment and outcomes for patients with skull base chordomas. We reviewed the pertinent literature for this study. Skull base chordomas usually arise in the clivus and are rarely completely resectable. Therefore, most are treated with radiotherapy (RT). Because of the risk of severe late complications, the dose is often limited with conventional photon RT, and the probability of cure is low. Proton RT alone or combined with photon RT (proton/photon RT) offers the advantage of improved dose distribution and the ability to treat the tumor to a higher dose without exceeding normal tissue tolerance. The 10-year local control rate after proton/photon RT is approximately 40% to 50%. The probability of local control is related to minimum tumor dose and dose inhomogeneity. Skull base chordoma is a rare neoplasm that is rarely cured after surgery alone or combined with conventional RT. Proton/photon RT offers the advantage of increasing the tumor dose while minimizing the dose to normal tissues, thus reducing the risk of late complications. The optimal treatment may be photon/proton RT alone or combined with a gross total resection, when feasible. 2005 Wiley Periodicals, Inc.
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              Surgical seeding of chordomas.

              Chordomas have a high propensity for local recurrence and progression, as well as for systemic and cerebrospinal fluid metastasis. The authors identified and analyzed a series of patients with chordomas, focusing on an underrecognized pathological entity-surgical seeding of tumor cells. In a retrospective analysis of 82 patients with chordomas treated over a 10-year period (1990-2000), the authors found six patients (7.3%) in whom surgical seeding had occurred. In five (83%) of these patients the primary tumor was located at the clivus. In one (17%), the tumor was present at the cervical spine. There were two male (33%) and four female patients (67%) whose mean age was 34 years. The seeding sites, which were separate from the primary tumor, were located along the operative route or in the abdomen where fat was removed. The seeding was diagnosed 5 to 15 months (mean 12 months) after surgery. One seeding site was present in five patients, and 17 seeding sites were present in one patient. The involved tissues included mucosa, bone, dura, muscle, and fat. After resection, all seedings were confirmed histologically. Seeding of chordomas occurs along the operative route and at distant locations where tissue is harvested. Early diagnosis and aggressive surgery are recommended. Based on the results of this study, the authors suggest that surgical techniques, postoperative radiotherapy, neuroradiological follow-up protocol, and even research on chordomas should be reevaluated.
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                Author and article information

                Journal
                J Surg Case Rep
                J Surg Case Rep
                jscr
                jscr
                Journal of Surgical Case Reports
                JSCR Publishing Ltd
                2042-8812
                1 January 2011
                January 2011
                : 2011
                : 1
                : 3
                Affiliations
                University of Toronto, Toronto, Canada
                Article
                10.1093/jscr/2011.1.3
                3649199
                8d76b25a-fa95-4e16-a421-1f279c721857
                © JSCR
                History
                Categories
                Head & Neck Surgery
                070

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