Aggressive Myeloid Sarcoma Causing Recurrent Spinal Cord Compression.

Area of Science:

• Oncology
• Neurology
• Hematology

Background:

• Myeloid sarcoma is a rare extramedullary tumor of myeloid precursors, often linked to acute myelogenous leukemia (AML).
• Shwachman-Diamond syndrome is a rare inherited disorder with significant hematologic implications, including AML.
• Spinal cord compression by extramedullary tumors presents a critical neuro-oncologic emergency.

Observation:

• A 20-year-old male with Shwachman-Diamond syndrome and AML presented with acute myelopathy due to a thoracic dorsal epidural myeloid sarcoma.
• Initial management involved immediate surgical decompression due to rapid neurological decline.
• Tumor recurrence leading to re-compression occurred 13 days postoperatively.

Findings:

• Emergent radiation therapy following surgical decompression resulted in resolution of cord compression and local disease control.
• The rapid recurrence highlights the aggressive nature of myeloid sarcoma in this context.
• No established guidelines exist for the optimal timing of postoperative radiation in spinal myeloid sarcoma.

Implications:

• This case suggests that prompt initiation of radiation therapy, as soon as pathology is confirmed, is critical for patients with neurological compromise from spinal myeloid sarcoma.
• The findings challenge the conventional 14-day postoperative radiation delay typically observed in neuro-oncology to ensure wound healing.
• Further research is needed to establish evidence-based protocols for managing spinal cord compression caused by myeloid sarcoma.