Epidural Chloroma and Spinal Cord Compression
Chloroma, also known as granulocytic sarcoma, is a rare solid tumor that arises from malignant myeloid blast cells outside the hematopoietic system. This tumor can occur in various locations, including the head and neck, skeletal system, breast, uterus, and central nervous system. It is particularly common in patients with leukemia. When chloroma presents as an epidural mass, it can lead to spinal cord compression, resulting in significant neurological symptoms. Spinal lesions associated with chloroma most frequently occur at the thoracic level, with primary symptoms often including low back pain and radiating pain in the legs.
A 68-year-old male patient from China, who had no other specific diseases, visited the Hematology Clinic of Seoul St. Mary’s Hospital for evaluation of chronic myeloid leukemia (CML). He was diagnosed with CML based on a bone marrow pathology report, which showed findings consistent with chronic myelogenous leukemia, BCR-ABL1 positive, chronic phase. The bone marrow cytogenetics report revealed a karyotype of 46 X,Y, t(9;22)(q34;q11.2). Following the diagnosis, the patient was treated with Nilotinib, starting at 300 mg twice daily from July 22 to August 13, 2015, and then adjusted to 400 mg once daily from August 28, 2015. A thoracolumbar magnetic resonance imaging (MRI) scan showed focal bone marrow-replacing lesions at T11 and the sacrum, which were suspected to be related to CML.
Despite ongoing chemotherapy, the patient began to experience low back pain and radiating pain in both legs. He also reported numbness in both feet and was referred to the pain clinic for management. A thoracolumbar computed tomography (CT) scan revealed mild discogenic endplate degeneration at T7–8, a small Schmorl node in the superior endplate of T12, and diffuse bulging discs with ligamentum flavum thickening from L1 to S1. The patient’s pain intensity was rated as 9/10 on a numeric rating scale. To manage the pain, a caudal epidural block was performed using 0.2% lidocaine, hyaluronic acid, dexamethasone, and a sacroiliac joint steroid injection with 0.4% lidocaine and triamcinolone. Gabapentin was also prescribed for pain control.
Two months later, the patient’s pain intensity had decreased from 9/10 to 3/10. However, he still experienced low back pain and radiating pain in both legs. Despite this, he was able to walk without a stick, and his pain had significantly improved compared to his initial visit. Six months after the first visit, the patient returned to the pain clinic. He had been pain-free for several months and had discontinued treatment. However, after working as a construction worker for one month, he began to experience low back pain again, along with radiating pain and numbness in both legs. He also complained of weakness in both legs, and his pain intensity was rated as 7/10. A caudal epidural block with 0.2% lidocaine, hyaluronic acid, dexamethasone, and transforaminal steroid injections at the L4–5 and L5–S1 levels were performed. Gabapentin was again prescribed for pain control.
Four days after the transforaminal steroid injection, the patient returned to the pain clinic with severe weakness in both legs, dysuria, and dyschezia. His pain intensity was rated as 4/10. A complete blood cell count, blood coagulation test, and blood chemistry were performed, all of which were normal. Physical examination revealed paresthesia and hypoesthesia below T12, motor weakness in both lower extremities, and the absence of voluntary anal contraction. A Foley catheter was inserted due to the patient’s inability to urinate independently. Emergency thoracolumbar MRI was performed, showing T2 hyperintensity with impeded water diffusivity in the epidural and paraspinal spaces of T11–T12, resulting in cord compression of T10–T12. These findings were suggestive of chloroma. Hemangiomas were also detected at T12 and L4.
Given the rapid progression to paraplegia and the MRI findings, emergency surgery for decompression was performed. The frozen section removed during surgery confirmed the presence of chloroma. The patient underwent urgent total laminectomy and extradural spinal cord tumor removal for decompression. Pathological findings revealed atypical small, round cell infiltration. Steroid therapy was continued, and the patient’s neurological dysfunction improved slightly. Additional palliative radiotherapy was performed at T9–T12, but the neurological dysfunction remained. The patient refused bone marrow transplantation and was discharged and transferred to a local hospital for conservative care 35 days after the operation.
In this case, urgent decompressive laminectomy and tumor removal were performed immediately, followed by radiotherapy. However, the paraplegia was not resolved. This case highlights the importance of suspecting spinal cord compression due to epidural chloroma in patients with known acute or chronic myeloid leukemia who present with progressive weakness and paresthesia. Chloroma is a localized proliferation of myelogenous leukemia cells and is often an initial manifestation of myelocytic leukemia. In this patient, the chloroma was located in the thoracic epidural area rather than the central nervous system, which is atypical. The patient did not exhibit triplegia but rather paraplegia, and the condition occurred in an older age group rather than in young adolescents.
The patient in this case was 68 years old and had been diagnosed with chronic myeloid leukemia. Initially, there was no evidence of chloroma in the spine on MRI, and the condition was thought to be cured. The patient was treated for a simple disc problem with a lumbosacral lesion. However, the condition recurred within six months, and the spine was invaded by chloroma. This suggests that earlier recognition and intervention could have had a better impact on the prognosis. This disease is uncommon in this age group, and the patient showed no signs of transformation into the acute stage. Paraplegia following the transforaminal injection necessitated MRI to diagnose common complications of nerve block; however, it revealed chloroma compressing the spinal cord. Surgery and radiation therapy had no effect, and the paraplegia was not resolved.
This case is similar to another reported case of occult epidural chloroma presenting with acute paraplegia after lumbar puncture. In that case, myelography was recommended for diagnosis rather than MRI. As seen in this case, lifestyle adjustment advice and patient education about their disease are crucial. If the patient had recognized the significance of fecal incontinence, which is an important clue for disease differentiation, he might have sought immediate medical attention and undergone MRI earlier. This case demonstrates how delays in treatment and misdiagnosis can occur due to the patient’s understanding and education about the disease.
The ideal treatment for chloroma remains unclear. Patients with acute or chronic myeloid leukemia who exhibit progressive neurological dysfunction should be suspected of having chloroma. Additionally, this tumor can occur without hematologic disease or hematologic involvement. Therefore, MRI may be a good diagnostic approach considering all possible cases.
doi.org/10.1097/CM9.0000000000000081
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