Introduction
Myeloid sarcoma (MS), previously called granulocytic sarcoma or chloroma, is the name of an extramedullary accumulation of malignant blasts of myeloid lineage that form a solid tumor in non-hematopoietic tissues, altering their normal architecture. In alignment with this definition, MS does not include leukemic infiltration of the liver, spleen, lymph nodes, pleural and pericardial fluid, cerebrospinal fluid (CSF), and non-solid mass tissue infiltration commonly seen in skin, gums, retina, and lungs. Most cases of MS present in the setting of underlying intramedullary acute myeloid leukemia (AML); however, it can also present de novo in the absence of intramedullary disease, as a manifestation of newly diagnosed or relapsed AML, or as progression of myelodysplastic syndromes (MDS) or myeloproliferative neoplasms (MPN) [ 1 - 3 ]. As of now, the Fifth Edition of the WHO classification of hematolymphoid tumors and the International Consensus Classification (ICC) of Myeloid Neoplasms and Acute Leukemias proposes MS as a unique clinical presentation within the category of AML, and although listed separately, all cases of MS should be approached similar to AML and investigated to classify them into a more specific AML subtype [ 4 , 5 ].
Circulating malignant myeloid blasts can virtually disseminate to any extramedullary site, and there have been reports of MS in many different anatomic locations. The most frequently reported anatomic sites affected by MS are connective and soft tissues, breast, and the digestive system; these locations account for approximately 50% of all MS cases [ 1 ]. The clinical presentations of MS are diverse and mainly depend on its anatomic location, where it can cause mass effect-related manifestations or even lead to organ dysfunction [ 6 ]. Spinal intra- and extramedullary disease is an infrequent presentation of MS, with less than 100 reported cases in the literature. These cases usually present with signs and symptoms of spinal cord compression, representing a medical emergency as it can lead to permanent neurologic deficits, including paralysis/paresis, sensory losses, and bladder and bowel dysfunction. There has not been consensus regarding adequate management of spinal cord compression caused by MS, given its rarity and lack of prospective randomized control trials (RCTs), and the scarce available evidence comes from relatively few published case reports. Most of the latter favor a combination of systemic chemotherapy (CTX), radiation therapy (RT), corticosteroids (CS), and decompressive surgery. Hereby, we present the case of a young adult who presented with signs and symptoms of spinal cord compression approximately one year after completing induction CTX for AML, along with a comprehensive literature review of available data regarding previously reported cases of spinal cord compression caused by MS.
Case presentation
An 18-year-old male presented to a community hospital in September 2023 with left-sided neck and mandible swelling associated with night sweats. Initial workup was remarkable for a complete blood count of peripheral blood with a WBC of 37.0 K/mm 3 with 11% of monocytes and 41% of undifferentiated blasts; a CT scan reported bilateral submandibular, anterior cervical, axillary, and inguinal lymphadenopathies. A cervical lymph node core biopsy reported the presence of atypical proliferation of medium-to-large cells with round-to-irregular nuclei and fine chromatin, with CD13+, CD33+, and lysozyme+ on flow cytometry and immunohistochemistry, consistent with lymph node involvement by myeloid blasts. He was subsequently referred to our institution. At presentation, he had significant leukocytosis of 40.0 K/mm 3 , and flow cytometry of peripheral blood reported a prominent (55%) blast population characterized by the expression of CD34, CD117 (partial), CD13, CD33, CD123, HLA-DR, CD38, TdT, CD7, and minimal expression of myeloperoxidase (MPO), with negativity for B-cell and other T-cell markers, immunophenotypically consistent with AML. Chromosome analysis reported a complex profile 46,XY,del(1)(p36.1),del(9) (q13q22),-10,inv(11)(q21q23),-12,-16,+mar1-3 in 18 out of 20 analyzed cells. Fluorescence in situ hybridization (FISH) analysis on the peripheral blood reported loss of 16q22.1 (CBFB), loss of 12p13.2 (ETV6), and gain of 19p13.3 (TCF3). Next-generation sequencing (NGS) reported MLLT10, PHF6, and PTPNH mutations. Given the high percentage of myeloid blasts in the peripheral blood, a bone marrow (BM) examination was deferred, and the patient received induction CTX with 7+3 regimen (seven days of cytarabine and three days of idarubicin). A BM examination on day 15 of induction revealed a hypocellular BM (<10%) with mildly increased blasts (approximately 10%). Given these findings and his poor-risk cytogenetics, the patient was recommended to receive high-dose cytarabine (HiDAC) for re-induction; however, he declined and opted to be discharged once he would not be transfusion-dependent. A subsequent BM examination performed as outpatient on day 30 after induction CTX reported maturating trilineage hematopoiesis with left-shifted myelopoiesis and no increase in blasts, consistent with complete morphologic remission; flow cytometry also reported a heterogeneous cell population and no increase in blasts; cytogenetics reported a karyotype (46, XY). The patient received one cycle of consolidation CTX with HiDAC and was referred for stem cell transplantation (SCT) consideration. However, the patient decided not to pursue a transplant due to concerns about potential side effects. Subsequently, he did not return to the clinic and was lost to follow-up.
In September 2024, the patient presented to a community hospital with a four-month history of slowly progressive right upper extremity paresthesia associated with posterior neck and mid-low back pain that eventually advanced to severe sensory deficits and paresis of both lower extremities and limited ambulation, along with urinary retention and constipation. He also had a 35-lb unintentional weight loss over three to four months. A whole spine MRI reported multilevel nerve root thickening and enhancement from C1 to T3 and L5 to S3, as well as multiple enhancing intradural extramedullary masses (Figure 1 ). The cervical lesions were causing significant thecal sac stenosis and cord compression, and the cauda equina was thickened with multiple foci of enhancement. The patient received three days of RT along with high-dose CS. Neurosurgery and neuro-interventional radiology were consulted to consider a potential biopsy of spinal masses; however, this was deferred as they considered it a high-risk procedure with a very high likelihood these lesions were representing MS in the setting of previously treated AML. He was then referred again to our institution.
Following RT, the patient reported partial improvement in his neurologic deficits. Physical examination revealed paresis and hypoesthesia in his right arm and bilateral lower extremities, with the right worse than the left. A complete blood count (Table 1 ) reported hemoglobin (Hgb) 12.0 g/dL, WBC 3.9 K/mm 3 , and platelet count 99 k/mm 3 , with a normal differential count and no circulating blasts. A computed tomography (CT) scan with intravenous contrast of the chest, abdomen, and pelvis showed no disease in these locations. A BM aspiration was performed, and he was started on systemic CTX for induction with MEC regimen (mitoxantrone, etoposide, and cytarabine) for five days. Unfortunately, the BM aspirate sample was inadequate for evaluation. His strength and sensation improved progressively over the next several days, and he could walk with a cane. On day 5 post-induction, a lumbar puncture for CSF analysis and intrathecal (IT) CTX (methotrexate, cytarabine, hydrocortisone) administration was performed. The CSF contained myeloid blasts, confirmed by cytology and flow cytometry, and was consistent with central nervous system (CNS) involvement by AML. A subsequent brain MRI showed abnormal enhancement along the left oculomotor nerve (Figure 2 ), raising concern for leptomeningeal disease. A repeat whole-spine MRI on day 15 post-induction reported a partial response demonstrated by shrinking the intraspinal masses, spine nerve roots, and cauda equina thickening. After improving the platelet count, he received another dose of IT CTX. After the patient achieved count recovery, he was discharged with the plan to continue his care close to home.
Table 1
Laboratory findings. BUN: blood ureic nitrogen; CBC: complete blood count; CTX: chemotherapy; RBC: red blood cells; WBC: white blood cells
| Test | Sep 2023 (at initial presentation) | Oct 2023 (on discharge after induction CTX) | Sep 2024 (at presentation) | Oct 2024 (after re-induction CTX) | Reference values |
| CBC with differential | |||||
| WBC | 37.0 x10 3 /µL | 1.5 x10 3 /µL | 3.9 x10 3 /µL | 1.4 x10 3 /µL | 3.6 x10 3 -10.6 x10 3 /µL |
| Neutrophils (%) | 4 | 8 | 62 | 69 | 40-60 |
| Band form (%) | 2 | 0 | 5 | 4 | 0-3 |
| Lymphocytes (%) | 41 | 81 | 24 | 19 | 20-40 |
| Monocytes (%) | 11 | 10 | 6 | 5 | 2-8 |
| Blasts (%) | 41 | 0 | 0 | 0 | 0 |
| RBC | 4.22 x10 6 /µL | 2.49 x10 6 /µL | 3.9 x10 6 /µL | 3.0 x10 6 /µL | 4.18 x10 6 -5.51 x10 6 /µL |
| Hemoglobin | 13.1 g/dL | 7.5 g/dL | 12 g/dL | 9.1 g/dL | 13.7-17.0 g/dL |
| Hematocrit (%) | 39.1 | 21.3 | 34.5 | 26.4 | 40-54 |
| Platelet count | 279 x10 3 /µL | 108 x10 3 /µL | 99 x10 3 /µL | 95 x10 3 /µL | 150 x10 3 -450 x10 3 /µL |
| Basic metabolic panel | |||||
| Sodium | 139 mEq/L | 138 mEq/L | 135 mEq/L | 136 mEq/L | 135-145 mEq/L |
| Potassium | 4.4 mEq/L | 3.3 mEq/L | 4.0 mEq/L | 3.5 mEq/L | 3.5-5.5 mEq/L |
| Chloride | 101 mEq/L | 103 mEq/L | 101 mEq/L | 101 mEq/L | 98-108 mEq/L |
| Bicarbonate | 32 mEq/L | 25 mEq/L | 26 mEq/L | 26 mEq/L | 22-29 mEq/L |
| Creatinine | 0.6 mg/dL | 0.65 mg/dL | 0.67 mg/dL | 0.57 mg/dL | 0.8-1.4 mg/dL |
| BUN | 15 mg/dL | 7 mg/dL | 20 mg/dL | 3 mg/dL | 0.5-20 mg/dL |
Discussion
Materials and methods
We conducted a comprehensive review of cases of MS presenting with signs and symptoms of spinal cord compression reported in the literature. We performed a literature review in November 2024 using PubMed as a database. We included case reports and case series articles that included one of the following search terms in their titles or abstracts: myeloid sarcoma, acute myeloid leukemia, granulocytic sarcoma, myeloblastoma, or chloroma, in combination with either one of the following search terms: spinal cord compression, myelopathy, paraplegia, or hemiplegia.
We found 155 articles that met our initial search criteria. We excluded non-English articles and those unavailable as full-text online articles. We also excluded articles that were not case reports or case series of MS presenting with manifestations of spinal cord compression.
Results
A total of 62 case reports and case series articles (making a total of 69 cases) were included in this literature review. A summary of these reported cases from 1973 to 2023 is presented in Table 2 . Demographic and patients' characteristics are presented in Table 3 . Fifty-five (80%) of the reported cases were male, and the median age at presentation was 29 years (14-47 years). Only 22 (32%) of patients had a known prior history of hematologic diseases such as AML (the most common one reported), MPN, or MDS. The most frequently reported clinical manifestations were paresis/paralysis (81%), sensory deficits (59%), back pain (59%), bladder dysfunction (40%), and bowel dysfunction (26%). Fifty-seven percent of cases were found to have BM disease at the time of presentation. Still, only 11% of cases reported CNS involvement (demonstrated by the presence of myeloid blasts on CSF analysis). Regarding management, patients mostly underwent systemic CTX (71%), RT (56%), and/or surgery (56%); other reported treatment strategies included IT CTX (19%) and SCT (13%). The median follow-up period between the initial presentation and the last evaluation before writing the case report was six months (3-12 months), and 46% of the patients were alive at the time of the report.
Table 2
Literature review of patients with spinal cord compression due to MS sarcoma. AML: acute myeloid leukemia; ARDS: acute respiratory distress syndrome; ATRA: all-trans retinoic acid; BM: bone marrow; BMT: bone marrow transplant; CML: chronic myeloid leukemia; CNS: central nervous system; CS: corticosteroids; CTX: chemotherapy; DIC: disseminated intravascular coagulation; DLI: donor lymphocyte infusion; HiDAC: high-dose cytarabine; IT: intrathecal; L: left; LE: lower extremity; LP: lumbar puncture; MDS: myelodysplastic syndrome; MPN: myeloproliferative neoplasm; NHL: non-Hodgkin lymphoma; PMF: primary myelofibrosis; R: right; RT: radiotherapy; SCT: stem-cell transplant; TKI: tyrosine kinase inhibitor; UE: upper extremity
| First author, year, reference | Sex, age | History of AML or MPN | Clinical presentation | Anatomic location | CNS disease at presentation | BM disease at presentation | Flow cytometry, cytogenetic and genetic hallmarks | Management | Interval follow-up | Outcome at time of publication |
| Stefansson, 1973 [ 7 ] | F, 11 | No | Acute lumbar pain, paraplegia, sphincter disturbances | T7 epidural mass | No | Yes | Not mentioned | Surgery, IT methotrexate, RT, and systemic CTX (purinethol, rubidomycin, cytarabine, prednisone) | Not mentioned | Alive. Partial response to treatment with minor improvement in the paraplegia |
| Hildebrand, 1980 [ 8 ] | M, 25 | Yes, AML | Rapidly progressive paraplegia and complete loss of sphincter control | T3-7 epidural mass | No | No | Not mentioned | Surgery, RT | 1 month | Died from pulmonary embolism |
| Petursson, 1981 [ 9 ] | M, 21 | No | Flu-like symptoms, hepatosplenomegaly, constipation, urinary retention, perineal sensory deficits, and sacral pain | L5-S1 mass | No | Yes | Not mentioned | RT, and systemic CTX (induction with daunorubicin and cytarabine, followed by re-induction and consolidation with vincristine, prednisone, cytarabine, and 6-thioguanine). Patient relapsed and underwent BMT | 4 months | Died from interstitial pneumonia following BMT |
| Kellie, 1984 [ 10 ] | M, 8 | No | Low back pain, bilateral LE dysesthesias and paresis | T4-6 epidural mass | No | Yes | Karyotype 45X,-Y, t(8;21)(q22;q22) | Surgery, systemic CTX (induction with cytarabine, 6-thioguanine, and daunorubicin, followed by consolidation with cyclophosphamide, doxorubicin), and IT CTX (cytarabine) | 5 months | Alive. Complete remission and resolution of neurologic deficits |
| Abe, 1986 [ 11 ] | M, 17 | No | Lumbar pain, paresthesias below the umbilicus, spastic paraplegia | T5-8 epidural mass | No | Yes | t(8;21) | Systemic CTX (two cycles of daunorubicin, cytarabine, 6-mercaptopurine, and prednisolone). Due to first relapse, patient had a course of systemic daunorubicin and enocitabine, followed by surgery (laminectomy with tumor resection) and one cycle of aclarubicin and enocitabine | 1 year and 6 months | Died after second relapse |
| Wodzinski, 1988 [ 12 ] | M, 14 | No | Paraplegia, T5 sensory level. Second relapse was associated with multiple 2-cm fleshy skin lesions that were demonstrated to be MS | T3-5 epidural mass | No | Yes | Karyotype 45X,-Y, t(8;21)(q22;q22) | Systemic CTX (daunorubicin, cytarabine, and 6-thioguanine), and surgery (laminectomy). Due to first relapse, patient received re-induction with mitoxantrone, cytarabine, and 6-thioguanine | 6 weeks | Died after second relapse |
| M, 19 | No | Back pain, absent right ankle reflex, and urinary retention | T7-11 paraspinal mass | No | Yes | Karyotype 45X,-Y, t(8;21)(q22;q22) | IT methotrexate, systemic CTX (daunorubicin, cytarabine, and thioguanine), and spine RT. Due to first relapse, patient received re-induction with mitoxantrone and cytarabine, followed by BMT | 6 months | Died after BMT | |
| M, 17 | Yes, AML | Bilateral LE weakness and urinary retention | T9-10 epidural mass | Not mentioned | Yes | Complex karyotype | Surgery, RT, and systemic CTX | Not mentioned | Not mentioned | |
| Brown, 1989 [ 13 ] | M, 12 | Yes, AML | Back pain, pleural effusion, post-LP urinary retention, bilateral LE paresis, and T5 sensory level | Paraspinal mid- and lower thoracic spinal mass with extension to the epidural space | Not mentioned | Yes | Not mentioned | CS, thoracic spine RT, and systemic CTX | 3 months | Died from persistent BM disease |
| Ripp, 1989 [ 14 ] | M, 58 | No | Back pain, paraparesis, T6 sensory level, perianal numbness, bladder and bowel dysfunction | T5-7 and L5-S1 epidural masses | Not mentioned | Yes | Not mentioned | Systemic CTX for presumed multiple myeloma, followed by local RT and induction CTX (daunorubicin, cytarabine, and 6-thioguanine) | 3 months | Died from sepsis |
| Kook, 1992 [ 15 ] | M, 10 | No | Gradual bilateral LE paresis hyperreflexia, T5 sensory level, loss of sphincter tone. | Epidural thoracic spinal mass | Not mentioned | Yes | MPO+, CD13+, CD33+ | Surgery, followed by RT, IT methotrexate, and systemic CTX (vincristine, doxorubicin, cyclophosphamide, CS) for presumed NHL. After BM showed AML, patient received CTX with aclacinomycin, cytarabine, and CS | 1 year and 4 months | Died from intracranial hemorrhage |
| Frohna, 1993 [ 16 ] | M, 77 | Yes, PMF | Low back pain and unsteady gait that progressed to paraplegia | T5-L2 epidural mass | Not mentioned | Yes | Not mentioned | None | 2 weeks | Died before diagnosis |
| Aizawa, 1996 [ 17 ] | M, 19 | No | Low back pain, paraparesis, urinary retention, T9 sensory level | T5-7 epidural space | Not mentioned | Yes | MPO+, Karyotype 45X,-Y, t(8;21) (q22;q22) | Surgery, systemic CTX | 6 months | Alive. Complete remission and resolution of neurologic deficits |
| Deme, 1997 [ 18 ] | M, 47 | No | Neck and arm pain, followed by quadriparesis and ataxia | C6-7 epidural space | Yes | No | CD45+, CD43+, MPO+, lysozyme+, CD20-, CD45RO- | Surgery, RT, IT methotrexate. After first relapse, the patient had systemic CTX with cytarabine followed by allo-SCT | 1 year | Died from aspiration related to methotrexate encephalopathy. |
| M, 49 | No | Low back pain, urinary retention, and right leg paresis and numbness | L3-L4 epidural, paraspinal, and foraminal regions | Not mentioned | Not mentioned | CD45+, CD43+, MPO+, CD20-, CD45RO- | Surgery, RT, and systemic CTX | Not mentioned | Alive. Patient getting CTX. Improvement of neurologic deficits | |
| M, 15 | No | Bilateral LE pain and paresis, 15-lb weight loss | L2 vertebral body with epidural extension | No | No | CD45+, CD43+, MPO+, lysozyme+, CD20-, CD45RO- | Surgery, systemic CTX, followed by autologous SCT. Re-induction CTX was given after relapse but was unsuccessful | 1 year | Died after relapse | |
| Sajjad, 1997 [ 19 ] | M, 22 | No | Urinary retention, loss of anal sphincter tone, bilateral LE sensory deficits, and weight loss | L4 and S1 peridural spaces | Not mentioned | No | Not mentioned | Not mentioned | Not mentioned | Not mentioned |
| Eser, 2000 [ 20 ] | F, 17 | No | Left facial nerve palsy, paraplegia, loss of rectal sphincter tone, and T4 sensory level | T4 extramedullary-intradural space | Not mentioned | Yes | LCA+, CD68+, lysozyme+ | Surgery and systemic CTX (doxorubicin and cytarabine) | Not mentioned | Died from sepsis after second course of chemotherapy |
| Ugras, 2001 [ 21 ] | M, 13 | No | Low back pain, bilateral LE pain, paraparesis, and L1 sensory level | T11-L1 epidural space | Not mentioned | No | MPO+, CD45+, CD68+, CD43, CD3- | Surgery | 1 year | Died |
| Landis, 2003 [ 22 ] | M, 29 | No | Lower thoracic back pain and soft tissue swelling | T7-9 spine | No | Yes | CD45+, CD43+, CD34+, MPO+, CD117+, CD20-, CD3-, t(8;21)(q22;q22) | Systemic CS, surgery, IT cytarabine and systemic CXT (induction with idarubicin and cytarabine, followed by consolidation with cytarabine) | 6 months | Alive. In remission |
| Hamadani, 2005 [ 23 ] | M, 33 | No | Thoracic spine pain, left leg paresis, sensory deficits, B-symptoms | T6-8 spine | No | Yes | MPO+, S100+, CD68+, CD3-, CD20-, CD1a-, CD30- | Systemic CXT (induction with daunorubicin and cytarabine, reinduction with HiDAC, and reinduction again with mitoxantrone plus HiDAC), followed by BMT x2 (due to engraftment failure of first BMT) | 3 months | Alive. In remission |
| Kalayci, 2005 [ 24 ] | M, 24 | No | Low back pain, paraparesis, T5 sensory level, and urinary retention | T3-5 epidural space | Not mentioned | No | MPO+, LCA+, lysozyme+ | Systemic CS, surgery, systemic CTX, spine RT | 8 months | Alive. In remission |
| Meltzer, 2005 [ 25 ] | M, 10 | No | Low back pain, paraparesis, bilateral LE sensory deficits | T2-6 prevertebral area with extension to epidural space, bone cranial involvement | No | Yes | Not mentioned | Systemic CS and CTX, surgery, spine RT | 1 year and 6 months | Alive. In remission |
| Seo, 2006 [ 26 ] | M, 59 | Yes, AML | Back pain, paraplegia, bilateral LE paresthesias | T8-11 epidural and paravertebral spaces | Not mentioned | No | CD45+, CD43+, CD34+, MPO+, lysozyme+, CD3-, CD20- | CS only | Not mentioned | Died from pneumonia |
| Widhalm, 2006 [ 27 ] | F, 35 | No | Paraparesis, T5 sensory level, sphincter dysfunction, and urinary retention | T3-5, and S1-S4 intradural space, right parietal extra- and intracranial regions with invasion of the sensory cortex | Not mentioned | No | CD34+, CD43+, CD117+, MPO+, t(8;21)(q22;q22) | Surgery, systemic CTX (cyclophosphamide) followed by whole-body RT and allo-BMT | 7 years | Alive. In remission |
| Balleari, 2007 [ 28 ] | F, 76 | No | Large inguino-crural mass, bilateral UE paresis and hypoesthesia | C7-T2 subdural space with extension to the subarachnoid space | Yes | Yes | MPO+, CD33+, CD34+, CD20- | Systemic CTX (induction with fludarabine, cytarabine, and idarubicin) | 3 months | Died from pneumonia and ARDS |
| Chauhan, 2007 [ 29 ] | F, 53 | Yes, CML | Paraparesis, right T10 sensory level, | T10-12 epidural space | Not mentioned | Not mentioned | Not mentioned | Not mentioned | Not mentioned | Not mentioned |
| Tsai, 2007 [ 30 ] | M, 29 | Yes, AML | Back pain, paraparesis, perineal anesthesia, fecal and urinary incontinence | Multiple lumbar subarachnoid lesions + S1-5 subdural space | Not mentioned | Not mentioned | Not mentioned | Regional RT, systemic CS, IT CTX (cytarabine) and systemic salvage CTX | 1 month | Died from febrile neutropenia and septic shock |
| Al-Sobhi, 2008 [ 31 ] | F, 23 | Yes, AML | Paraplegia | T4-5 and T8-9 paraspinal region | Not mentioned | Yes | t(8;21) | Systemic CS, CTX (re-induction with anthracycline and cytarabine, followed by three cycles of consolidation with HiDAC), and adjuvant RT | 1 year and 1 month | Died after second relapse |
| Amalraj, 2009 [ 32 ] | F, 14 | No | Back pain, paraparesis, T4 sensory level, urinary symptoms | T3-11 epidural space | No | Yes | Not mentioned | Systemic CS, CTX, and local RT | Not mentioned | Alive. Improvement of neurologic deficits |
| Olcay, 2009 [ 33 ] | M, 12 | No | Left cheek swelling, bilateral LE pain, S1 sensory level, fecal and urinary incontinence | Left maxillary sinus, cauda equina nerve roots | No | Yes | CD45+, CD15+, CD33+, CD117+, CD34+, CD13+, CD19+, CD20+ | Systemic CTX (induction with cytarabine, mitoxantrone, etoposide, and CS, followed by consolidation with cytarabine and daunorubicin), IT CTX (methotrexate, cytarabine, prednisolone), and regional RT | Not mentioned | Died from pneumonia and DIC |
| Takeda, 2009 [ 34 ] | M, 13 | No | Exophthalmos, back pain, paraparesis | Left temporal lobe, left orbit, T5-6 and epidural L5-S5 space | Not mentioned | Yes | MPO+, CD13+, CD33+, CD34+, CD45+, CD56+ | Systemic CS, surgery, systemic CXT, and BMT | 18 months | Alive. In remission and with complete recovery of neurologic deficits |
| Verra, 2009 [ 35 ] | M, 45 | Yes, AML | Back and R leg pain, gait disturbance, R dorsi-flexion weakness. After second relapse, patient had R flank pain with dysesthesias in dermatomes T11-12, and vision loss | L5-S2 intraspinal extra-medullary. Multifocal nerve roots of thoracic spine, and multifocal intracranial lesions | Yes | No | Not mentioned | Surgery, RT, systemic CTX (HiDAC), and DLI. IT CTX (cytarabine) after relapse, followed by second DLI. | 3 months | Alive. With refractory disease and persistent neurologic symptoms |
| Amritanand, 2010 [ 36 ] | M, 15 | No | Back pain, paraparesis | Epidural T8-10 space | Not mentioned | No | MPO+, CD43+, CD99+, CD20-, CD30-, CD3- | Surgery, RT | 9 months | Died |
| Pacilli, 2010 [ 37 ] | M, 38 | Yes, AML | Chest pain, paraparesis | Extramedullary intraspinal T6-8 | Not mentioned | No | MPO+, CD117+, CD45+, CD34-, CD79a-, CD20-, CD3-, CD15-, CD56-, t(15;17) (q22;q21-q22), PML-RAR𝝰 rearrangement | Surgery, RT, systemic CXT (ATRA and arsenic trioxide) | 1 year and 8 months | Alive. In remission |
| Serefhanoglu, 2010 [ 38 ] | M, 22 | No | Back pain, paraparesis, eosinophilia | Supratentorial and epidural space of all skull bones, C2-3, C7-T10, and L5 epidural and, prevertebral and paravertebral spaces with involvement of the spinal cord | Yes | No | MPO+, CD34+, CD2-, CD3-, CD5-, CD20-, CD30-, CD56-, Tdt- | Cranial and cervical RT followed by systemic CTX (idarubicin + cytarabine) | Not mentioned | Died from septic shock |
| F, 43 | No | Left arm and back pain | C6-7 epidural space | Yes | No | MPO+, CD3-, CD5-, CD7-, CD10-, CD20-, Tdt-, normal cytogenetics | Spinal and cranial RT, systemic CTX (mitoxantrone + cytarabine), IT CTX | Not mentioned | Died due to ventricular fibrillation and cardiac arrest | |
| Chargari, 2011 [ 39 ] | M, 41 | No | Back pain, paraplegia, T10 sensory level, loss of sphincter tone | T8 epidural mass | Not mentioned | No | MPO+, CD20-, CD79a-, CD1a-, CD3-, CD5- | Surgery, spinal RT | 1 year | Relapsed with AML and was referred for induction CTX |
| Baikaidi, 2012 [ 40 ] | F, 75 | No | Back and L leg pain, gait imbalance | T12 paravertebral mass | Not mentioned | Yes | CD43+, CD117+, CD30+, CD34-, CD20-, CD79a-, TdT-, CD3-, CD2-, hyperdiploid, t(9;22), BCR-ABL rearrangement | Systemic CS, RT, systemic CTX (decitabine), and TKI (imatinib) | Not mentioned | Alive |
| Cornfield, 2012 [ 41 ] | M, 79 | Yes, MDS | Back pain, paraparesis, weight loss | T7 epidural and paravertebral masses | Not mentioned | Yes | CD71, glycophorin A, CD117, and vimentin positivity (consistent with erythroblastic sarcoma) | RT and supportive therapy | 2 weeks | Died |
| Kyaw, 2012 [ 42 ] | M, 26 | No | Back pain, paraplegia, T4 sensory level | T2-4 and T12-L2 epidural masses | No | Yes | MPO+, CD45+, CD13+, CD33+, CD117+, CD64+, CD34-, CD14-, CD19-, CD79a-, CD3-, t(15;17), PML/RAR𝝰 rearrangement | Regional RT, systemic CTX (induction with ATRA and idarubicin followed by consolidation), IT CTX | Not mentioned | Alive. In remission, undergoing maintenance CTX |
| Chamberlain, 2013 [ 43 ] | M, 23 | Yes, AML | Left buttock and thigh pain | S2 nerve root mass | Not mentioned | Yes | Not mentioned | Systemic reinduction CTX and whole-body RT followed by allo-BMT | 1 year | Alive. In remission, with resolution of neurologic symptoms |
| Ganapule, 2014 [ 44 ] | M, 43 | No | Back pain, paraplegia with bladder and anal sphincter dysfunction | T6-12 paravertebral lesions | Not mentioned | Yes (CML on chronic phase) | MPO+, CD43+, CD34+, CD20-, CD3-, t(9;22), BCR-ABL rearrangement | Hydroxyurea, spinal RT, and TKI (imatinib) | 2 months | Alive. With complete hematologic response |
| Isshiki, 2014 [ 45 ] | M, 59 | No | Chest pain, bilateral LE pain and paresthesias, paraplegia, bowel and bladder incontinence | T7-10 epidural mass | Yes | Yes | CD25+, CD33+, CD34+, CD3-, CD13-, CD19-, CD20-, FLT3-ITD mutation, karyotype 46,XY, dup(1)(q21;q23) | IT CTX (methotrexate, cytarabine, CS), systemic CTX (induction and consolidation with HiDAC) | 4 months | Alive |
| Kurian, 2014 [ 46 ] | M, 39 | No | Paraplegia, bladder involvement, T6 sensory level | T6 epidural mass | Not mentioned | No | MPO+, CD117+, CD68+, CD34-, CD56- | Surgery, systemic CTX (daunorubicin + cytarabine) | Not mentioned | Died from sepsis |
| Joseph, 2015 [ 47 ] | M, 20 | Yes, AML in the setting of Shwachman-Diamond syndrome | Back pain, paraparesis, T7 sensory level, urinary retention | T4-9 epidural mass | Not mentioned | Not mentioned | Weak CD68+, partial CD43 and CD117 | Surgery, RT | 5 months | Died from liver failure due to suspected leukemic infiltration |
| Krishnan, 2015 [ 48 ] | M, 22 | No | Back pain, gait ataxia, paraparesis, T10 sensory deficit | T6-8 epidural mass | Not mentioned | No | MPO+, CD43+, CD3-, S100- | Systemic CTX for initially suspected lymphoma followed by CS, surgery, and RT due to worsening neurologic deficits. Systemic CTX with fludarabine and cytarabine after diagnosis of MS | 2 years | Alive. In remission with resolution of all neurologic deficits |
| Asano, 2016 [ 49 ] | M, 29 | No | Bilateral LE pain, paraplegia, T10 sensory level, absence of anal sphincter tone, visual field deficits followed by blindness, bilateral pleural effusion | Intramedullary T6 lesion, T6-9 paravertebral mass, pituitary mass, bilateral orbital cavity masses | Yes | Yes | Not mentioned | Surgery, systemic CTX (daunorubicin plus cytarabine) | 1 year | Alive. In remission, but neurologic deficits (paraplegia and blindness) persisted |
| Dhakar, 2016 [ 50 ] | M, 55 | Yes, AML | Paraparesis | Multiple osseous and extra-osseous paravertebral masses with infiltration into the epidural space | Not mentioned | Yes | Not mentioned | None, patient went into hospice | Not mentioned | Not mentioned |
| Horvath, 2016 [ 51 ] | M, 66 | Yes, MDS | B-symptoms, paraplegia | T1-3 epidural mass | Not mentioned | Yes | LCA+, MPO+, CD15+, CD117+, CD99+ | Surgery, systemic CTX with cytarabine due to progression from MDS to AML | 5 weeks | Died |
| Kucukapan, 2016 [ 52 ] | M, 13 | No | Back pain, paraparesis, and bilateral LE paresthesias | T10-12 epidural mass | Not mentioned | Yes | Not mentioned | RT and systemic CTX (HiDAC) | 10 days | Alive, total remission of spine lesion |
| Sahu, 2016 [ 53 ] | F, 38 | Yes, CML and history of scalp MS | Quadriplegia, bowel and bladder incontinence, and T4 sensory level | T4-7 epidural mass | Not mentioned | Yes | CD13+, CD33+, CD117+, t(9;22), BCR-ABL M351T mutation | CS, RT, and TKI (nilotinib) | Not mentioned | Patient died from pneumonia-related sepsis |
| Yang, 2016 [ 54 ] | F, 42 | Yes, AML | Paraplegia, T4 sensory level, fecal incontinence | T5-12 bilateral lateral and posterior funiculi lesions in the spinal cord | Yes | No | Not mentioned | Not mentioned | Not mentioned | Died from pneumonia |
| Arslantas, 2018 [ 55 ] | M, 4 | No | New presentation. Paraplegia | T10-12 epidural mass | Not mentioned | Yes | Not mentioned | RT, systemic CS, and CTX (AML-BMF 2012 protocol) | Not mentioned | Alive. In remission |
| Koh, 2019 [ 56 ] | M, 68 | Yes, CML | Back and bilateral LE pain, paraparesis, T12 sensory level, urinary retention, and anal sphincter dysfunction | T11-12 epidural and paraspinal masses | Not mentioned | Not mentioned | Not mentioned | Surgery, regional RT, and CS | 35 days | Alive. Transferred to local hospital for conservative therapy |
| Gupta, 2020 [ 57 ] | M, 59 | No | Back pain, paraplegia, bilateral LE hypoesthesia, and urinary incontinence | T7-10 and L2 epidural masses | Not mentioned | Yes | CD45+, CD117+, CD43+, MPO+, CD34-, NMP1 mutation | Surgery, systemic CTX (FLAG-IDA), and IT CTX (cytarabine) | Not mentioned | Alive. In remission |
| Kumar, 2020 [ 58 ] | F, 64 | Yes, AML in the setting of PMF | Paraparesis, bowel and urinary incontinence | T3-10 posterior epidural masses | Not mentioned | Not mentioned | Not mentioned | Regional RT and systemic CTX | Not mentioned | Died |
| Bai, 2021 [ 59 ] | M, 29 | No | Bilateral LE pain, dysuria | L2-L4 lumbar canal mass | Not mentioned | Not mentioned | CD33+, MPO+, partial CD68+ | Surgery, systemic CTX (daunorubicin + cytarabine) | 10 months | Alive. In remission |
| Bhandohal, 2021 [ 60 ] | M, 47 | No | Back pain, left LE pain, paresis and numbness | L4-L5 intrathecal mass with invasion of L5 nerve root | Not mentioned | No | CD33+, CD34+, CD45+, MPO+, CD117+, FLI1+, CD99+ | Surgery, regional RT | 6 months | Alive, improvement in neurologic deficits, and decreased size of mass |
| Shah, 2021 [ 61 ] | M, 47 | No | Back and right LE pain, anorexia, weight loss, mild sacral bulging | L4-S5 paraspinal mass | Not mentioned | No | MPO+, CD30+, CD13+ | Systemic CTX (induction with cytarabine and daunorubicin, followed by consolidation with HiDAC), regional RT | Not mentioned | Not mentioned |
| M, 24 | No | Paraplegia with bilateral LE sensory loss | T4-7 paravertebral mass | Not mentioned | Yes (CML in chronic phase) | t(9;22), BCR-ABL rearrangement | Surgery, RT, and TKI (imatinib) | Not mentioned | Alive | |
| F, 32 | No | Paraparesis | T6-8 paravertebral mass | Not mentioned | No | Not mentioned | Systemic CTX (daunorubicin + cytarabine), followed by surgery, local RT, and consolidation CTX (HiDAC) | 2 years | Alive. In remission | |
| Han, 2022 [ 62 ] | M, 37 | Yes, CML | Back and chest pain | T2-L2 epidural mass | Not mentioned | No | CD15, CD3, CD20, MPO, CD79a | Surgery, readjustment of TKI therapy (switch from imatinib to dasatinib) | 1 year | Alive. In remission |
| Panda, 2022 [ 63 ] | M, 13 | No | Urinary retention and overflow incontinence, constipation, saddle anesthesia, loss of anal reflex | L5-S4 spinal canal mass | Not mentioned | Yes | CD34+, MPO+ | Surgery, systemic CTX | Not mentioned | Alive. Receiving systemic CTX |
| Shao, 2022 [ 64 ] | M, 15 | No | Paraparesis, T6 sensory level, back and chest pain | T3-8 spinal space-occupying mass | Not mentioned | Yes | CD13+, CD33+, CD34+, CD38+, CD56+, CD117+, HLA-DR+, MPO+ | Systemic CTX (idarubicin + cytarabine) | 1 year | Alive. In remission |
| Fujikawa, 2023 [ 65 ] | M, 14 | No | Back pain, paraplegia, T9 sensory level, bladder and bowel dysfunction | T7-9 posterior epidural mass | No | Yes, AML (82.5% of myeloblasts) | MPO+, CD34+, t(8;21), RUNX1-RUNX1T1 rearrangement | Surgery, systemic CTX (cytarabine, mitoxantrone, etoposide) | 8 months | Alive. In remission, incomplete paraplegia and bladder and bowel dysfunction persisted |
| Nair, 2023 [ 66 ] | M, 36 | Yes, CML | Back pain, unsteady gait, paraparesis | T5-11 epidural, pelvic, and bilateral proximal femoral masses | Not mentioned | Not mentioned | MPO+, CD34+, CD117+ | Surgery, systemic CTX | Not mentioned | Not mentioned |
| Osman, 2023 [ 67 ] | M, 20 | Yes, AML | Back pain, paraparesis, T4 sensory level, and urinary retention | T2-6 epidural mass extending to the subdural space | Not mentioned | Not mentioned | MPO+, CD43+ | CS, surgery | Not mentioned | Not mentioned |
| Patel, 2023 [ 68 ] | F, 87 | No | Paraparesis | T4-7 epidural mass | Not mentioned | Yes | CD68+ (MS with monocytic differentiation) | Surgery, declined further CTX or RT | 3 months | Died |
Table 3
Demographics and patients' characteristics. AML: acute myeloid leukemia; BM: bone marrow; BMT: bone marrow transplant; CNS: central nervous system; CML: chronic myeloid leukemia; CTX: chemotherapy; IQR: interquartile ration; IT: intrathecal; MDS: myelodysplastic syndrome; MPN: myeloproliferative neoplasm; MS: myeloid sarcoma; PMF: primary myelofibrosis; RT: radiotherapy
| Variable | |
| Patient number, n | 69 |
| Gender | |
| Male, n (%) | 55 (80) |
| Age | |
| Age, median (IQR) | 29 years (14-47) |
| History of AML, MPN, or MDS | |
| No, n (%) | 47 (68) |
| Yes, n (%) | 22 (32) |
| AML, n (%) | 14 (20) |
| CML, n (%) | 5 (7) |
| MDS, n (%) | 2 (3) |
| PMF, n (%) | 1 (1) |
| Signs and symptoms at presentation | |
| Paresis/paralysis, n (%) | 56 (81) |
| Sensory deficits, n (%) | 41 (59) |
| Back pain, n (%) | 41 (59) |
| Bladder dysfunction, n (%) | 28 (40) |
| Bowel dysfunction, n (%) | 18 (26) |
| CNS disease at presentation | |
| Yes, n (%) | 8 (11) |
| No, n (%) | 15 (22) |
| Not mentioned, n (%) | 46 (67) |
| BM disease at presentation | |
| Yes, n (%) | 39 (57) |
| No, n (%) | 21 (30) |
| Not mentioned, n (%) | 9 (13) |
| Treatment | |
| Systemic CTX | 49 (71) |
| RT | 39 (56) |
| Surgery | 39 (56) |
| IT CTX | 13 (19) |
| BMT | 9 (13) |
| Outcomes at time of publication | |
| Alive, n (%) | 32 (46) |
| Interval follow-up, median (IQR) | 6 months (3-12) |
Discussion
MS is a rare extramedullary manifestation of AML or other MPN and MDS that can present in virtually any anatomical location, including the spinal cord. Spinal MS, although rare, represents a critical clinical challenge due to the potential for spinal cord compression, which can lead to severe neurological deficits that might become permanent if not promptly managed. Our case report and literature review showcase these severe neurologic deficits that can arise in patients with spinal MS, such as muscle weakness, sensory deficits, and bladder and bowel dysfunction. The delay in diagnosis due to the insidious onset of symptoms emphasizes the need for high clinical suspicion, especially in patients with a history of hematologic malignancies.
MRI plays a crucial role in the diagnosis of MS, particularly in cases with spinal cord involvement. The whole spine MRI findings (intradural enhancing masses with associated nerve root thickening) were critical in establishing the diagnosis in our patient, especially in the setting of the previous history of AML. Other reported cases also highlight the importance of prompt imaging studies, MRI in particular, in patients presenting with signs of spinal cord compression, as it supports an early diagnosis and timely interventions. These imaging findings help plan therapeutic strategies, including potential surgical decompression, RT, and systemic CTX [ 69 , 70 ]. Despite the high yield of imaging in the diagnostic workup of MS, the definitive diagnosis often relies on a pathology assessment of a biopsy specimen.
Given its rarity and lack of RCTs, there is no consensus regarding the standard management of spinal MS; however, it generally requires a multimodal approach. According to our literature review, most patients were treated with some combination of systemic or IT CTX, RT, CS, and/or surgery. In this case, our patient received systemic and IT CTX, as well as regional spinal RT. RT and steroids may provide relief of the spinal cord compression; however, as virtually all cases with MS ultimately progress to AML, treatment protocols for AML (e.g., systemic CTX with subsequent allogeneic SCT) are usually considered the most reasonable approach [ 1 , 71 ]. Our patient would ultimately benefit from more CTX with a CNS-penetrating agent such as HiDAC, followed by allogeneic SCT.
The literature review highlights poor outcomes for patients with MS presenting with spinal cord compression, with only 46% of patients alive at the time of publication and none of them in remission. The median follow-up of six months reflects the aggressive nature of this disease. The rarity of MS with spinal cord compression limits the feasibility of RCTs, and current treatment recommendations rely heavily on case reports and expert opinion. This underscores the need for collaborative studies and registries to establish optimal management strategies formally. Additionally, advancements in molecular diagnostics and targeted therapies may provide opportunities for improved outcomes. Integrating novel targeted agents, such as FLT3 inhibitors or monoclonal antibodies, into treatment protocols could potentially enhance responses while mitigating toxicities.
Conclusions
Although rare, spinal MS should be suspected in a patient with a history of myeloid neoplasia presenting with new-onset neurologic deficits, particularly if imaging shows mass lesions involving the spinal cord. Management typically involves a multimodal approach, including a combination of CTX, RT, CS, and/or surgery. Despite treatment, outcomes are poor, and further collaborative studies are essential to develop standardized management strategies and improve prognosis in this aggressive disease.
Disclosures
Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study.
Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following:
Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work.
Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work.
Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.
Author Contributions
Concept and design: Raul Gregg-Garcia, Hamid Sayar
Acquisition, analysis, or interpretation of data: Raul Gregg-Garcia, Hamid Sayar
Drafting of the manuscript: Raul Gregg-Garcia, Hamid Sayar
Critical review of the manuscript for important intellectual content: Raul Gregg-Garcia, Hamid Sayar
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