Comparison of Primary Spinal Central and Peripheral Primitive Neuroectodermal Tumors in Clinical and Imaging Characteristics and Long-Term Outcome

Qi, Wei; Deng, Xiaofeng; Liu, Tie; Hou, Ying-Zhi; Yang, Chenlong; Wu, Liang; Fang, Jingyi; Tong, Xianzeng; Yang, Jun; Xu, Yulun

doi:10.1016/j.wneu.2015.12.033

Cited by 17 publications

(15 citation statements)

References 27 publications

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“…Limb weakness (70%) and pain (50%), as well as incontinence (20%), were the most common initial symptoms, which is largely consistent with previous reports (5, 9). The mean duration of symptoms before the first operation was 42 days, which is longer than that of previous reports (5, 10). The lesions were generally located in the thoracic spine (60.0%), which is consistent with previous reports (11).…”

Section: Discussioncontrasting

confidence: 54%

“…Since ES/pPNETs have the character of local infiltration, the local recurrence rate will be high if initial surgery is inadequate. Previous studies have demonstrated that gross total resection can result in better prognosis than subtotal resection (5, 10). In our study cohort, resection mode included partial resection, subtotal resection, total piecemeal resection, and total en bloc resection.…”

Section: Discussionmentioning

confidence: 99%

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Treatment Outcomes and Prognostic Factors of Patients With Primary Spinal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors

Zheng

Fan

et al. 2019

Front. Oncol.

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Purpose: Primary spinal Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare, and the current understanding of these tumors is poor. The authors aimed to illustrate the clinical characteristics of primary spinal ES/pPNETs and to discuss prognostic factors by survival analysis. Methods: A total of 40 patients who were pathologically diagnosed with primary spinal ES/pPNETs between 2000 and 2018 were enrolled in this study. Progression-free survival (PFS) and overall survival (OS) were estimated by the Kaplan–Meier method to identify potential prognostic factors. Factors of p ≤ 0.1 in the Log-rank tests were subjected to multivariate analysis by Cox regression analysis. Results: The mean follow-up period was 23.8 (range, 2–93) months, and 24 (60.0%) patients had local recurrence and 11 (27.5%) patients had distant metastasis. The 1-, 2-, and 5-year PFS rates were 57.7, 30.4, and 9.5%, respectively. The 1-, 2-, and 5-year OS rates were 74.8, 50.7, and 12.2%, respectively. The univariate analysis suggested that resection mode, postoperative Frankel score, adjuvant chemotherapy and adjuvant radiotherapy were potential prognostic factors for OS and PFS. However, after these factors were subjected to multivariate analyses, only adjuvant radiotherapy and resection mode remained as independent prognostic factors. Conclusions: Total en bloc resection can significantly improve PFS for primary spinal ES/pPNETs and adjuvant radiotherapy was a favorable factor for PFS. Total en bloc resection and adjuvant radiotherapy considerably improve OS for patients with primary spinal ES/pPNETs.

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Section: Discussioncontrasting

confidence: 54%

Section: Discussionmentioning

confidence: 99%

Treatment Outcomes and Prognostic Factors of Patients With Primary Spinal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors

Zheng

Fan

et al. 2019

Front. Oncol.

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“…The radiographic ndings of intraspinal PNET are usually lack of speci city, and radiographic diagnosis may often indicate astrocytoma, ependeoma or schwannoma. Although imaging data is nonspeci c, the possibility of PNET should be considered in pediatric patients when MRI reveals large, well-de ned margin intraspinal space-occupying lesions [14].…”

Section: Discussionmentioning

confidence: 99%

Intraspinal Space-Occupying Lesions in Children: Clinical Features, Neuroimaging and Surgical Outcomes of 27 Cases

Chang

Jiang

Wang

et al. 2021

Preprint

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Background: Pediatric intraspinal space-occupying lesions are relatively uncommon. However, these lesions can result in neurological disabilities due to misdiagnosis and delayed treatment. The main goal of the present study is to evaluate the clinical and radiological features and treatment options of pediatric intraspinal space-occupying lesions in order to improve the clinical recognition and management. Methods: Clinical data of 27 children with intraspinal space-occupying lesions who underwent surgery treatment in a tertiary-care hospital between 2010 and 2018 were retrospectively reviewed and analyzed. Results: Of these 27 patients, 14 (51.85%) were girls and 13 (48.15%) were boys. The most common age group affected was 10~14 years (62.96%, 9 girls and 8 boys in this age group). The mean age was 10.11 years old. Pain and weakness were the most common clinical symptoms. Preoperative magnetic resonance imaging (MRI) identified intramedullary (10 cases, 37.04%), intradural extramedullary (10 cases, 37.04%) and extradural (7 cases, 25.92%) lesions, respectively. The majority of the lesions were intraspinal tumors (23 cases, 85.19%). The histological diagnosis of tumors included embryonic residual tumors (6 cases, 22.22%), ependymoma (5 cases, 18.52%), primitive neuroectodermal tumors (PNET) (3 cases, 11.11%), schwannomas (2 cases, 7.4%), ganglioneuroma (1 case, 3.7%), Ewing’s sarc (1 case, 3.7%), B-cell non Hodgkin lymphoma (1 case, 3.7%), Hodgkin lymphoma (1 case, 3.7%), chondrosarcoma (1 case, 3.7%), ganglioglioma (1 case, 3.7%), and glioma (1 case, 3.7%). Conclusions: The incidence of pediatric intraspinal space-occupying lesions is low, and the clinical manifestation is lack of specificity. The prognosis for children with malignant tumors is poor and surgical resection is still the primary treatment option.

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“…Radiotherapy as local treatment approach is used when complete local resection is not feasible with a functional organ, a difficult anatomic location, or with very large tumor volume not amenable to radical surgery even after neoadjuvant chemotherapy, and in case of a metastatic disease. Post-operative radiotherapy has been implied to decrease local recurrence and provide prolonged survival 25,26 . The selection of local treatment modality is considerably biased by several factors, including tumor location, tumor volume, sensitivity to chemotherapy, patient general status, and institutional protocol.…”

Section: Discussionmentioning

confidence: 99%

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor

Liu

Yuan

Zhang

et al. 2020

Sci Rep

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Peripheral primitive neuroectodermal tumors (PNETs) constitute very rare and aggressive malignancies. To date, there are no standard guidelines for management of peripheral PNETs due to the paucity of cases arising in various body sites. Therapeutic approach is derived from Ewing sarcoma family, which currently remains multimodal. Our study retrospectively analyzed 86 PNET patients from February 1, 1998 to February 1, 2018 at Peking Union Medical College Hospital with an additional 75 patients from review of literature. The clinicopathologic and treatment plans associated with survival was investigated. Surgery, chemotherapy, female sex, small tumor size, no lymph node metastasis, R0 surgical resection, (vincristine + doxorubicin + cyclophosphamide)/(isophosphamide + etoposide) regimen, and more than 10 cycles of chemotherapy were associated with improved overall survival in univariate analysis. Surgery, more than 10 cycles of chemotherapy, and small tumor size were independent prognostic factors for higher overall survival. Our data indicates that multimodal therapy is the mainstay therapeutic approach for peripheral PNET.

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Comparison of Primary Spinal Central and Peripheral Primitive Neuroectodermal Tumors in Clinical and Imaging Characteristics and Long-Term Outcome

Cited by 17 publications

References 27 publications

Treatment Outcomes and Prognostic Factors of Patients With Primary Spinal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors

Treatment Outcomes and Prognostic Factors of Patients With Primary Spinal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors

Intraspinal Space-Occupying Lesions in Children: Clinical Features, Neuroimaging and Surgical Outcomes of 27 Cases

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor

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