Abstract | November 16, 2023
A Rare Presentation of Metastatic Sclerosing Epithelioid Fibrosarcoma with Spinal Cord Compression
Learning Objectives
- Upon completion of this lecture, learners should be better prepared to diligently monitor the patient with sclerosing epithelioid fibrosarcoma and remain vigilant to prevent or promptly identify any potential cancer metastasis in the long term. Sclerosing epithelioid fibrosarcoma is an extremely uncommon soft tissue tumor that can arise in various locations throughout the body. Recurrence of sclerosing epithelioid fibrosarcoma is not infrequent, and metastasis to distant organs can occur, even following successful treatment, as illustrated in this case.
- Upon completion of this lecture, learners should be better prepared to maintain a high level of suspicion when patients with a history of cancer present with deteriorating back pain, particularly when accompanied by neurological abnormalities. Metastatic spinal cord compression is an oncological emergency that requires emergent management. It manifests as back pain and neurological abnormalities, including weakness, numbness, or cramping in extremities. When cord compression occurs at the level of cauda equina, it can also cause cauda equina syndrome.
- Upon completion of this lecture, learners should be better prepared to treat the patient with metastatic cord compression timely. Patients presenting with this metastatic spinal cord compression should promptly receive intravenous dexamethasone and undergo cord decompression treatment. Available treatment modalities include surgical decompression and radiation therapy, as exemplified in this case. The selection of a definite treatment should be personalized and take the account the degree of neurologic compromise, radiosensitivity of cancer, and systemic burden of cancer. Failure to prompt treatment results in dire permanent damage to neurological function.
Introduction:
Sclerosing epithelioid fibrosarcoma is an extremely rare malignant soft tissue tumor that predominantly affects adults and can manifest at any anatomical site. Previous studies have suggested that approximately 30% to 53% of patients experience local recurrence following treatment. The rate of distant metastasis can vary considerably, ranging from 20% to 86%. This case illustrates a rare case of metastatic cord compression of sclerosing epithelioid fibrosarcoma after definitive surgical treatment.
Case Presentation:
A 68-year-old male patient with a past medical history of skin cancer removal on the left flank in 2019, which was pathologically confirmed as sclerosing epithelioid fibrosarcoma, presented to the emergency department due to worsening back pain. He reported a weight loss of 25 pounds over the past several months, but denied experiencing fever, chills, nausea, or vomiting. Upon physical examination, bilateral lower extremities weakness (grade 2/5) was noted, while no significant tenderness or skin changes along the spine were observed. Vital signs were normal. Routine lab work was unremarkable.
The previous PET/CT and bone scan, which took place one year after the surgery, were reviewed and negative for any abnormal uptake. However, metastatic cord compression was strongly suspected considering the past medical history and symptoms. Thoracic MRI taken during this admission revealed multiple large enhancing masses with spinal cord compression at T6, indicative of metastatic bone lesions. Additionally, lumbar MRI showed multiple enhancing lesions, with the largest lesion observed at L2. CT scans of the chest, abdomen, and pelvis did not reveal any signs of metastasis. The patient underwent emergent palliative radiation therapy and intravenous dexamethasone to manage cord compression with the working diagnosis of metastatic cord compression. Surgical decompression was not considered due to extensive lesions on the spine.
Final Diagnosis:
A CT-guided biopsy of the T6 vertebral body was performed, confirming the presence of metastatic sclerosing epithelioid fibrosarcoma.
Management and follow-up:
The patient completed a 5-day course of palliative radiation therapy and steroid therapy, which resulted in improvement of symptoms. Hematology/oncology was consulted, and outpatient chemotherapy was planned. Subsequently, the patient was discharged to a rehabilitation facility for additional physical therapy.
References and Resources
- Yahyaoui, M., Benhammou, M., Aharram, S., Amghar, J., Sadougui, M., Agoumi, O., & Daoudi, A. (2020). Sclerosing epithelioid fibrosarcoma: rare and serious. The Pan African medical journal, 36, 131. https://doi.org/10.11604/pamj.2020.36.131.18668
- Murshed, K. A., Al-Bozom, I., & Ammar, A. (2021). Sclerosing epithelioid fibrosarcoma: In-depth review of a genetically heterogeneous tumor. APMIS, 129(8), 455-460. https://doi.org/10.1111/apm.13157
- Sodji, Q., Kaminski, J., Willey, C., Kim, N., Mourad, W., Vender, J., & Dasher, B. (2017). Management of Metastatic Spinal Cord Compression. Southern medical journal, 110(9), 586–593. https://doi.org/10.14423/SMJ.0000000000000700
