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.

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.

Choriocarcinoma masquerading as lumbar spinal tumor: Case report and literature review.

RATIONALE: Choriocarcinoma is a highly invasive gestational trophoblastic neoplasm, usually metastasis to lung and brain, but occurrence of choriocarcinoma following spontaneous abortion presenting as a vertebral tumor is extremely rare, to the best of our knowledge. Because of the poor diagnosis and high malignancy, the low progression-free survival follows up. PATIENT CONCERNS: We here are reporting a case of choriocarcinoma that presented with vertebral tumor induced paralysis of limbs and incontinence of urine. DIAGNOSIS: Combined with the childbearing history, high ß-human chorionic gonadotrophinin levels, and imaging examination, a clinical diagnosis was made exactly. Till the pathological results after the operation of lumbar spinal canal tumorectomy, the diagnosis was exactly clear. INTERVENTIONS: After performing the laminectomy, the fierce bleeding follows up, just did the temporary limited decompression. Because of the vertebral artery embolization, lumbar spinal canal tumorectomy, spinal canal and root canal decompression, subdural decompression and hematoma removal were performed. OUTCOMES: After performing the operation and chemotherapy timely and positively, the patient lost consciousness and died due to the pulmonary embolism at last. LESSONS: This is the first case report describing choriocarcinoma with metastases to the spine amongst Chinese population as well. Early metastasis is one of the marked tendencies of choriocarcinoma, but spine metastasis and the related spinal oppressional symptoms were found instead of vaginal bleeding in this case, which is indeed rare.

Lower thoracic schwannoma presenting as pseudotumor cerebri: a case report.

Primary Spinal tumors presenting as increased intracranial pressure is a rare and intradural extramedullary (IDEM) spinal schwannoma with unique presentation of pseudotumour cerebri (PTC) is extremely rare. Here, we describe a case of 48 years old male patient who presented to us with six months' history of headache and visual disturbances and was found to have bilateral papilledema. CT scan of brain was normal and CSF opening pressure on Lumbar puncture (LP) was 30 cm of H2O with raised protein level. His headache and visual symptoms settled down after LP (Lumbo-peritoneal) shunt was performed. Three days postoperatively, patient complained of hypoesthesia and weakness of left leg that became an established complain after 2 weeks. A Lumbosacral MRI was performed with concerns of any postoperative complications of LP Shunt, which revealed IDEM spinal tumour at D11-D12 level. Patient underwent second surgery for excision of tumor. We reviewed the relevant literatures and discuss the possible mechanism of such atypical presentation of spinal tumors.

Evaluation of different scoring systems for spinal metastases based on a Chinese cohort.

INTRODUCTIONS: The spine is one of the most common sites of metastasis for malignancies. This study aimed to compare the predictive performance of seven commonly used prognostic scoring systems for surgically treated spine metastases. It is expected to assist surgeons in selecting appropriate scoring systems to support clinical decision-making and better inform patients. METHODS: We performed a retrospective study involving 268 surgically treated patients with spine metastases between 2017 and 2020 at a single regional oncology center in China. The revised Tokuhashi, Tomita, modified Bauer, revised Katagiri, van der Linden, Skeletal Oncology Research Group (SORG) nomogram, and SORG machine-learning (ML) scoring systems were externally validated. The area under the curve (AUC) of the receiver operating characteristic curve was used to evaluate sensitivity and specificity at different postoperative time points. The actual survival time was compared with the reference survival time provided in the original publication. RESULTS: In the present study, the median survival was 16.6 months. The SORG ML scoring system demonstrated the highest accuracy in predicting 90-day (AUC: 0.743) and 1-year survival (AUC: 0.787). The revised Katagiri demonstrated the highest accuracy (AUC: 0.761) in predicting 180-day survival. The revised Katagiri demonstrated the highest accuracy (AUC: 0.779) in predicting 2-year survival. Based on this series, the actual life expectancy was underestimated compared with the original reference survival time. CONCLUSIONS: None of the scoring systems can perform optimally at all time points and for all pathology types, and the reference survival times provided in the original study need to be updated. A cautious awareness of the underestimation by these models is of paramount importance in relation to current patients.

Aggressive Vertebral Hemangioma and Spinal Cord Compression: A Particular Direct Access Case of Low Back Pain to Be Managed-A Case Report.

Hemangiomas are the most common benign tumours affecting the spine, with an incidence of 10-12% of the general population. Although most hemangiomas are asymptomatic, there are aggressive forms which can develop symptoms, leading patients to show signs of disability. This case report aims to highlight the importance of red flags screening, and to report the physiotherapist's clinical reasoning that led him to refer his patient to other healthcare professionals. This case also illustrated the pre- and post-surgical treatment of a specific low back pain case in a patient affected by aggressive vertebral hemangioma and spinal cord compression. The patient is a 52-year-old man, who reported intense pain in his sacral region about three months prior, which worsened while in sitting position. The physiotherapist proceeded with a complete medical history investigation and clinical examination. After an impaired neurological examination, the patient was referred to another health professional, who diagnosed multiple vertebral hemangiomas in the patient's lumbosacral tract. The therapeutic intervention included the patient's post-surgical rehabilitation following a vascular embolization. This case report shows the importance of proper patient screening. Indeed, during patients' assessment, it is paramount to recognize red flags and to investigate them appropriately. An early referral of patients with conditions that require the support and expertise of other professionals can lead to a timely diagnosis and avoid costly and unnecessary rehabilitation procedures. In this case, the interdisciplinary collaboration between physiotherapist and neurosurgeon was crucial in guiding the patient towards recovery.

Laparoscopic Approach to Presacral Recurrence of Sacrococcygeal Teratoma.

A 6-month-old girl was presented to our hospital due to a presacral mass found 5 months after surgery of sacrococcygeal teratoma. The original tumor was a 63 x 50 mm sized round cyst connecting to the coccyx, observed with computed tomography. The initial operation was performed with en bloc removal of the tumor along with the coccyx in the prone position. During a routine follow up, ultrasonography indicated a possible local recurrence, 5 months after the initial operation. The magnetic resonance imaging revealed a polycystic formation with a diameter of 20 x 11 x 17 mm in the presacral space. The laparoscopic operation was conducted with the patient in the lithotomy and Trendelenburg position. The broad ligament of uterus was fixed to the abdominal skin and the rectum was mobilized to identify the tumor, which was resected laparoscopically. A histopathological examination showed the tumor to be a mature cystic teratoma. We observed her without any additional treatment and no recurrence is seen after 6 months.