Spinal Cord Tumors: Reportable or Not?

Suzanne Neve, RHIA, CTR - Director, Cancer Registry Inpatient Coding Leave a Comment

Spinal Cord Tumors: Reportable or Not?

Deciding whether a spinal cord tumor is reportable or not can be a daunting task for even experienced cancer registrars. The decision-making process used for correct case-finding must begin with an understanding of the anatomy, terms and definitions used, and how benign and malignant spinal cord tumors are classified.    

Spinal cord tumors are defined by their position relative to the meninges, or the thick membrane composed of three layers, which surround and protect the brain and spinal cord (Figure 1). Tumors can be either extradural, i.e., outside the dura mater, or intradural, meaning within or below the dura mater.

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Figure 1: Anatomy of the three layers of the meninges around the brain and spinal cord.

Intradural tumors are further classified as either intramedullary or extramedullary.

  • Intramedullary tumors begin in the cells within the spinal cord itself, and typically includes includes gliomas, astrocytomas, or ependymomas. (Figure 2c and 2d)
  • Extramedullary tumors arise in either the membrane surrounding the spinal cord or the nerve roots that reach out from the spinal cord. While extramedullary tumors do not arise within the spinal cord, they may affect spinal cord function by causing compression or other problems. Types of extramedullary tumors include meningiomas, neurofibromas, schwannomas and nerve sheath tumors. (Figure 2b)

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Figure 2: Schematic demonstration of normal (a), extradural (b), intradural-extramedullary (c) and intramedullary (d) tumors of the spinal cord. (Bhatt, et al).

The Benign Brain Tumors Cancer Registries Amendment Act, Public Law 107-260, refers to CNS tumors as “brain-related tumors” and is the standard definition adopted by all cancer registry standard-setters. The law describes these as a reportable primary tumors, whether malignant or benign that occur in any of the following sites:

  • Brain, meninges, spinal cord, cauda equina, a cranial nerve or nerves, or any other part of the central nervous system.
  • The pituitary gland, pineal gland, or craniopharyngeal duct.

 

Based on the information above and per SEER SINQ #20210031, spinal cord tumors are reportable when the tumor arises in the intradural space. Tumors tumors outside the dura or arising in the extradural space or peripheral nerves are not reportable.

Various parts of the brain function as the “control centers” for important body functions and the presenting symptoms in the clinical setting may be related to the site of origin. For example, the patient may experience symptoms on the right side of the body if the tumor is found on the left side of the brain, and vice versa. The left side of the brain is where the speech center or functions are found and a tumor in this area might mean that a patient has difficulty saying or recalling words even though they are fully capable of understanding what is being said. The frontal lobe controls intellectual functions such as thought process, behavior and memory. Other symptoms that may be found at the time of presentation include, but are not limited to:

  • Blurred or double vision,
  • Weakness in the arms or legs, stumbling, falling or lack of coordination,
  • Impaired memory,
  • Slurring of speech or difficulty speaking,
  • Altered or inability to smell or hear.

 

Benign, borderline, and malignant tumors of the brain and CNS have separate, and rather complex, sets of rules for casefinding, abstracting, histology and determination of multiple primaries.   Cancer Registrars abstracting for accredited programs also need to review the abstracting and coding requirements published by the Commission on Cancer (CoC). And, other elements may apply, such as the timing or interval between the diagnosis of two tumors, laterality, grade, or transformation from a benign to malignant morphology. Paying careful attention to the information in the medical record and frequent referencing of the abstracting manuals will ensure accurate abstracting of the case. State Cancer Registries may also have specific requirements for reporting benign or malignant tumors of the brain and CNS.

 

In summary, when deciding reportability of a spinal cord tumor, it is important to distinguish between intra- and extradural origin of the tumor. Benign and malignant intradural tumors of the brain and CNS are reportable. Careful attention to the clinical, radiographic and pathologic elements are also needed to correctly and accurately suspense and abstract the case.   Registrars should consult the abstracting and standards manuals to ensure accuracy and completeness.  

 

Table 1: Definitions of the terms used to describe spinal cord tumors

Term

Definition

arachnoid

a fine, delicate membrane, the middle one of three membranes or meninges that surround the brain and spinal cord. The arachnoid is situated between the dura mater and the pia mater

dura mater

the tough, outermost membrane enveloping the brain and spinal cord

extramedullary

situated or occurring outside the spinal cord or the medulla oblongata

intradural

within or beneath the dura mater

intramedullary

within a medulla of an organ or structure, e.g., the inner part of the spinal cord, the medulla oblongata

leptomeninges

the inner two meninges, the arachnoid and the pia mater, between which circulates the cerebrospinal fluid

meninges

the three membranes, the dura mater, arachnoid, and pia mater, which line the skull and vertebral canal and enclose the brain and spinal cord

pia mater

the delicate, innermost membrane enveloping the brain and spinal cord

 

References:

  • Figure 1: SEER Site-Specific Modules, Brain & Other Nervous System Tumors, Brain & CNS Tumors, Meninges. U.S. National Institutes of Health, National Cancer Institute, October 17, 2021. URL: https://training.seer.cancer.gov.
  • Figure 2: Bhatt G, Jain A, Bhatt A, Civelek AC. Intramedullary spinal cord metastases and whole body F-FDG PET-CT-A Case Report. Quant Imaging Med Surg 2019;9(3):530-534.
  • SEER SINQ, Question #20210031, date last changed 5/15/2021. URL: SEER Inquiry System - Question 20210031 Details (cancer.gov).

 

Resources for Cancer Registrars:  

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