Extramedullary Tumor Research Articles

A Case of Acute Promyelocytic Leukemia Presenting as Sternal Infiltrate

Granulocytic sarcoma (GS) or myeloid sarcoma (MS) is a localized extramedullary tumor composed of immature myeloid cells which can develop de novo or concurrently with acute myeloid leukemia (AML), myeloproliferative disorders (MPN) or myelodysplastic syndrome (MDS). It can present before, along with or after the diagnosis of acute myeloid leukemia, MPN, MDS or may present as a manifestation of relapse in a previously treated AML patient. The occurrence of MS in an acute promyelocytic leukemia (APL) patient is a very uncommon clinical event especially as initial or primary presentation. APL is often associated with a life threatening coagulopathy making prompt diagnosis and initiation of therapy critical. Unusual clinical localization may lead to misdiagnosis, or delayed diagnosis and treatment. Hence being aware of such atypical presentations of APL is crucial. Here we report a patient with acute promyelocytic leukemia who presented with sternal infiltrate but without any systemic symptoms. The diagnostic challenges are of particular interest given the unusual clinical presentation along with atypical morphology of the abnormal promyelocytes.

RNA sequencing of myeloid sarcoma, shed light on myeloid sarcoma stratification.

Myeloid sarcoma (MS) is a rare, extramedullary tumor consisting of myeloid blasts. Little is known about the genetic background of MS and the prognostic value of genetic abnormalities in MS. In particular, the broad variety of gene fusions that occur in MS is marginally covered by traditional testing methods due to lack of fresh tumor specimens. Here, we analyzed the clinical and genetic features of 61 MS cases. We performed RNA sequencing (RNA-seq) on formalin-fixed paraffin-embedded (FFPE) or fresh samples to analyze fusion genes in 26 cases. In addition, we performed genetic abnormalities-based risk stratification using fusion genes and gene mutations. A total of 305 fusion genes were identified in 22 cases, including the following five recurrent fusion genes: RUNX1-RUNX1T1, CBFβ-MYH11, ETV6-MECOM, FUS-ERG, and PICALM-MLLT10. The prognosis in the adverse-risk group was significantly worse than that in the favorable/intermediate-risk group (median survival: 12 months vs. not reached; p = 0.0004). These results indicated the efficacy of RNA-seq using FFPE-derived RNA as a clinical routine for detecting fusion genes, which can be used as markers for risk stratification in MS.

Clinical efficacy of resection of extramedullary subdural tumor in thoracic spinal canal via muscle-pedicle open-door laminoplasty approach

In order to evaluate the efficacy of resecting extramedullary subdural tumors, located in the thoracic spinal canal, via the muscle-pedicle open-door laminoplasty approach, clinical data of fifteen patients with thoracic spinal tumors admitted to the Department of Spinal Surgery in the Affiliated Hospital of Qingdao University from December 2018 to June 2021 were analyzed retrospectively. All patients received surgical treatment to remove the tumors via the muscle-pedicle open-door laminoplasty approach. The clinical data of the patient was recorded. The Japanese Orthopedic Association (JOA) score, the thoracic kyphosis angle in the standing position before and after the operation, and the visual analog score (VAS) of thoracic back incision pain before and after the operation were compared. CT scan was performed three months after the surgery and at the last follow-up to measure the healing of the lamina. In addition, MRI was used to detect the healing of the posterior ligament at the last follow-up. The tumors of all fifteen patients were completely removed, the operating time was (91±15) minutes, blood loss was (117±56) ml. Cerebral spinal fluid leakage occurred in three patients, but the incisions healed smoothly following compression and no complication was observed. All patients were followed-ups for more than 6 months. The VAS score of thoracic back incision pain was (3±1.3) points at three days post operation and it dropped to (1.3±0.9) points three months post-operation. The JOA score improved significantly after the operation, it was (11.9±2.4) points before operation, and it was (14.0±1.6) points three months post operation and increased to (16.1±0.7) points at the last follow-up (both P<0.001). The Cobb angle was 10.1°±5.3° before the operation and it was 10.4°±6.2° at the last follow-up (P=0.420). Three months after operation, partial callus formation or even complete healing on the muscle pedicle side was observed on the CT scans of all patients. At the last follow-up, CT scans showed complete healing on the muscle pedicle side. There were different degrees of sclerosis and healing on the open-door side. There was no necrosis, displacement, or secondary spinal canal stenosis in any vertebral lamina. It indicated that the muscle-pedicle open-door laminoplasty approach is an effective way to resection extramedullary subdural tumors located in the thoracic spinal canal.

Pediatric Myeloid Sarcoma, More than Just a Chloroma: A Review of Clinical Presentations, Significance, and Biology.

Myeloid sarcomas (MS), commonly referred to as chloromas, are extramedullary tumors of acute myeloid leukemia (AML) with varying incidence and influence on outcomes. Pediatric MS has both a higher incidence and unique clinical presentation, cytogenetic profile, and set of risk factors compared to adult patients. Optimal treatment remains undefined, yet allogeneic hematopoietic stem cell transplantation (allo-HSCT) and epigenetic reprogramming in children are potential therapies. Importantly, the biology of MS development is poorly understood; however, cell-cell interactions, epigenetic dysregulation, cytokine signaling, and angiogenesis all appear to play key roles. This review describes pediatric-specific MS literature and the current state of knowledge about the biological determinants that drive MS development. While the significance of MS remains controversial, the pediatric experience provides an opportunity to investigate mechanisms of disease development to improve patient outcomes. This brings the hope of better understanding MS as a distinct disease entity deserving directed therapeutic approaches.

Pure endoscopic minimally invasive surgery with a non‑expandable tubular retractor for intradural extramedullary spinal tumors.

Minimally invasive spinal surgery (MISS) for intradural extramedullary (IDEM) spinal tumors is a safe and effective surgical strategy. Currently, various tubular retractors are widely used in the MISS of IDEM spinal tumors, primarily relying on microscopic visualization. To the best of the authors' knowledge, there is no report of pure endoscopic surgery with parallel non-expandable tubular retractors for IDEM spinal lesions. The present study reports a case series of IDEM spinal tumors that were treated via pure endoscopic MISS with a parallel non-expandable tubular retractor. The extent of tumor resection was evaluated by comparing preoperative and postoperative magnetic resonance imaging (MRI). The initial and follow-up clinical conditions were assessed according to the visual analog scale for pain and the modified McCormick scale for neurological status. Postoperative MRI demonstrated that all cases had achieved a gross total resection. After the operation, the clinical symptoms of all patients were significantly improved and there were no serious postoperative complications. At the initial follow-up, the pain experienced by the patients was significantly reduced or had even disappeared, and the neurological deficit was improved by at least one grade on the modified McCormick scale. The present report indicates that pure endoscopic MISS with a parallel non-expandable tubular retractor may be an effective and safe surgical strategy for IDEM spinal tumor resection.

Role of Posterior Longitudinal Ligament Complex in Spinal Deformity Secondary to Surgical Resection of the Intradural Tumor.

In most cases, complete resection of the intradural tumor is accompanied by long-term neurological complications. Postoperative spinal deformity is the most common complication after surgical resection of intradural tumors, and posterior longitudinal ligament complex (PLC) plays an important role in postoperative spinal deformity. In this study, we investigated the role of PLC in spinal deformity after the surgical treatment of intradural tumors. We analyzed the data of 218 consecutive patients who underwent intradural tumor resection from 2000 to 2018 in this retrospective study. Before 2010, patients underwent laminoplasty without maintaining the integrity of PLC (laminoplasty group, n=155). After 2010, patients performed single-port laminoplasty to maintain the integrity of PLC (laminoplasty retain posterior ligament complex group, n=63). The score of quality of life, painful cortex, spinal cord movement, progressive kyphosis or scoliosis, perioperative morbidity, and neurological results were analyzed in the laminoplasty group and laminoplasty retain posterior ligament complex group. The distributed variable was shown as mean ± standard deviation and an independent t-test or one-way analysis of variance was calculated. There are 155 patients (71.1%) included in the laminoplasty group, and 63 patients (28.9%) in the laminoplasty retain posterior ligament complex group. The average age of patients was 42 ± 2.3 years, and the average modified McCormick score was 2. There were 158 (72.4%) patients with intramedullary tumors and 115 (52.7%) patients with extramedullary tumors. The length of hospital stays (8 days vs. 6 days; p=0.023) and discharge to inpatient rehabilitation (48.4% vs. 26.9%; p=0.012) were significantly lower in the laminoplasty retain posterior ligament complex group than the laminoplasty group. There was no significant difference in the risk of progressive deformity between the two groups at 18months after surgery (relative risk 0.12; 95% confidence interval [CI] 0.43-1.25; p=0.258) and at 20months after surgery (relative risk 0.24; 95% CI 0.21-2.1). Laminoplasty retains posterior ligament complex showed no impact on the spinal deformities compared with laminoplasty, but significantly improved the postoperative spinal activity, alleviated pain symptoms, and reduced hospital recovery time.

Intradural extramedullary tumors. Retrospective cohort study assessing prognostic factors for functional outcome in adult patients

ObjectiveThe purpose of this study is to analyze a series of patients with intradural extramedullary tumors (IDEM) and assess factors that may modify or determine the final long term outcome and management. Materials and methodsSingle Center, retrospective study of a series of surgical patients with IDEM lesions from our Institution operated between 2010 and 2021. Patients with less than 6 months of follow up were excluded. Several preoperative demographics, clinical, imaging and surgical features, as well as histopathology, recurrence and adjuvancy were assessed. Patients’ final clinical outcome was categorized using the McCormick scale. ResultsA total of 203 patients with a mean follow-up of 30.50 months (range 6–130) were included. 57.64% of the analyzed population was female and the mean age was 50.51 years.The most frequent location of the tumors was dorsal (34.98%) followed by the lumbar region (32.02%). Total resection was achieved in 84.24% of cases, and the most frequent histopathology was Schwannoma (36.45%), followed by Meningioma (30.05%). Pain was the most usual initial symptom (63.05%).In our analysis, functional outcome after surgery was associated with statistical significance with preoperative McCormick grade, tumor type, EOR and postoperative complications such as hematoma and sphincter involvement. ConclusionThe management of these lesions depends on many factors. It is worthy of mention that clinical presentation, EOR, histopathology and postoperative complications have shown significant prognostic value for the final outcome. Early treatment with the intention of achieving GTR when possible, using carefully tailored approaches, should be considered before the onset of significant symptoms.

Myeloid Sarcoma: Current Approach and Treatment Strategies

Myeloid sarcoma (MS) is a rare extramedullary tumor of immature myeloid cells with associated tissue damage. Although MS can manifest as isolated form without blood and marrow involvement, in the majority of the cases it is associated with acute myeloid leukemia (AML). Rarely, it may present in other myeloid disorders. It should, therefore, be considered in a differential diagnosis of any atypical soft tissue mass. MS can develop at any site, resulting in varied clinical manifestations. Identification and diagnosis of MS is highly challenging and depends on a high index of suspicion as well as on imaging, histopathology, immunophenotyping, and genetic analyses. Owing to the lack of prospective clinical trials, there is not enough data in the field to develop a consensus therapeutic regimen for MS. Majority of the patients with MS, including isolated MS, respond to upfront systemic chemotherapy using AML-like regimens that should be commenced early. Surgical resection and/or radiation therapy can be employed for symptomatic lesions or tumors causing local organ dysfunction or obstruction. Allogeneic hematopoietic stem cell transplantation has demonstrated promising results as consolidation therapy after complete remission with induction chemotherapy. Recent development in sequencing analysis has provided significant insights into the development of novel targeted therapies. At present, there are no validated prognostic factors for MS which can help in risk stratification of patients and treatment planning. As there is limited knowledge regarding the clinical approach and management strategies of MS, this review aims to update current knowledge about MS.

Cervical ganglioneuroma arising from the dorsal root ganglion: illustrative case

Ganglioneuroma is a benign and well-differentiated tumor derived from neural crest cells, which occurs infrequently, with most patients being female and adolescents. While predilection sites are the posterior mediastinum and retroperitoneal cavity, ganglioneuroma originating from the dorsal root ganglion is very rare. Here the authors report a case with C2 dorsal root ganglion-derived ganglioneuroma with some literature review. A 45-year-old male patient complained of persistent right-side throbbing occipital headache for more than a year. Magnetic resonance imaging (MRI) of the cervical spine revealed a dumbbell-shaped intradural extramedullary tumor from the C2 posterior surface of the odontoid to right C1-2 intervertebral foramen with high T2- and low T1-weighted signal intensities. The tumor displayed homogeneous contrast enhancement by MRI. The authors suspected schwannoma and performed a tumorectomy for both diagnosis and treatment purposes. Intraoperative findings showed that the tumor originated from the dorsal root ganglion, and pathological examination revealed ganglioneuroma. Immediately after the tumorectomy, the throbbing occipital headache disappeared and the patient was discharged from the hospital without major complications. Although ganglioneuroma derived from the dorsal root ganglion is very rare, a differential diagnosis of the ganglioneuroma should be made, when schwannoma is suspected.

Intradural extramedullary tumor location in the axial view affects the alert timing of intraoperative neurophysiologic monitoring.

Intraoperative neurophysiologic monitoring (IONM) reportedly helps prevent postoperative neurological complications following high-risk spinal cord surgeries. There are negative and positive reports about using IONM for intradural extramedullary (IDEM) tumors. We investigated factors affecting alerts of IONM in IDEM tumor surgery. We analyzed 39 patients with IDEM tumors who underwent surgery using IONM at our hospital between January 2014 and March 2021. Neurological symptoms were evaluated pre- and postoperatively using the manual muscle test (MMT). All patients were evaluated to ascertain the tumor level and location in the axial view, the operative time, intraoperative bleeding volume, and histological type. Additionally, the intraoperative procedure associated with significant IONM changes in transcranial electrical stimulation muscle-evoked potential was investigated. There were 11 false-positive and 16 true-negative cases. There was one true-positive case and one false-negative case; the monitoring accuracy achieved a sensitivity of 50%, a specificity of 59%, a positive predictive value of 8%, and a negative predictive value of 94%. In the 22 alert cases, if the tumor was located anterolateral in the axial view, alerts were triggered with a significant difference (p = 0.02) during tumor resection. Alerts were generated for fifteen patients during tumor resection; nine (60%) showed waveform improvement by intervention and were classified as rescue cases. Alert is probably triggered during tumor resection for anterolaterally located tumors. Alerts during tumor resection procedures were more likely to be rescued than other procedures in IDEM tumor surgery.

MRI Evaluation of Intradural Extramedullary Spinal Tumors with Histopathological Correlation - A Cross Sectional Study

Introduction: Tumors of nerve sheath, neurofibroma and schwannoma represent 30% of intradural extramedullary tumors. Schwannomas and neurofibromas are histologically composed of Schwann cells. Schwannomas almost always arise from the dorsal sensory roots and form well- encapsulated firm masses that compress adjacent tissue, without invading the involved nerve. Neurofibromas are more complex and are composed of Schwann cells mixed with fibroblast they involve the parent nerve. Meningiomas comprise approximately 25% of primary intraspinal tumors and are second only to nerve sheath tumors in frequency. Spinal cord meningiomas are originate from meningothelial cells near the distal root ganglia. Most menongiomas are intradural extramedullary, only 5% are extradural. Aim of the Study: The aim of this study was to evaluate the role of MRI in diagnosis of intradural extramedullary spinal tumors compared to histopathological findings. Methods: This was a cross sectional study and was conducted in the Department of Radiology and Imaging, Sylhet M.A.G. Osmani Medical College, Sylhet in collaboration with Department of Neurosurgery &amp; Pathology of the same hospital, Sylhet, Bangladesh during the period from September, 2018 to August 2020. Result: In total 35 patients completed the study. In our study we found the Mean ± SD of age was 41.71±13.9 in years. Majority (54%) of our patients presented with spinal tumor were male compared to female (46%).We found schwannoma was diagnosed by (42.9%), meningioma (31.4%), neurofibroma (25.7%) respectively. Neurofibroma &amp; schwannoma showed male predominance with 66.67% &amp; 60% while meningioma showed female predominance with 63.6%. We found majority (55.6%, 60% &amp; 72.7%) of neurofibroma, schwannoma &amp; meningioma was in dorsal region respectively. Conclusion: In our study we found that MRI is a very sensitive and effective imaging procedure of suspected spinal schwannoma tumor of the spinal cord for accurate pre-operative diagnosis and ........

Clinical evaluation of paraspinal mini-tubular technique vs. laminoplasty for spinal intradural extramedullary tumors: Study protocol for a multicenter, randomized controlled trial.

ChiCTR2100047582.

Spinal oncologic paraparesis: Analysis of neurological and surgical outcomes in patients with intramedullary, extramedullary, and extradural tumors.

Paraparesis due to oncologic lesions of the spine warrants swift neurosurgical intervention to prevent permanent disability and hence maintain independence of affected patients. Clinical parameters that predict a favorable outcome after surgical intervention could aid decision-making in emergency situations. Patients who underwent surgical intervention for paraparesis (grade of muscle strength <5 according to the British Medical Research Council grading system) secondary to spinal neoplasms between 2006 and 2020 were included in a single-center retrospective analysis. Pre- and postoperative clinical data were collected. The neurological status was assessed using the modified McCormick Disability Scale (mMcC) Score. In a univariate analysis, patients with favorable (discharge mMcC improved or stable at <3) and non-favorable outcome (discharge mMcC deteriorated or stable at >2) and different tumor anatomical compartments were statistically compared. 117 patients with oncologic paraparesis pertaining to intramedullary lesions (n=17, 15%), intradural extramedullary (n=24, 21%) and extradural lesions (n=76, 65%) with a mean age of 65.3 ± 14.6 years were included in the analysis. Thoracic tumors were the most common (77%), followed by lumbar and cervical tumors (13% and 12%, respectively). Surgery was performed within a mean of 36±60 hours of admission across all tumors and included decompression over a median of 2 segments (IQR:1-3) and mostly subtotal tumor resection (n=83, 71%). Surgical and medical complications were documented in 9% (n=11) and 7% (n=8) of cases, respectively. The median hospital length-of-stay was 9 (7-13) days. Upon discharge, the median mMcC score had improved from 3 to 2 (p<0.0001). At last follow-up (median 180; IQR 51-1080 days), patients showed an improvement in their mean Karnofsky Performance Score (KPS) from 51.7±18.8% to 65.3±20.4% (p<0.001). Localization in the intramedullary compartment, a high preoperative mMcC score, in addition to bladder and bowel dysfunction were associated with a non-favorable outcome (p<0.001). The data presented on patients with spinal oncologic paraparesis provide a risk-benefit narrative that favors surgical intervention across all etiologies. At the same time, they outline clinical factors that confer a less-favorable outcome like intramedullary tumor localization, a high McCormick score and/or bladder and bowel abnormalities at admission.

Imaging findings of an extramedullary epidural metastatic small cell lung cancer tumor: a case report

Background: Extramedullary epidural metastatic tumors of small cell lung cancer (SCLC) are rare, and their clinical symptoms and imaging features lack specificity. This study was aimed at improving understanding of epidural metastatic SCLC tumors. Case report: We present the case of a 75-year-old patient with an extramedullary epidural metastatic SCLC tumor that was misinterpreted as a primary intraspinal tumor according to preoperative CT and MRI resonance imaging. Laboratory test results for CA-153 (28.30 U/mL) were substantially abnormal. A solid, well-defined, soft tissue mass approximately 0.3 cm × 1.5 cm in diameter at the seventh and eighth thoracic canals was observed on CT and MRI images. A dural tail sign was observed on contrast-enhanced magnetic MRI. Because the tumor compressed the spinal cord, the intraspinal mass was resected, and the vertebral canal was decompressed. Pathological examination confirmed the diagnosis of an extramedullary epidural metastatic SCLC tumor. Conclusions: Extramedullary epidural metastatic SCLC tumors lack clinical specificity. Imaging is helpful for early diagnosis, treatment, prediction of the disease course, and evaluation of curative effects. Ultimately, pathological examination and biopsy are required to confirm the diagnosis.

Spinal Intradural Extramedullary Tumors: A Retrospective Analysis on Ten-Years' Experience of Minimally Invasive Surgery and a Comparison with the Open Approach.

Spinal intradural extramedullary (ID-EM) tumors are pathologies widely treated through a classical open approach. However, minimally invasive surgery is gaining traction as a comparable treatment option because it carries less morbidity and may reduce healthcare costs.This study aimed to compare the clinical and functional outcomes of open versus minimally invasive approaches for patients with ID-EM tumors. We performed a retrospective analysis on prospectively collected data from patients with ID-EM tumors submitted to surgery. Baseline features and operative variables were compared, including surgery duration and estimated blood loss (EBL). Postoperative data covered tumor histology, length of stay (LOS), complication(s), and neurological status (Medical Research Council (MRC) scale) at the last follow-up.In total, 46 patients were included: 30 (65.2%) operated through an open approach and 16 (34.8%) through a minimally invasive surgical (MIS) approach. The predominant histology type was schwannomas (43.5%). Lesions more frequently affected the lumbar spine (34.8%). The tumor dimensions were similar in both cohorts. The minimally invasive approach was on average 76.7 min faster and correlated positively with less EBL (140 mL less than that of the open approach). Patients in the MIS group had shorter LOSs (5.63 days vs. 17.27 days) and had fewer postoperative complications. No significant difference in functional outcome was found.MIS is as effective as the traditional approach in achieving comparable functional outcomes, with advantages such as shorter surgery durations, less blood loss, and shorter hospital LOSs.

Extramedullary spinal tumors: Cross-sectional study of the demographic characteristics and treatment outcomes of surgically treated patients from Isfahan (Iran)

Extramedullary spinal tumors: Cross-sectional study of the demographic characteristics and treatment outcomes of surgically treated patients from Isfahan (Iran)

The potential for further exploration of extramedullary spinal tumors in Iranian patients.

The potential for further exploration of extramedullary spinal tumors in Iranian patients.

Minimally Invasive Resection of Intradural Extramedullary Tumors Using Tubular Retractors.

Minimally Invasive Resection of Intradural Extramedullary Tumors Using Tubular Retractors.

25, 50 and 75 years ago

25, 50 and 75 years ago

Minimally Invasive Resection of Intradural Extramedullary Tumors Using Tubular Retractors.

Minimally Invasive Resection of Intradural Extramedullary Tumors Using Tubular Retractors.