Jacob R Joseph1, D Andrew Wilkinson1, Nathanael G Bailey2, Andrew P Lieberman2, Christina I Tsien3, Daniel A Orringer4. 1. Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan, USA. 2. Department of Pathology, University of Michigan, Ann Arbor, Michigan, USA. 3. Department of Radiation Oncology, University of Michigan, Ann Arbor, Michigan, USA. 4. Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan, USA. Electronic address: dorringe@med.umich.edu.
Abstract
OBJECTIVE: Myeloid sarcoma is a rare extramedullary solid tumor comprised of immature myeloid precursor cells, most commonly associated with acute myelogenous leukemia (AML). We present the case of a patient with a history of Shwachman-Diamond syndrome and AML who presented with myeloid sarcoma causing acute spinal cord compression. CASE DESCRIPTION: The patient was a 20-year-old man who presented with acute onset weakness and numbness in his lower extremities. Magnetic resonance imaging revealed a thoracic dorsal epidural mass. Despite the history of AML, we elected to forego image-guided biopsy and up-front radiation due to the rapidly progressive nature of his myelopathy. Immediate surgical decompression was performed, but the patient had recurrence of tumor leading to further compression 13 days postoperatively. Subsequently, emergent radiation was performed, leading to resolution of cord compression and local disease control. CONCLUSIONS: To our knowledge, there are no randomized controlled trials examining the appropriate timing for postoperative radiation. Because most typical neuro-oncologic cases have no need for immediate postoperative radiation, our practice has been to wait 14 days to initiate postoperative radiation to ensure wound healing. One unique feature of our case was the rapid recurrence of symptoms due to tumor progression. Given this observation, we believe that radiation therapy should be considered as soon as possible after confirmatory pathology diagnosis for patients presenting with neurological compromise due to myeloid sarcoma of the spine.
OBJECTIVE:Myeloid sarcoma is a rare extramedullary solid tumor comprised of immature myeloid precursor cells, most commonly associated with acute myelogenous leukemia (AML). We present the case of a patient with a history of Shwachman-Diamond syndrome and AML who presented with myeloid sarcoma causing acute spinal cord compression. CASE DESCRIPTION: The patient was a 20-year-old man who presented with acute onset weakness and numbness in his lower extremities. Magnetic resonance imaging revealed a thoracic dorsal epidural mass. Despite the history of AML, we elected to forego image-guided biopsy and up-front radiation due to the rapidly progressive nature of his myelopathy. Immediate surgical decompression was performed, but the patient had recurrence of tumor leading to further compression 13 days postoperatively. Subsequently, emergent radiation was performed, leading to resolution of cord compression and local disease control. CONCLUSIONS: To our knowledge, there are no randomized controlled trials examining the appropriate timing for postoperative radiation. Because most typical neuro-oncologic cases have no need for immediate postoperative radiation, our practice has been to wait 14 days to initiate postoperative radiation to ensure wound healing. One unique feature of our case was the rapid recurrence of symptoms due to tumor progression. Given this observation, we believe that radiation therapy should be considered as soon as possible after confirmatory pathology diagnosis for patients presenting with neurological compromise due to myeloid sarcoma of the spine.