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248 - Severe Functional Decline: A Rare Case of Central Nervous System Extraskeletal Myxoid Chondrosarcoma

Friday, February 28, 2025

5:00 PM - 6:30 PM MST

Presenting Author(s)

HV

Hallie E. Vanney, n/a

MD Candidate
University of Minnesota
Blaine, Minnesota, United States
AH

Allison Hemmesch, n/a

MD Candidate
University of Minnesota
New London, Minnesota, United States

Co-Author(s)

AG

Aaron Greenblat, DO

Resident Physician
University of Minnesota
Lakeville, Minnesota, United States
FJ

Florence John, MD, MPH

Faculty Physician
University of Minnesota
Eden Prairie, Minnesota, United States

Case Diagnosis: Central nervous system extraskeletal myxoid chondrosarcoma

Case Description: A 25 year old female with a history of central nervous system extraskeletal myxoid chondrosarcoma presented to clinic with functional decline resulting in dependence for mobility and activities of daily living. She was first diagnosed with an intracranial lesion at twelve years of age with initial recurrence occurring within 16 months of tumor resection. Subsequently, she developed multiple intracranial tumors requiring resection, radiation, and ventriculo-peritoneal shunt placement. Ten years after her diagnosis, she developed headaches and visual symptoms. Magnetic resonance imaging (MRI) revealed multiple brain tumors that were treated with a multimodal approach. She later developed metastatic lesions throughout her spine that resulted in a non-traumatic spinal cord injury causing paraplegia. Due to the extent of disease, no further cancer-directed treatment was recommended. Rehabilitation care included management of neurogenic bowel and bladder and a multidisciplinary approach involving palliative care, physical therapy, occupational therapy, social work and psychology.

Discussions: Extraskeletal myxoid chondrosarcoma (EMC) is an incredibly rare soft tissue sarcoma that arises most commonly in the proximal extremities of older adults. Primary intracranial EMC is even rarer with only 15 cases reported in literature. The standardized treatment is surgery with wide resection and adjuvant radiation. Studies have shown that EMC has a high recurrence rate at roughly 50%. Impairments are exacerbated with each recurrence and resulting treatment making rehabilitation services crucial for recovery and quality of life.

Conclusions:
The age, severity of functional decline and poor prognosis in our patient highlight the intersection of rehabilitation with palliative care, and the importance that rehabilitation measures play in patients with a limited life expectancy. Management of functional decline early in the disease process is critical to achieving patient goals for mobility, comfort and daily activities. Early intervention with a multidisciplinary rehabilitation approach is essential to optimize function and well-being.