Extremely Rare Spine Disease Frequency Revealed New Study | Orthopedics This Week

Extremely Rare Spine Disease Frequency Revealed New Study

Source: Wikimedia Commons and Petty Officer 3rd Class Madailein Abbott, U.S. Navy

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.

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.


1 thought on “Extremely Rare Spine Disease Frequency Revealed New Study

  1. In 1964 while at AFIP Dr Kenneth Earle and I wrote a paper, published two years later in the Journal of Neurosurgery, in which we attempted to define the natural history of ependymal tumors and classify them, following the Kernohan and Sayers scheme , according to their degree of malignancy. Our finding regarding those occurring in the spinal canal were in accord with that reported in this paper. Predominantly in the cauda equina were the myxopapillary ependymomas with the more solid variety found in the cord itself. One disturbing factor in our findings, was that at that point in history, “ependymoma” served as a reservoir for a hodgepodge of otherwise unclassified glial tumors. We hoped that confining the diagnosis “ependymoma” to those tumors which fit our rigid criteria, we could end this misuse of the term.

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