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Low back pain and lumbar radiculopathy as harbingers of acute myeloid leukemia recurrence in a patient with myeloid sarcoma
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Case Reports / Journal of Clinical Neuroscience 19 (2012) 1040–1041
Low back pain and lumbar radiculopathy as harbingers of acute myeloid leukemia recurrence in a patient with myeloid sarcoma Timothy R. Smith ⇑, Nicholas Slimack, Jamal McClendon Jr., Albert Wong, Richard G. Fessler Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, 676 N. St Clair Street, Suite 2210, Chicago, IL 60611, USA
a r t i c l e
i n f o
Article history: Received 31 October 2011 Accepted 5 November 2011
Keywords: AML Chloroma Granulocytic sarcoma Low back pain Myeloid sarcoma Radiculopathy Spinal compression
a b s t r a c t Myeloid sarcoma (MS) is an extra-osseous, solid collection of myeloblasts. It is associated with myeloid leukemias, and rarely affects the spine. The most common clinical presentation of MS in spine patients is some form of pain related to compression of neural elements. Given that MS is rare, and its imaging characteristics are similar to other more common diagnoses, it is frequently missed on initial presentation. We present a 28-year-old female, in her fifth year of remission from AML, with low back pain and right lumbar radiculopathy. Initially, the leading diagnosis was schwannoma in preference to neurofibroma; however, intra-operative pathology and subsequent bone marrow biopsy revealed the tumor to be MS. This report highlights the difficulties of diagnosis of MS in patients in remission from acute myeloid leukemia. Thus, in patients with a history of leukemia, MS should be considered in the differential diagnosis of any epidural or nerve root tumor. Timely diagnosis and treatment are key to optimal outcomes. Published by Elsevier Ltd.
1. Case report A 28-year-old woman in remission for five years from acute myeloid leukemia (AML) was admitted to hospital in August 2010 for an elective lumbar nerve root tumor exploration, biopsy, and possible resection. Prior to admission, she reported three months of progressive localized low back pain, shooting pain down the back and medial aspect of her right leg, and parasthesias in the right leg. In follow-up for this pain, a contrasted MRI of the lumbar spine was obtained, which revealed an enhancing mass infiltrating the right fourth lumbar nerve root (Fig. 1). The patient was taken to the operating theater for a minimally invasive right L4 to L5 far lateral approach for exploration, biopsy, and possible resection of the tumor. Frozen sections suggested that this was not a neurofibroma or a schwannoma. The decision was made, therefore, not to attempt a gross total resection. The final pathology ultimately revealed that this was indeed myeloid sarcoma (MS) (Figs. 2 and 3). This finding was confirmed by an iliac crest bone marrow biopsy (acute myelogenous leukemia in a hypercellular bone marrow).
2. Discussion MS is an extra-osseous, solid collection of myeloblasts (that is, an extramedullary manifestation of AML). It is associated with myeloid leukemias (AML, acute promyelocytic leukemia, and acute myelogenous leukemia [ACL]), myeloproliferative and myelodysplastic disorders, but it can also occur in isolation. King referred to this tumor as a ‘‘chloroma’’ because of its greenish appearance (from the Greek, chloros, meaning green), which is caused by the high levels of myeloperoxidase granules in the malignant myeloid cells.1 Now, in the absence of myeloperoxidase, these tumors are referred to as myeloid sarcomas, myeloblastomas, or granulocytic sarcomas.2 The prevalence of granulocytic sarcoma of the spine is approximately 1.0% of all patients with AML/chronic myelogenous leukemia.3 MS in the spine often presents clinically as radiculopathy4 or as a multiple nerve root entrapment with or without motor deficits.5 ⇑ Corresponding author. Tel.: +1 312 695 0087; fax: +1 312 695 0225. E-mail address: [email protected] (T.R. Smith).
The differential is broad for MS, and in the absence of circulating myeloblasts, MS is rarely considered initially in patients with isolated spinal involvement.6 Typically, the actual diagnosis is made only after surgical biopsy or mounting evidence of acute leukemia.6 In a seminal review of 154 patients with primary extramedullary leukemia, almost half (47%) were initially misdiagnosed.7 In this series, there were numerous incorrect diagnoses including rhabdomyosarcoma, schwannoma, neurofibroma, Ewings sarcoma, cytosarcoma phyllode, infiltrating ductal carcinoma, undifferentiated carcinoma, and carcinoid tumor, but lymphoma was the most common misdiagnosis. Imaging characteristics can be helpful in discriminating among the differential diagnoses for MS in the spine, which includes lymphoma, metastasis, extramedullary hematopoiesis, neurofibroma, and an extramedullary neurogenic tumor (i.e. schwannoma).8 In association with leukemia, imaging findings of multiple or contiguous multilevel extramedullary spinal masses with diffuse, abnormal bone marrow signal intensity is suggestive of MS.8 MS should be considered when both a solitary dumbbell-shaped mass in the intervertebral foramen and diffuse bone marrow infiltration are seen on MRI. MS can be confused easily with neurogenic tumors such as schwannomas. Without evidence of bone marrow infiltration, MR signal intensities can be used to help distinguish schwannomas from granulocytic sarcomas. MS tends to have intermediate signal intensity (slightly less than high intensity) on T2-weighted MRI, and iso-intensity on T1-weighted MRI (Fig. 1).9 The present report of a patient with MS presenting with low back pain and lumbar radiculopathy illustrates the difficulties of diagnosis of MS in patients in AML remission. Thus, MS should be considered in the differential diagnosis of any epidural or nerve root tumor (with or without compression) in all patients with a history of leukemia, but especially in those with AML.
Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2011.11.012.
References 1. King A. A case of chloroma. Mon J Med 1853;17:97.
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1040–1041
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Fig. 1. MRI of the lumbar spine showing a compressive mass surrounding the right fourth lumbar nerve root: (A) axial T1-weighted MRI; (B) sagittal gadolinium enhanced T1-weighted MRI; (C) axial T2-weighted MRI; and (D) sagittal T2-weighted MRI.
Fig. 2. Intra-operative fluoroscopy showing: (A) minimally invasive working channel docked on the right facet of L4–L5; and (B) expanded minimally invasive retractors positioned over the right facet of L4–L5.
doi:10.1016/j.jocn.2011.11.012
2. Rappaport H. Tumors of the hematopoietic system. Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1967. p. 241–7. 3. Byrd JC, Weiss RB, Arthur DC, et al. Extramedullary leukemia adversely affects hematologic complete remission rate and overall survival in patients with t(8;21)(q22;q22): results from Cancer and Leukemia Group B 8461. J Clin Oncol 1997;15:466–75. 4. Kim FS, Rutka JT, Bernstein M, et al. Intradural granulocytic sarcoma presenting as a lumbar radiculopathy. Case report. J Neurosurg 1990;72:663–7. 5. Verra WC, Snijders TJ, Seute T, et al. Myeloid sarcoma presenting as a recurrent, multifocal nerve root entrapment syndrome. J Neurooncol 2009;91:59–62. 6. Kook H, Hwang TJ, Kang HK, et al. Spinal intramedullary granulocytic sarcoma: magnetic resonance imaging. Magn Reson Imaging 1993;11:135–7. 7. Byrd JC, Edenfield WJ, Shields DJ, et al. Extramedullary myeloid cell tumors in acute nonlymphocytic leukemia: a clinical review. J Clin Oncol 1995;13:1800–16. 8. Seok JH, Park J, Kim SK, et al. Granulocytic sarcoma of the spine: MRI and clinical review. AJR Am J Roentgenol 2010;194:485–9. 9. Fritz J, Vogel W, Bares R, et al. Radiologic spectrum of extramedullary relapse of myelogenous leukemia in adults. AJR Am J Roentgenol 2007;189:209–18.
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1040–1041
Low back pain and lumbar radiculopathy as harbingers of acute myeloid leukemia recurrence in a patient with myeloid sarcoma Timothy R. Smith ⇑, Nicholas Slimack, Jamal McClendon Jr., Albert Wong, Richard G. Fessler Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, 676 N. St Clair Street, Suite 2210, Chicago, IL 60611, USA
a r t i c l e
i n f o
Article history: Received 31 October 2011 Accepted 5 November 2011
Keywords: AML Chloroma Granulocytic sarcoma Low back pain Myeloid sarcoma Radiculopathy Spinal compression
a b s t r a c t Myeloid sarcoma (MS) is an extra-osseous, solid collection of myeloblasts. It is associated with myeloid leukemias, and rarely affects the spine. The most common clinical presentation of MS in spine patients is some form of pain related to compression of neural elements. Given that MS is rare, and its imaging characteristics are similar to other more common diagnoses, it is frequently missed on initial presentation. We present a 28-year-old female, in her fifth year of remission from AML, with low back pain and right lumbar radiculopathy. Initially, the leading diagnosis was schwannoma in preference to neurofibroma; however, intra-operative pathology and subsequent bone marrow biopsy revealed the tumor to be MS. This report highlights the difficulties of diagnosis of MS in patients in remission from acute myeloid leukemia. Thus, in patients with a history of leukemia, MS should be considered in the differential diagnosis of any epidural or nerve root tumor. Timely diagnosis and treatment are key to optimal outcomes. Published by Elsevier Ltd.
1. Case report A 28-year-old woman in remission for five years from acute myeloid leukemia (AML) was admitted to hospital in August 2010 for an elective lumbar nerve root tumor exploration, biopsy, and possible resection. Prior to admission, she reported three months of progressive localized low back pain, shooting pain down the back and medial aspect of her right leg, and parasthesias in the right leg. In follow-up for this pain, a contrasted MRI of the lumbar spine was obtained, which revealed an enhancing mass infiltrating the right fourth lumbar nerve root (Fig. 1). The patient was taken to the operating theater for a minimally invasive right L4 to L5 far lateral approach for exploration, biopsy, and possible resection of the tumor. Frozen sections suggested that this was not a neurofibroma or a schwannoma. The decision was made, therefore, not to attempt a gross total resection. The final pathology ultimately revealed that this was indeed myeloid sarcoma (MS) (Figs. 2 and 3). This finding was confirmed by an iliac crest bone marrow biopsy (acute myelogenous leukemia in a hypercellular bone marrow).
2. Discussion MS is an extra-osseous, solid collection of myeloblasts (that is, an extramedullary manifestation of AML). It is associated with myeloid leukemias (AML, acute promyelocytic leukemia, and acute myelogenous leukemia [ACL]), myeloproliferative and myelodysplastic disorders, but it can also occur in isolation. King referred to this tumor as a ‘‘chloroma’’ because of its greenish appearance (from the Greek, chloros, meaning green), which is caused by the high levels of myeloperoxidase granules in the malignant myeloid cells.1 Now, in the absence of myeloperoxidase, these tumors are referred to as myeloid sarcomas, myeloblastomas, or granulocytic sarcomas.2 The prevalence of granulocytic sarcoma of the spine is approximately 1.0% of all patients with AML/chronic myelogenous leukemia.3 MS in the spine often presents clinically as radiculopathy4 or as a multiple nerve root entrapment with or without motor deficits.5 ⇑ Corresponding author. Tel.: +1 312 695 0087; fax: +1 312 695 0225. E-mail address: [email protected] (T.R. Smith).
The differential is broad for MS, and in the absence of circulating myeloblasts, MS is rarely considered initially in patients with isolated spinal involvement.6 Typically, the actual diagnosis is made only after surgical biopsy or mounting evidence of acute leukemia.6 In a seminal review of 154 patients with primary extramedullary leukemia, almost half (47%) were initially misdiagnosed.7 In this series, there were numerous incorrect diagnoses including rhabdomyosarcoma, schwannoma, neurofibroma, Ewings sarcoma, cytosarcoma phyllode, infiltrating ductal carcinoma, undifferentiated carcinoma, and carcinoid tumor, but lymphoma was the most common misdiagnosis. Imaging characteristics can be helpful in discriminating among the differential diagnoses for MS in the spine, which includes lymphoma, metastasis, extramedullary hematopoiesis, neurofibroma, and an extramedullary neurogenic tumor (i.e. schwannoma).8 In association with leukemia, imaging findings of multiple or contiguous multilevel extramedullary spinal masses with diffuse, abnormal bone marrow signal intensity is suggestive of MS.8 MS should be considered when both a solitary dumbbell-shaped mass in the intervertebral foramen and diffuse bone marrow infiltration are seen on MRI. MS can be confused easily with neurogenic tumors such as schwannomas. Without evidence of bone marrow infiltration, MR signal intensities can be used to help distinguish schwannomas from granulocytic sarcomas. MS tends to have intermediate signal intensity (slightly less than high intensity) on T2-weighted MRI, and iso-intensity on T1-weighted MRI (Fig. 1).9 The present report of a patient with MS presenting with low back pain and lumbar radiculopathy illustrates the difficulties of diagnosis of MS in patients in AML remission. Thus, MS should be considered in the differential diagnosis of any epidural or nerve root tumor (with or without compression) in all patients with a history of leukemia, but especially in those with AML.
Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2011.11.012.
References 1. King A. A case of chloroma. Mon J Med 1853;17:97.
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1040–1041
1041
Fig. 1. MRI of the lumbar spine showing a compressive mass surrounding the right fourth lumbar nerve root: (A) axial T1-weighted MRI; (B) sagittal gadolinium enhanced T1-weighted MRI; (C) axial T2-weighted MRI; and (D) sagittal T2-weighted MRI.
Fig. 2. Intra-operative fluoroscopy showing: (A) minimally invasive working channel docked on the right facet of L4–L5; and (B) expanded minimally invasive retractors positioned over the right facet of L4–L5.
doi:10.1016/j.jocn.2011.11.012
2. Rappaport H. Tumors of the hematopoietic system. Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1967. p. 241–7. 3. Byrd JC, Weiss RB, Arthur DC, et al. Extramedullary leukemia adversely affects hematologic complete remission rate and overall survival in patients with t(8;21)(q22;q22): results from Cancer and Leukemia Group B 8461. J Clin Oncol 1997;15:466–75. 4. Kim FS, Rutka JT, Bernstein M, et al. Intradural granulocytic sarcoma presenting as a lumbar radiculopathy. Case report. J Neurosurg 1990;72:663–7. 5. Verra WC, Snijders TJ, Seute T, et al. Myeloid sarcoma presenting as a recurrent, multifocal nerve root entrapment syndrome. J Neurooncol 2009;91:59–62. 6. Kook H, Hwang TJ, Kang HK, et al. Spinal intramedullary granulocytic sarcoma: magnetic resonance imaging. Magn Reson Imaging 1993;11:135–7. 7. Byrd JC, Edenfield WJ, Shields DJ, et al. Extramedullary myeloid cell tumors in acute nonlymphocytic leukemia: a clinical review. J Clin Oncol 1995;13:1800–16. 8. Seok JH, Park J, Kim SK, et al. Granulocytic sarcoma of the spine: MRI and clinical review. AJR Am J Roentgenol 2010;194:485–9. 9. Fritz J, Vogel W, Bares R, et al. Radiologic spectrum of extramedullary relapse of myelogenous leukemia in adults. AJR Am J Roentgenol 2007;189:209–18.