First incidence of extrarenal wilms tumor within the spinal canal in the adult population: a novel case report and literature review.

BACKGROUND: Wilms tumor (WT), also known as nephroblastoma, is rare in adults, accounting for merely 3% of all nephroblastomas or 0.2 cases per million individuals. Extrarenal Wilms tumor (ERWT) emerges outside the renal boundaries and comprises 0.5 to 1% of all WT cases, with even rarer incidences in adults. Oncogenic mutations associated with ectopic nephrogenic rests (NR) may contribute to ERWT development. Diagnosis involves surgical resection and pathology examination. Due to scarce cases, adults often rely on pediatric guidelines. We thoroughly searched PubMed, Scopus, and Web of Science databases to establish our case's uniqueness. To the best of our knowledge, this is the first documented incidence of extrarenal Wilms tumor within the spinal canal in the adult population. CASE PRESENTATION: A 22-year-old woman with a history of congenital lipo-myelomeningocele surgery as an infant presented with a 6-month history of back pain. This pain gradually resulted in limb weakness, paraparesis, and loss of bladder and bowel control. An MRI showed a 6 × 5 × 3 cm spinal canal mass at the L4-S1 level. Consequently, a laminectomy was performed at the L4-L5 level to remove the intramedullary tumor. Post-surgery histopathology and immunohistochemistry confirmed the tumor as ERWT with favorable histology without any teratomatous component. CONCLUSION: This report underscores the rarity of extrarenal Wilms tumor (ERWT) in adults, challenging conventional assumptions about its typical age of occurrence. It emphasizes the importance of clinical awareness regarding such uncommon cases. Moreover, the co-occurrence of spinal ERWTs and a history of spinal anomalies warrants further investigation.

Structure-sparing resection for the management of cervical chordomas: a retrospective institutional series.

OBJECTIVE: Chordomas are a rare and relatively slow-growing malignancy of notochordal origin with a nearly 50% recurrence rate. Chordomas of the cervical spine are particularly challenging tumors given surrounding vital anatomical structures. Although standard in other areas of the spine, en bloc resection of cervical chordomas is exceedingly difficult and carries the risk of significant postoperative morbidity. Here, the authors present their institutional experience with 13 patients treated with a structure-sparing radical resection and adjuvant radiation for cervical chordomas. METHODS: Records of the standing senior author and institutional database of spinal surgeries were retrospectively reviewed for surgically managed cervical and high thoracic chordomas between 1997 and 2022. Chordomas whose epicenter was cervical but touched the clivus or had extension to the thoracic spine were included in this series. Clinical and operative data were gathered and analyzed for the index surgery and any revisions needed. Outcome metrics such as recurrence rates, complication rates, functional status, progression-free interval (PFI) and overall survival (OS) were evaluated. RESULTS: The median patient age at diagnosis was 57 (range 32-80) years. The median modified Rankin Scale (mRS) score at the time of presentation was 1 (range 0-4). Approximately 40% of tumors were located in the upper cervical spine (occiput-C2). The median time from diagnosis to surgery was 74.5 (range 10-483) days. Gross-total resection was achieved in just under 40% of patients. All patients received adjuvant radiotherapy. The mean duration of follow-up was 4.09 years, with a mean PFI of 3.80 (range 1.16-13.1) years. Five patients experienced recurrence (38.5%). The mean OS was 3.44 years. Three patients died during the follow-up period; 2 due to disease progression and 1 died in the immediate postoperative period. One patient was lost to follow-up. A significant positive relationship was identified between high cervical tumor location and disease recurrence (p = 0.021). CONCLUSIONS: While en bloc resection is appropriate and feasible for tumors in the sacral spine, the cervical region poses a significant technical challenge and is associated with increased postoperative morbidity. Radical resection may allow for achievement of negative operative margins and, along with sparing postoperative morbidity following resection of cervical chordomas, maintaining a similar rate of recurrence when compared with en bloc resection while preserving quality of life.

Characterizing the presentation, management, and clinical outcomes of patients with intradural spinal chordomas: a systematic review.

OBJECTIVE: Chordomas are locally aggressive neoplasms of the spine or skull base that arise from embryonic remnants of the notochord. Intradural chordomas represent a rare subset of these neoplasms, and few studies have described intradural chordomas in the spine. This review evaluates the presentation, management, and outcomes of intradural spinal chordomas. METHODS: A systematic review of PubMed/MEDLINE, EMBASE, Cochrane Library, Scopus, and Web of Science was performed. Studies describing at least 1 case of intradural chordomas anywhere in the spine were included. Extracted details included presenting symptoms, radiological findings, treatment course, follow-up, and disease progression. RESULTS: Thirty-one studies, with a total of 41 patients, were included in this review. Seventy-six percent (31/41) of patients had primary intradural tumors, whereas 24% (10/41) presented with metastasis. The most common signs and symptoms were pain (n = 27, 66%); motor deficits (n = 20, 49%); sensory deficits (n = 17, 42%); and gait disturbance (n = 10, 24%). The most common treatment for intradural chordoma was resection and postoperative radiotherapy. Sixty-six percent (19/29) of patients reported improvement or complete resolution of symptoms after surgery. The recurrence rate was 37% (10/27), and the complication rate was 25% (6/24). The median progression-free survival was 24 months (range 4-72 months). Four patient deaths were reported. The median follow-up time was 12 months (range 13 days-84 months). CONCLUSIONS: Treatment of intradural spinal chordomas primarily involves resection and radiotherapy. A significant challenge and complication in management is spinal tumor seeding after resection, with 9 studies proposing seeding as a mechanism of tumor metastasis in 11 cases. Factors such as tumor size, Ki-67 positivity, and distant metastasis may correlate with worse outcomes and demonstrate potential as prognostic indicators for intradural spinal chordomas. Further research is needed to improve understanding of this tumor and develop optimal treatment paradigms for these patients.

Multidisciplinary surgical considerations for en bloc resection of sacral chordoma: review of recent advances and a contemporary single-center series.

OBJECTIVE: Contemporary management of sacral chordomas requires maximizing the potential for recurrence-free and overall survival while minimizing treatment morbidity. En bloc resection can be performed at various levels of the sacrum, with tumor location and volume ultimately dictating the necessary extent of resection and subsequent tissue reconstruction. Because tumor resection involving the upper sacrum may be quite destabilizing, other pertinent considerations relate to instrumentation and subsequent tissue reconstruction. The primary aim of this study was to survey the surgical approaches used for managing primary sacral chordoma according to location of lumbosacral spine involvement, including a narrative review of the literature and examination of the authors' institutional case series. METHODS: The authors performed a narrative review of pertinent literature regarding reconstruction and complication avoidance techniques following en bloc resection of primary sacral tumors, supplemented by a contemporary series of 11 cases from their cohort. Relevant surgical anatomy, advances in instrumentation and reconstruction techniques, intraoperative imaging and navigation, soft-tissue reconstruction, and wound complication avoidance are also discussed. RESULTS: The review of the literature identified several surgical approaches used for management of primary sacral chordoma localized to low sacral levels (mid-S2 and below), high sacral levels (involving upper S2 and above), and high sacral levels with lumbar involvement. In the contemporary case series, the majority of cases (8/11) presented as low sacral tumors that did not require instrumentation. A minority required more extensive instrumentation and reconstruction, with 2 tumors involving upper S2 and/or S1 levels and 1 tumor extending into the lower lumbar spine. En bloc resection was successfully achieved in 10 of 11 cases, with a colostomy required in 2 cases due to rectal involvement. All 11 cases underwent musculocutaneous flap wound closure by plastic surgery, with none experiencing wound complications requiring revision. CONCLUSIONS: The modern management of sacral chordoma involves a multidisciplinary team of surgeons and intraoperative technologies to minimize surgical morbidity while optimizing oncological outcomes through en bloc resection. Most cases present with lower sacral tumors not requiring instrumentation, but stabilizing instrumentation and lumbosacral reconstruction are often required in upper sacral and lumbosacral cases. Among efforts to minimize wound-related complications, musculocutaneous flap closure stands out as an evidence-based measure that may mitigate risk.

Rare Columnar Cell Variant of Papillary Thyroid Carcinoma with Cervical Spine Metastasis: A Case Report.

BACKGROUND: Columnar cell carcinoma is a rare subtype of papillary thyroid carcinoma (CCV-PTC) that accounts for only 0.15% to 0.2% of all Papillary Thyroid Carcinomas (PTCs). It has aggressive behavior but a better prognosis than anaplastic thyroid carcinoma. CASE PRESENTATION: A 64-year-old female presented with a huge thyroid mass resulting in compressive myelopathy and was diagnosed as CCV-PTC, not anaplastic carcinoma. After multidisciplinary discussions, we decided to proceed with otolaryngological, thoracic, and orthopaedic surgery. All tumours were unresectable, and we planned to proceed with R2 resection to resolve the gait disturbance and anterior fusion to resolve spinal instability. CONCLUSION: Advanced-stage thyroid cancer is relatively uncommon, but desirable treatment effects can be expected through accurate pathological diagnosis. Immunohistochemical staining and tissue-specific markers can be helpful.

Vertebral hemangiomas: a review on diagnosis and management.

BACKGROUND: Vertebral hemangiomas (VHs) are the most common benign tumors of the spinal column and are often encountered incidentally during routine spinal imaging. METHODS: A retrospective review of the inpatient and outpatient hospital records at our institution was performed for the diagnosis of VHs from January 2005 to September 2023. Search filters included "vertebral hemangioma," "back pain," "weakness," "radiculopathy," and "focal neurological deficits." Radiographic evaluation of these patients included plain X-rays, CT, and MRI. Following confirmation of a diagnosis of VH, these images were used to generate the figures used in this manuscript. Moreover, an extensive literature search was conducted using PubMed for the literature review portion of the manuscript. RESULT: VHs are benign vascular proliferations that cause remodeling of bony trabeculae in the vertebral body of the spinal column. Horizontal trabeculae deteriorate leading to thickening of vertical trabeculae which causes a striated appearance on sagittal magnetic resonance imaging (MRI) and computed tomography (CT), "Corduroy sign," and a punctuated appearance on axial imaging, "Polka dot sign." These findings are seen in "typical vertebral hemangiomas" due to a low vascular-to-fat ratio of the lesion. Contrarily, atypical vertebral hemangiomas may or may not demonstrate the "Corduroy" or "Polka-dot" signs due to lower amounts of fat and a higher vascular component. Atypical vertebral hemangiomas often mimic other neoplastic pathologies, making diagnosis challenging. Although most VHs are asymptomatic, aggressive vertebral hemangiomas can present with neurologic sequelae such as myelopathy and radiculopathy due to nerve root and/or spinal cord compression. Asymptomatic vertebral hemangiomas do not require therapy, and there are many treatment options for vertebral hemangiomas causing pain, radiculopathy, and/or myelopathy. Surgery (corpectomy, laminectomy), percutaneous techniques (vertebroplasty, sclerotherapy, embolization), and radiotherapy can be used in combination or isolation as appropriate. Specific treatment options depend on the lesion's size/location and the extent of neural element compression. There is no consensus on the optimal treatment plan for symptomatic vertebral hemangioma patients, although management algorithms have been proposed. CONCLUSION: While typical vertebral hemangioma diagnosis is relatively straightforward, the differential diagnosis is broad for atypical and aggressive lesions. There is an ongoing debate as to the best approach for managing symptomatic cases, however, surgical resection is often considered first line treatment for patients with neurologic deficit.

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.

Intracranial ependymoma with extremely rare extraneural metastasis.

Ependymomas account for 1-8% of overall brain tumors. They are most common at the age of 3-4 years. Their metastasis is very rare, and extraneural metastasis is even more unusual. In this report, the ependymoma localized in the posterior fossa with metastasis into femoral diaphysis in a 27-year-old male patient, who was treated in 2001, is presented. As we did not have any other cases of patients having a brain and spinal tumor with extraneural metastases even after 21 years, until 2022, this case was found worthy of being presented. When the literature was examined, it was observed that there is still no standard treatment after surgery for ependymomas and their metastasis. Due to their rarity, the general treatment of extraneural metastasis of ependymomas is also under discussion. It is recommended that clinicians consider admitting patients with rare or hard-to-treat tumors to ongoing clinical trials.

Vertebral Hemangiomas: Yucesoy-Yilmaz Classification System with Corresponding Therapeutic Options.

OBJECTIVE: Vertebral hemangiomas (VHs) are relatively common, symptomatic benign tumors of the spine with a reported estimated incidence up to 11%. They usually appear in the body of the vertebrae; however, they can extend into pedicles, laminae, and epidural space. They may cause pain, neurologic deficits. and fractures. METHODS: In this retrospective, single-center study, we reviewed our cases with VH and we propose the novel classification system that evaluates these lesions per their views on magnetic resonance imaging and clinical findings under 4 main categories. RESULTS: Our novel classification system proposes that grade I lesions occupy less than 50% of the vertebral body, whereas grade II lesions occupy more than 50% and grade III lesions occupy the whole corpus. Grade IV lesions show an epidural and pedicular extension. We propose that grade I lesions may not be worthwhile for follow-up, whereas asymptomatic grade II (a) lesions to be worthy for a biannual imaging and symptomatic thoracolumbar grade II (b) and thoracolumbar grade III lesions to be considered for percutaneous vertebroplasty. We imply that decompression, posterior spinal instrumentation, and open vertebroplasty may be performed for thoracolumbar grade IV lesions. We further consider cervical grade IIb, III, and grade IV lesions as operable because of the disadvantages of percutaneous vertebroplasty. CONCLUSIONS: We suggest that our novel classification system may be useful for the determination of diagnostic and therapeutic procedures in the management of VH. Further multicentric trials on larger series are warranted to validate this system and popularize its utility in larger populations.

Preoperative elastoplasty of aggressive vertebral hemangiomas in elderly patients: a new strategy for reducing intraoperative bleeding and complications.

PURPOSE: Preoperative elastoplasty could be an alternative strategy for treating aggressive vertebral hemangiomas (VHs) in frail patients needing for spinal cord decompression, combining the advantages of embolization and vertebroplasty. METHODS: Three elderly patients with spinal cord compression from thoracic aggressive VHs underwent XperCT-guided percutaneous injection of silicone (VK100), filling the whole affected vertebra, followed by a decompressive laminectomy. At 12-months follow-up no recurrences, vertebral collapse or segmental kyphosis were noted at the CT scans, with patients reporting an improvement of preoperative neurological deficits, VAS and Smiley-Webster pain scale (SWPS) parameters. RESULTS: With its elastic modulus, non-exothermic hardening, and lower viscosity than PMMA, VK100 allowed a preoperative augmentation of the affected vertebral body, pedicles, and laminae without complications, with a controlled silicone delivery even in part of VH's epidural components thanks to XperCT-guidance. CONCLUSION: When facing highly bony erosive VH encroaching the spinal canal, VK100 combines the advantages of embolization and vertebroplasty especially in elderly patients, permeating the whole VH's angioarchitecture, significantly reducing tumor.

Spinal lymphoma in a goat.

A 3-year-old Pygmy Wether was presented for chronic hindlimb paralysis. A neurological exam revealed nonambulatory paraplegia with absent deep pain nociception, lack of hindlimb withdrawal reflexes, and paraspinal pain on palpation with T3 to L3 neurolocalization. MRI of the lumbar spine revealed an extensive, dorsal to dorsolateral, severely compressive, heterogeneously contrast-enhancing extradural lesion of the lumbar spine with intervertebral foraminal extension into the surrounding paraspinal musculature. Vertebral bone marrow involvement was also noted in the L5 and L6 vertebrae. A diagnosis of lymphoma was obtained after cytological sampling. This is the first case report describing specific MRI findings (signal characteristics, enhancement pattern, and perilesional changes) in a goat with paraspinal lymphoma.

Multimodality Imaging for 3D Printing and Surgical Rehearsal in Complex Spine Surgery.

Surgery is the mainstay treatment of symptomatic spinal tumors. It aids in restoring functionality, managing pain and tumor growth, and improving overall quality of life. Over the past decade, advancements in medical imaging techniques combined with the use of three-dimensional (3D) printing technology have enabled improvements in the surgical management of spine tumors by significantly increasing the precision, accuracy, and safety of the surgical procedures. For complex spine surgical cases, the use of multimodality imaging is necessary to fully visualize the extent of disease, including both soft-tissue and bone involvement. Integrating the information provided by these examinations in a cohesive manner to facilitate surgical planning can be challenging, particularly when multiple surgical specialties work in concert. The digital 3-dimensional (3D) model or 3D rendering and the 3D printed model created from imaging examinations such as CT and MRI not only facilitate surgical planning but also allow the placement of virtual and physical surgical or osteotomy planes, further enhancing surgical planning and rehearsal. The authors provide practical information about the 3D printing workflow, from image acquisition to postprocessing of a 3D printed model, as well as optimal material selection and incorporation of quality management systems, to help surgeons utilize 3D printing for surgical planning. The authors also highlight the process of surgical rehearsal, how to prescribe digital osteotomy planes, and integration with intraoperative surgical navigation systems through a case-based discussion. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Two-step osteotomy/discectomy through cannulated screw (TOCS) technique for en bloc resection of spine tumor: surgical technique and preliminary results.

PURPOSE: We have developed a novel technique for osteotomy/discectomy during en bloc resection of spine tumors named two-step osteotomy/discectomy through cannulated screw (TOCS). This study aims at describing the procedure of TOCS technique and assessing its efficiency and safety. METHODS: We retrospectively reviewed fourteen patients who underwent en bloc resection for spine tumors using TOCS technique in our center between August 2018 and September 2022. The technique was based on a specially designed "slotted" cannulated screw which was a cannulated screw with a longitudinal slot to provide the accessibility of T-saw. During osteotomy/discectomy, the "slotted" cannulated screw was inserted obliquely along the plane between the dura and the posterior wall of spine in light of the planned osteotomy/discectomy plane under routine fluoroscopic imaging guidance. The T-saw was introduced through the screw, and the osteotomy/discectomy was performed sequentially in two steps under the guidance of the screw by turning the slot away and toward the dura. The intra-/perioperative complication, neurological function (determined by Frankel grading), surgical margin (determined by a pathologist using AJCC R system), follow-up details were documented. RESULTS: The mean duration of surgery was 599.3 (360-890) min with a mean volume of intra-operative hemorrhage of 2021.4 (800-5000) mL. The intra-/perioperative complications were found in four patients (28.6%). R0 and R1 resections were achieved in nine and five patients, respectively. There was no R2 resection. After a mean follow-up period of 30.6 (10-67) months, all patients were alive except one patient died ten months after surgery due to unrelated cause. No recurrence and implant failure were found. Thirteen patients (92.9%) exhibited completely normal neurological function same as their preoperative neurological status. CONCLUSION: Using TOCS technique can facilitate a precise, complete and safe osteotomy/discectomy procedure during en bloc resection for spine tumor without the aid of intra-operative navigation.

Percutaneous Vertebroplasty for Cervical Symptomatic Hemangiomas and Spinal Metastases: A Case Series and Literature Review.

OBJECTIVE: Percutaneous vertebroplasty (PVP) is a commonly used technique for the treatment of spinal diseases, but it is rarely employed for cervical lesions. This study presents a case series and a literature review to evaluate the efficacy of cervical PVP. METHODS: From August 2013 to January 2023, 14 patients underwent cervical PVP in the author's institution. The mean postoperative follow-up time was 20.3 ± 12.1 months (ranging from 5 to 41 months). The pain status and quality of life were assessed preoperatively, postoperatively, and during follow-up using the Visual Analog Scale and Neck Disability Index. Additionally, complications that occurred during the study period were documented. RESULTS: The series of cases included 9 cases of hemangiomas and 5 cases of spinal metastases. The common symptom was axial pain in the neck. All patients were successfully treated with PVP. Visual analog scale scores decreased from 6.6 ± 0.8 preoperatively to 1.9 ± 0.8 at 24 hours postoperatively and to 2.4 ± 1.2 at the last follow-up (P < 0.01). Neck Disability Index decreased from 22.3% ± 8.9% preoperatively to 7.6% ± 8.1% at 24 hours postoperatively and to 6.0% ± 7.2% at 12-month follow-up (P < 0.01). After the operation, a case of dysphagia occurred, but no major complications were observed during the follow-up period. CONCLUSIONS: Cervical PVP via the anterolateral approach is a safe option for the treatment of cervical symptomatic hemangiomas and spinal metastases with limited invasiveness. It is effective in relieving pain and improving quality of life.

A Novel and Reproducible Classification of Cervical Dumbbell Tumors to Inform Surgical Approach and Reconstruction Techniques.

STUDY DESIGN: A retrospective case series. OBJECTIVE: This study developed a novel classification system based on imaging and anatomy to select optimal surgical approaches and reconstruction strategies to achieve total resection of cervical dumbbell tumors and restore spinal stability. SUMMARY OF BACKGROUND DATA: Total resection is necessary to decrease the recurrence rate of cervical dumbbell tumors. Previous cervical dumbbell tumor classifications are insufficient for determining surgical strategies; therefore, a practical classification is needed. MATERIALS AND METHODS: This study included 295 consecutive patients with cervical dumbbell tumors who underwent total surgical resection. A novel classification of cervical dumbbell tumors was developed based on magnetic resonance imaging and computed tomography. Continuous variables were expressed as mean±SD and were compared using an unpaired two-tailed Student t test. The χ 2 test or the Fisher exact test was used for categorical variables. Kendall's W test assessed three independent raters' inter-rater and intrarater reliabilities on 140 cervical dumbbell tumors. RESULTS: The inter-rater and intrarater consistency coefficient was 0.969 (χ 2 =404.3, P <0.001) and 0.984 (χ 2 =273.7, P <0.001). All patients with type I and II tumors underwent single-posterior surgeries to achieve total resection. Of the patients in this study, 86.1%, 25.9%, 75.9%, and 76.9% underwent posterior surgeries for types IIIa, IIIb, IVa, and V tumors, respectively. All patients with type IVb tumors underwent a combined anterior and posterior approach. Posterior internal fixation was used for all patients in posterior surgery. Anterior reconstruction was applied for patients with type IVb tumors (20/20, 100%) and some with type V tumors (3/13, 23.1%). The mean follow-up duration was 93.6±2.6 months. A recurrence was observed in 19 (6.4%) patients. CONCLUSION: The authors describe a novel classification system that is of practical use for planning the complete resection of cervical dumbbell tumors.

Essentials of Spinal Tumor Ablation.

Recent advances in percutaneous minimally invasive thermal ablation and vertebral augmentation provide radiologists with important arsenal for treatment of selected patients with spinal metastases. These interventions have proven to be safe, effective, and durable in treatment of selected patients with vertebral metastases. Attention to procedure techniques, including choice of ablation modality, vertebral augmentation technique, and thermal protection, is essential for improved patient outcomes. A detailed knowledge of such interventions and implementation of procedural safety measures will further heighten radiologists' role in the management of patients with spinal metastases.