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1.
J Pak Med Assoc ; 74(4): 794-796, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38751282

RESUMO

Enbloc Sacrectomy is the procedure of choice for aggressive sacral lesions but not widely practiced in Pakistan, both by Neurosurgeons and Orthopaedic surgeons. Only one case has been mentioned in indexed local literature so far and that too not operated in Pakistan. The case of a 27 year old neurologically intact male is presented. He had a huge residual mass and midline non-healing wound after two attempts at intralesional debulking and one full course of local irradiation. He presented to the Mayo Hospital, Lahore on 29th December 2021 for a redo surgery of sacral chordoma. A marginal excision was achieved utilizing posterior only approach. This case will help to understand the key steps in enbloc mid-Sacrectomy and importance of involving multidisciplinary team for ensuring adequate wound closure.


Assuntos
Cordoma , Reoperação , Sacro , Neoplasias da Coluna Vertebral , Humanos , Cordoma/cirurgia , Cordoma/diagnóstico por imagem , Masculino , Sacro/cirurgia , Adulto , Neoplasias da Coluna Vertebral/cirurgia , Reoperação/métodos
2.
Neurosurg Focus ; 56(5): E10, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38691855

RESUMO

OBJECTIVE: Chordoma is a primary bone tumor with limited literature on its management because of its rarity. Resection, while considered the first-line treatment, does not always provide adequate tumor control. In this systematic review, the authors aimed to provide comprehensive insights by managing these tumors with stereotactic radiosurgery (SRS). METHODS: A systematic review was conducted according to PRISMA guidelines using the PubMed, Scopus, Web of Science, Embase, and Cochrane Library databases. Search terms included chordoma and radiosurgery and their equivalent terms. Data on baseline characteristics, SRS details, and outcomes were extracted. The Joanna Briggs Institute checklist was used to assess risk of bias. A meta-analysis was performed on relevant variables. RESULTS: A total of 33 eligible studies encompassing 714 patients with skull base chordomas were included. Most studies had a low risk of bias. Patients, predominantly male (57.37%) with a mean age of 46.54 years, exhibited a conventional chordoma subtype (74.77%) and primary lesions (77.91%), mainly in the clivus (98.04%). The mean lesion volume was 13.49 cm3, and 96.68% of patients had undergone prior surgical attempts. Gamma Knife radiosurgery (88.76%) was the predominant SRS method. Radiologically, 27.19% of patients experienced tumor regression, while 55.02% showed no signs of disease progression at the latest follow-up. Progression occurred after a mean of 48.02 months. Symptom improvement was noted in 27.98% of patients. Radiosurgery was associated with a relatively low overall adverse event rate (11.94%), mainly cranial nerve deficits (8.72%). Meta-regression revealed that age and primary lesion type influenced symptom improvement, while factors like extent of resection, radiotherapy, and SRS type affected adverse event rates. CONCLUSIONS: This systematic review provides evidence on the safety and effectiveness of radiosurgery in the management of skull base chordomas. Local tumor control was achieved in the majority of patients treated with SRS. Various baseline characteristics and SRS features have been analyzed to identify modifying factors for each outcome to provide a framework for informed decision-making when managing these patients.


Assuntos
Cordoma , Radiocirurgia , Neoplasias da Base do Crânio , Radiocirurgia/métodos , Humanos , Cordoma/cirurgia , Cordoma/radioterapia , Cordoma/diagnóstico por imagem , Neoplasias da Base do Crânio/cirurgia , Neoplasias da Base do Crânio/radioterapia , Neoplasias da Base do Crânio/diagnóstico por imagem , Resultado do Tratamento
3.
Neurosurg Focus ; 56(5): E14, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38691865

RESUMO

OBJECTIVE: Chordomas are locally aggressive neoplasms of the spine or skull base that arise from embryonic remnants of the notochord. Intradural chordomas represent a rare subset of these neoplasms, and few studies have described intradural chordomas in the spine. This review evaluates the presentation, management, and outcomes of intradural spinal chordomas. METHODS: A systematic review of PubMed/MEDLINE, EMBASE, Cochrane Library, Scopus, and Web of Science was performed. Studies describing at least 1 case of intradural chordomas anywhere in the spine were included. Extracted details included presenting symptoms, radiological findings, treatment course, follow-up, and disease progression. RESULTS: Thirty-one studies, with a total of 41 patients, were included in this review. Seventy-six percent (31/41) of patients had primary intradural tumors, whereas 24% (10/41) presented with metastasis. The most common signs and symptoms were pain (n = 27, 66%); motor deficits (n = 20, 49%); sensory deficits (n = 17, 42%); and gait disturbance (n = 10, 24%). The most common treatment for intradural chordoma was resection and postoperative radiotherapy. Sixty-six percent (19/29) of patients reported improvement or complete resolution of symptoms after surgery. The recurrence rate was 37% (10/27), and the complication rate was 25% (6/24). The median progression-free survival was 24 months (range 4-72 months). Four patient deaths were reported. The median follow-up time was 12 months (range 13 days-84 months). CONCLUSIONS: Treatment of intradural spinal chordomas primarily involves resection and radiotherapy. A significant challenge and complication in management is spinal tumor seeding after resection, with 9 studies proposing seeding as a mechanism of tumor metastasis in 11 cases. Factors such as tumor size, Ki-67 positivity, and distant metastasis may correlate with worse outcomes and demonstrate potential as prognostic indicators for intradural spinal chordomas. Further research is needed to improve understanding of this tumor and develop optimal treatment paradigms for these patients.


Assuntos
Cordoma , Neoplasias da Medula Espinal , Humanos , Cordoma/cirurgia , Cordoma/diagnóstico por imagem , Neoplasias da Medula Espinal/cirurgia , Neoplasias da Medula Espinal/terapia , Resultado do Tratamento , Neoplasias da Coluna Vertebral/cirurgia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Gerenciamento Clínico
4.
Neurosurg Focus ; 56(5): E7, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38691863

RESUMO

OBJECTIVE: Contemporary management of sacral chordomas requires maximizing the potential for recurrence-free and overall survival while minimizing treatment morbidity. En bloc resection can be performed at various levels of the sacrum, with tumor location and volume ultimately dictating the necessary extent of resection and subsequent tissue reconstruction. Because tumor resection involving the upper sacrum may be quite destabilizing, other pertinent considerations relate to instrumentation and subsequent tissue reconstruction. The primary aim of this study was to survey the surgical approaches used for managing primary sacral chordoma according to location of lumbosacral spine involvement, including a narrative review of the literature and examination of the authors' institutional case series. METHODS: The authors performed a narrative review of pertinent literature regarding reconstruction and complication avoidance techniques following en bloc resection of primary sacral tumors, supplemented by a contemporary series of 11 cases from their cohort. Relevant surgical anatomy, advances in instrumentation and reconstruction techniques, intraoperative imaging and navigation, soft-tissue reconstruction, and wound complication avoidance are also discussed. RESULTS: The review of the literature identified several surgical approaches used for management of primary sacral chordoma localized to low sacral levels (mid-S2 and below), high sacral levels (involving upper S2 and above), and high sacral levels with lumbar involvement. In the contemporary case series, the majority of cases (8/11) presented as low sacral tumors that did not require instrumentation. A minority required more extensive instrumentation and reconstruction, with 2 tumors involving upper S2 and/or S1 levels and 1 tumor extending into the lower lumbar spine. En bloc resection was successfully achieved in 10 of 11 cases, with a colostomy required in 2 cases due to rectal involvement. All 11 cases underwent musculocutaneous flap wound closure by plastic surgery, with none experiencing wound complications requiring revision. CONCLUSIONS: The modern management of sacral chordoma involves a multidisciplinary team of surgeons and intraoperative technologies to minimize surgical morbidity while optimizing oncological outcomes through en bloc resection. Most cases present with lower sacral tumors not requiring instrumentation, but stabilizing instrumentation and lumbosacral reconstruction are often required in upper sacral and lumbosacral cases. Among efforts to minimize wound-related complications, musculocutaneous flap closure stands out as an evidence-based measure that may mitigate risk.


Assuntos
Cordoma , Sacro , Neoplasias da Coluna Vertebral , Humanos , Cordoma/cirurgia , Cordoma/diagnóstico por imagem , Cordoma/patologia , Sacro/cirurgia , Sacro/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/patologia , Masculino , Pessoa de Meia-Idade , Feminino , Idoso , Adulto , Procedimentos de Cirurgia Plástica/métodos
5.
Neurosurg Focus ; 56(5): E8, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38691866

RESUMO

OBJECTIVE: Skull base chordomas are rare, locally osseo-destructive lesions that present unique surgical challenges due to their involvement of critical neurovascular and bony structures at the craniovertebral junction (CVJ). Radical cytoreductive surgery improves survival but also carries significant morbidity, including the potential for occipitocervical (OC) destabilization requiring instrumented fusion. The published experience on OC fusion after CVJ chordoma resection is limited, and the anatomical predictors of OC instability in this context remain unclear. METHODS: PubMed and Embase were systematically searched according to the PRISMA guidelines for studies describing skull base chordoma resection and OC fusion. The search strategy was predefined in the authors' PROSPERO protocol (CRD42024496158). RESULTS: The systematic review identified 11 surgical case series describing 209 skull base chordoma patients and 116 (55.5%) who underwent OC instrumented fusion. Most patients underwent lateral approaches (n = 82) for chordoma resection, followed by midline (n = 48) and combined (n = 6) approaches. OC fusion was most often performed as a second-stage procedure (n = 53), followed by single-stage resection and fusion (n = 38). The degree of occipital condyle resection associated with OC fusion was described in 9 studies: total unilateral condylectomy reliably predicted OC fusion regardless of surgical approach. After lateral transcranial approaches, 4 studies cited at least 50%-70% unilateral condylectomy as necessitating OC fusion. After midline approaches-most frequently the endoscopic endonasal approach (EEA)-at least 75% unilateral condylectomy (or 50% bilateral condylectomy) led to OC fusion. Additionally, resection of the medial atlantoaxial joint elements (the C1 anterior arch and tip of the dens), usually via EEA, reliably necessitated OC fusion. Two illustrative cases are subsequently presented, further exemplifying how the extent of CVJ bony elements removed via EEA to achieve complete chordoma resection predicts the need for OC fusion. CONCLUSIONS: Unilateral total condylectomy, 50% bilateral condylectomy, and resection of the medial atlantoaxial joint elements were the most frequently described independent predictors of OC fusion in skull base chordoma resection. Additionally, consistent with the occipital condyle harboring a significantly thicker joint capsule at its posterolateral aspect, an anterior midline approach seems to tolerate a greater degree of condylar resection (75%) than a lateral transcranial approach (50%-70%) prior to generating OC instability.


Assuntos
Vértebras Cervicais , Cordoma , Osso Occipital , Neoplasias da Base do Crânio , Fusão Vertebral , Humanos , Cordoma/cirurgia , Cordoma/diagnóstico por imagem , Neoplasias da Base do Crânio/cirurgia , Neoplasias da Base do Crânio/diagnóstico por imagem , Osso Occipital/cirurgia , Osso Occipital/diagnóstico por imagem , Fusão Vertebral/métodos , Vértebras Cervicais/cirurgia , Vértebras Cervicais/diagnóstico por imagem , Feminino , Articulação Atlantoccipital/cirurgia , Articulação Atlantoccipital/diagnóstico por imagem , Masculino , Adulto , Pessoa de Meia-Idade
6.
Neurosurg Focus ; 56(5): E3, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38691859

RESUMO

OBJECTIVE: The mainstay of treatment for skull base chordoma (SBC) is maximal safe resection followed by radiotherapy. However, even after gross-total resection (GTR), the recurrence rate is high due to microscopic disease in the resection margins. Therefore, supramarginal resection (SMR) could be beneficial, as has been shown for sacral chordoma. The paradigm of postoperative radiation therapy for every patient has also begun to change, as molecular profiling has shown variability in the risk of recurrence. The aim of this study was to present the concept of SMR applied to SBC, along with an individualized decision for postoperative radiation therapy. METHODS: This is a retrospective analysis of all SBCs operated on by the senior author between 2018 and 2023. SMR was defined as negative histological margins of bone and/or dura mater, along with evidence of bone resection beyond the tumor margins in the craniocaudal and lateral planes on postoperative imaging. Tumors were classified into 3 molecular recurrence risk groups (group A, low risk; group B, intermediate risk; and group C, high risk). Postoperative radiation therapy was indicated in group C tumors, in group B chordomas without SMR, or in cases of patient preference. RESULTS: Twenty-two cases of SBC fulfilled the inclusion criteria. SMR was achieved in 12 (55%) cases, with a mean (range) amount of bone resection beyond the tumor margins of 10 (2-20) mm (+40%) in the craniocaudal axis and 6 (1-15) mm (+31%) in the lateral plane. GTR and near-total resection were each achieved in 5 (23%) cases. Three (19%) tumors were classified as group A, 12 (75%) as group B, and 1 (6%) as group C. Although nonsignificant due to the small sample size, the trends showed that patients in the SMR group had smaller tumor volumes (13.9 vs 19.6 cm3, p = 0.35), fewer previous treatments (33% vs 60% of patients, p = 0.39), and less use of postoperative radiotherapy (25% vs 60%, p = 0.19) compared to patients in the non-SMR group. There were no significant differences in postoperative CSF leak (0% vs 10%, p = 0.45), persistent cranial nerve palsy (8% vs 20%, p = 0.57), and tumor recurrence (8% vs 10%, p = 0.99; mean follow-up 15 months) rates between the SMR and non-SMR groups. CONCLUSIONS: In select cases, SMR of SBC appears to be feasible and safe. Larger cohorts and longer follow-up evaluations are necessary to explore the benefit of SMR and individualized postoperative radiation therapy on progression-free survival.


Assuntos
Cordoma , Neoplasias da Base do Crânio , Humanos , Cordoma/cirurgia , Cordoma/radioterapia , Cordoma/diagnóstico por imagem , Neoplasias da Base do Crânio/cirurgia , Neoplasias da Base do Crânio/radioterapia , Neoplasias da Base do Crânio/diagnóstico por imagem , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Estudos Retrospectivos , Idoso , Resultado do Tratamento , Procedimentos Neurocirúrgicos/métodos , Recidiva Local de Neoplasia/cirurgia , Recidiva Local de Neoplasia/diagnóstico por imagem , Adulto Jovem , Margens de Excisão
7.
Neurosurg Focus ; 56(5): E6, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38691869

RESUMO

OBJECTIVE: Chordomas are a rare and relatively slow-growing malignancy of notochordal origin with a nearly 50% recurrence rate. Chordomas of the cervical spine are particularly challenging tumors given surrounding vital anatomical structures. Although standard in other areas of the spine, en bloc resection of cervical chordomas is exceedingly difficult and carries the risk of significant postoperative morbidity. Here, the authors present their institutional experience with 13 patients treated with a structure-sparing radical resection and adjuvant radiation for cervical chordomas. METHODS: Records of the standing senior author and institutional database of spinal surgeries were retrospectively reviewed for surgically managed cervical and high thoracic chordomas between 1997 and 2022. Chordomas whose epicenter was cervical but touched the clivus or had extension to the thoracic spine were included in this series. Clinical and operative data were gathered and analyzed for the index surgery and any revisions needed. Outcome metrics such as recurrence rates, complication rates, functional status, progression-free interval (PFI) and overall survival (OS) were evaluated. RESULTS: The median patient age at diagnosis was 57 (range 32-80) years. The median modified Rankin Scale (mRS) score at the time of presentation was 1 (range 0-4). Approximately 40% of tumors were located in the upper cervical spine (occiput-C2). The median time from diagnosis to surgery was 74.5 (range 10-483) days. Gross-total resection was achieved in just under 40% of patients. All patients received adjuvant radiotherapy. The mean duration of follow-up was 4.09 years, with a mean PFI of 3.80 (range 1.16-13.1) years. Five patients experienced recurrence (38.5%). The mean OS was 3.44 years. Three patients died during the follow-up period; 2 due to disease progression and 1 died in the immediate postoperative period. One patient was lost to follow-up. A significant positive relationship was identified between high cervical tumor location and disease recurrence (p = 0.021). CONCLUSIONS: While en bloc resection is appropriate and feasible for tumors in the sacral spine, the cervical region poses a significant technical challenge and is associated with increased postoperative morbidity. Radical resection may allow for achievement of negative operative margins and, along with sparing postoperative morbidity following resection of cervical chordomas, maintaining a similar rate of recurrence when compared with en bloc resection while preserving quality of life.


Assuntos
Vértebras Cervicais , Cordoma , Neoplasias da Coluna Vertebral , Humanos , Cordoma/cirurgia , Cordoma/diagnóstico por imagem , Pessoa de Meia-Idade , Feminino , Adulto , Estudos Retrospectivos , Idoso , Masculino , Vértebras Cervicais/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Idoso de 80 Anos ou mais , Recidiva Local de Neoplasia/cirurgia , Resultado do Tratamento , Procedimentos Neurocirúrgicos/métodos
8.
Neurosurg Focus ; 56(5): E2, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38691870

RESUMO

OBJECTIVE: The aim of this study was to describe the natural history of incidental benign-appearing notochordal lesions of the skull base with specific attention to features that can make differentiation from low-grade chordoma more difficult, namely contrast uptake and bone erosion. METHODS: In this retrospective case series, the authors describe the clinical outcomes of 58 patients with incidental benign-appearing notochordal lesions of the clivus, including those with minor radiological features of bone erosion or contrast uptake. RESULTS: All lesions remained stable during a median follow-up of almost 3 years. Thirty-seven (64%) patients underwent contrast-enhanced MRI; lesions in 14 (38%) of these patients exhibited minimal contrast enhancement. Twenty-seven (47%) patients underwent CT; lesions in 6 (22%) of these patients exhibited minimal bone erosion. CONCLUSIONS: These data make the case for monitoring selected cases of benign-appearing notochordal lesions of the clivus in the first instance even when there is minor contrast uptake or minimal bone erosion.


Assuntos
Achados Incidentais , Imageamento por Ressonância Magnética , Notocorda , Neoplasias da Base do Crânio , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto , Notocorda/diagnóstico por imagem , Idoso , Neoplasias da Base do Crânio/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Cordoma/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Seguimentos , Adulto Jovem , Fossa Craniana Posterior/diagnóstico por imagem
9.
Medicine (Baltimore) ; 103(14): e37678, 2024 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-38579025

RESUMO

2-Deoxy-2-[18F]fluoro-D-glucose ([18F]FDG) positron emission tomography (PET)/computed tomography (CT) is known to be a helpful imaging modality for sacral chordoma, but its detailed characteristics have not been fully described. The purpose of our study was to identify the [18F]FDG PET/CT imaging characteristics of sacral chordoma and compare them with other sacral malignancy. This retrospective study included patients who underwent [18F]FDG PET/CT because of a mass involving the sacrum. Investigated visual findings included visual score and distribution, and semiquantitative parameters measured included standardized uptake values (SUVmax, SUVpeak, SUVmean), tumor-to-liver ratio (TLR), metabolic tumor volume (MTV), total lesion glycolysis (TLG), and tumor size. Comparison studies and receiver operating characteristics (ROC) curve analysis were performed to differentiate between sacral chordoma and other sacral malignancy. Ten patients with sacral chordoma were finally included (M:F = 6:4, median age = 67 yr). On [18F]FDG PET/CT, sacral chordomas presented as a mass with minimal-moderate uptake with a usually heterogenous distribution. Compared with 12 patients with other sacral malignancies (M:F = 4:8, median age 42 yr), sacral chordoma showed a significantly lower TLR (median value 2.1 vs 6.3, P = .021). In ROC curve analysis, TLR showed the largest area under the curve (AUC) of 0.79 (cutoff ≤ 4.0; sensitivity 100.0%, specificity 58.3%; P = .004), and SUVmax showed the second largest AUC of 0.73 (cutoff ≤ 6.9; sensitivity 80.0%, specificity 66.7%; P = .034). [18F]FDG PET/CT of sacral chordoma showed minimal-moderate uptake. The TLR of [18F]FDG PET/CT was significantly lower than that of other sacral malignancy and was the most useful parameter for differentiating sacral chordoma, with the largest AUC. SUVmax could be another helpful semiquantitative parameter.


Assuntos
Cordoma , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Humanos , Idoso , Adulto , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Fluordesoxiglucose F18 , Cordoma/diagnóstico por imagem , Diagnóstico Diferencial , Sacro/diagnóstico por imagem , Estudos Retrospectivos , Carga Tumoral , Compostos Radiofarmacêuticos
11.
Med Arch ; 78(1): 68-70, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38481587

RESUMO

Background: Chordoma is a rare malignant neoplasm that predominantly arises from the axial skeleton, but can also develop in unusual locations. However, there are also rare cases of "NOS" chordoma involving the oropharyx and epithelial-myoepithelial carcinoma of the parotid gland in the same patient. According to contemporary research, chordoma is a rare malignant neoplasm that arises from the embryonic remnants of the notochord. and typically involves the clivus, sacrococcygeal bones or vertebrae. Studies have shown that the incidence of chordoma has been estimated to be one per one million people per year. Chordoma can occur at any age, but most commonly it is diagnosed in the 40-60 year old age group with the male predominance. Objective: The aim of this article was to review the case of a 74-year-old female patient with epithelial-myoepithelial carcinoma of the parotid gland and a case of "NOS" chordoma involving the oropharyx. Methods: Diagnostic methods were used to examine a female patient with two primary malignant tumors: CT neck scan, CT of paranasal sinuses, ultrasound examination, scintigraphy and operative finding. Case presentzation: Due to the anatomy complexity, complete resection of the tumor through a transoral-transpharyngeal approach was not possible. Intraoperative palpation of the mass revealed well defined submucosal lesion 20x43x46mm beginning at the level of the oro- and hypopharynx and extending superiorly to the nasopharynx, and posteriorly into the spinal canal and intervertebral foramen causing near complete occlusion of the oro and hypopharynx. The patient also underwent extracapsular dissection of the parotid tumor. Postoperative palliative radiotherapy was performed. Conclusion: Surgical treatment remains the mainstay of treatment for EMC and radiation is imperative for patients who refuse surgery and for those with advanced or inoperable diseases.


Assuntos
Carcinoma , Cordoma , Humanos , Masculino , Feminino , Idoso , Adulto , Pessoa de Meia-Idade , Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Orofaringe/patologia , Coluna Vertebral/patologia
12.
Neuroradiology ; 66(6): 955-961, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38407581

RESUMO

PURPOSE: Cranial nerve involvement (CNI) influences the treatment strategies and prognosis of head and neck tumors. However, its incidence in skull base chordomas and chondrosarcomas remains to be investigated. This study evaluated the imaging features of chordoma and chondrosarcoma, with a focus on the differences in CNI. METHODS: Forty-two patients (26 and 16 patients with chordomas and chondrosarcomas, respectively) treated at our institution between January 2007 and January 2023 were included in this retrospective study. Imaging features, such as the maximum diameter, tumor location (midline or off-midline), calcification, signal intensity on T2-weighted image, mean apparent diffusion coefficient (ADC) values, contrast enhancement, and CNI, were evaluated and compared using Fisher's exact test or the Mann-Whitney U-test. The odds ratio (OR) was calculated to evaluate the association between the histological type and imaging features. RESULTS: The incidence of CNI in chondrosarcomas was significantly higher than that in chordomas (63% vs. 8%, P < 0.001). An off-midline location was more common in chondrosarcomas than in chordomas (86% vs. 13%; P < 0.001). The mean ADC values of chondrosarcomas were significantly higher than those of chordomas (P < 0.001). Significant associations were identified between chondrosarcomas and CNI (OR = 20.00; P < 0.001), location (OR = 53.70; P < 0.001), and mean ADC values (OR = 1.01; P = 0.002). CONCLUSION: The incidence of CNI and off-midline location in chondrosarcomas was significantly higher than that in chordomas. CNI, tumor location, and the mean ADC can help distinguish between these entities.


Assuntos
Condrossarcoma , Cordoma , Neoplasias da Base do Crânio , Humanos , Feminino , Masculino , Estudos Retrospectivos , Pessoa de Meia-Idade , Cordoma/diagnóstico por imagem , Cordoma/patologia , Adulto , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/patologia , Idoso , Neoplasias da Base do Crânio/diagnóstico por imagem , Meios de Contraste , Adolescente , Imageamento por Ressonância Magnética/métodos
14.
World Neurosurg ; 181: e620-e627, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37898264

RESUMO

INTRODUCTION: Skull-base chordomas are aggressive tumors with a propensity for recurrence/progression. Even with standard of care (SoC), 5-year recurrence rates are variable (19%-54%). This high recurrence/progression rate correlates with increased morbidity and mortality. We sought to analyze a multicenter cohort of skull base chordomas to identify predictors of progression in patients receiving SoC. METHODS: The [Blinded]-Neurosurgery data registry was queried for skull base chordomas treated from 2008-2020. Patients with the histopathologic diagnosis of chordoma were included. The cohort was composed of patients with preoperative and postoperative magnetic resonance imaging. Tumor volume and radiologic characteristics were obtained from axial T2 sequences using a Digital Imaging and Communications in Medicine viewer. Survival analysis was performed using Kaplan-Meier method, and time-to-event multivariate regression was performed to identify independent predictors of progression. RESULTS: The cohort included 195 patients, of which 66 patients met inclusion criteria; median age was 44, and 28 (42%) were females. Fifty-four (82%) received SoC, 7 (11%) resection only, and 5 (8%) radiotherapy only. Median preoperative and postoperative tumor volumes were 11.55 cm3 (0.33-54.89) and 0.34 cm3 (0-42.52), respectively. Recurrence rate with SoC was 37%. Postoperative tumor volume (P = 0.010) correlated with progression. A postoperative volume of >4.9 cm3 (P = 0.044), ≤81.3% of tumor resection (P = 0.02), and lower-clivus location (P < 0.005) correlated with decreased time to progression. CONCLUSIONS: Skull base chordomas can be challenging to resect. Even though maximal resection and radiotherapy improve rate of tumor progression, many of these lesions eventually recur. We have identified a postoperative tumor volume of ≥4.9 cm3 and extent of resection of ≤81.3% in this cohort as predictors of progression in patients receiving SoC.


Assuntos
Cordoma , Neoplasias da Base do Crânio , Feminino , Humanos , Masculino , Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Cordoma/patologia , Seguimentos , Imageamento por Ressonância Magnética/métodos , Estudos Retrospectivos , Base do Crânio/patologia , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/cirurgia , Neoplasias da Base do Crânio/patologia , Análise de Sobrevida , Resultado do Tratamento , Adulto
15.
JCO Precis Oncol ; 7: e2300243, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38127828

RESUMO

PURPOSE: Chondrosarcomas arise from the lateral pelvis; however, midline chondrosarcomas (10%) display similar imaging features to chordoma, causing a diagnostic challenge. This study aims to determine the diagnostic accuracy of apparent diffusion coefficient (ADC)-based radiomic features and two novel diffusion indices for differentiating sacral chordomas and chondrosarcomas. METHODS: A retrospective, multireader review was performed of 82 pelvic MRIs (42 chordomas and 40 chondrosarcomas) between December 2014 and September 2021, split into training (n = 69) and validation (n = 13) data sets. Lesions were segmented on a single slice from ADC maps. Eight first-order features (minimum, mean, median, and maximum ADC, standard deviation, skewness, kurtosis, and entropy) and two novel indices: restriction index (RI, proportion of lesions with restricted diffusion) and facilitation index (FI, proportion of lesions with facilitated diffusion) were estimated. One hundred seven radiomic features comparing patients with chondrosarcoma versus chordoma were sorted based on mean group differences. RESULTS: There was good to excellent interobserver reliability for eight of the 10 ADC metrics on the training data set. Significant differences were observed (P < .005) for RI, FI, median, mean, and skewness using the training data set. Optimal cutpoints for diagnosis of chordoma were RI > 0.015; FI < 0.25; mean ADC < 1.7 × 10-3 mm2/s; and skewness >0.177. The optimal decision tree relied on FI. In a secondary analysis, significant differences (P < .00047) in chondrosarcoma versus chordoma were found in 18 of 107 radiomic features, including six first-order and 12 high-order features. CONCLUSION: The novel ADC index, FI, in addition to ADC mean, skewness, and 12 high-order radiomic features, could help differentiate sacral chordomas from chondrosarcomas.


Assuntos
Neoplasias Ósseas , Condrossarcoma , Cordoma , Humanos , Cordoma/diagnóstico por imagem , Cordoma/patologia , Estudos Retrospectivos , Reprodutibilidade dos Testes , Radiômica , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/patologia , Neoplasias Ósseas/diagnóstico por imagem
18.
World Neurosurg ; 179: e288-e295, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37625639

RESUMO

BACKGROUND: Sacral masses can be removed using anterior, posterior, or combined approaches. Achieving total sacrectomy through a posterior-only approach results in a shorter procedure time, minimal tissue damage, and a reduced risk of complications. In this study, we aimed to share our experience with performing total sacrectomy using a posterior-only approach in 26 patients and to assess their clinical outcomes at our center. MATERIALS AND METHODS: This retrospective study examines the clinical progression, surgical response, and outcomes of 26 patients with various sacral mass pathologies. We accessed patient information from our hospital records. RESULTS: The study included 14 men (54%) and 12 women (46%), with an average age of 49.8 years. Most cases had a normal body mass index, while 6 were overweight. Sacrectomy was performed at a high level in 12 patients and at a middle level in 14 patients. In addition to pain, motor deficits were observed in 9 patients, and sphincter dysfunction was found in 5. Preoperative embolization was conducted for 11 patients. The most prevalent lesions were chordoma (8 patients), malignant peripheral nerve sheath tumor (4 patients), giant cell tumor (3 patients), and solitary plasmacytoma (3 patients). Only 1 patient experienced a temporary partial motor deficit after surgery. There were no instances of cerebrospinal fluid leakage. Five patients experienced local recurrence, and 1 had distant metastasis. CONCLUSIONS: Performing sacrectomy for large or giant sacral tumors through a posterior approach is both feasible and safe, resulting in reduced morbidity and no significant change in overall survival.


Assuntos
Cordoma , Neoplasias da Coluna Vertebral , Masculino , Humanos , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Sacro/diagnóstico por imagem , Sacro/cirurgia , Sacro/patologia , Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Dor
19.
No Shinkei Geka ; 51(4): 679-687, 2023 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-37491065

RESUMO

Skull base chordoma is a rare bone tumor that is predominantly found in the clival area and considered to originate from the notochordal remnants. Chordoma is characterized by an aggressive nature and has a high risk of repeated recurrence despite multimodal treatments, including extensive surgical resection and high-dose radiotherapy. Thus, extensive surgical resection of the tumor and adjacent bony structures is strongly recommended. However, surgery was difficult because of the deep location of the lesion and involvement of important anatomies such as the cranial nerves and internal carotid arteries. Recent advent of endoscopic technology has changed visibility of the surgical field and accessibility, and surgical outcomes of this intractable tumor have dramatically changed. In this article, we present our surgical strategy of skull base chordoma aiming for radical surgical resection, focusing on the neuroendoscopic skull base surgery.


Assuntos
Cordoma , Neuroendoscopia , Neoplasias da Base do Crânio , Humanos , Resultado do Tratamento , Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Procedimentos Neurocirúrgicos , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/cirurgia , Fossa Craniana Posterior/cirurgia , Base do Crânio/cirurgia
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