Elsevier

World Neurosurgery

Volume 149, May 2021, Pages e316-e328
World Neurosurgery

Original Article
Incidence, Management, and Outcomes of Adult Patients with Spinal Chondrosarcoma in the United States

https://doi.org/10.1016/j.wneu.2021.02.030Get rights and content

Objective

Spinal chondrosarcomas are rare primary malignant neoplasms composed of cartilage-producing cells. They are slow-growing but locally aggressive lesions that have high rates of recurrence and progression after treatment. We provide the largest comprehensive analysis of prognostic factors, treatment modalities, and survival outcomes in patients with spinal chondrosarcoma using a large, prospectively collected national database.

Methods

Patients with diagnosis codes specific for chondrosarcoma of the spine, sacrum, and coccyx were queried from the National Cancer Database (NCDB) during 2004–2016. Outcomes were investigated using Cox univariate and multivariate regression analyses, and survival curves were generated for comparative visualization.

Results

A total of 1843 individuals were identified with a diagnosis of chondrosarcoma, 82.1% of which were at the sacrum or coccyx and 17.9% at the spine. The mean overall survival of patients in our cohort was 7.91 years. Increased age, larger tumor, dedifferentiated histology, and presence of metastases were associated with worsened overall survival. Regarding management, 77.7% of patients received surgical intervention and both partial and radical resection were associated with significantly improved overall survival (P < 0.001). Neither radiotherapy nor chemotherapy administration improved overall survival; however, among patients who received radiation, those who received higher-dose radiation had significantly improved overall survival compared with those who received lower-dose radiation.

Conclusions

Surgical resection significantly improves overall survival in patients with spinal chondrosarcoma. In those patients receiving radiation, those who receive high doses have improved overall survival compared with those who receive lower doses. Further studies into optimal radiation modality and doses are required.

Introduction

Chondrosarcomas are among the most common primary malignant bone tumors in adults,1,2 accounting for an estimated 26% of primary osseous spine tumors.3 Overall, spinal chondrosarcomas are rare (the estimated incidence is 1 in 200,000)3, which has resulted in a low density of reports over time. These neoplasms represent management challenges to multidisciplinary teams because of their aggressive, yet anatomically constrained surgical requirements and high resistance to radiation and chemotherapy.2, 3, 4, 5, 6, 7 These heterogeneous cartilaginous malignancies that often develop de novo within physiologic bone may also arise secondarily from malignant transformation of benign tumors.2,3,7

Chondrosarcomas are predominantly identified in men, with a reported male/female ratio between 1.5 and 4 to 1.3,8, 9, 10 The highest incidence is reportedly between ages 30 and 70 years, and the mean age at diagnosis is between 33 and 51 years.2,3,8,9 Chondrosarcomas are frequently located within the thoracolumbar spine,2,9,10 with a predilection for posterior elements and common extension into the vertebral body.2,3,8 Because of their slow-growing nature, most present with pain and a palpable mass, and about half of patients also show neurologic deficits such as radiculopathy, myelopathy, or cauda equina syndrome.2,8,9 In addition, these patients require long-term monitoring because of a high tendency for progression and recurrence, which may be seen up to 10 years or more after resection.1,2,4,5,7,8,11 En bloc resection is a standard therapy2,4,5,8,9; however, despite technological advancements, this approach remains challenging because of the critical need for complete excision with negative margins and for preserving vital adjacent neurologic, vascular, and visceral structures.1,2,4,5,7 Effective radiotherapy (RT) dosing is not tolerated well by the spinal cord, although radiation likely has adjuvant and palliative roles.5, 6, 7,9,11

Prognostic factors include histologic subtype, grade, tumor size (before and after resection), extent of disease, recurrence, metastasis, and patient age and race.2,3,6, 7, 8, 9 Treatment modality, resection extent including positive or negative margin status, and radiation timing and dose are also believed to affect outcome.1,2,4, 5, 6,8, 9, 10, 11 A lack of research of these rare tumors has provided an inadequate basis to understand the optimal treatment regimen. We aim to assess the effects of epidemiologic, histopathologic, and treatment characteristics on survival outcomes and to formulate management recommendations based on these data.

Section snippets

Study Cohort

Data for this investigation were derived from the National Cancer Database (NCDB), a prospectively collected cancer registry maintained jointly by the American College of Surgeons and the American Cancer Society. This database is sourced from >1500 cancer centers and represents >70% of newly diagnosed cancer cases and >34 million historical records. The data used in the study are derived from a de-identified NCDB file. The American College of Surgeons and the Commission on Cancer have not

Baseline Population Characteristics

Over the period between 2004 and 2016, the search criteria identified 1843 adults in the NCDB who had been diagnosed with spinal chondrosarcoma. Patients were most commonly aged 36–54 years (n = 650, 35.3%), followed by ≥65 years (n = 543, 29.5%). The study population was predominantly white (n = 1582, 85.8%), male (n = 1120, 60.8%), and had relatively few comorbidities, with a Charlson/Deyo score of 0 (n = 1541, 83.6%). Histologically, most tumors were classified as conventional

Baseline Population Characteristics

Most patients in the present study were between 36 and 54 years old and >65 years old, and this later age of diagnosis is consistent with that reported in the literature.12 The 2 peaks in incidence may partially be explained by the fact that the most common histologic subtype analyzed in the current study was conventional chondrosarcoma, which can be subclassified as conventional central chondrosarcomas, which usually present in patients older than 50 years, and conventional peripheral

Conclusions

Spinal chondrosarcoma is a rare primary malignant bone tumor that is locally aggressive, with high rates of recurrence and progression after treatment. The anatomic location of these lesions does not always permit complete radical resection without neurologic consequences. Surgical resection is considered the gold standard of treatment, with a goal of wide, en bloc resection; however, the balance for surgical cure with significant lifestyle modification requires extensive discussion when

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    Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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