Elsevier

World Neurosurgery

Volume 84, Issue 3, September 2015, Pages 866.e7-866.e10
World Neurosurgery

Case Report
Aggressive Myeloid Sarcoma Causing Recurrent Spinal Cord Compression

https://doi.org/10.1016/j.wneu.2015.04.017Get rights and content

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.

Introduction

Myeloid sarcoma, previously known as granulocytic sarcoma or chloroma (9), is a rare extramedullary solid tumor comprised of immature myeloid precursor cells. Tumor locations are widely variable, but have been known to involve the spinal canal. They are most commonly associated with a previous diagnosis of acute myelogenous leukemia (AML), and have an incidence of 2.9%–3.1% in these patients 11, 14. They can also precede the diagnosis of AML (16). Myeloid sarcoma has also been associated with other myelodysplastic and myeloproliferative disorders, but may occur in isolation (2). Presence of myeloid sarcoma is generally regarded as a poor prognostic indicator from a hematologic standpoint (18), although nonleukemic myeloid sarcoma may have increased survival in comparison with AML (20). We present a case of recurrent, aggressive myeloid sarcoma presenting as acute spinal cord compression in a patient with a history of Shwachman-Diamond syndrome and AML.

Section snippets

History and Physical Examination

A 20-year-old man with a history of Shwachman-Diamond syndrome and refractory AML presented with acute onset weakness and numbness in his lower extremities. Previous treatment history included chemotherapy and bone marrow transplant. Approximately 1 week before presentation, he developed midthoracic pain that migrated to his left scapular region and was initially resolved with nonsteroidal anti-inflammatory agents. On the day before presentation, the patient noticed onset of right lower

Discussion

Myeloid sarcoma is relatively uncommon, occurring in 3% of patients with AML 11, 14. Those involving the spinal column or spinal cord are rare, and the optimal treatment for cases presenting with neurological complications has not been established. A recent case series of the literature identified 55 cases with myeloid sarcoma and spinal complications, with most receiving a combination of surgery, chemotherapy, or radiotherapy (12). However, the timing of these therapies in the acute setting of

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  • Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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