Autonomic dysreflexia in patients with cancer and spinal cord injury: a case series.

INTRODUCTION: Autonomic dysreflexia (AD) is a potentially life-threatening syndrome that can occur in patients with traumatic injury to the spinal cord; however, it has not been well described in patients with non-traumatic spinal cord injury (SCI) from cancer and its treatments. CASE PRESENTATION: We report four cases of autonomic dysreflexia secondary to primary spinal cord tumors and metastatic disease to the spine, and as sequela to cancer treatment. The clinical characteristics, diagnostic considerations, and therapeutic strategies used to mitigate the symptoms are discussed. DISCUSSION: The case series shows that AD is a rare but important condition to consider among patients with cancer-related SCI. There is a need for close monitoring and early identification of this syndrome in this population. Therapeutic strategies are available to mitigate these symptoms and risks of complications.

Decoding pediatric spinal tumors: a single-center retrospective case series on etiology, presentation, therapeutic strategies, and outcomes.

INTRODUCTION: Spinal tumors (ST) often result in dire prognosis, carrying risks such as permanent paralysis, sensory loss, and sphincter dysfunction. Data on their incidence and etiology in pediatric populations are markedly scant. Our study investigates the etiology, clinical manifestation, treatment, and outcomes of pediatric ST. METHODS: We conducted a retrospective review of our institutional pediatric oncology and neurosurgery database, examining 14 patients under 18 years admitted with ST due to oncological diseases since 2005. We analyzed the clinical presentations, evaluations, molecular diagnostics and treatments for these patients. RESULTS: The study spanned 15 years and included 14 pediatric patients, each diagnosed with distinct spinal tumor entity. The mean patient age was approximately 19.6 ± 10.1 months. Severe axial pain along the vertebral column was observed in 13 patients, while acute neurological deterioration manifested in 7 patients. As a first-line intervention, 13 patients underwent decompressive surgery through laminectomy and tumor resection, and only one patient received chemotherapy solely. Before surgery, seven patients were unable to walk; post-surgery, six of them regained their ability to ambulate. The diagnosis encompassed a range of neoplasms: two instances of Ewing sarcoma, 3 instances of teratoma, one case presenting an atypical teratoid Rhabdoid tumor, two instances each of low-grade astrocytoma and neuroblastoma, and single instances of ependymoma, meningioma, rhabdomyosarcoma, and embryonal tumors with multilayered rosettes (ETMRs). Three patients succumbed two years after initiating therapy. CONCLUSION: Despite their rarity, intraspinal tumors in pediatric patients pose substantial therapeutic challenges. The intertwined complexities of the disease entity and the patient's neurological status demand swift initiation of an individualized therapeutic strategy. This crucial step helps optimize outcomes for this patient cohort, who frequently grapple with debilitating health conditions. Inclusion of these patients within a registry is mandatory to optimize treatment outcomes due to their rarity in pediatric population.

Spinal Atypical Teratoid Rhabdoid Tumor in a 14-Year-old Child With Down Syndrome: A Case Report.

Individuals with 21 trisomy or Down syndrome (DS) are known to have an increased risk of acute leukemia, while they rarely develop solid or central nervous system (CNS) tumors. Atypical teratoid rhabdoid tumor (ATRT) is a highly aggressive CNS-WHO grade 4 neoplasm, which has never been reported in association with Down syndrome. We present a case study of a 14-year-old female with Down syndrome, diagnosed with intradural-extramedullary spinal ATRT. The chief complaints included bilateral lower limb weakness, constipation, and urinary incontinence for 2 weeks. Surgery was scheduled, and a biopsy was taken. The histopathology, immunohistochemistry, and molecular analysis confirmed the diagnosis of the ATRT-MYC/group 2B subgroup. This report highlights the challenges of managing a patient with complex medical conditions. Moreover, it adds to the existing literature on CNS tumors in patients with Down syndrome.

Unusual case of spinal cord compression revealed a double primary Hodgkin's lymphoma of the spinal cord.

Hodgkin's lymphoma revealed by a medullary compression with a double primary vertebral localisation is extremely rare. We report the case of a boy in middle childhood who was presented with slow progression of medullary compression syndrome over 9 months, ultimately leading to paraplegia with loss of sphincter tone. The spinal MRI showed two tumour processes at T9 and L1 with epidural extension. An anatomical-pathological examination of the biopsy of the tumour mass, along with immunohistochemical analysis, confirmed the diagnosis of a lymphocyte-rich classic Hodgkin's lymphoma, stage IV according to the Ann Arbor classification. The therapeutic strategy was based on chemotherapy. This study aims to report a unique clinical presentation of Hodgkin's lymphoma in a paediatric patient and underscores the diagnostic challenges encountered in such an uncommon scenario.

Simultaneous L1-2 Bulged Disc and Mobile Spinal Schwannoma Causing Cauda Equina Syndrome: A Rare Case Report.

BACKGROUND Aside from the rarity of mobile spinal schwannomas, the coexistence of these tumors with herniated intervertebral disc is also scarce. Furthermore, cauda equina syndrome (CES), as a manifestation of intraspinal schwannomas has been reported rarely. Described here is a case of simultaneous lumbar disc bulge and mobile spinal schwannoma presented with intermittent symptoms of CES. CASE REPORT A 62-year-old man presented with severe but intermittent leg pain for 2 weeks, which later progressed to an episode of lower extremity weakness and difficulty in urination. Magnetic resonance imaging revealed an intraspinal tumor that moved in position relative to the L1-2 disc bulge on scans 6 h apart, with associated spontaneous regression in symptoms. The tumor was found to be a mobile spinal schwannoma, originated from a nerve root. A standard microdissection technique was used to remove the tumor through a spinous process-sparing unilateral approach, with complete laminectomy of L1. Use of intraoperative ultrasound facilitated the accurate tumor localization. Postoperatively, the patient no longer had symptoms. CONCLUSIONS This report presents a combination of a common spinal pathology, intervertebral disc herniation, alongside a rare condition, mobile spinal schwannoma, whose uncommon clinical manifestations, such as CES can cause irreversible neurological deficits. Surgeons need to remain vigilant of potential atypical scenarios when treating patients. Surgical treatment challenges regarding the mobility of tumors, such as accurate localization, should be addressed using intraoperative imaging to avoid wrong-level surgery. To mitigate the irreversible neurological complications, patients should receive comprehensive information for alarming signs of CES.

Acontractile detrusor as an initial presentation of sacral spinal cord lesion: Case series.

OBJECTIVES: This study aims to investigate cases of acontractile bladder as the initial presentation of benign and malignant spinal conditions. The focus is on the challenges in making a diagnosis and the importance of a thorough neurological evaluation. METHODS: We conducted a retrospective case series involving three patients who exhibited symptoms of acontractile bladder. Detailed clinical histories, urodynamic studies, and imaging techniques such as lumbosacral magnetic resonance imaging (MRI) were analyzed. Histopathological findings from relevant biopsies were also taken into account. RESULTS: Case 1: A 14-year-old female presented with urinary retention, back pain, and an acontractile bladder on urodynamic study. Further examination, including lumbosacral MRI and histopathology, confirmed a diagnosis of metastatic Ewing's Sarcoma. Case 2: A 39-year-old female with urinary incontinence and elevated post-void residual exhibited delayed bladder sensation. Lumbar spine MRI revealed a grade I Schwannoma after surgical resection. Case 3: A 15-year-old male with lower urinary tract symptoms and an acontractile detrusor on urodynamic study was found to have a Tarlov cyst on lumbosacral MRI. CONCLUSION: Atonic or Underactive bladder syndrome may be the initial presentation of a serious spinal condition. Complete neurological evaluation is mandatory if no obvious clinical cause.

Intraoperative View of a Multinodal, Paralysis-Inducing Spinal Melanocytoma: Case Report and Literature Review.

Melanocytomas arising from the leptomeningeal melanocytes within the central nervous system are a rare occurrence, accounting for 0.06%-0.1% of brain tumors and having an incidence of 1/10 million people per year.1-14 Here, we describe the case of 68-year-old male presenting with bilateral lower extremity weakness progressing to paralysis and urinary incontinence (Video 1). Upon examination, this gentleman had no sensation below T11. Magnetic resonance imaging showed multiple contrast-enhancing lesions with a major intradural lesion at level T11 arising from the ventrolateral surface and causing severe spinal cord compression. The multifocal nature of this tumor further adds to its rarity. Interdisciplinary indication for surgical resection of the intradural lesion was made. This was accomplished through a T11 laminectomy and concomitant T11-12 stabilization with neuromonitoring. Pathologic analysis of the resected tumor identified an S100+, HMB45+, pigmented melanocytoma. No complications occurred during the procedure. The patient was discharged to rehabilitation with persistent neurologic deficits. Routine follow-up is indicated given the high rates of recurrence and the multiple remaining tumor nodules.14.

Is perioperative blood transfusion associated with postoperative thromboembolism or infection after metastatic spinal tumor surgery?

STUDY DESIGN: Retrospective cohort. SUMMARY OF BACKGROUND DATA: Patients with metastatic spine disease who undergo surgical intervention have a high risk of requiring red blood cell (RBC) transfusion. Perioperative transfusion has been independently associated with increased risk of venous thromboembolic (VTE) and infectious complications following orthopedic procedures and degenerative spinal intervention; however, literature within spine oncology is limited. OBJECTIVE: To determine the association between perioperative RBC transfusion and postoperative VTE or infection following spinal tumor surgery. METHODS: A total of 153 patients who underwent surgery for spinal metastases between April 2012 and April 2022 were included. Medical records were reviewed to identify RBC transfusion administered either intraoperatively or within 96 h following surgery. The primary endpoints were: 1) development of any VTE or 2) development of any infection within 30 days following surgery. Any VTE was defined as deep vein thrombosis or pulmonary embolism, and any infection was defined as pneumonia, meningitis, Clostridium difficile infection, urinary tract infection, surgical site infection, or sepsis. Logistic regression analyses were performed. RESULTS: Of the 153 patients included in the study, 43 % received a perioperative RBC transfusion. The overall incidence of postoperative VTE and infection was 15 % and 22 %, respectively. In univariate analysis, perioperative transfusion was not associated with postoperative VTE (odds ratio [OR] 2.41; 95 % confidence interval [CI] 0.97-6.00; p = 0.058) but was associated with infection (OR 3.02; 95 % CI 1.36-6.73; p = 0.007). After adjusting for confounders such as performance status, operative time, and surgical extent, transfusion was not associated with both VTE (OR 1.25; 95 % CI 0.36-4.32; p = 0.727) or infection (OR 1.86; 95 % CI 0.70-4.92; p = 0.210). While not statistically significant, sub-analyses demonstrated a trend towards increased VTE incidence in patients requiring transfusion earlier (within 24 h) as opposed to later postoperatively. CONCLUSIONS: We found that perioperative transfusion was not an independent predictor of 30-day postoperative VTE or infection in patients undergoing metastatic spinal surgery. Further exploration of time-dependent transfusion outcomes is warranted.

Sacral ependymoma presents 20 years after initial posterior fossa lesion.

Posterior fossa ependymomas (PFEs) are designated histologically as low-grade neoplasms. Despite being characterised as benign, cases of metastasis have been reported only a few times with the patients concurrently diagnosed with the primary tumour. Interval drop metastasis or spontaneous second distal tumours are extremely rare and, in most cases, are diagnosed within a few months of primary tumour resection. Here, we report a patient with a grade 2 paediatric PFE exhibiting a 20-year interval to a second sacral ependymoma. The patient was initially diagnosed with a PFE at the age of 10 years and underwent tumour resection and postoperative radiotherapy. In their late 20s, the patient presented with basilar artery occlusion complicated by life-threatening epistaxis. Post-thrombolysis, the patient presented with a large sacral grade 1 myxopapillary ependymoma with cauda equina syndrome-like symptoms. Here, we present a rare case of two ependymomas with a 20-year interval in the same patient with compounding comorbidities.

Neurological Outcome and Respiratory Insufficiency in Intramedullary Tumors of the Upper Cervical Spine.

Background and Objectives: Intramedullary spinal cord tumors (IMSCT) are rare entities. A location in the upper cervical spine as a highly eloquent region carries the risk of postoperative neurological deficits, such as tetraparesis or respiratory dysfunction. Evidence for respiratory dysfunction is scarce. This study aimed to describe these highly eloquent tumors' early and late postoperative clinical course. Materials and Methods: This is a single-center retrospective cohort study. We included 35 patients with IMSCT at levels of the craniocervical junction to C4 who underwent surgical treatment between 2008 and 2022. The authors analyzed the patients' preoperative status, tumor- and surgery-specific characteristics, and follow-up functional status. Results: The study cohort included twenty-two patients with grade II ependymoma (62.9%), two low-grade astrocytomas (5.7%), two glioblastomas (5.7%), six hemangioblastomas (17.1%), two metastases (5.7%), and one patient with partially intramedullary schwannoma (2.9%). Gross total resection was achieved in 76% of patients. Early dorsal column-related symptoms (gait ataxia and sensory loss) and motor deterioration occurred in 64% and 44% of patients. At a follow-up of 3.27 ± 3.83 years, 43% and 33% of patients still exhibited postoperative sensory and motor deterioration, respectively. The median McCormick Scale grade was 2 in the preoperative and late postoperative periods, respectively. Only three patients (8.6%) developed respiratory dysfunction, of whom, two patients, both with malignant IMSCT, required prolonged invasive ventilation. Conclusions: More than 60% of the patients with IMSCT in the upper cervical cord developed new neurological deficits in the immediate postoperative period, and more than 40% are permanent. However, these deficits are not disabling in most cases since most patients maintain functional independence as observed by unchanged low McCormick scores. The rate of respiratory insufficiency is relatively low and seems to be influenced by the rapid neurological deterioration in high-grade tumors.

Does the degree of preoperative gait disturbance remain after tumor resection in patients with intradural extramedullary spinal cord tumors?

STUDY DESIGN: Retrospective comparative study. OBJECTIVE: This study aimed to determine whether the degree of preoperative gait disturbance remains following surgical resection in patients with intradural extramedullary spinal cord tumors (IDEMSCTs), and to investigate any factors that may influence poor improvement in postoperative gait disturbance. SETTING: The single institution in Japan. METHODS: In total, 78 IDEMSCTs patients who required surgical excision between 2010 and 2019 were included. According to the degree of preoperative gait disturbance using modified McCormick scale (MMCS) grade, they were divided into the Mild and Severe groups. The mean postoperative follow-up period was 50.7 ± 17.9 months. Data on demographic and surgical characteristics were compared between the two groups. RESULTS: There was no significant difference in terms of age at surgery, sex, tumor size, surgical time, estimated blood loss, tumor histopathology, and postoperative follow-up period between the Mild and Severe groups. At the final follow-up, 84.6% of IDEMSCTs patients were able to walk without support. Gait disturbance improved after surgery in most of the patients with preoperative MMCS grades II-IV, but remained in approximately half of patients with preoperative MMCS grade V. Age at surgery was correlated with poor improvement in postoperative gait disturbance in the Severe group. CONCLUSIONS: Regardless of the degree of preoperative gait disturbance, it improved after tumor resection in most of the IDEMSCTs patients. However, in the preoperative MMCS grade III-V cases, older age at surgery would be an important factor associated with poor improvement in postoperative gait disturbance.

Laminectomy with Laminar Reconstruction for Resection of Intradural Tumors at the Thoracolumbar Junction: A Technical Note.

OBJECTIVE: To introduce a new laminar reconstruction technique to treat primary spinal cord tumors. METHODS: Laminectomy and laminoplasty techniques have been used to treat intradural spinal tumors. The advantage of laminectomy is its superior exposure of the spinal cord, whereas the advantage of laminoplasty is the reconstruction of the dorsal roof of the spine. In this technical note, we present a technique that combines a full laminectomy to maximize exposure, with a reconstructive technique to repair the lamina. This technique restores the posterior ligamentous complex to preserve spinal biomechanics. RESULTS: In this illustrative case, a 55-year-old woman with severe back pain radiating to the right lower extremity was found to have an intradural tumor at the T12-L1 spinal level. Given the transitional level of the spine and potentially high biomechanical stresses on the posterior support structures, we used a T12 laminectomy to resect the tumor, followed by reconstruction using miniplates. The patient tolerated the surgery well, without any complications. She was discharged home and was doing well during the 3 months follow-up visit. Appropriate patient consent was obtained. CONCLUSIONS: Laminectomy and laminar reconstruction allow maximum visualization and manipulation of the tumor, followed by restoration of the dorsal roof of the spinal ring, and is an effective technique for treating spinal cord tumors.

Non-dysraphic intramedullary spinal cord lipoma of the child: Report of 3 cases.

BACKGROUND AND PURPOSE: Non-dysraphic intramedullary spinal cord lipomas (NDSCL) represent 1% of spinal cord tumors. They are less frequent than dysraphic spinal cord lipomas and clinical presentation is unspecific. There are no guidelines on surgical management. MATERIAL AND METHODS: We report three observations of NDSCL in children, focusing on the clinical presentation, surgical management and postoperative outcome. RESULTS: The patients, one female and two males, aged from 5 months to 10 years presented with neurological deterioration, pain, spinal rigidity and in two cases, a subcutaneous mass. Spinal MRI found intradural lipomas without spina bifida, located in the cervico-thoracic area in all cases. The lipoma extended to the medulla oblongata in two cases and was in the lumbar region in the third. These lipomas were massive, requiring decompression surgery. Surgery confirmed the lipoma to be subpial. We performed debulking of the lipoma without attempting total resection, and with or without dural plasty and laminoplasty, followed by minerva cast in two cases, and avoidance of standing in the youngest. Satisfactory recovery occurred in all three cases. After a follow-up between 4 months and 9 years, the outcome was favorable in all cases, and no patient presented with secondary spinal deformation or lipoma progression. CONCLUSION: NDSCL is a rare entity, which often manifests with progressive pain and neurological deficits. In our experience, partial resection with or without dural plasty and laminoplasty has been associated with satisfactory postoperative outcomes and no recurrence of symptoms. We should be attentive to the risk of postoperative spinal deformity in these young patients.

Chronic Intramedullary Spinal Cord Abscess Mimicking Intramedullary Spinal Cord Tumor-"Phantom Tumor": A Case Report.

CASE: A 33-year-old woman with back pain and radiculopathy had presented with bilateral ankle weakness. MRI showed an intramedullary conus lesion suggestive of neoplasm, but posterior midline durotomy revealed only pus. Pus samples showed Staphylococcus aureus, which was treated with 6 weeks of antibiotics. Two-year follow-up showed complete neurological recovery with no clinicoradiological signs of recurrence. CONCLUSION: Usually, intramedullary spinal cord abscess (ISCA) has an acute presentation and warrants an emergent line of treatment with a risk of mortality. Very rarely chronic ISCA can mimic intramedullary spinal cord tumor. It is the first case reported in the literature of chronic ISCA mimicking conus IMST.