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Purpose: Aggressive giant cell tumor (GCT) is an important subtype of GCT and is relatively rare in the spine. There is little published information regarding this subject. The objective of our study is to discuss prognostic factors for primary aggressive GCT in the spine., Methods: A retrospective study of patients who had primary aggressive GCT in the spine and underwent surgical treatment at our center between 2000 and 2012 was conducted. Univariate and multivariate analyses were performed to identify the factors that might affect recurrence. T test, Chi-square test and rank sum test were used to analyze a single factor for recurrence, and factors with P ≤ 0.1 were subjected to multivariate analyses by binary logistic regression analyses. P values of ≤0.05 were considered statistically significant., Results: A total of 71 patients with primary aggressive GCT in the spine were included in the study. The mean follow-up period was 73.9 (range 23-167) months. Recurrence was detected in 24 patients after the initial surgery in our center with a recurrence rate of 33.8 %. The statistical analyses suggested that age more than 40 years, pathology grade III, total en bloc spondylectomy, and bisphosphonate treatment were independent prognostic factors for recurrence of primary aggressive GCT in the spine., Conclusion: Total en bloc spondylectomy together with bisphosphonate treatment could significantly decrease recurrence risk of primary aggressive GCT in the spine. Jaffe grade III was an adverse prognostic factor for recurrence, while age less than 40 years was a favorable prognostic factor.

Background Context: Desmoplastic fibroma (DF) is a benign, yet locally aggressive, tumor of the connective tissue. Desmoplastic fibroma in the spine is extremely rare, and only a few cases have been reported. Although surgical resection of DF arising in the spine is commonly regarded as a recommended treatment, it is difficult to achieve satisfactory results., Purpose: This study reviews the clinical patterns and follow-up data of patients with DF in the spine who underwent surgical treatment. We attempted to correlate surgical treatment and outcomes over time., Study Design: A retrospective clinical study of the surgical managements, including subtotal resection, total spondylectomy, and en bloc resection, for DF in the spine. Desmoplastic fibroma of the spine treatment occurred from 2004 to 2009 at the Department of Bone Tumor Surgery, AA Hospital., Patient Sample: Twelve consecutive cases of DF of the spine underwent surgical treatment at our center between 2004 and 2009., Outcome Measures: Neurologic outcomes were evaluated using Frankel score system and recurrence and metastasis were evaluated by computed tomography or magnetic resonance imaging of the surgical segments involved. Imaging was performed 3, 6, and 12 months after surgery, every 6 months for the next 2 years, and then annually for life., Methods: Overall, two different surgery protocols were applied. One protocol involved subtotal resection followed by radiotherapy (n=4), whereas the other involved total tumor resection (n=8). Postoperative radiotherapy was administered in six cases. Clinical data and surgery efficacy were analyzed via chart review., Results: Eleven patients were disease-free during their follow-up period, whereas one patient experienced recurrence without metastasis. Radicular pain nearly disappeared, and patients suffering from spinal cord compression recovered well. Local recurrence was detected in one-fourth (25%) of the cases that underwent subtotal resection and was not detected in any of the cases involving total spondylectomy., Conclusions: Local recurrence of DF is not uncommon after insufficient removal. Therefore, total excision, while also preserving neural function, is recommended. In our study, patients who underwent a total spondylectomy had significantly lower local recurrence rates for DF in the spine. Radiotherapy may be an acceptable alternative therapy, whereas en bloc resection has the potential to result in significant functional impairment., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: The aim of this study was to discuss the clinical presentation, imaging findings, treatments received, and outcome of therapies for patients with epidural spinal cord compression caused by thyroid spinal metastases, with the goal of emphasizing the importance of surgery in this setting and discussing therapeutic plan for treating these patients., Methods: A total of 22 patients with spinal cord compression due to thyroid tumor spinal metastases who received surgery in our department were identified from 2004 to 2011. The series of 22 patients collected from our institution over the past 7 years was used to discuss treatment options for thyroid cancer spinal metastases on the basis of literature review and our own extensive experience., Results: The mean age of the patients in this study was 57 years (range 37-78 years). The duration of the preoperative symptoms was 1-24 months, with an average of approximately 6 months. All patients attained improvement of at least one level of the Frankel classification after surgery. Two patients received more than one operation at our institution. Two patients died during follow-up, two patients had stable disease, and all other patients maintained a disease-free status during follow-up., Conclusions: As thyroid tumor spinal metastases have a favorable prognosis, a radical therapeutic attitude should be considered in decision-making. Dorsal spinal decompression through curettage and stabilization can preserve or restore neurological function for most patients. For patients who have more than one metastatic lesion of the spine, surgeries can be sequentially performed based on the urgency of the case. In addition to treatment of primary disease, surgery and bisphosphonate treatment are the most important therapies for these patients.

• Spinal GCTTS mainly involves the posterior bone elements with the onset around 30–40 years old. • Soft tissue masses centering on the facet joint with osteolytic bony destruction. • A few cases presented as the intramuscular lesions without bone destruction. • T2-weighted dark signals, homogeneous or heterogeneous enhancement. • Gross-total resection is an effective means for treating spinal GCTTS. This study aimed to characterize giant cell tumor of the tendon sheath (GCTTS) in spine more fully and further validate the therapeutical effect of surgcial resection for treating this neoplasm. Patients diagnosed with spinal GCTTS and received surgical resection in our hospital between January 2009 and September 2021 were identified retrospectively. The clinical data and radiological images were summarized and the clinical outcomes of patients with a follow-up period of more than 12 months were analyzed. Thirty patients with benign GCTTS and one with malignant GCTTS were included. Preoperative radiological images were available in 28 of 30 benign cases. Benign lesions were revealed as soft tissue masses centering on the facet joint with osteolytic bone destruction in 26 patients on CT, and as prevertebral or intramuscular masses without bone erosion in 2. MRI showed the signal of isointensity or hypointensity on T1 weighted images (T1WI) in 25 patients and slightly hyperintense in three. On T2 weighted images (T2WI), 17 lesions displayed homogeneous hypointense signal, and eight lesions possessed heterogeneous signals. The remaining three lesions featured slightly hyperintense signal on T2WI. Follow-up data were available in 23 of 30 benign cases treated with gross-total resection, and two patients experienced recurrence. Spinal GCTTS should be suspected in cases with features such as the mass mainly involving the posterior bone elements, the lack of intralesional calcification, T2-weighted dark signals, and free of any cancer. Gross-total resection is an effective means for treating spinal GCTTS. [ABSTRACT FROM AUTHOR]

Abstract: Background context: Langerhans cell histiocytosis (LCH) of the spine has been well documented in the literature, but most studies concern management of the disease. No focused report on the differences in clinical and radiographic features of spinal LCH among children, adolescents, and adults exists. Purpose: To review and stress the clinical and imaging differences of spinal LCH in children, adolescents, and adults to avoid false diagnosis. Study design: A retrospective study of children and adults with LCH of the spine. Patient sample: Consecutive patients treated at our institution. Outcome measures: Visual analog scale for pain, Frankel scale for neurologic status, and X-ray, computed tomography (CT) and magnetic resonance imaging (MRI) for imaging. Methods: Seventy-six patients with pathology-proven LCH involving the spine were treated at our institution between 1996 and 2010. Only patients with spine involvement pathologically and/or radiographically were included. Two groups were identified based on the age. Group I comprised children and adolescents (age <18 years; n=40) and Group II comprised adults (age ≥18 years; n=36). Analysis included age and gender distribution, clinical presentation, and imaging features and diagnosis. Pathologic diagnosis was performed by needle or open biopsy of the lesions. Results: Of the 76 patients, 55 were male and 21 were female (ratio of 2.62:1). Neck or back pain was the most common symptom in all patients and was the only presenting symptom in some patients. Restricted motion of spine was the most frequent symptom secondary to pain. Thirty-seven patients presented with neurologic symptoms. Adult patients were more likely to suffer neurologic deficits (p<.005). The distribution of lesions revealed predominance in the cervical spine, followed by thoracic and lumbosacral spine. Plain radiology of children and adolescents with spinal LCH usually revealed a typical vertebral plana, but the adult patients represented different severity of vertebral collapse without typical features. The images of CT scans between the two groups were similar, and all revealed lytic lesions in vertebral bodies and/or posterior elements. In Group I, lesions showed hypointense on T1-weighted images in 15 cases and isointense in 25 cases. Nineteen patients presented as intermediate to slight high signal on T2-weighted images, and the remaining patients presented as hyperintense on T2-weighted images. In Group II, lesions showed hypointense on T1-weighted images in 29 cases, isointense on T1-weighted images in seven cases, and hyperintense on T2-weighted images in 36 cases. Paraspinal soft tissue mass was detected in 28 and 23 cases in Group I and Group II, respectively. Fifteen children and adolescent patients versus 23 adult patients had epidural spinal cord compression. Oversleeve-like or dumbbell sign was observed in 21 cases in Group I but only in four cases in Group II. Conclusions: The most common clinical manifestations of LCH of the spine were neck or back pain, followed by restricted motion of spine, neurologic symptoms, and deformity. Neurologic deficits were more frequent in adult patients. Vertebral plana is the typical imaging feature in children and adolescent patients but seldom in adults. Computed tomography is best for characterizing anatomy of the involved vertebra, and MRI is best for delineating marrow and soft tissue. The oversleeve-like sign on MRI may be a feature of spinal LCH as well as vertebra plana in children and adolescents. Needle biopsy under CT guidance should be performed before a treatment strategy is determined. [Copyright &y& Elsevier]

Benign fibrous histiocytoma (BFH) is extremely rare in the spine. We present a 52-year-old patient diagnosed with BFH in the thoracic spine and treated with total en bloc spondylectomy. The related literature was also reviewed. A total of 19 spinal BFHs have been reported, all involving the posterior elements of spine. Here we present the first BFH with total vertebral involvement. To our knowledge, this is the first case of total vertebral BFH. Spinal BFH is a considered a non-aggressive benign lesion. Surgical intervention is usually sufficient for managing BFH with both recurrence and metastasis uncommon. [ABSTRACT FROM AUTHOR]

Cellular schwannoma (CS) is a rare tumor that accounts for 2.8%–5.2% of all benign schwannomas. There is a dearth of up-to-date information on spinal CS in the literature. The aims of this study were to identify the proportion of CS cases amongst spinal benign schwannoma, describe the clinical features of spinal CS, and identify prognostic factors for local recurrence by analyzing data from 93 consecutive CS cases. Retrospective review. We analyzed 93 PSGCT screened from 1,706 patients with spine CS who were treated at our institute between 2008 and 2021. Demographic, radiographic, operative and postoperative data were recorded and analyzed. We compared the clinical features of spinal CS from the cervical, thoracic, lumbar and sacral segments. Prognostic factors for local recurrence-free survival (RFS) were identified by the Kaplan-Meier method. Factors with p≤.05 in univariate analysis were subjected to multivariate analysis by Cox regression analysis. The proportion of spinal CS in all benign schwannomas was 6.7%. The mean and median follow-up times for the 93 patients in this study were 92.2 and 91.0 months respectively (range 36–182 months). Local recurrence was detected in 11 cases, giving an overall recurrence rate of 11.7%, with one patient death. Statistical analysis revealed that tumor size ≥5 cm, intralesional resection, and Ki-67 ≥5% were independent negative prognostic factors for RFS in spinal CS. Whenever possible, en bloc resection is recommended for spinal CS. Long-term follow-up should be carried out for patients with tumor size ≥5 cm and postoperative pathological Ki-67 ≥5%. [ABSTRACT FROM AUTHOR]

Spinal phosphaturic mesenchymal tumor (PMT) is a rare disorder but can be cured once the diagnosis is clear and a complete removal by surgery is performed. To the best of our knowledge, only 22 cases in the spine have been described, and we report a case with the largest number of spinal segments (T12-L5) affected among spine PMT cases. A comprehensive literature search was performed until May 23, 2023, following the Preferred Reporting Items for Systematic Reviews guidelines. Studies were chosen through relevant PubMed, Web of Science, and EMBASE searches to prioritize obtaining the largest studies. The Medical Subject Headings and Boolean operators employed for this search were ("PMT" or "TIO" or "Tumor-induced osteomalacia" or "phosphaturic mesenchymal tumor") and ("spine" or "spinal"). Two researchers (L.S.Z. and D.B.C) independently reviewed and evaluated the included articles. Any differing opinions were discussed until a consensus was reached. A total of 18 studies were included. A case report is also presented. We report a case of spinal PMT. The full text of the relevant articles was construed. A total of 18 studies were reviewed and consolidated. These articles are roughly divided into the following 5 subcategories: 1) clinical features and baseline distribution, 2) laboratory and imaging findings, 3) pathological manifestations, and 4) surgical methods and treatment options. Spinal PMT is very rare with a high rate of misdiagnosis and debilitating complications, so it is of significance to increase awareness of the disease among spine surgeons consulted by patients with spinal PMT. 68Ga-DOTATOC-PET/CT shows very high sensitivity to the spinal PMT but there is no way to exactly determine the location of the tumor. PMT has unique immunohistochemical characteristics and malignant PMT is rare. Once diagnosed, complete surgical excision is the recommended treatment. Burosumab is one of the available options, especially in cases that are recurrent and difficult to surgically resect. [ABSTRACT FROM AUTHOR]

Study Design: Retrospective analysis.Objective: The study was designed to: (1) figure out risk factors of metastasis; (2) explore prognostic factors and develop a nomogram for pelvis and spine Ewing sarcoma (PSES).Summary Of Background Data: Tools to predict survival of PSES are still insufficient. Nomogram has been widely developed in clinical oncology. Moreover, risk factors of PSES metastasis are still unclear.Methods: The data were collected and analyzed from the Surveillance, Epidemiology, and End Results (SEER) database. The optimal cutoff values of continuous variables were identified by X-tile software. The prognostic factors of survival were performed by Kaplan-Meier method and multivariate Cox proportional hazards modeling. Nomograms were further constructed for estimating 3- and 5-year cancer-specific survival (CSS) and overall survival (OS) by using R with rms package. Meanwhile, Pearson χ2 test or Fisher exact test, and logistic regression analysis were used to analyze the risk factors for the metastasis of PSES.Results: A total of 371 patients were included in this study. The 3- and 5-year CSS and OS rate were 65.8 ± 2.6%, 55.2 ± 2.9% and 64.3 ± 2.6%, 54.1 ± 2.8%, respectively. The year of diagnosis, tumor size, and lymph node invasion were associated with metastasis of patients with PSES. A nomogram was developed based on identified factors including: age, tumor extent, tumor size, and primary site surgery. The concordance index (C-index) of CSS and OS were 0.680 and 0.679, respectively. The calibration plot showed the similar trend of 3-year, 5-year CSS, and OS of PSES patients between nomogram-based prediction and actual observation, respectively.Conclusion: PSES patients with earlier diagnostic year (before 2010), larger tumor size (>59 mm), and lymph node invasion, are more likely to have metastasis. We developed a nomogram based on age, tumor extent, tumor size, and surgical treatments for determining the prognosis for patients with PSES, while more external patient cohorts are warranted for validation.Level of Evidence: 3. [ABSTRACT FROM AUTHOR]

Background Context: Giant cell tumors (GCTs) of the bone are benign but locally aggressive. Pediatric spine giant-cell tumors (PSGCTs) have been infrequently reported in the literature because of the rarity of the disease.Purpose: The purpose of this study was to define the overall occurrence rate of PSGCTs among all spinal GCTs in our center and investigate the clinical features and prognostic factors of this rare disease.Study Design: A retrospective review.Patient Sample: Thirty-one PSGCT patients, screened from 226 patients with spine GCTs who received treatment in our center between 1998 to 2017.Outcome Measures: The clinical symptoms, neurologic status, radiologic manifestations, treatment, outcome, and complications were recorded and analyzed.Methods: The postoperative recurrence-free survival (RFS) rate was estimated by the Kaplan-Meier method. Factors with p values ≤.1 were subjected to multivariate analysis for RFS by proportional hazard analysis, among which p values ≤.05 were considered statistically significant.Results: A total of 31 (31 of 226, 13.7%) PSGCTs patients (9 male and 22 female) were included in the study, with a mean age of 15.9 years and a mean follow-up period of 85.1 (median 84.0; range 12-221) months. The majority of patients (80.6 %) were 14-18 years of age. Recurrence was detected in 12 (38.7%) of the 31 patients. Univariate and multivariate analyses suggested that Jaffe grade II-III was an adverse prognostic factor for RFS, while total spondylectomy and bisphosphonate treatment were positive prognostic factors.Conclusions: Total en bloc spondylectomy (TES) is associated with excellent prognosis for PSGCTs, and total piecemeal spondylectomy is a viable alternative if total en bloc spondylectomy is unfeasible. Long-term bisphosphonate administration could significantly reduce the recurrence risk of PSGCTs. Denosumab treatment is recommended, especially for advanced PSGCTs. Jaffe grade II-III is an adverse prognostic factor for recurrence. [ABSTRACT FROM AUTHOR]

Study Design: Retrospective survival analysis of 44 undifferentiated high grade pleomorphic sarcoma (UPS) of the spine.Objective: To identify factors related to overall survival (OS) and help decision making in the treatment of undifferentiated high grade pleomorphic sarcoma of the spine.Summary Of Background Data: UPS is an aggressive malignant tumor rarely originating from the spine. Due to its scarcity, only a few studies had been reported to describe the clinical features, treatments, and outcomes of sporadic cases, devoid of evaluation on prognostic factors.Methods: Enrolled in this survival analysis were 44 patients who underwent surgery and adjuvant therapies from January 1999 to December 2015. Kaplan-Meier methods were applied to estimate the overall survival. A multivariate Cox algorithm was applied to recognize factors independently associated with overall survival.Results: Multivariate analysis suggested that age greater than or equal to 55 years (hazard ratio [HR], 3.923, P < 0.001), Eastern Cooperative Oncology Group (ECOG) score four (HR, 4.656, P < 0.001), and subtotal resection or piecemeal total resection (HR, 4.375, P < 0.001) were independently associated with poor overall survival.Conclusion: We identified independent prognostic factors of UPS of the spine. Subtotal resection or piecemeal total resection, age more than or equal to 55 years and ECOG score four are factors adversely affecting overall survival of patients with UPS of the spine.Level Of Evidence: 4. [ABSTRACT FROM AUTHOR]

Study Design: Retrospective study.Objective: The aim of this study was to compare the clinical outcomes between adolescent and young adult (AYA) patients and old adult patients with cervical chordoma who were treated surgically and present the surgical consideration for adolescents and young adults with cervical chordoma.Summary Of Background Data: With predominance in senior patients, chordoma is distinctively rare in AYAs. Because of the rarity of AYA chordoma, individual case report represents most of the literature on this disease entity on mobile spine and lack of long-term follow up, which leads to the paucity of clinical evidence for treatment planning and prognosis prediction.Methods: A retrospective study was conducted to investigate the prognosis of AYA patients with cervical chordoma who were treated surgically. We collected the clinical data of these patients and their older counterparts, and further compared the prognosis of the patients in different age groups. To estimate survival curves, Kaplan-Meier method was used, and significance was assessed using a log-rank test.Results: Forty consecutive patients with chordoma of the cervical spine treated in our institution were included in the study. Two groups were identified according to age. Group 1 comprised children and adolescents (age ≤ 25 yrs; n = 9) and Group 2 comprised adults (age > 25 years; n = 31). In comparison, Group 1 was featured by significantly higher rate of recurrence and shorter overall survival, although no difference found in the surgical modality between two groups.Conclusion: There is a dismal prognosis in young patients with chordoma, and thus support the notion that as radical a total en bloc spondylectomy (TES) of the lesions as possible may benefit the overall survival of these young patients. Although the ensuing neurological deficits may be devastating, it will be worth sacrificing if the life expectancy of these young patients is prolonged.Levels Of Evidence: 4. [ABSTRACT FROM AUTHOR]

Study Design: A retrospective study.Objective: The aim of this study was to discuss the factors that may affect surgical outcomes of patient with diffuse large B-cell lymphoma (DLBCL)-associated metastatic spinal cord compression (MSCC).Summary Of Background Data: DLBCL in the spine is rare and minimal information has been published in the literature regarding this subject. Although DLBCL is highly sensitive to both chemotherapy and radiotherapy, surgical decompression is recommended in the treatment of DLBCL-associated MSCC. However, the prognostic factors affecting surgical outcomes of patients with DLBCL-associated MSCC remain unknown.Methods: We conducted a retrospective study to investigate the impact of surgical decompression on recovery from neurological deficit caused by DLBCL-associated MSCC. Univariate and multivariate analyses were performed to identify prognostic factors for overall survival of spinal DLBCL. The survival rate was estimated by the Kaplan-Meier method, and differences were analyzed by the log-rank test. Factors with P values of 0.1 or less were subjected to multivariate analysis for survival rate by multivariate Cox proportional hazards analysis.Results: A total of 37 patients with spinal DLBCL from 2003 to 2014 were included in the study. International Prognostic Index (IPI) (0-1/2-5), duration of preoperative symptoms (≤2m/>2m), and lactate dehydrogenase (normal/abnormal) were suggested as the potential prognostic factors through univariate analysis. However, as they were submitted to the multivariate Cox regression model, only IPI and duration of preoperative symptoms were found as independent prognostic factors.Conclusion: Surgical decompression improves recovery from neurological deficit. Patient IPI score plays an important role in decision making for surgical intervention, as it affects the length of survival and functional outcome. Patients with duration of preoperative symptoms no more than 2 months confer a very poor prognosis.Level Of Evidence: 4. [ABSTRACT FROM AUTHOR]