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An Intramedullary Enigma

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.

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B. Radiation recall myelopathy

Given this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.

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Article Information

Corresponding Author: Danielle S. Bitterman, MD, Dana-Farber Cancer Institute, Department of Radiation Oncology, Brigham and Women’s Hospital, 75 Francis St, Boston, MA 02115 (danielle_bitterman@dfci.harvard.edu).

Published Online: March 31, 2022. doi:10.1001/jamaoncol.2022.0462

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Matthew Desalvo, MD, Brigham and Women's Hospital, for his assistance with radiology review in this case. He was not compensated.

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Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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