Optimizing radiotherapy strategies for skull base chordoma: a comprehensive meta-analysis and systematic review of treatment modalities and outcomes.

OBJECTIVE: In the treatment of skull base chordoma (SBC) surgery is considered the mainstay approach, and gross-total resection has an established relationship with progression-free survival (PFS) and overall survival (OS). However, the tumor's location often interferes with attempts at complete resection. In this case, surgery for maximal resection followed by high-dose radiotherapy has been demonstrated to be the standard treatment. In this context, various modalities are available, yet no consensus exists on the most effective. This systematic review and meta-analysis aimed to evaluate the efficacy and safety of different radiotherapy modalities for SBC. METHODS: Following PRISMA guidelines, the authors systematically searched for the treatment of SBC with radiation modalities in the PubMed, Cochrane, Web of Science, and EMBASE databases. Outcomes assessed for each modality were as follows: OS, PFS, local control (LC), and complications. The random-effects model was adopted. A single-proportion analysis with 95% CI was used to measure the effects in single-arm analysis. For the comparative analysis, the OR with 95% CI was used to compare outcome treatment effects. Heterogeneity was assessed using I2 statistics, and statistical significance was defined as p < 0.05. RESULTS: A total of 32 studies comprising 3663 patients, with 2322 patients who were treated with radiotherapeutic modalities, were included. Regarding 5-year OS findings in each modality study, the findings were as follows: in photon fractionated radiotherapy, an estimated rate of 77% (69%-84%, 568 patients); in conventional fractionated radiotherapy, 76% (65%-87%, 517 cases); in proton-based + carbon ion-based radiotherapy, 85% (82%-88%, 622 cases); and in a comparative analysis of proton-based and carbon ion-based therapy, there was an OR of 1.2 (95% CI 0.59-2.43, 306 cases). Regarding the 5-year PFS estimate, the rates were as follows: 35% (26%-45%, 95 cases) for photon fractionated therapy; 35% (25%-45%, 85 cases) for stereotactic radiotherapy; 77% (50%-100%, 180 cases) for proton-based and carbon ion-based radiotherapy; and 74% (45%-100%, 102 cases) for proton-based radiotherapy. Regarding LC in periods of 3 and 5 years after proton- and carbon ion-based therapy, the overall estimated rates were 84% (78%-90%, 326 cases) and 75% (65%-85%, 448 cases), respectively. For proton-based radiotherapy and carbon ion-based therapy, the 5-year LC rates were 76% (67%-86%, 259 cases) and 75% (59%-91%, 189 cases), respectively. CONCLUSIONS: The analysis highlights the finding that particle-based modalities like proton beam radiotherapy and carbon ion radiotherapy are the most effective radiation therapies available for the treatment of SBC. Furthermore, it reinforces the idea that surgery followed by radiotherapy constitutes the standard treatment.

Chordoma: demographics and survival analysis with a focus on racial disparities and the role of surgery, a U.S. population-based study.

BACKGROUND: Chordoma is a rare malignant tumor of notochordal origin that may appear anywhere in the axial skeleton from the skull base to the sacrum. This study presents findings from a large database query to highlight the demographic, clinical, and pathological factors, prognosis, and survival of chordomas. METHODS: The Surveillance, Epidemiology, and End Results (SEER) data based was used to identify patients with a "chordoma" diagnosis from 200 to 2018. RESULTS: In a total of 1600 cases, the mean age at diagnosis was 54.47 years (standard deviation, SD ± 19.62 years). Most cases were male (57.1%) and white (84.5%). Tumor size was found to be > 4 cm in 26% of cases. Histologically, 33% with known features had well-differentiated Grade I tumors, and 50.2% of the tumors were localized. Metastasis at the time of to the bone, liver, and lung was observed at a rate of 0.5%, 0.1%, and 0.7%, respectively. The most common treatment received was surgical resection (41.3%). The overall 5-year overall survival observed was 39% (confidence interval, CI 95% 37-41; p = 0.05) with patients who received surgery having a 5-year survival rate of 43% (CI 95% 40-46; p = 0.05). Multivariate analysis showed independent factors that contributed to worse prognosis chemotherapy only as a treatment modality and no surgery as a treatment modality. CONCLUSION: Chordomas are more common in white males and appear between the 5th and 6th decades of life. Factors that contributed to a worse prognosis were Asian, Pacific Islander, American Indian, or Alaska Native races.

The prognostic significance of different degrees of resection of skull base chordoma.

BACKGROUND: Complete resection combined with postoperative radiotherapy is ideal for skull base chordomas. The recent literature suggests that the degree of surgical resection is the most important prognostic factor. METHODS: We retrospectively analyzed the clinical data of 16 patients with initial chordoma treated at our center between August 2015 and December 2021 and conducted a retrospective study on the prognosis of surgical treatment of skull base chordoma between 2013 and 2022. RESULTS: According to the Kaplan-Meier method, there was a significant difference in PFS between patients aged > 50 years and < 50 years, and no significant difference was observed in PFS for tumor involvement of the internal carotid artery, dura, or superior or inferior clivus. However, there was still a correlation with prognosis. As observed in the included literature, the 5-year overall survival rate was significantly higher in patients undergoing total skull base chordoma resection than in those undergoing subtotal resection (STR), which in turn was significantly higher than in those undergoing partial resection (PR). Patients undergoing subtotal resection had significantly better 5-year PFS rates than those undergoing PR. CONCLUSION: Our study shows that gross total resection and STR have better survival in patients with skull base chordomas compared to PR.

Spontaneous Regression of a Clival Chordoma: An Unusual Case Report.

INTRODUCTION: Chordoma is a malignant and aggressive tumor originating from remnants of the primitive notochord and usually involving the axial skeleton. Spontaneous regression of clival chordomas was described recently. We present the third case report of spontaneous regression of a clival chordoma and discuss similarities of cases and implications for clinical practice. CASE DESCRIPTION: We present the case of a previously healthy 21-year-old Caucasian woman who presented with progressive holocranial headache for 3 months, which encouraged image investigation. Magnetic resonance imaging (MRI) revealed an osteolytic clival lesion hyperintense in T2 and hypointense in T1 images. After 2 months of initial evaluation and surgical proposal, she repeated MRI to allow use for intraoperative neuronavigation. Surprisingly, there was tumor regression. DISCUSSION: The present reported case is somehow different from previous ones and does not share an underlying inflammatory/immunological recognizable fact, being interpreted by us as a spontaneous partial regression of the tumor. We highlight the need for continuous investigation of chordoma regression to uncover the underlying mechanisms.

Resection of Clival Chordoma Through the Anterior Clivectomy: 2-Dimensional Operative Video.

Clival chordomas are rare malignant behaving tumors that grow, locally invade, metastasize, and seed, and they have a high recurrence rate.1,2 The longest disease control is achieved by radical resection followed by high doses of radiation therapy, commonly proton beam.3 To achieve radical tumor removal, multiple surgical procedures through different approaches might be required.4 Since the chordoma's origin is, and remains, extradural, an extradural approach is preferred, and can lead to intradural extension. Anterior approach is frequently utilized to remove the midline-located tumor and the eroded clivus.5 Several midline approaches were utilized, including the transbasal, transfacial, transcervical, open door, and Lefort's maxillotomies1; however, the same tumor removal can be achieved with a simple extension of the trans-sphenoidal approach, by resecting the anterior maxillary wall, of the contralateral to the lesion preponderant side.5 This approach coupled with the use of neuronavigation on mobile head and endoscopic-assisted technique allowed to achieve a wide and direct exposure, with the ability to resect extra- and intradural tumors.2,5 Lately, the endonasal endoscopic technique became popular as an alternative4; however, we found a great advantage in the ability to combine the stereoscopic microsurgical technique with the endoscopic dissection, in addition to avoiding the extensive nasal dissection and its complications. We present a case of a 63-yr old woman with an upper clivus chordoma compressing the brainstem who underwent a gross total resection by endoscopic-assisted microscopic techniques through an anterior clivectomy approach. Patient consented to the procedure and publication of her images.

Zygomatic Approach to Cavernous Sinus Chordoma: 2-Dimensional Operative Video.

Chordoma is not a benign disease. It grows invasively, has a high rate of local recurrence, metastasizes, and seeds in the surgical field.1 Thus, chordoma should be treated aggressively with radical resection that includes the soft tissue mass and the involved surrounding bone that contains islands of chordoma.2-5 High-dose radiation, commonly by proton beam therapy, is administered after gross total resection for long-term control. About half of chordoma cases occupy the cavernous sinus space and resecting this extension is crucial to obtain radical resection. Fortunately, the cavernous sinus proper extension is the easier part to remove and pre-existing cranial nerves deficit has good chance of recovery. As chordomas originate and are always present extradurally (prior to invading the dura), an extradural access to chordomas is the natural way for radical resection without brain manipulation. The zygomatic approach is key to the middle fossa, cavernous sinus, petrous apex, and infratemporal fossa; it minimizes the depth of field and is highly advantageous in chordoma located mainly lateral to the cavernous carotid artery.6-12 This article demonstrates the advantages of this approach, including the mobilization of the zygomatic arch alleviating temporal lobe retraction, the peeling of the middle fossa dura for exposure of the cavernous sinus, the safe dissection of the trigeminal and oculomotor nerves, and total control of the petrous and cavernous carotid artery. Tumor extensions to the sphenoid sinus, sella, petrous apex, and clivus can be removed. The patient is a 30-yr-old who consented for surgery.

Experience With the Endoscopic Contralateral Transmaxillary Approach to the Petroclival Skull Base.

OBJECTIVES/HYPOTHESIS: The contralateral transmaxillary (CTM) approach is a new surgical approach that improves the surgical trajectory relative to the petrous segment of the internal carotid artery (ICA). Here, we present our clinical experience with the CTM approach to the petroclival region of the skull base. STUDY DESIGN: Retrospective review. METHODS: A retrospective review of 29 patients who underwent a CTM approach for skull base pathology from 2015 to 2020 was performed. Assessment of gross total resection (GTR) was based on postoperative imaging. RESULTS: The male:female ratio was 15:14, with an average age of 52 years (range = 19-78 years). Diagnoses included: 12 chondrosarcomas, 11 chordomas, two meningiomas, one schwannoma, one metastasis, one petrous apicitis, and one arachnoid cyst. CTM was performed in addition to a transclival approach and ipsilateral transpterygoid approach in all patients. Reconstruction of surgical defects included a vascularized flap in all but two patients: 24 nasoseptal flaps and three lateral nasal wall flaps. The reconstructive flap was on the same side as the CTM approach in 22 of 28 (79%) patients. There were no ICA injuries. In a subset of patients with chondromatous tumors, GTR of the targeted area was achieved in 16 of 22 (73%) evaluable chondromatous tumors. With a median follow-up of 13 months, 64% of these patients are without disease or dead of other causes; the remainder are alive with disease. CONCLUSIONS: The CTM approach improves the degree of resection of skull base tumors involving the petroclival region using an endoscopic endonasal approach and may minimize risk to the ICA. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:294-298, 2021.

Recidiva of sacrococcygeal chordoma. Multidisciplinary collaboration.

Los cordomas son tumores derivados de los remanentes embrionarios de la notocorda. Son localmente invasivos y en estadios localizados la resección completa es el tratamiento de elección. Presentamos el caso de un varón de 77 años con recidiva de cordoma sacrococcígeo, intervenido por un grupo multidisciplinario formado por cirujanos coloproctólogos, plásticos y neurocirujanos, obteniendo resección en bloque y márgenes negativos. A pesar de que recidivan localmente un 66-75% a los 5 años, la mayoría de los pacientes pueden aumentar su supervivencia si se obtienen márgenes libres. La cirugía debe conseguir una exéresis R0 y minimizar el riesgo de lesión rectal.Chordomas are tumors arising from the embryonic remnants of the notochord. They are locally invasive and in localized stages, complete resection is the treatment of choice. We present the case of a 77-year-old man with recurrent sacrococcygeal chordoma, he was operated by a multidisciplinary group including colorectal-, plastic- and neurosurgeons, obtaining complete en bloc resection with tumor-free margins. Even though 66-75% recur locally in 5 years, most of the patients can increase their survival if margins are tumor-free. The surgery must achieve the R0 exeresis minimizing the risk of rectal injury.

Partial Sacrectomy for Resection of a Sacral Chordoma: 2-Dimensional Operative Video.

Sacral chordomas are infrequent tumors that arise from remnants of the notochord. They are most often found in the sacrum and skull-base.1,2 These lesions rarely metastasize and usually have an indolent and oligosymptomatic clinical course. Chordomas show low sensitivity to standard radiation therapy and chemotherapy. Operative resection with wide resection margins offers the best long-term prognosis, including longer survival and local control.1,3 However, achieving a complete resection with oncological margins may be difficult because of the anatomic complexity of the sacrococcygeal region.4 The main complications of sacral resection include infections, wound closure defects, and anorectal and urogenital dysfunction. The rate of these complications is significantly increased when the tumor involves the S2 level or above. We report the case of a 64-yr-old male who presented with progressive sacrococcygeal pain and a feeling of incomplete evacuation. A heterogeneous, osteolytic lesion was found at the sacrococcygeal region. Full body imaging tests were negative for other lesions. A computed tomography (CT) guided biopsy was made. We usually use the midline approach in case we have to include the needle path in the resection. The pathology confirmed a sacrococcygeal, low-grade chordoma. We decided to perform an en bloc resection. A posterior, partial sacrectomy was planned distal to the S4 level.

Diffuse spinal spreading following previous intracranial intradural chordoma resection: A rare case report.

INTRODUCTION: Chordoma is a malignant tumor that usually involves the axial skeleton. Intradural chordomas are even rarer and 37 cases have been reported to the best of our knowledge. We present a case of a patient with an atypical metastatic diffuse intradural spinal involvement. CASE DESCRIPTION: We present a 33-year-old woman previously submitted to five brain surgeries to treat a posterior fossa intradural chordoma since December 2012. Currently, she presented almost with right and left hemiplegia (grade 2 bilaterally) and also left oculomotor, adbucent and facial nerve paresis. We performed neuroaxis magnetic resonance MR which disclosed stability of posterior fossa tumor which was previously irradiated. However, there were new intradural lesions at the level of C3, T11/T12 and L4/L5/S1 vertebrae. DISCUSSION: With the advent of contemponaeous surgery, radiotherapy options and even available chemotherapy to treat Chordomas (Imatinib), patients may experience enlarged survival and thus face complications such as drop metastases along neuroaxis. Our case illustrates a late (6 years) follow-up presentation of an initial posterior fossa intradural chordoma. It suggests that whole neuraxis involvement may be the final presentation of all patients harbouring chordomas and surviving after adequate initial treatment.

Neuronavigation-guided Endoscopic Endonasal Excision of Schwannoma-like Chordoma of Meckel's Cave: A Case Report.

Chordomas are locally aggressive malignant tumors due to their recurrence potential and originate from embryonic notochord remnants. Chordomas can originate anywhere on the axial skeleton. They are extradural and spread by bone destruction. Chordomas are locally aggressive tumors that invade the dura mater, and may also present with secondary intradural growth. The Meckel's cave location of chordomas has been very rarely reported in the literature. Chordomas located in Meckel's cave can be radiologically confused with trigeminal schwannomas. Herein, we report a case of Meckel's cave chordoma that was successfully excised through neuronavigation-guided endoscopic endonasal excision, a technique commonly used in skull base surgeries.

Three-Dimensional Volume Imaging to Increase the Accuracy of Surgical Management in a Case of Recurrent Chordoma of the Clivus.

BACKGROUND The clivus is a depression in the anterior occipital bone of the skull base, posterior to the dorsum sellae, at the junction with the sphenoid bone. Chordoma is a rare tumor arising from embryonic remnants of the notochord and can be locally aggressive with a tendency to recur. The optimal management of this rare tumor remains controversial. A report of a case of recurrent chordoma of the clivus is presented to illustrate the value of volumetric three-dimensional (3-D) reconstruction with computed tomography (CT) and magnetic resonance imaging (MRI) to determine optimal surgical management. CASE REPORT A 53-year-old man presented with pain in the right orbital cavity, right proptosis, swelling of the right cheek, and bilateral loss of vision. He also had adrenal insufficiency. CT and contrast-enhanced (gadolinium) T1-weighted MRI with multiplanar acquisition were performed with volumetric 3-D reconstruction of the tumor, to increase the chances of treatment success. Surgical resection was performed to remove the tumor and reduce the risk of recurrence. Histology of the tumor was consistent with chordoma, supported by positive immunohistochemical staining for S-100 and epithelial membrane antigen (EMA). CONCLUSIONS This report highlighted the value of 3-D volume imaging in the diagnosis and treatment planning in a rare case of recurrent chordoma of the clivus. Analysis of tumor volume may be an indicator of the efficacy of surgery, complementing the Response Evaluation Criteria In Solid Tumors (RECIST) system and as a valuable tool to predict treatment outcome.

Reto neuroquirúrgico actual del cordoma cervical / Current neurosurgical challenge of the cervical chordoma

El cordoma es un tumor osteocartilaginoso raro, de lento crecimiento, con una tasa de incidencia global de 8,4 casos por cada 10 millones de habitantes. Comúnmente aparece en la quinta y sexta década de la vida, predomina en el sexo masculino. Se presenta un paciente masculino de 47 años de edad, con cervicobraquialgia, cuadriparesia y masa tumoral palpable en región anterolateral del cuello. El estudio de resonancia magnética demostró la presencia de una lesión retrofraríngea con destrucción vertebral y compresión extradural. Se decidió resección quirúrgica de la lesión. El diagnóstico histopatológico por inmunohistoquímica arrojó como resultado, un cordoma(AU)

Chordoma is a rare, slow-growing osteocartilaginous tumor with an overall incidence rate of 8.4 cases per 10 million inhabitants. Commonly appears in the fifth and sixth decade of life, predominates in the male sex. We present a 47-year-old male patient with cervicobrachialgia, quadriparesis and palpable tumor mass in the anterolateral region of the neck. The magnetic resonance study showed the presence of a retropharyngeal lesion with vertebral destruction and extradural compression. Surgical resection of the lesion was decided. The histopathological diagnosis by immunohistochemistry resulted in a chordoma(AU)

Sacrectomía parcial por abordaje posterior único / Partial sacrectomy by single posterior approach

Introducción: Los tumores del sacro representan <7% de los tumores espinales, prevalecen los tumores secundarios por mieloma múltiple o carcinomas de próstata, mama, pulmón o colón. El cordoma es el tumor maligno primario más frecuente y el tumor de células gigantes es la lesión benigna más común. Por su evolución, compromiso de estructuras extraóseas y la escasa respuesta a los tratamientos coadyuvantes, la cirugía es el tratamiento más utilizado, la vía de abordaje y la necesidad de instrumentación dependerán del tumor por tratar. Los objetivos de este trabajo son: evaluar el uso de la resección parcial del sacro, analizar la técnica quirúrgica y reconocer las complicaciones. Materiales y Métodos: Cuatro pacientes con diagnóstico de tumor sacro ubicado por debajo de S1, que consultan por dolor y cuyas imágenes confirman la lesión. A todos se les realiza una resección en bloque por vía posterior preservando S1. Se describe la técnica. Resultados: Los estudios anatomopatológicos revelaron: un cordoma, un tumor maligno de vaina nerviosa, un condrosarcoma y una metástasis de carcinoma prostático. Se preservó la función de S1 en todos los pacientes; uno tiene disfunción vesical permanente. Se observaron una dehiscencia de la herida, una infección y una fístula de líquido cefalorraquídeo. Todos permanecen sin la enfermedad tras un seguimiento de entre 6 y 24 meses. Conclusiones: La resección parcial del sacro por abordaje posterior único se puede indicar cuando la lesión compromete desde S2 hacia distal y no hay compromiso sacroilíaco. La preservación de raíces es de vital importancia para garantizar mejores resultados posoperatorios y una menor tasa de infección. Nivel de Evidencia: IV

Introduction: Tumors of the sacrum represent less than 7% of spinal tumors. Secondary tumors due to multiple myeloma or prostate, breast, lung and colon carcinomas predominate. Chordoma is the most frequent primary malignant tumor and giant cell tumor is the most common benign lesion. For its evolution, involvement of extra bone structures and poor response to the adyuvant therapy, surgery is the most commonly used treatment, the approach and the need for instrumentation depend on tumor to treat. The objectives of this study are to evaluate the use of partial resection of sacrum, analyze the surgical technique and assess complications. Methods: Four patients with diagnosis of sacral tumor below S1, who presented with pain and images confirming the injury. All underwent an en bloc resection by posterior via preserving S1. The technique is described. Results: The pathological results were: a chordoma, a malignant nerve sheath tumor, a chondrosarcoma and a metastasis of prostatic carcinoma. S1 function was preserved in all patients; one has permanent bladder dysfunction. Wound dehiscence, infection and cerebrospinal fluid fistula were detected. Patients are free of disease after a follow-up of 6-24 months. Conclusions: Partial resection of the sacrum using a unique posterior approach may be indicated when the injury involves from S2 distally and no sacroiliac involvement is observed. Root preservation is vital to guarantee the best postoperative results and a lower rate of infection. Level of Evidence: IV

Endoscopic Endonasal Management of Skull Base Chordomas: Surgical Technique, Nuances, and Pitfalls.

Chordoma is a rare primary bone tumor derived from transformed notochord remnants. It has a local aggressive behavior and high recurrence rates. Treatment of skull base chordomas is complex and challenging. Control of the disease relies mainly on surgical excision of the tumor, sometimes followed by high-dose radiation therapy. The main surgical goal is to achieve maximal tumor removal with minimal morbidity. Development of the expanded endoscopic endonasal approach has improved surgical and prognostic results of skull base chordomas. This article highlights important aspects of approach selection, technique, and nuances of surgical management of this tumor.

Natural history and surgical treatment of chordoma: a retrospective cohort study / História natural e tratamento cirúrgico de cordoma: um estudo retrospectivo de coorte

CONTEXT AND OBJECTIVE: Chordoma is a rare tumor with a high risk of locoregional recurrences. The aim of this study was analyze the long-term results from treating this pathological condition. DESIGN AND SETTING: Cohort study in a single hospital in São Paulo, Brazil. METHODS: This was a retrospective cohort study on 42 patients with chordoma who were treated at Hospital A. C. Camargo between 1980 and 2006. The hospital records were reviewed and a descriptive analysis was performed on the clinical-pathological variables. Survival curves were estimated using the Kaplan-Meier method and these were compared using the log-rank test. RESULTS: Nineteen patients were men and 23 were women. Twenty-five tumors (59.5%) were located in the sacrum, eleven (26.2%) in the skull base and six (14.3%) in the mobile spine. Surgery was performed on 28 patients (66.7%). The resection was considered to have negative margins in 14 cases and positive margins in 14 cases. The five-year overall survival (OS) was 45.4%. For surgical patients, the five-year OS was 64.3% (82.2% for negative margins and 51.9% for positive margins). In the inoperable group, OS was 37.7% at 24 months and 0% at five years. CONCLUSION: Complete resection is related to local control and definitively has a positive impact on long-term survival. .

CONTEXTO E OBJETIVO: Cordoma é um tumor raro e com alto risco de recidiva locorregional. O objetivo deste estudo foi analisar os resultados a longo prazo do tratamento dessa doença. TIPO DE ESTUDO E LOCAL: Estudo de coorte realizado em um único hospital em São Paulo, Brasil. MÉTODOS: Estudo de coorte retrospectivo com 42 pacientes com cordoma tratados de 1980 e 2006 no Hospital A. C. Camargo. Os prontuários foram revistos e foi realizada a análise descritiva das variáveis clínicas e patológicas. As curvas de sobrevida foram estimadas pelo método de Kaplan-Meier e a comparação entre elas, pelo teste de log-rank. RESULTADOS: Dezenove pacientes eram homens e 23, mulheres. Vinte e cinco tumores (59,5%) estavam localizados no sacro, 11 (26,2%) na base do crânio e 6 (14,3%), na coluna móvel. A cirurgia foi realizada em 28 pacientes (66,7%). A ressecção foi considerada como tendo margens negativas em 14 casos e margens comprometidas em 14 pacientes. A sobrevida global (SG) em 5 anos foi de 45,4%. Para os pacientes cirúrgicos, a SG em 5 anos foi de 64,3% (82,2% para as margens negativas e 51,9% de margens positivas). No grupo inoperável, a SG em 24 meses foi de 37,7% e 0% em 5 anos. CONCLUSÃO: A ressecção completa está relacionada com o controle local e, definitivamente, tem impacto positivo na sobrevida a longo prazo. .

Natural history and surgical treatment of chordoma: a retrospective cohort study.

CONTEXT AND OBJECTIVE: Chordoma is a rare tumor with a high risk of locoregional recurrences. The aim of this study was analyze the long-term results from treating this pathological condition. DESIGN AND SETTING: Cohort study in a single hospital in São Paulo, Brazil. METHODS: This was a retrospective cohort study on 42 patients with chordoma who were treated at Hospital A. C. Camargo between 1980 and 2006. The hospital records were reviewed and a descriptive analysis was performed on the clinical-pathological variables. Survival curves were estimated using the Kaplan-Meier method and these were compared using the log-rank test. RESULTS: Nineteen patients were men and 23 were women. Twenty-five tumors (59.5%) were located in the sacrum, eleven (26.2%) in the skull base and six (14.3%) in the mobile spine. Surgery was performed on 28 patients (66.7%). The resection was considered to have negative margins in 14 cases and positive margins in 14 cases. The five-year overall survival (OS) was 45.4%. For surgical patients, the five-year OS was 64.3% (82.2% for negative margins and 51.9% for positive margins). In the inoperable group, OS was 37.7% at 24 months and 0% at five years. CONCLUSION: Complete resection is related to local control and definitively has a positive impact on long-term survival.

Sacral chordoma: management of a rare disease in a tertiary hospital.

INTRODUCTION: Chordoma is a rare malignant bone tumor. The outcome depends on the adequate surgical treatment with complete excision of the tumor with microscopic clear margins. MATERIALS AND METHODS: We performed a retrospective review of 13 cases of chordomas operated in our center between 1988 and 2009. RESULTS: All cases were treated with wide excision. Inadequate margins were found in seven patients. There were complications in ten patients and long-term sequels in 11 patients. Nine had recurrence within the first 2 years, six of which had inadequate margins. Five had metastatic disease, three of them with inadequate margins. During the follow-up, four patients died. In a posterior review, three patients survive and all of them had clear margins. CONCLUSIONS: In our experience, the best treatment is complete excision of the tumor with clear microscopic margins. When this is not possible, local recurrences increase and survival rate decreases.