Unifocal Primary Bone Lymphoma of the Lateral Mass of C1.

Primary bone lymphoma is an infrequently encountered tumor of the spine that has a better prognosis than other primary spinal malignancies. The understanding of this entity and its differences from other secondary bone lymphomas have evolved over time. The thoracic spine is the commonly reported site of the lesions. However, it is seldom considered as a first diagnosis when the patient presents to the neurosurgeon. A case of this uncommon tumor in a 68-year-old woman at an extremely rare location-the lateral mass of C1-is used to illustrate the detailed evaluation, nuances in treatment, and outcomes of primary bone lymphomas.

Primary intraspinal neuroendocrine tumor: A case report and literature review.

RATIONALE: Neuroendocrine tumors (NET) refer to a group of uncommon tumors arising in the neuroendocrine system. Most NETs occur in the digestive tract and bronchi but are rare in the central nervous system, especially in the spinal canal. NET in the central nervous system mainly metastasize from other systems, with non-specific clinical symptoms. In this study, we report the diagnosis and treatment of intraspinal NET to provide clinical guidance as well as to avoid misdiagnosis and missed diagnosis. PATIENT CONCERNS: A 59-year-old male patient, presented with recurrent right lower limb pain for half a year, accompanied by numbness and weakness for 4 months and aggravation for 2 months. Lumbar spine magnetic resonance imaging (MRI) revealed a space-occupying lesion in the spinal canal. The diagnosis of primary intraspinal NET was confirmed by topathological examination. DIAGNOSIS: Primary intraspinal NET tumor. INTERVENTIONS: Surgical resection. OUTCOMES: Significant improvements in right lower limb pain, numbness, and weakness were observed, and lumbar spine MRI was performed again to dynamically observe the changes in intraspinal NET. CONCLUSIONS: Surgical resection may be an effective treatment for intraspinal NETs. LESSONS: Intraspinal NETs are relatively rare and mostly manifest as limb numbness, weakness, and pain. Due to its nonspecific clinical symptoms, intraspinal NETs are easily misdiagnosed as lumbar disc herniation with radiculopathy and lumbar spondylolisthesis. Therefore, in patients with long-term symptoms, in addition to common lumbar neuromuscular diseases, lumbar MRI should be performed promptly to exclude the possibility of lumbar NETs.

Unmasking Pott Disease: A Diagnostic Challenge Mimicking Metastatic Lung Cancer - A Case Report.

BACKGROUND Tuberculosis spondylitis, also known as Pott disease, is a rare form of the ancient infectious disease tuberculosis. It bears a complex clinical and radiological profile, often necessitating an extensive differential diagnostic approach for accurate identification. The disease was named in honor of the first diagnosed patient, highlighting its historical significance. CASE REPORT We report a case involving a 69-year-old male initially admitted to the Pulmonology Department under the suspicion of a left lung tumor, as indicated by a chest X-ray. A subsequent CT scan revealed a tumor-hilar mass, enlarged subcarineal lymph nodes, and a pathological mass at the C6/C7 vertebral level. Despite negative tuberculosis tests, the patient was misdiagnosed with disseminated lung cancer with spinal metastases. Following radiotherapy targeting the cervical and thoracic spine, the definitive diagnosis of spinal tuberculosis was confirmed via histopathological examination from an open biopsy of the C6 and C7 vertebrae. CONCLUSIONS Tuberculosis can present with an insidious and misleading clinical picture, often mimicking other diseases such as cancer. Early and accurate diagnostic processes are crucial for effective treatment. This case underscores the importance of considering tuberculosis in the differential diagnosis, especially when clinical presentations are ambiguous.

Bizarre parosteal osteochondromatous proliferation (Nora lesion) involving the spine: a case report and systematic review.

Bizarre parosteal osteochondromatous proliferation (BPOP), also termed Nora lesion, is a rare, benign tumor most often located in the hands and feet. We herein present the second reported case of BPOP affecting the spine, an uncommon location. One year after surgical excision, the patient was pain-free and showed no evidence of recurrence. We reviewed a total of 323 cases of BPOP among 101 articles, providing the first systematic update on the latest knowledge of BPOP. The age of patients with BPOP ranges from 3 months to 87 years, peaking in the second and third decades of life. The hands are the most common location of BPOP (58.39%), followed by the feet (20.81%). Imaging features play a key role in the diagnosis of BPOP, but histopathologic diagnosis remains the gold standard. Differential diagnosis of BPOP should be based on the epidemiologic and clinical features as well as clinical examination findings. Surgical resection is the most extensively used treatment for BPOP. Recurrence is common (37.44%) and can be treated with re-excision. This article can deepen our understanding of BPOP and will be helpful for the diagnosis and treatment of BPOP in clinical practice.

[PRIMARY CHONDROSARCOMA OF THE SPINE].

INTRODUCTION: PRIMARY CHONDROSARCOMA OF THE SPINE.

Musculoskeletal misdiagnoses in pediatric patients with spinal tumors.

OBJECTIVE: Childhood spinal tumors often present with musculoskeletal symptoms, potentially causing a misdiagnosis and delays in diagnosis and treatment. This study aims to identify, characterize, and compare children with spinal tumors who had prior musculoskeletal misdiagnoses to those without, analyzing clinical presentation, diagnostic interval, and outcome. STUDY DESIGN: This retrospective cohort study evaluated all children aged 0-14 years diagnosed with a spinal tumor in Denmark from 1996 to 2018. The cohort was identified through the Danish Childhood Cancer Registry, and the registry data were supplemented with data from medical records. The survival was compared using the Kaplan-Meier method. RESULTS: Among 58 patients, 57% (33/58) received musculoskeletal misdiagnoses before the spinal tumor diagnosis. Misdiagnoses were mostly nonspecific (64%, 21/33), involving pain and accidental lesions, while 36% (12/33) were rheumatologic diagnoses. The patients with prior misdiagnosis had less aggressive tumors, fewer neurological/general symptoms, and 5.5 months median diagnostic interval versus 3 months for those without a misdiagnosis. Those with prior misdiagnoses tended to have a higher 5-year survival of 83% (95% confidence interval [CI]: 63%-92%) compared to 66% (95% CI: 44%-82%) for those without (p = .15). CONCLUSION: Less aggressive spinal tumors may manifest as gradual skeletal abnormalities and musculoskeletal symptoms without neurological/general symptoms, leading to misdiagnoses and delays.

Dermoscopy of hypopigmented macules unveiling genetic diagnosis of tuberous sclerosis complex type 2 in an infant presenting with sacral chordoma.

A 2-month-old male with surgically resected sacral chordoma presented with multiple hypopigmented macules showing characteristic patchy, sharply demarcated areas of pigment network on dermoscopy. These dermoscopic findings were suggestive of the ash-leaf macules of tuberous sclerosis over other common hypopigmented macules in neonates. Chordomas presenting in early childhood in the sacral location have been reported as a rare manifestation of tuberous sclerosis complex. The combination of these findings led to a diagnosis of tuberous sclerosis, confirmed with the finding of a heterozygous TSC2 gene deletion; treatment with sirolimus resulted in regression of cardiac rhabdomyomas and hypopigmented macules.

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.

Estrategias de tratamiento en la metástasis vertebral. Necesidad de comités multidisciplinarios desde la perspectiva del cirujano. Narración de la literatura / Treatment strategies in vertebral metastasis. Need for multidisciplinary committees from the perspective of the surgeon. Narration of literatura

Las mejoras en el diagnóstico y tratamiento del cáncer han mejorado la supervivencia. Secundariamente también aumenta el número de estos pacientes que presentan una metástasis vertebral y el número con alguna morbilidad en relación con estas metástasis. Fractura vertebral, compresión radicular o lesión medular causan un deterioro de su calidad de vida. El objetivo en el tratamiento de las mismas ha de ser el control del dolor, mantenimiento función neurológica y de la estabilidad vertebral, teniendo presente que en muchos casos será un tratamiento paliativo.El tratamiento de estas complicaciones presenta un enfoque multidisciplinario, radiólogos, radiólogos intervencionistas, oncólogos y radioterapeutas, cirujanos de raquis, pero también Unidad de Rehabilitación o Unidad de Dolor. Recientes trabajos muestran que un enfoque multidisciplinario de estos pacientes puede mejorar calidad de vida e incluso pronóstico.En el presente trabajo se realiza una revisión y lectura de la bibliografía sobre el manejo multidisciplinario de estos pacientes.(AU)

Improvements in cancer diagnosis and treatment have improved survival. Secondarily, the number of patients who present a vertebral metastasis and the number with some morbidity in relation to these metastases also increases. Vertebral fracture, root compression or spinal cord injury cause a deterioration of their quality of life.The objective in the treatment of the vertebral metastasis must be the control of pain, maintenance of neurological function and vertebral stability, bearing in mind that in most cases it will be a palliative treatment.The treatment of these complications needs a multidisciplinary approach, radiologists, interventional radiologists, oncologists and radiation therapists, spine surgeons, but also rehabilitation or pain units. Recent studies show that a multidisciplinary approach of these patients can improve quality of life and even prognosis.In the present article, a review and reading of the literature on the multidisciplinary management of these patients is carried out.(AU)

[Translated article] Treatment strategies in vertebral metastasis. Need for multidisciplinary committees from the perspective of the surgeon. Narration of literature / Estrategias de tratamiento en la metástasis vertebral. Necesidad de comités multidisciplinarios desde la perspectiva del cirujano. Narración de la literatura

Las mejoras en el diagnóstico y tratamiento del cáncer han mejorado la supervivencia. Secundariamente también aumenta el número de estos pacientes que presentan una metástasis vertebral y el número con alguna morbilidad en relación con estas metástasis. Fractura vertebral, compresión radicular o lesión medular causan un deterioro de su calidad de vida. El objetivo en el tratamiento de las mismas ha de ser el control del dolor, mantenimiento función neurológica y de la estabilidad vertebral, teniendo presente que en muchos casos será un tratamiento paliativo.El tratamiento de estas complicaciones presenta un enfoque multidisciplinario, radiólogos, radiólogos intervencionistas, oncólogos y radioterapeutas, cirujanos de raquis, pero también Unidad de Rehabilitación o Unidad de Dolor. Recientes trabajos muestran que un enfoque multidisciplinario de estos pacientes puede mejorar calidad de vida e incluso pronóstico.En el presente trabajo se realiza una revisión y lectura de la bibliografía sobre el manejo multidisciplinario de estos pacientes.(AU)

Improvements in cancer diagnosis and treatment have improved survival. Secondarily, the number of patients who present a vertebral metastasis and the number with some morbidity in relation to these metastases also increases. Vertebral fracture, root compression or spinal cord injury cause a deterioration of their quality of life.The objective in the treatment of the vertebral metastasis must be the control of pain, maintenance of neurological function and vertebral stability, bearing in mind that in most cases it will be a palliative treatment.The treatment of these complications needs a multidisciplinary approach, radiologists, interventional radiologists, oncologists and radiation therapists, spine surgeons, but also rehabilitation or pain units. Recent studies show that a multidisciplinary approach of these patients can improve quality of life and even prognosis.In the present article, a review and reading of the literature on the multidisciplinary management of these patients is carried out.(AU)

Association between immunohistochemical markers and tumor progression following resection of spinal chordomas: a multicenter study.

OBJECTIVE: Chordomas are slow-growing tumors derived from notochord remnants. Despite margin-negative excision and postoperative radiation therapy, spinal chordomas (SCs) often progress. The potential of immunohistochemical (IHC) markers, such as epithelial membrane antigen (EMA), combined with machine learning algorithms to predict long-term (≥ 12 months) postoperative tumor progression, has been understudied. The authors aimed to identify IHC markers using trained tree-based algorithms to predict long-term (≥ 12 months) postoperative tumor progression. METHODS: The authors reviewed the records of patients who underwent resection of SCs between January 2017 and June 2021 across the Mayo Clinic enterprise. Demographics, type of treatment, histopathology, and other relevant clinical factors were abstracted from each patient's record. Low tumor progression was defined as more than a 94.3-mm3 decrease in the tumor size at the latest radiographic follow-up. Decision trees and random forest classifiers were trained and tested to predict the long-term volumetric progression after an 80/20 data split. RESULTS: Sixty-two patients diagnosed with and surgically treated for SC were identified, of whom 31 were found to have a more advanced tumor progression based on the tumor volume change cutoff of 94.3 mm3. The mean age was 54.3 ± 13.8 years, and most patients were male (62.9%) and White (98.4%). The most common treatment modality was subtotal resection with radiation therapy (35.5%), with proton beam therapy being the most common (71%). Most SCs were sacrococcygeal (41.9%), followed by cervical (32.3%). EMA-positive SCs had a postoperative progression risk of 67%. Pancytokeratin-positive SCs had a progression rate of 67%; however, patients with S100 protein-positive SCs had a 54% risk of progression. The accuracy of this model in predicting the progression of unseen test data was 66%. Pancytokeratin (mean minimal depth = 1.57), EMA (mean minimal depth = 1.58), cytokeratin A1/A3 (mean minimal depth = 1.59), and S100 protein (mean minimal depth = 1.6) predicted the long-term volumetric progression. Multiway variable importance plots show the relative importance of the top 10 variables based on three measures of varying significance and their predictive role. CONCLUSIONS: These IHC variables with tree-based machine learning tools successfully demonstrate a high capacity to identify a patient's tumor progression pattern with an accuracy of 66%. Pancytokeratin, EMA, cytokeratin A1/A3, and S100 protein were the IHC drivers of a low tumor progression. This shows the power of machine learning algorithms in analyzing and predicting outcomes of rare conditions in a small sample size.

Malignant spinal cord compression in the paediatric population-a systematic review, meta-analysis.

BACKGROUND: Malignant spinal cord compression (MSCC) has been noted in 3-5% of children with primary tumours. MSCC can be associated with permanent neurological deficits and prompt treatment is necessary. Our aim was to perform a systematic review on MSCC in children < 18 years to help formulate national guidelines. METHODS: A systematic review of the English language was undertaken using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Search criteria included 'MSCC in children, paediatric and metastases' for papers published between January1999 and December 2022. Isolated case reports/case series with < 10 patients were excluded. RESULTS: From a total of 17 articles identified, a final 7 were analysed (Level III/IV). Neuroblastoma constituted the most common cause for MSCC in children (62.7%) followed by sarcoma (14.2%). Soft tissue sarcomas were the most frequent cause of MSCC in children > 5 years old, while for neuroblastomas, the mean age of presentation was 20 months. The median age at time of diagnosis for the entire cohort of patients was 50.9 months (14.8-139). The median follow-up duration was 50.7 months (0.5-204). Motor deficits were the presenting symptom in 95.6% of children followed by pain in 65.4% and sphincter disturbance in 24%. There was a delay of about 26.05 days (7-600) between the onset of symptoms and diagnosis. A multimodality approach to treatment was utilised depending on the primary tumour. The prognosis for neurological recovery was found to be inversely proportional to the degree of neurological deficits and duration of symptoms in four studies. CONCLUSION: Neuroblastoma is the most common cause for MSCC in children (62.7%) followed by sarcoma (14.2%), whilst soft tissue sarcomas constituted the most frequent cause of MSCC in children > 5 years old. The majority of patients presented with motor deficit, followed by pain. In children with neuroblastoma /lymphoma, chemotherapy was the primary treatment. Early surgery should be a consideration with rapid deterioration of neurology despite chemotherapy. A multimodality approach including chemo-radiotherapy and surgery should be the treatment of choice in metastatic sarcomas. It is worth noting that multi-level laminectomy/decompression and asymmetrical radiation to the spine can lead to spinal column deformity in the future.

Multiple Myeloma Spinal Lesion Care: Management of a Primary Bone Malignancy Rather Than a Spinal Metastasis.

OBJECTIVE: Multiple myeloma (MM) is too often wrongly categorized as a spinal metastasis (SpM), although it is distinguishable from SpM in many aspects, such as its earlier natural history at the time of diagnosis, its increased overall survival (OS), and its response to therapeutic modalities. The characterization of these 2 different spine lesions remains a main challenge. METHODS: This study compares 2 consecutive prospective oncologic populations of patients with spine lesions: 361 patients treated for MM spine lesions and 660 patients treated for SpM between January 2014 and 2017. RESULTS: The mean time between the tumor/MM diagnosis and spine lesions was respectively 0.3 (standard deviation [SD] 4.1) and 35.1 months (SD 21.2) for the MM and SpM groups. The median OS for the MM group was 59.6 months (SD 6.0) versus 13.5 months (SD 1.3) for the SpM group (P < 0.0001). Regardless of Eastern Cooperative Oncology Group (ECOG) performance status, patients with MM always have a significantly better median OS than do patients with SpM: ECOG 0, 75.3 versus 38.7 months; ECOG 1, 74.3 versus 24.7 months; ECOG 2, 34.6 versus 8.1 months; ECOG 3, 13.5 versus 3.2 months and ECOG 4, 7.3 versus 1.3 months (P < 0.0001). The patients with MM had more diffuse spinal involvement (mean, 7.8 lesions; SD 4.7) than did patients with SpM (mean, 3.9; SD 3.5) (P < 0.0001). CONCLUSIONS: MM must be considered as a primary bone tumor, not as SpM. The strategic position of the spine in the natural course of cancer (i.e., nurturing cradle of birth for MM vs. systemic metastases spreading for SpM) explains the differences in OS and outcome.

Aggressive thoracic vertebral hemangioma: case report and literature review.

INTRODUCTION: Vertebral hemangiomas are common and typically benign vascular lesions, with a prevalence of 10-12% in the general population and 2-3% in all spine tumors. A small subset of vertebral hemangiomas can be categorized as "aggressive" if they exhibit extraosseous expansion that compress the spinal cord, causing pain and various neurologic symptoms. This report details a case of aggressive thoracic hemangioma resulting in worsening pain and paraplegia to draw attention to this rare condition, including identification and treatment. CASE PRESENTATION: In this case, we present a 39 year-old female with a history of progressively worsening pain and paraplegia caused by compression of the spinal cord from an aggressive thoracic vertebral hemangioma. Clinical presentation, imaging, and biopsies, confirmed the diagnosis. A combined surgical and endovascular treatment strategy was implemented, and the patient's symptoms improved. DISCUSSION: Aggressive vertebral hemangioma is a rare condition that may cause symptoms that diminishes the quality of life, including pain and various neurological symptoms. Given the low number of such cases, and significant impact on lifestyle, it is beneficial to identify cases of aggressive thoracic hemangiomas to ensure timely and accurate diagnosis and help development of treatment guidelines. This case highlights the importance of identifying and diagnosing this rare but serious disease.

Surgical management of symptomatic vertebral hemangiomas: a single institution experience and literature review.

Vertebral hemangiomas (VHs), formed from a vascular proliferation in bone marrow spaces limited by bone trabeculae, are the most common benign tumors of the spine. While most VHs remain clinically quiescent and often only require surveillance, rarely they may cause symptoms. They may exhibit active behaviors, including rapid proliferation, extending beyond the vertebral body, and invading the paravertebral and/or epidural space with possible compression of the spinal cord and/or nerve roots ("aggressive" VHs). An extensive list of treatment modalities is currently available, but the role of techniques such as embolization, radiotherapy, and vertebroplasty as adjuvants to surgery has not yet been elucidated. There exists a need to succinctly summarize the treatments and associated outcomes to guide VH treatment plans. In this review article, a single institution's experience in the management of symptomatic VHs is summarized along with a review of the available literature on their clinical presentation and management options, followed by a proposal of a management algorithm.

Separation Surgery and Postoperative Intensity-Modulated Radiation Therapy for a High-Grade Myxofibrosarcoma Involving the Spine: A Case Report.

BACKGROUND Myxofibrosarcoma involving the spine is a rare and intractable disease. Although wide surgical resection is the mainstay of treatment, it is often difficult to complete marginal en-bloc resection due to adjacent neurovascular components in the spine. Separation surgery, a partial resection to achieve circumferential separation and high-dose irradiation such as postoperative intensity-modulated radiation therapy, has received much attention as a new therapy for spinal tumors. However, little evidence regarding separation surgery with intensity-modulated radiation therapy for a spinal myxofibrosarcoma exists. CASE REPORT We present a case of a 75-year-old man with progressive myelopathy. Radiological examination revealed severe spinal cord compression due to an unknown widespread multiple tumor in the cervical and thoracic spine. Computed tomography-guided biopsy showed high-grade sarcoma. Positron emission tomography detected no other tumors in the body. Separation surgery was therefore performed with posterior stabilization. Hematoxylin and eosin staining showed storiform cellular infiltrates and pleomorphic cell nuclei. Histopathology identified high-grade myxofibrosarcoma. Postoperative intensity-modulated radiation therapy of 60 Gy in 25 fractions was completed without any adverse effects. The patient had greatly improved neurological function, was capable of walking with a cane, and had no recurrence for at least 1 year after surgery. CONCLUSIONS We reported a case of an unresectable high-grade myxofibrosarcoma of the spine successfully treated with the combination of separation surgery and postoperative intensity-modulated radiation therapy. This combination therapy is a relatively safe and effective treatment option in patients with impending neurological damage by unresectable sarcomas when total en-bloc resection is challenging due to the size, location, or adhesion.

A current review of spinal meningiomas: epidemiology, clinical presentation and management.

PURPOSE: To provide an up-to-date review of the epidemiology, histopathology, molecular biology, and etiology of spinal meningiomas, as well as discuss the clinical presentation, clinical evaluation, and most recent treatment recommendations for these lesions. METHODS: PubMed and Google Scholar search was performed for studies related to meningiomas of the spine. The terms "meningioma," "spinal meningioma," "spine meningioma," "meningioma of the spine," "benign spinal tumors," and "benign spine tumors," were used to identify relevant studies. All studies, including primary data papers, meta-analyses, systematic reviews, general reviews, case reports, and clinical trials were considered for review. RESULTS: Eighty-four studies were identified in the review. There were 22 studies discussing adverse postoperative outcomes, 21 studies discussing tumor genetics, 19 studies discussing epidemiology and current literature, 9 studies discussing radiation modalities and impact on subsequent tumor development, 5 studies on characteristic imaging findings, 5 studies discussing hormone use/receptor status on tumor development, 2 discussing operative techniques and 1 discussing tumor identification. CONCLUSION: Investigations into spinal meningiomas generally lag behind that of intracranial meningiomas. Recent advancements in the molecular profiling of spinal meningiomas has expanded our understanding of these tumors, increasing our appreciation for their heterogeneity. Continued investigation into the defining characteristics of different spinal meningiomas will aid in treatment planning and prognostication.