One of the rarest diseases of the spine, primary malignant non-osseous spinal tumors, has finally been quantified in a new study which highlights the importance of both registries and data analysis in medicine.
Extremely Rare Spine Disease Frequency Revealed New Study
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The study, “Epidemiology of Primary Malignant Non-Osseous Spinal Tumors in the United States,” was published online on March 4, 2022, in The Spine Journal.
“Primary malignant non-osseous spinal tumors are relatively rare and this has led to a paucity of studies specifically examining the epidemiology of malignant spinal tumors,” the study authors wrote.
Because of this, they sought to provide updated and more comprehensive data on the epidemiology and relative survival of these tumors.
The researchers analyzed retrospective data from the Central Brain Tumor Registry of the United States. All the primary malignant non-osseous spinal tumor cases had been diagnosed between 2000 and 2017. Primary outcome measures were incidence rates, relative survival rates, and hazard ratios.
Incidence rates were only determined for those cases that were histologically-confirmed. Relative survival estimates were calculated between 2001 and 2016 for death from any cause.
There were approximately 587 new cases of malignant non-osseous spinal tumors every year in the U.S. during this study period. The overall incidence rate was 0.178 per 100,000 persons.
Ependymomas were the most commonly diagnosed tumor in all age groups. The 10-year relative survival rates were 94.1%, 62.1% and 13.3% for ependymomas, lymphomas, diffuse astrocytomas, and high grade astrocytomas, respectively.
According to their analysis, females have a significantly lower risk of death compared with males for ependymomas (HR: 0.74, p <.001) and diffuse astrocytomas (HR: 0.70, p = .005). In addition, Black Americans have a significantly higher risk of death compared with whites for ependymomas (HR: 1.52, p = .009) or lymphomas (HR: 1.55, p = .009).
“Primary malignant non-osseous spinal tumors are primarily diagnosed in adulthood or late adulthood. Ependymal tumors are the most commonly diagnosed primary malignant non-osseous spinal tumors and have the highest 10-year relative survival rates. High grade astrocytomas are rare and portend the worst prognosis,” the researchers wrote.
The study authors include Arbaz A. Momin, M.S., Precious Ovem, B.S., Pranay Soni, M.D., Tamia O. Potter, B.S., Pablo F. Recinos, M.D., Varun R. Kshettry, M.D., and Michael P. Steinmetz, M.D., of Case Western Reserve University in Cleveland.
Nirav Patil, M.B.B.S., M.P.H, Gino Cioffi, Kristin Waite, Ph.D., Quinn Ostrom, Ph.D., Caro Kruchko, B.A., and Jill S. Barnholtz-Sloan, Ph.D. all of the Central Brain Tumor Registry of the United States, also contributed to the study.
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This is a fascinating development. In my practice we've seen similar outcomes with the revised protocol. The key differentiator seems to be patient selection criteria. Has anyone else noticed the correlation with BMI thresholds?
Great point. I'd push back slightly on the conclusion, the sample size in the cited study is too small to draw population-level inferences. That said, the directional signal is compelling and worth a larger RCT.
We implemented a similar approach last year. Early results are promising but we're still gathering 12-month follow-up data. Happy to share our protocol if anyone is interested.
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