Notochordal Tumors

Yamaguchi, Takehiko; Imada, Hiroki; Iida, S.; Szuhai, Károly

doi:10.1016/j.path.2017.04.008

Cited by 32 publications

(14 citation statements)

References 85 publications

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“…This would inform future targeted therapies and clinical trials making use of our initial findings. Moreover, while molecular targeting therapies have been investigated in chordoma but have largely been underwhelming, the cyclin E1 should be investigated as a novel therapeutic strategy for chordoma patients (40)(41)(42)(43).…”

Section: Discussionmentioning

confidence: 99%

“…There are some limitations in our study. First, 75 chordoma cases is comparatively large sample size for this rare disease as compared to other previous studies on investigating prognostic biomarkers in chordoma (7,41,42). However, future studies to increase the number of specimens are needed to validate cyclin E1 as a biomarker for chordoma prognosis.…”

Section: Discussionmentioning

confidence: 99%

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Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study

et al. 2020

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Purpose Chordomas are rare, slow-growing sarcomas without any accepted prognostic biomarkers. Owing to their proximity to critical neurovascular structures, discovering predictive biomarkers in chordoma has been a significant research effort because it may potentially reduce risky therapies in patients with less aggressive tumors. In response, because cyclin E1 overexpression correlates with patient prognosis in several malignancies, we investigated its expression in chordoma and whether it informs patient prognosis. Methods Seventy-five chordoma patient specimens were enrolled in a tissue microarray (TMA) to evaluate cyclin E1 expression via immunohistochemical staining. Western blot was used to assess cyclin E1 expression in chordoma cell lines and fresh tissues. We then correlated cyclin E1 staining intensity in the TMA to clinicopathological features and chordoma patient outcomes. Results Sixty-three percent of the chordoma patient specimens in the TMA, fifty-six percent of the fresh chordoma tissues, and all chordoma cell lines showed high cyclin E1 expression. In TMA analysis, cyclin E1 expression positively correlated to chordoma patient disease status. By survival analysis, high cyclin E1 expression was an independent prognostic risk factor for chordoma patients along with advanced disease status and positive surgical margin. Conclusion Cyclin E1 is a promising biomarker predicting chordoma patient prognosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study

et al. 2020

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“…Chordomas are slow-growing, locally invasive tumors arising at the cranial (32%), spinal (32%), and sacral (29%) sites occurring in 8.4 per 10 million patients [ 1 ]. Historically hypothesized as originating from notochordal remnants, recent research revealed that some developed from benign notochordal cell tumors (BCNT) [ 2 ]. The classification system is based on location: osseous extradural (Type I), extraosseous extradural (Type II), osseous intradural (Type III), and extraosseous intradural (Type IV) chordomas [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

Isolated cervical extraosseous intradural chordoma attached to the C5 nerve root: a case report

et al. 2019

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Background: As chordomas are slow growing and locally invasive with high recurrence rates, initial recommendations include complete surgical resection with or without radiation therapy. A large proportion of recurrences occur years after initial resection necessitating lengthy follow-up. The novel biomarker brachyury and the repurposing of pharmaceutical products have the potential to substantially impact long-term recurrence rates. Case presentation: A 43-year-old woman presented with an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root underwent a C3-5 laminectomy, C3-5 lateral mass screw instrumentation, and mass resection. All symptoms resolved by the 12-month postoperative follow-up visit. Conclusions: This is the first report of an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root, and this case adds to the previous six Type IV chordomas in the literature. Unfortunately, the very rare form of extraosseous intradural chordoma is poorly understood: the lack of detailed knowledge in how they are differentiated from other forms of chordoma confounds the development of optimal treatment strategies and follow-up guidelines.

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“…It can be particularly difficult to deliver sufficient radiation doses in cases of chondrosarcoma and chordoma due to nearby organs being at risk. Preoperative radiation therapy in these cases is generally not utilized (84)(85)(86)(87). Modern treatment regimens for classic osteosarcoma include preoperative chemotherapy to eradicate micrometastatic disease.…”

Section: Malignant Tumors Of Bone and Cartilagementioning

confidence: 99%

“…Although its benefit in craniofacial osteosarcoma (CFOS) is controversial, preoperative chemotherapy has been associated with improved survival in patients with high-grade CFOS (88)(89)(90)(91)(92)(93)(94)(95)(96)(97). Preoperative chemotherapy is not advocated in cases of chondrosarcoma and chordoma as these tumors are resistant (84,86,87).…”

Section: Malignant Tumors Of Bone and Cartilagementioning

confidence: 99%

The Role of Adjuvant Treatment in Craniofacial Malignancy: A Critical Review

et al. 2020

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Background: Tumors originating from the craniofacial region usually present in a locally advanced stage with frequent involvement of adjacent sites and have a strong tendency for local recurrence in the absence of adjuvant therapy, even when the original surgical resection was presumed to be radical. In the past decades, several advances in the radiological diagnosis and treatment of craniofacial malignancies have been introduced. There are, however, no randomized trials that define the optimal multimodal treatment of these tumors because of their rarity as well as heterogeneity in both histology and site of origin. The aim of this study was to conduct a critical review of the role of adjuvant therapy in the treatment of craniofacial malignancy. Method: We conducted a critical review of the past and contemporary literature available, focusing on adjuvant oncological treatments of the most common craniofacial malignancies. Results: Preoperative radiotherapy can have a documented role in the treatment of olfactory neuroblastoma and soft tissue sarcoma, while preoperative chemotherapy can be advocated in the treatment of sinonasal undifferentiated carcinoma, neuroendocrine carcinoma, olfactory neuroblastoma, and craniofacial sarcoma (both soft-tissue and high-grade osteosarcoma). Postoperative radiotherapy has a well-established role in the treatment of most craniofacial malignancies. The role of postoperative chemotherapy is unclear in most histologies, but is commonly used during the treatment of well-selected cases of paranasal sinus carcinoma, olfactory neuroblastoma, mucosal melanoma, soft tissue sarcoma and high-grade craniofacial osteosarcoma. Discussion: Alongside developments in surgery, there have also been improvements in diagnostics, radiotherapy, and chemotherapy. Implementation of novel radiation techniques allows delivery of higher radiation doses while minimizing irradiation-related morbidity. Better understanding of tumor biology allows the construction of more König et al. Adjuvant Treatment in Craniofacial Malignancy complex treatment strategies, incorporating adjuvant chemotherapy either pre-or postoperatively. In the era of personalized targeted therapy, rapid strides are being made to identify specific tumor-targets for use of novel biologic agents, with the potential to change current management paradigms.

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Notochordal Tumors

Cited by 32 publications

References 85 publications

Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study

Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study

Isolated cervical extraosseous intradural chordoma attached to the C5 nerve root: a case report

The Role of Adjuvant Treatment in Craniofacial Malignancy: A Critical Review

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