Factors influencing the development of recurrence and continued growth of primary extramedullary tumors removed using Nd:YAG laser

Objective. To assess the significance of clinical factors of extramedullary tumors and new methods of their resection as potential predictors of their recurrence and continued growth.Material and Methods. The long-term results of removal of primary extramedullary tumors in 412 patients operated on in 1998–2014 were analyzed comparing the use of standard methods of microsurgical technique for tumor removal (277 patients) and of those with additional use of neodymium laser radiation (135 patients).Results. The use of laser technologies for resection of extramedullary tumors can significantly reduce the number of their recurrences and continued growth, along with other clinical factors is a significant prognostic indicator in determining the nature of the disease course and can be a predictor of their occurrence. The most reliable clinical factors determining the prognosis of a decrease in the incidence of recurrences and continued growth when using laser techniques of surgical resection were repeated operations (p = 0.002), the presence of ependymomas of the cone and cauda equina (p = 0.017), operations for primary tumors in the thoracic spine (p = 0.039) and extramedullary tumors with Grade I anaplasia (p = 0.007). An increase in the number of these conditions was associated with operations on the cervical spine (p = 0.027), the presence of a tumor with Grade II anaplasia (p = 0.007), and a primary extramedullary tumor involving more than three vertebrae (p = 0.017).Conclusion. The use of the laser is indicated for reoperations when removing neoplasms, that have arisen as a result of recurrence or continued growth of extramedullary tumors of any level and length after removal of primary neoplasms with a Grade I malignancy confirmed by intraoperative cytological examination involving no more than three vertebrae in the thoracic, lumbar and sacral spine and during resection of ependymomas with extramedullary growth.

Spinal metastasis of parotid acinic cell carcinoma followed by intradural extramedullary recurrence: illustrative case

BACKGROUND The diagnosis and management of acinic cell carcinoma (ACC) is often challenging given its similarity to benign tumors, high incidences of late recurrence and distant metastasis, and tendency to be resistant to systemic chemotherapy. A primary parotid ACC resulting in an intradural extramedullary mass has not been reported. OBSERVATIONS The authors describe such a case that presented as a progressive cervical myelopathy 29 years after initial diagnosis. The tumor, located at the C2–C3 level, infiltrated the dura and contained both extradural and intradural components. This occurred 18 months after the incomplete resection of an extradural metastasis at the same location. LESSONS Although intracranial and extradural metastases of various primary malignancies are well reported, secondary spinal intradural malignancies are rare. As a result, there are no established guidelines for the surgical management of intradural extramedullary metastases and prognosis may be difficult to establish. In this case, treatment options were limited because systemic therapy options had been exhausted and repeated radiation to the area was not recommended. We report on this case to highlight the clinical course of a rare local recurrence after spinal metastasis leading to an intradural extramedullary tumor and to show that surgical intervention can lead to improvement of neurological symptoms.

Spine Myeloid Sarcoma: A Case Series with Review of Literature

Myeloid sarcoma (MS) is a malignant extramedullary tumor consisting of immature cells of myeloid origin. It may precede, present concurrently or follow acute myeloid leukemia (AML) in de novo case or may also be present and might be the only manifestation of recurrent AML, myelodysplastic syndrome, or chronic myeloid leukemia. It frequently involves skin, orbit, bone, periosteum, lymph nodes, and gastrointestinal tract, soft tissue, central nervous system, and testis. Because of its different localization and symptoms, and the lack of diagnostic algorithm, MS is a real diagnostic challenge particularly in patients without initial bone marrow involvement. The correct diagnosis of MS is important for optimum therapy, which is often delayed because of a high misdiagnosis rate. We reported three cases of MS derived from spine presented with back pain, paraplegia, paraparesis, respectively, and reviewed the relevant literature.

MNG-2 Meningioma with aseptic meningitis

The patient, a woman in her seventies, visited the Department of Neurology at our hospital one month ago with transient right hemiparesis, and was referred to our department because a CT scan showed a 4cm extramedullary lesion in the left convexity. She was judged to have symptomatic epilepsy associated with the lesion and was started on antiepileptic drugs. The lesion showed low signal on T1WI, equal signal on T2WI, and homogeneous contrast on Gd contrast T1WI, suggesting a meningioma, but the surrounding left frontal lobe subarachnoid space was also contrasted, suggesting the possibility of seeding or other diseases. After that, the contrast area of the subarachnoid space increased in a short period of time, and the control of epileptic seizures was poor. Preoperative spinal fluid examination showed an elevated cell count and findings of aseptic meningitis. A left parietal craniotomy was performed to remove the extramedullary tumor as much as possible. The subarachnoid space of the left frontal lobe adjacent to the tumor was covered with extensive pale yellow apparently abscess-like tissue. The pathological diagnosis of the extramedullary tumor was angiomatous meningioma (WHO Grade 1), and the pale yellow tissue that filled the subarachnoid space was necrotic tissue containing neutrophils and no tumor component. IgG4 was positive in about 10% of the tumor. The postoperative course of the patient was good, the contrast area of the left frontal lobe subarachnoid space was reduced on MRI, aseptic meningitis was improved, and she was discharged home with no neurological deficits. The patient has been under outpatient observation for 2 years without recurrence of aseptic meningitis or appearance of contrast-enhancing lesions in the subarachnoid space. This case is thought to be a possible IgG4-related disease, and we report it with a discussion of the literature.

Myeloid Sarcoma Type of Acute Promyelocytic Leukemia With a Cryptic Insertion of RARA Into FIP1L1: The Clinical Utility of NGS and Bioinformatic Analyses

BackgroundAcute promyelocytic leukemia (APL) is characterized by the presence of coagulopathy at onset and translocation t (15; 17) (q22; 21), meanwhile, other translocation variants of APL have also been reported. The FIP1L1–RARA fusion gene has recently been reported as a novel RARA-associated fusion gene.ObjectivesWe report a case of de novo myeloid sarcoma (MS) type of APL with FIP1L1–RARA found by next-generation sequencing (NGS) that was not detected by conventional analyze analysis for RARA translocations.MethodsWe performed typical morphological, magnetic resonance imaging (MRI), conventional tests for PML–RARA dual-fusion translocation probe, high-through sequencing and NGS. Meanwhile, bioinformatics analyses were done by using public repositories, including ONCOMINE, COSMIC, and GeneMANIA analysis.ResultsA 28-month-old girl with a complex karyotype that includes 46,XX,t(4;17)(q12;q22)[9]/46,idem,del(16)(q22)[3]/45,idem,-x,-4,-9,-15,del(16)(q22),+marl,+mar2,+mar3[7]/46,xx[3], c.38G>A (p.Gly13Asp) in the KRAS gene, and a cryptic insertion of RARA gene into the FIP1L1 gene was diagnosed with APL complicated by the de novo MS.ConclusionWe report a FIP1L1–RARA fusion in a child with APL who presented with an extramedullary tumor in the skull without the classic karyotype using NGS, whom we treated with good results. NGS analysis should be considered for APL variant cases. Further experimental studies to the association between the mutation in KRAS gene and FIP1L1–RARA fusion on the clinical phenotype and progression of APL are needed to identify more effective therapeutic targets for APL.

Laminectomy versus Unilateral Hemilaminectomy for the Removal of Intraspinal Schwannoma: Experience of a Single Institution and Review of Literature

Background and Study Aims Spinal schwannomas are benign slow-growing tumors, and gross total resection is the gold standard of treatment. The conventional surgical approach is laminectomy, which provides a wide working area. Today minimally invasive surgery (MIS) is popular because it is associated with shorter hospital stay, less operative blood loss, minimized tissue traumas and relative postoperative pain, and, and spine surgery, avoidance of spinal instability. Material and Methods From January 2016 to December 2019, we operated on 40 patients with spinal intradural extramedullary tumor (schwannoma) with laminectomy or hemilaminectomy. Baseline medical data, including patients' sex and age, tumor location, days of postoperative bed rest, operative time, length of hospitalization, and 1-month visual analog scale (VAS) value were collected and analyzed. Data analysis was performed using STATA/IC 13.1 statistical package (StataCorp LP, College Station, Texas, United States). Results Hemilaminectomy was associated with faster operative time (p < 0.001), shorter postoperative time spent in bed (p < 0.001), and shorter hospitalization (p < 0.001). At 1-month follow-up, the mean VAS score was 4.6 (1.7) among the laminectomy patients and 2.5 (1.3) among the hemilaminectomy patients (p < 0.001). Postoperative complications occurred in 1 (7.7%) and 7 (25.9%) patients in the hemilaminectomy and laminectomy groups, respectively (p = 0.177). Conclusions Unilateral hemilaminectomy has significant advantages compared with laminectomy in spinal schwannoma surgery including shorter operative time, less time spent in bed, shorter hospitalization, and less postoperative pain.

Hypermutated phenotype in gliosarcoma of the spinal cord

Gliosarcoma is a variant of glioblastoma with equally poor prognosis and characterized by mixed glial and mesenchymal pathology. Metastasis is not uncommon but the involvement of the spinal cord is rare, and comprehensive genetic characterization of spinal gliosarcoma is lacking. We describe a patient initially diagnosed with a low-grade brain glioma via biopsy, followed by adjuvant radiation and temozolomide treatment. Nearly 2 years after diagnosis, she developed neurological deficits from an intradural, extramedullary tumor anterior to the spinal cord at T4, which was resected and diagnosed as gliosarcoma. Whole-exome sequencing (WES) of this tumor revealed a hypermutated phenotype, characterized by somatic mutations in key DNA mismatch repair (MMR) pathway genes, an abundance of C>T transitions within the identified somatic single nucleotide variations, and microsatellite stability, together consistent with temozolomide-mediated hypermutagenesis. This is the first report of a hypermutator phenotype in gliosarcoma, which may represent a novel genomic mechanism of progression from lower grade glioma.

Primary Ewing’s Sarcoma of the Lumbosacral Spine: A Case Report

Ewing's sarcoma is a rare tumor first discovered by James Ewing in 1921. It is more common in bone or skeletal component compared to soft tissue or extraosseous Ewing's sarcoma. Among soft tissue Ewing’s, spinal cord involvement is rarer with only nine cases reported. We report a case of nine-year-old Malay girl who presented with low back pain for two months following a fall with progressive neurological deficits of bilateral lower limb. Magnetic resonance imaging was suggestive of a well-defined margin of intradural extramedullary tumor. With nerve sheath tumor in mind, surgical excision with laminectomy L2-S1 was performed. Intraoperative finding was an extradural mass from L3-L5 with extension to bilateral neuroforamen. Histopathology report defined a round cell tumour of Ewing’s sarcoma from the mass.