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      Primary extradural tumors of the spinal column: A comprehensive treatment guide for the spine surgeon based on the 5 th Edition of the World Health Organization bone and soft-tissue tumor classification

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          Abstract

          Background:

          In 2020, the World Health Organization (WHO) published the 5 th version of the soft tissue and bone tumor classification. Based on this novel classification system, we reviewed the current knowledge on all tumor entities with spinal manifestations, their biologic behavior, and most importantly the appropriate treatment options as well as surgical approaches.

          Methods:

          All tumor entities were extracted from the WHO Soft-Tissue and Bone Tumor Classification (5 th Edition). PubMed and Google Scholar were searched for the published cases of spinal tumor manifestations for each entity, and the following characteristics were extracted: Growth pattern, ability to metastasize, peak age, incidence, treatment, type of surgical resection indicated, recurrence rate, risk factors, 5-year survival rate, key molecular or genetic alterations, and possible associated tumor syndromes. Surgical treatment strategies as well as nonsurgical treatment recommendations are presented based on the biologic behavior of each lesion.

          Results:

          Out of 163 primary tumor entities of bone and soft tissue, 92 lesions have been reported along the spinal axis. Of these 92 entities, 54 have the potential to metastasize. The peak age ranges from conatal lesions to 72 years. For each tumor entity, we present recommended surgical treatment strategies based on the ability to locally destruct tissue, to grow, recur after resection, undergo malignant transformation as well as survival rates. In addition, potential systemic treatment recommendations for each tumor entity are outlined.

          Conclusion:

          Based on the 5 th Edition of the WHO bone and soft tumor classification, we identified 92 out of 163 tumor entities, which potentially can have spinal manifestations. Exact preoperative tissue diagnosis and interdisciplinary case discussions are crucial. Surgical resection is indicated in a significant subset of patients and has to be tailored to the specific biologic behavior of the targeted tumor entity based on the considerations outlined in detail in this article.

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          Most cited references189

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          Changes in incidence and survival of Ewing sarcoma patients over the past 3 decades: Surveillance Epidemiology and End Results data.

          The US population-based cancer registry Surveillance, Epidemiology, and End Results (SEER) database provides an opportunity to evaluate the incidence and survival rates of Ewing sarcoma (ES) for the past 3 decades. This analysis reflects trends for the diagnosis of localized versus metastatic disease and changes in ES survival in a setting of wide-ranging cancer care institutions across the United States, which is expected to be different from clinical trials published to date. Data from the SEER public-access database were reviewed for the diagnosis of ES of the bone among patients of 1 to 19 years of age between 1973 and 2004. Age-adjusted incidence was analyzed for the entire group and for localized and metastatic disease separately over the past 3 decades. Actuarial survival rates were examined for 3 intervals: 1973 to 1982, 1983 to 1992, and 1993 to 2004. The overall incidence of ES seemed to remain unchanged with an average of 2.93 cases/1,000,000 reported annually between 1973 and 2004. The proportion of patients with distant metastasis among all ES cases remained in the 26% to 28% range, whereas the percent of localized cases slightly increased from 57% in 1973 to 1982 to 67% in 1993 to 2004 and the proportion of unstaged cases decreased from 17% to 5%. The 5-year survival of localized disease increased from 44% in the first study decade to 68% in the period after 1993, whereas 5-year survival of metastatic disease increased from 16% to 39%. The corresponding 10-year survival increased from 39% to 63% for localized disease and from 16% to 32% for metastatic ES. The incidence of ES has not increased appreciably over the last 30 years. A marked decrease in the proportion of unstaged cases may be reflective of diagnostic improvement or changes in reporting. There is a clear improvement in survival for both localized and metastatic disease. Poorer outcome of metastatic patients still warrants intensification of therapy, which is currently being tested in several ongoing trials.
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            A System for the Surgical Staging of Musculoskeletal Sarcoma

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              Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model.

              Solitary fibrous tumor represents a spectrum of mesenchymal tumors, encompassing tumors previously termed hemangiopericytoma, which are classified as having intermediate biological potential (rarely metastasizing) in the 2002 World Health Organization classification scheme. Few series have reported on clinicopathological predictors with outcome data and formal statistical analysis in a large series of primary tumors as a single unified entity. Institutional pathology records were reviewed to identify primary solitary fibrous tumor cases, and histological sections and clinical records reviewed for canonical prognostic indicators, including patient age, tumor size, mitotic index, tumor cellularity, nuclear pleomorphism, and tumor necrosis. Patients (n=103) with resected primary solitary fibrous tumor were identified (excluding meningeal tumors). The most common sites of occurrence were abdomen and pleura; these tumors were larger than those occurring in the extremities, head and neck or trunk, but did not demonstrate significant outcome differences. Overall 5- and 10-year metastasis-free rates were 74 and 55%, respectively, while 5- and 10-year disease-specific survival rates were 89 and 73%. Patient age, tumor size, and mitotic index predicted both time to metastasis and disease-specific mortality, while necrosis predicted metastasis only. A risk stratification model based on age, size, and mitotic index clearly delineated patients at high risk for poor outcomes. While small tumors with low mitotic rates are highly unlikely to metastasize, large tumors ≥ 15 cm, which occur in patients ≥ 55 years, with mitotic figures ≥ 4/10 high-power fields require close follow-up and have a high risk of both metastasis and death.
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                Author and article information

                Journal
                J Craniovertebr Junction Spine
                J Craniovertebr Junction Spine
                JCVJS
                Journal of Craniovertebral Junction & Spine
                Wolters Kluwer - Medknow (India )
                0974-8237
                0976-9285
                Oct-Dec 2021
                11 December 2021
                : 12
                : 4
                : 336-360
                Affiliations
                [1]Department of Orthopedic Surgery, Icahn School of Medicine – The Mount Sinai Hospital, New York, USA
                [1 ]Department of Neurosurgery, Cantonal Hospital Lucerne, Lucerne, Switzerland
                [2 ]Department of Radiation Oncology, North Shore University Hospital, Manhasset, USA
                [3 ]Department of Pathology, North Shore University Hospital, Manhasset, USA
                [4 ]Department of Orthopedics, Balgrist University Hospital, Zurich, Switzerland
                [5 ]Department of Radiology, University Hospital Zurich, Zurich, Switzerland
                [6 ]Department of Neurosurgery, North Shore University Hospital, Manhasset, USA
                Author notes
                Address for correspondence: Dr. Alexander Spiessberger, Department of Neurosurgery, North Shore University Hospital, 300 Community Drive, Manhasset, NY 11030, USA. E-mail: alexander.s.spiessberger@ 123456gmail.com
                Article
                JCVJS-12-336
                10.4103/jcvjs.jcvjs_115_21
                8740815
                35068816
                dedf3ffd-4b70-4c9b-8507-6c08ab99eabd
                Copyright: © 2021 Journal of Craniovertebral Junction and Spine

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 16 August 2021
                : 06 November 2021
                Categories
                Review Article

                Neurology
                chordoma,primary spinal tumors,sarcoma
                Neurology
                chordoma, primary spinal tumors, sarcoma

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