Primary Spinal Intradural Extramedullary Mesenchymal Chondrosarcoma

Saito, Ryu; Senbokuya, Nobuo; Yagi, Takashi; Yoshioka, Hideyuki; Kanemaru, Kazuya; Kinouchi, Hiroyuki

doi:10.1016/j.wneu.2020.10.007

Cited by 6 publications

(18 citation statements)

References 26 publications

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“…Spinal intradural extramedullary MCS is an extremely rare malignant tumour that has been described in only a few case reports. Including our case, the clinical information of 20 cases of primary intradural MCS in the literature is summarized in (Table 1) (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). Although primary intraspinal MCS can occur at any age and in any organ or tissue in the body, the disease tends to occur in younger people, with a mean age of 11 years (18), and most commonly affects the upper lumbar and lower thoracic spine.…”

Section: Discussionmentioning

confidence: 99%

“…Including our case, the clinical information of 20 cases of primary intradural MCS in the literature is summarized in (Table 1) (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). Although primary intraspinal MCS can occur at any age and in any organ or tissue in the body, the disease tends to occur in younger people, with a mean age of 11 years (18), and most commonly affects the upper lumbar and lower thoracic spine. These tumours are usually solitary and tend to be located on the right side of the spine.…”

Section: Discussionmentioning

confidence: 99%

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Rare malignant primary spinal intradural extramedullary mesenchymal chondrosarcoma: a case report and literature review

Wang¹,

Meng²,

Liu³

et al. 2022

Transl Cancer Res TCR

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Background: Mesenchymal chondrosarcoma (MCS) is a rare malignant chondrosarcoma with a high propensity for recurrence and distant metastasis. MCS usually arises from bone tissue, and rarely occurs outside the bone. MCS in the subdural and extramedullary regions of the spinal cord is especially rare. In this article, we report a case of spinal intradural extramedullary MCS with herpes virus infection, which is the first such case reported in East China.Case Description: A 13-year-old male complained of intermittent low-grade fever, sweating, progressive constipation with weakness of both lower extremities and bilateral hypoesthesia after a 5-month history of herpes virus infection. Spinal magnetic resonance imaging (MRI) revealed a subdural-extramedullary solid nodular mass with isointensity on T1-weighted imaging and hyperintensity on T2-weighted imaging that was located behind the superior margin of the T5 vertebral body. The patient was initially diagnosed with thoracic meningioma and underwent spinal cord tumour resection followed by adjuvant chemotherapy.Histopathological examination revealed that the tumour was mainly composed of round or oval cells and mesenchymal chondroid matrix, and gene analysis showed the fusion of HEY1 exon 4 to NCOA2 exon 13.Both test results were consistent with the diagnosis of primary intraspinal MCS. At the 1-year follow-up, the patient received adjuvant chemotherapy, and the reexamination images revealed no evidence of tumour in situ tumour recurrence or distant metastasis.Conclusions: As more research has been done on MCS, it has been found that the disease is more likely to occur in adolescents, but is often overlooked due to its lack of imaging characterization. Therefore, the misdiagnosis rate can be reduced only by closely considering clinical manifestations with pathology and imaging findings. Although MCS is a highly malignant tumour, early primary spinal intradural extramedullary MCS can cause neurological symptoms, early detection and treatment can achieve basic total surgical resection. Postoperative adjuvant chemoradiotherapy can further reduce recurrence.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Rare malignant primary spinal intradural extramedullary mesenchymal chondrosarcoma: a case report and literature review

Wang¹,

Meng²,

Liu³

et al. 2022

Transl Cancer Res TCR

View full text Add to dashboard Cite

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“…Although most of intraspinal MCS showed dural attachment in surgery, the dural tail sign was much rarer observed on enhanced MR images. Saito reviewed 19 intradural MCS from the literature, 12 showed dural attachment and only two had the dural tail sign [ 11 ]. It seems to be difficult to distinguish MCS from spinal meningioma if the dural tail sign is also present on enhanced MRI.…”

Section: Discussionmentioning

confidence: 99%

Primary intra- and extradural extramedullary mesenchymal chondrosarcoma with isolated punctate calcification: case report and literature review

Chen

Lai

2022

BMC Neurol

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Background Mesenchymal chondrosarcoma (MCS) is an ultra-rare, high-grade subtype of chondrosarcoma affecting both bone and soft tissues. Extra-skeletal MCS rarely occurs in intra- and extradural regions. Case presentation We presented a case of intraspinal dumbbell-shaped MCS at the T12-L2 level with isolated punctate calcification in a 19-year-old male complaining of progressive lower back pain. Surgical treatment for removal of the tumor was performed. The postoperative pathological result confirmed MCS. The patient showed symptomatic improvement and follow-up MRI showed no evidence of recurrence or metastasis for nearly 1 year after surgery. Conclusions CT and MRI play an important role in differential diagnosis for intraspinal MCS. MCS should be added to the differential diagnosis of intraspinal dumbbell-shaped tumors, especially when radiological examinations reveal punctate calcification in a homogeneous enhanced tumor without dural tail sign. However, the final diagnosis depends on histopathological results. Despite the good prognosis of intraspinal MCS, close follow-up after operation is still necessary.

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“…Although MBT is not common, their mortality is still high. The three most common MBT in elderly patients include chondrosarcoma, osteosarcoma, and chord sarcoma, of which chondrosarcoma is the most common ( 5 , 6 ). Our study also found that most elderly patients with MBT over 60 years old are chondrosarcoma.…”

Section: Discussionmentioning

confidence: 99%

“…The prognosis of patients with osteosarcoma metastasis is inferior, and the overall 5-year survival rate is 20–30% ( 4 ). Chondrosarcoma accounts for about 9.2% of all primary MBT, with an annual incidence of 1/200,000 and an average age of onset of about 50 years old ( 5 , 6 ). It is the second most common bone malignant tumor after osteosarcoma.…”

Section: Introductionmentioning

confidence: 99%

A Web-Based Prediction Model for Overall Survival of Elderly Patients With Malignant Bone Tumors: A Population-Based Study

Tang

Wang

Pan

2022

Front. Public Health

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Background: Malignant bone tumors (MBT) are one of the causes of death in elderly patients. The purpose of our study is to establish a nomogram to predict the overall survival (OS) of elderly patients with MBT.Methods: The clinicopathological data of all elderly patients with MBT from 2004 to 2018 were downloaded from the SEER database. They were randomly assigned to the training set (70%) and validation set (30%). Univariate and multivariate Cox regression analysis was used to identify independent risk factors for elderly patients with MBT. A nomogram was built based on these risk factors to predict the 1-, 3-, and 5-year OS of elderly patients with MBT. Then, used the consistency index (C-index), calibration curve, and the area under the receiver operating curve (AUC) to evaluate the accuracy and discrimination of the prediction model was. Decision curve analysis (DCA) was used to assess the clinical potential application value of the nomogram. Based on the scores on the nomogram, patients were divided into high- and low-risk groups. The Kaplan-Meier (K-M) curve was used to test the difference in survival between the two patients.Results: A total of 1,641 patients were included, and they were randomly assigned to the training set (N = 1,156) and the validation set (N = 485). The univariate and multivariate analysis of the training set suggested that age, sex, race, primary site, histologic type, grade, stage, M stage, surgery, and tumor size were independent risk factors for elderly patients with MBT. The C-index of the training set and the validation set were 0.779 [0.759–0.799] and 0.801 [0.772–0.830], respectively. The AUC of the training and validation sets also showed similar results. The calibration curves of the training and validation sets indicated that the observed and predicted values were highly consistent. DCA suggested that the nomogram had potential clinical value compared with traditional TNM staging.Conclusion: We had established a new nomogram to predict the 1-, 3-, 5-year OS of elderly patients with MBT. This predictive model can help doctors and patients develop treatment plans and follow-up strategies.

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Primary Spinal Intradural Extramedullary Mesenchymal Chondrosarcoma

Cited by 6 publications

References 26 publications

Rare malignant primary spinal intradural extramedullary mesenchymal chondrosarcoma: a case report and literature review

Rare malignant primary spinal intradural extramedullary mesenchymal chondrosarcoma: a case report and literature review

Primary intra- and extradural extramedullary mesenchymal chondrosarcoma with isolated punctate calcification: case report and literature review

A Web-Based Prediction Model for Overall Survival of Elderly Patients With Malignant Bone Tumors: A Population-Based Study

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