Association between serum interleukin-17 levels and ectopic bone formation in OPLL patients with DISH.

OBJECTIVE: To investigate the relationship between the severity and morphology of heterotopic ossification in the spinal ligaments including sacroiliac (SI) joints, and serum interleukin-17 (IL-17) levels in patients with ossification of the posterior longitudinal ligament (OPLL) with or without diffuse idiopathic skeletal hyperostosis (DISH), as well as a non-OPLL group. METHODS: A total of 103 patients with OPLL (DISH (-), n = 50; DISH (+), n = 53) and 53 age- and gender-matched controls were included. The serum levels of IL-17 were analyzed, and the severity of ectopic ossification and the morphology of ectopic bone formation were evaluated. The SI joint morphological variations were categorized into four types. RESULTS: No significant differences were found in serum IL-17 levels between the OPLL and control groups. However, the DISH (+) group showed higher IL-17 levels than the DISH (-) group, especially in female patients (p = 0.003). Additionally, IL-17 levels were positively correlated with the number of Flat vertebral units, meaning one of the characteristics of DISH ossification type (R2 = 0.199, p = 0.012). IL-17 levels in type 4 were significantly higher in the DISH (+) group than in the DISH (-) group. CONCLUSIONS: The morphological characteristics of paravertebral bone formation in the entire spine, including the SI joint, are likely associated with serum IL-17 levels in OPLL. These findings provide pathological and serological evidence of local inflammation contributing to paravertebral ossification of OPLL patients.

Morphological characteristics of DISH in patients with OPLL and its association with high-sensitivity CRP: inflammatory DISH.

OBJECTIVES: To characterize and clarify evidence as to whether the ectopic bone formations of DISH in patients with ossification of the posterior longitudinal ligament (OPLL) are caused by inflammatory or degenerative processes. METHODS: Whole-spine CT and serum high-sensitivity CRP (hs-CRP) levels were obtained from 182 cervical OPLL patients (DISH+, n = 104; DISH-, n = 78). In the DISH+ group, ectopic bone formations were categorized into Flat and Jaggy types, then further divided into three subgroups: group 1 (Jaggy-dominant pattern), group 2 (Equivalence of pattern) and group 3 (Flat-dominant pattern). Data were compared between the DISH+ and DISH- groups, and among the three subgroups. RESULTS: The upper thoracic spine was most affected by the Flat type, whereas the Jaggy type was more frequent in the middle and lower thoracic regions. There was no difference in hs-CRP levels between the DISH+ and DISH- groups. Among the three subgroups, hs-CRP levels in group 3 [mean (s.d.) 0.16 (0.09) mg/dl] were significantly higher than in group 1 [0.04 (0.02) mg/dl] and group 2 [0.08 (0.06) mg/dl]. Higher levels of hs-CRP were associated with a greater number of vertebral units with Flat-type formations (ß = 0.691, P < 0.0001) and with a lesser number of vertebral units with Jaggy-type formations (ß = -0.147, P = 0.036). CONCLUSION: The Flat type in DISH might be caused by an inflammatory pathogenesis rather than a degenerative process presented in the Jaggy type.

Sacroiliac joint variation associated with diffuse idiopathic skeletal hyperostosis.

BACKGROUND: Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by the ossification of vertebral bodies and peripheral entheses. However, variations in sacroiliac (SI) joint change in patients with DISH have not been fully clarified. The purpose of this study was to evaluate SI joint variation in patients with DISH in comparison with a non-DISH population. METHODS: A total of 342 SI joints in 171 patients (DISH+, n = 86; DISH-, n = 85) who had undergone lumbar spine surgery were analyzed by computed tomography examination. SI joint variations were classified into four types: Type 1, normal or tiny peripheral bone irregularity; Type 2, subchondral bone sclerosis and osteophytes formation; Type 3, vacuum phenomenon; and Type 4, bridging osteophyte and bony fusion. The type of bridging osteophyte in SI joints and the prevalence of ossification in each spinal segment from C1 to SI joint were also examined. RESULTS: The most common SI joint variation in the DISH+ group was bony fusion (Type 4), with 71.6% exhibiting anterior paraarticular bridging. On the other hand, SI joint vacuum phenomenon (Type 3) was the most frequent change (57.1%) in the DISH- group. The middle to lower thoracic spine and SI joints were highly affected in DISH and caused bony ankylosis. CONCLUSIONS: Anterior paraarticular bridging was the most common type of SI joint change in patients with DISH who underwent lumbar spine surgery. The present results regarding variations of SI joint changes in DISH should help understand the etiology of DISH.

The effect of multiple lesions in patients with ossification of the posterior longitudinal ligament of the cervical spine.

PURPOSE: Ossification of the posterior longitudinal ligament of the cervical spine (cervical OPLL) is associated with the lesions at the thoracic and/or lumbar spine. Multiple spinal lesions cause additional neurological deficit, affecting the outcomes of cervical laminoplasty. This study aimed to clarify the effect of multiple lesions on the outcomes of cervical laminoplasty and to compare the results with data from patients without them. METHODS: From April 1981 to October 2015, 201 patients underwent laminoplasty for cervical OPLL; however, 167 patients were followed for >2 years. Twenty-four patients underwent additional surgery for multiple lesions due to spinal stenosis. The pathologies of the lesions were assessed. The patients were divided into two groups: the thoracic and thoraco-lumbar group (T-group: 8 patients) and the lumbar group (L-group: 16 patients). One-hundred patients without an additional surgery served as the control group. The maximum Japanese Orthopaedic Association (JOA) score and the most recent score for recovery was compared between the multiple and control groups. RESULTS: The maximum score and recovery rate and the score and recovery rate at the last follow-up in the multiple group were lower than those in the control group. There was no significant difference in the postoperative JOA score and recovery rate between the T-group and the L-group. CONCLUSIONS: Neurological recovery in patients with multiple lesions was poorer than in those without lesions. Therefore, special attention should be paid to cervical OPLL with multiple spinal lesions.

Clinical and imaging characteristics in patients undergoing surgery for lumbar epidural lipomatosis.

BACKGROUND: Lumbar epidural lipomatosis (LEL) is characterized by abnormal accumulation of unencapsulated adipose tissue in the spinal epidural space. Such accumulation compresses the dural sac and nerve roots, and results in various neurological findings. However, the pathophysiology of LEL remains unclear. This study examined the associations between imaging and clinical findings in detail, and investigated the mechanisms underlying symptom onset by measuring intraoperative epidural pressures in LEL. METHODS: Sixteen patients (all men; mean age, 68.8 years) were enrolled between 2011 and 2015. Mean body mass index was 26.5 kg/m2. Four cases were steroid-induced, and the remaining 12 cases were idiopathic. All patients presented with neurological deficits in the lower extremities. Cauda equina syndrome (CES) alone was seen in 8 patients, radiculopathy alone in 4, and both radiculopathy and CES (mixed CES) in 4. All patients subsequently underwent laminectomy with epidural lipomatosis resection and were followed-up for more than 1 year. We investigated the clinical course and imaging and measured epidural pressures during surgery. RESULTS: Subjective symptoms improved within 1 week after surgery. Mean Japanese Orthopaedic Association (JOA) score was 15.2 ± 2.8 before surgery, improving to 25.4 ± 2.5 at 1 year after surgery. On magnetic resonance imaging, all lipomatosis lesions included the L4-5 level. On preoperative computed tomography, saucerization of the laminae was not observed in radiculopathy cases, whereas saucerization of the posterior vertebral body was observed in all radiculopathy or mixed CES cases. Intraoperative epidural pressures were significantly higher than preoperative subarachnoid pressures. The results suggest that high epidural pressure resulting from the proliferation of adipose tissue leads to saucerization of the lumbar spine and subsequent symptoms. CONCLUSIONS: Clinical courses were satisfactory after laminectomy. In LEL, epidural pressure increases and symptoms develop through the abnormal proliferation of adipose tissue. Higher epidural pressures induce saucerization of the laminae and/or posterior vertebral body. Furthermore, the direction of proliferative adipose tissue (i.e., site of saucerization) might be related to the types of neurological symptoms.

Teriparatide versus low-dose bisphosphonates before and after surgery for adult spinal deformity in female Japanese patients with osteoporosis.

PURPOSE: Complications of adult spinal deformity surgery are problematic in osteoporotic individuals. We compared outcomes between Japanese patients treated perioperatively with teriparatide vs. low-dose bisphosphonates. METHODS: Fifty-eight osteoporotic adult Japanese female patients were enrolled and assigned to perioperative teriparatide (33 patients) and bisphosphonate (25 patients) groups in non-blinded fashion. Pre- and post-operative X-ray and computed tomography imaging were used to assess outcome, and rates were compared between the groups and according to age. Pain scores and Oswestry Disability Indices (ODI) were calculated before and 2 years after surgery. RESULTS: Adjacent vertebral fractures and implant failure, fusion failure, and poor pain and ODI outcomes were significantly more common in the bisphosphonates group than the teriparatide group. CONCLUSIONS: Perioperative administration of teriparatide is more effective than that of low-dose bisphosphonates in preventing complications and maintaining fusion rates in osteoporotic Japanese females with spinal deformities undergoing surgery.

Fibroma of tendon sheath around large joints: clinical characteristics and literature review.

BACKGROUND: Fibroma of tendon sheath (FTS) is a benign tumor arising from the synovium of the tendon sheath that occurs mostly around small joints such as the fingers, hands, and wrist. However, FTS rarely arises around a large joint (knee, shoulder, elbow, and ankle) with intra-articular or extra-articular involvement. The clinical characteristics of FTS arising around a large joint are unclear. An additional 3 cases of FTS arising around a large joint are presented. Furthermore, the published cases and the present cases are reviewed with respect to their clinical characteristics and imaging and histopathology findings. METHODS: The 43 reported cases including the present 3 patients were summarized, and the patients' profiles, symptoms, sites and locations in the joint involved by FTS, magnetic resonance imaging (MRI) findings, surgical procedures, clinical courses, and cytogenetic analyses were reviewed. RESULTS: The average age of 26 cases was 40.9 years (range 13-69 years), and about 60% of the patients were male. About 10% of the patients had a past history of trauma to the knee joint. Of the present 3 cases, one case was extra-articular around the elbow joint, one case was extra-articular around the knee joint, and one case was intra-articular involving the knee joint. The common symptoms were pain (62.5%), swelling or palpable mass (54.2%), and limited range of motion of the involved joint (50%). The most commonly involved joint was the knee, with 32 cases (74.4%), followed by the elbow in 5 cases (11.6%), ankle in 4 (9.3%), and shoulder in 2 (4.7%). The tumor typically exhibited iso to low signal intensity on T1-weighted MRI. T2-weighted images showed various patterns, but mostly low signal intensity relative to muscle. The surgical margin was marginal resection in all cases. There were no recurrences after surgery. On chromosomal analysis, only the present Case 3 showed an abnormality. CONCLUSIONS: A total of 43 FTS cases that occurred around large joints were summarized. The most common site was around the knee joint. In FTS cases around large joints, it is necessary to distinguish between various fibroblastic and/or fibrohistiocytic tumors.

Lumbar disc degeneration progression in young women in their 20's: A prospective ten-year follow up.

BACKGROUND: Lumbar disc degeneration (LDD) is known to be the main cause of low back pain, although it is unclear how or when LDD progresses. The purpose of this study was to clarify the process and features of LDD progression in young women. METHODS: We enrolled 480 nursing students and carried out a prospective cohort study in 84 nursing students. MRI of the lumbar spine was taken once during their time as a student and again at 9.8 years (7-14) after the first MRI when they were working as nurses. The grade of LDD was determined according to Schneiderman's grade. The progression of disc degeneration was evaluated by the change in the degenerative disc disease (DDD) score (the summation of DDD score at each disc level). The subjects were divided into two groups based on MRI findings: Group A, those without disc degeneration at the first MRI (n = 58) and Group B, those showing disc degeneration on the first MRI (n = 26). We evaluated the change in DDD score and assessed the particular disc levels that showed Schneiderman's grade worsening in each group. RESULTS: At the time of the 2nd MRI, the DDD score increased from 5.6 (5-9) to 6.3 (5-11). The L5/S disc was the most frequent level showing the progression of LDD. In Group A, 18 subjects (31.0%) and in Group B, 15 subjects (57.7%) had degeneration progression (p = 0.02). CONCLUSIONS: This study revealed that 31% of the young adult subjects already had disc degeneration in 20's (time of first MRI) and the disc degeneration rapidly progressed in these subjects.

The clinical outcome of pazopanib treatment in Japanese patients with relapsed soft tissue sarcoma: A Japanese Musculoskeletal Oncology Group (JMOG) study.

BACKGROUND: Because the efficacy and safety of pazopanib in Japanese patients with soft tissue sarcoma (STS) had not been evaluated previously in a large-scale cohort, the authors investigated the efficacy and safety of pazopanib in 156 Japanese patients with relapsed STS. This was a retrospective study based on the collection of real-life, postmarketing surveillance data. METHODS: Patients received pazopanib with the objective of treating local recurrence (n = 20), metastasis (n = 104), and both (n = 32). The patient median age was 53.8 years. The primary objective of this study was to clarify the efficacy of pazopanib for patients with STS. RESULTS: The median treatment duration was 28.7 weeks, and the average dose intensity of pazopanib was 609 mg. Adverse events occurred in 127 patients (81.4%). In addition to the main common toxicities, such as hypertension and liver disorder, pneumothorax (n = 11) and thrombocytopenia (n = 16) also were observed. The median progression-free survival for all patients was 15.4 weeks. The median progression-free survival for patients with leiomyosarcoma, synovial sarcoma, undifferentiated pleomorphic sarcoma, and liposarcoma was 18.6 weeks, 16.4 weeks, 15.3 weeks, and 8 weeks, respectively. The median survival for all patients was 11.2 months. The median survival for patients with leiomyosarcoma, synovial sarcoma, undifferentiated pleomorphic sarcoma, and liposarcoma was 20.1 months, 10.6 months, 9.5 months, and 7.3 months, respectively. CONCLUSIONS: There were apparent differences in the efficacy of pazopanib treatment among histologic types of STS. Pazopanib treatment is a new treatment option; however, adverse events like pneumothorax and thrombocytopenia, which did not occur frequently in the PALETTE study (pazopanib for metastatic soft-tissue sarcoma), should be taken into consideration. Cancer 2016;122:1408-16. © 2016 The Authors. Cancer published by Wiley Periodicals, Inc. on behalf of American Cancer Society.

Characteristics of ossification of the spinal ligament; incidence of ossification of the ligamentum flavum in patients with cervical ossification of the posterior longitudinal ligament - Analysis of the whole spine using multidetector CT.

BACKGROUND: Ossification of the posterior longitudinal ligament (OPLL) and ossification of the ligamentum flavum (OLF) are characterized by replacement of ligamentous tissue by ectopic new bone formation. Although the background of both diseases might be similar, there are some differences between two diseases. Some patients have both OPLL and OLF. However, the incidence of both OPLL and OLF is still unclear and the precise lesions have not been investigated, yet. This study was conducted to evaluate OLF of the whole spine in patients with cervical OPLL and to analyze the relationship of the ossified lesions between OLF and OPLL. METHODS: One hundred seventy eight patients who were diagnosed as cervical OPLL by plain radiographs were included. CT images of the whole spine were taken. Ossified lesions were checked at each level of vertebral body and intervertebral disc. The ossification index of OPLL (OPLL OS index) was determined by the sum of the levels of vertebral bodies and intervertebral discs where OPLL existed. The same index was applied for detecting the level of OLF (OLF OS index). Age, gender and OPLL characteristics were compared between the OLF(+) group, OLF was seen at any levels of the spinal canal, and the OLF(-) group, OLF was not seen. RESULTS: The most frequent level of OPLL was at C5 vertebral level and OLF was predominant at upper and lower thoracic levels. Seventeen patients (9.6%) had OPLL and OLF at the same spinal level. The averaged OPLL OS index of the total spine in these patients was 8.7 ± 6.1, ranged from 1 to 36. The averaged OLF OS index of the total spine was 3.1 ± 2.2 (ranged from 1 to 13) in the patients who had OLF at any levels of the whole spine. One hundred fifteen patients (64.6%) with cervical OPLL had OLF at any levels of the whole spine. No relationship was found between the OPLL OS index and the OLF OS index. There was no significant difference among the data between the OLF(+) group and the OLF(-) group. CONCLUSIONS: This study demonstrated 64.6% of the patients with cervical OPLL had OLF, mainly in the thoracic spine. However, there was no relationship regarding the severity of the ossified lesions between OPLL and OLF. CT analysis of the whole spine should be carried out for the early detection of OPLL and OLF in patients with cervical OPLL.

Clinical significance of high intramedullary signal on T2-weighted cervical flexion-extension magnetic resonance imaging in cervical myelopathy.

BACKGROUND: In cervical myelopathy, significant findings are seen in flexion-extension MRI due to the increased likelihood of cord compression during neck extension. In addition, a high intramedullary signal on T2-weighted MR images has been reported to be a prognostic factor in this condition. However, the relationship between Japanese Orthopaedic Association (JOA) scores and the signal intensity in preoperative cervical flexion-extension T2-weighted images has not been evaluated. The purpose of this study was to evaluate whether preoperative flexion-extension MRI may be used to predict surgical outcomes in patients with cervical myelopathy. METHODS: A total of 121 patients who underwent surgery for cervical myelopathy were included. All patients underwent preoperative cervical flexion-extension MRI followed by cervical decompression surgery, with or without spinal fusion, and postoperative follow-up for at least 2 years. Pre- and postoperative (2 years after surgery) JOA scores were recorded, and the degree of postoperative improvement was calculated. The relationship between intramedullary signal intensity on preoperative cervical dynamic MRI findings and degree of clinical recovery was examined. RESULTS: Patients with a high intramedullary signal on the extension MRI had significantly better neurological recovery than those with a high signal on the flexion MRI (p < 0.000005). There was no significant difference in neurological recovery between patients with and without a high intramedullary signal on extension MRI. CONCLUSIONS: A preoperative high intramedullary signal on flexion MRI was associated with a poor surgical outcome, while no such association was seen with extension MRI.

Surgical treatment of lumbar vertebral collapse in a patient with Parkinson's disease: a case report.

Parkinson's disease (PD) frequently develops postural abnormalities including extreme neck flexion and trunk flexion. Patients with PD sometimes have osteopenia and vertebral deformity due to the destruction of fragile bone can be also associated with the spinal deformity. Surgical treatment for these patients is very difficult. We encountered a patient with PD presenting severe trunk sagittal and frontal deformity. The patient had cauda equina syndrome due to progressive vertebral collapse of the lumbar spine. We performed anterior reconstruction surgery at first in order to achieve improved lordotic alignment of the lumbar spine. Then, we performed 2 posterior surgeries, resulting in total long fusion from T4 to S1. The clinical findings of this patient were presented, and the treatment options were discussed.

Relationship between postoperative axial symptoms and the rotational angle of the cervical spine after laminoplasty.

INTRODUCTION: An axial symptom is one of the postoperative problems after cervical laminoplasty. The range of motion (ROM) of the cervical spine decreases after laminoplasty. It is speculated that the reduction in neck ROM is one of the causes of axial symptoms. However, most reports have focused on ROM of the cervical spine in flexion and extension, and few papers describe the rotational motion after surgery. It is impossible to assess the rotational motion in plain X-rays. This study was undertaken for the following two purposes: (1) to compare segmental rotational angles of the cervical spine between control subjects and patients with cervical laminoplasty ; (2) to analyze the relationship between postoperative axial symptoms and the range of cervical rotational angle after laminoplasty. METHODS: Eleven young volunteers (control 1 group) and 10 elderly subjects (control 2 group) who did not have any neck problems and 23 patients who had en bloc cervical laminoplasty due to cervical spondylotic myelopathy were included. Dynamic CT scan was conducted in neutral, right, and left maximum rotational positions from C1 to T1. We measured the rotational angle in each vertebral segment. The axial symptoms, such as limited neck motion and neck stiffness, were assessed by a questionnaire. The rotational angle of the cervical spine was compared between the patients with axial symptoms and the subjects without. RESULTS: The rotational angle in control 1 group was greater than that in control 2 group. The rotational angle was markedly decreased in the patients with cervical laminoplasty compared to that in control 2 group. The reduction was observed at the upper vertebral segment. Twelve patients were judged to have axial symptom after cervical laminoplasty (symptom + group), and 11 patients did not have symptoms (symptom - group). The rotational angle in the symptom + group was significantly smaller than that in symptom - group. The reduction in the rotational angle was found below C2 level, whereas the C1-2 angle was not significantly different in the two symptom groups. CONCLUSION: Reduction of the rotational angle in the cervical spine was clearly observed in patients with cervical laminoplasty, compared to that in the controls. The marked reduction might be related to the axial symptoms after laminoplasty.

Assessment of Cervical Myelopathy Risk in Ossification of the Posterior Longitudinal Ligament Patients With Spinal Cord Compression Based on Segmental Dynamic Versus Static Factors.

OBJECTIVE: Using segmental dynamic and static factors, we aimed to elucidate the pathogenesis and relationship between ossification of the posterior longitudinal ligament (OPLL) and the severity of cervical myelopathy. METHODS: Retrospective analysis of 163 OPLL patients' 815 segments. Imaging was used to evaluate each segmental space available for the spinal cord (SAC), OPLL diameter, type, bone space, K-line, the C2-7 Cobb angle, each segmental range of motion (ROM), and total ROM. Magnetic resonance imaging was used to evaluate spinal cord signal intensity. Patients were divided into the myelopathy group (M group) and the without myelopathy group (WM group). RESULTS: Minimal SAC (p = 0.043), (C2-7) Cobb angle (p = 0.004), total ROM (p = 0.013), and local ROM (p = 0.022) were evaluated as an independent predictor of myelopathy in OPLL. Different from the previous report, the M group had a straighter whole cervical spine (p < 0.001) and poorer cervical mobility (p < 0.001) compared to the WM group. Total ROM was not always a risk factor for myelopathy, as its impact depended on SAC, when SAC > 5 mm, the incidence rate of myelopathy decreased with the increase of total ROM. Lower cervical spine (C5-6, C6-7) showing increased "Bridge-Formation," along with spinal canal stenosis and segmental instability (C2-3, C3-4) in the upper cervical spine, could cause myelopathy in M group (p < 0.05). CONCLUSION: Cervical myelopathy is linked to the OPLL's narrowest segment and its segmental motion. The hypermobility of the C2-3 and C3-4, contributes significantly to the development of myelopathy in OPLL.

Rod Rotation with Outrigger Is Substantial for Correcting Apical Hypokyphosis in Patients with Adolescent Idiopathic Scoliosis: Novel Outrigger Device for Concave Rod Rotation.

The apical hypokyphosis of scoliotic patients is thought to lead to decreased lung capacity and cause shortness of breath. Additionally, concave rod curve reduction is a problem in the correction of apical hypokyphosis in posterior spinal fusion surgery in adolescent idiopathic scoliosis (AIS). We investigated the contributions of rod rotation (RR) with an outrigger device, followed by differential rod contouring (DRC) with the outrigger attached to the concave rod, designed to prevent concave rod curve-flattening. We analyzed and compared the results of segmental pedicle screw fixation without the outrigger in 41 AIS patients with thoracic curves (Lenke type I, 25; type II, 16) to those corrected using the outrigger in 36 patients (Lenke type I, 24; type II,12). The changes in the Cobb angle, apical kyphosis of five vertebrae, thoracic kyphosis (TK, T4-12), correction rate, correction angle of apical vertebral rotation, spinal penetration index (SPi), and rib hump index (RHi) before and after surgery were measured, and the contribution of the outrigger was analyzed. The mean scoliosis correction rates without and with the outrigger were 72.1° and 75.6°, respectively (p = 0.03). Kyphosis of the five apical vertebrae and TK were significantly greater in the surgery with the outrigger (p = 0.002). Significantly greater improvements in SPi and RHi were also noted in the surgery with the outrigger (p < 0.05). The use of concave RR and convex DRC with the outrigger appear to be advantageous for correcting apical hypokyphosis, followed by the subsequent formation of TK. As a result, breathing problems are less likely to occur during daily life because of improvements in SPi and RHi.

Sacroiliac Joint Variation in Patients With Ossification of the Posterior Longitudinal Ligament.

STUDY DESIGN: Retrospective Cohort Study. OBJECTIVES: Ossification of the posterior longitudinal ligament (OPLL) reveals heterotopic ossification in the spinal ligament. OPLL also tends to ossify ligaments and entheses throughout the body. However, hallmarks of sacroiliac (SI) joint ossification and its variation in OPLL have not been clarified. Here, we investigated the morphological changes in SI joints in individuals with and without OPLL. METHODS: We included 240 age- and sex-matched patients (OPLL+, 120; OPLL-, 120) in the study. SI joint variations were classified into 4 types: Type 1, normal or small peripheral bone irregularity; Type 2, subchondral bone sclerosis and osteophyte formation; Type 3, vacuum phenomenon; and Type 4, bridging osteophyte and bony fusion. Type 4 was further divided into 3 subgroups as previously described. Interactions between the ossified spinal region in OPLL and morphological changes in the SI joint were evaluated. RESULTS: SI joint ankylosis occurs more frequently in patients with OPLL (51.7%) than in those without (non-OPLL) (33.3%). The SI joint vacuum phenomenon (49.2%) was the main finding in non-OPLL. SI joint ankylosis in OPLL was characterized by anterior bridging and intra-articular fusion. OPLL patients with multilevel ossification tend to develop degeneration and ankylosis of the SI joints. CONCLUSIONS: OPLL conferred a high risk of SI joint ossification compared with non-OPLL, and patients with extensive ossification had a higher rate of SI joint ankylosis. Understanding SI joint variation could help elucidate OPLL etiology and clarify the phenotypic differences in the SI joint between OPLL and other spinal disorders.

Association of Inflammation, Ectopic Bone Formation, and Sacroiliac Joint Variation in Ossification of the Posterior Longitudinal Ligament.

Ossification of the posterior longitudinal ligament (OPLL) is considered a multifactorial condition characterized by ectopic new bone formation in the spinal ligament. Recently, its connections with inflammation as well as sacroiliac (SI) joint ankylosis have been discussed. Nevertheless, whether inflammation, spinal ligament ossification, and SI joint changes are linked in OPLL has never been investigated. In this study, whole-spinal computed tomography and serum high-sensitive C-reactive protein (hs-CRP) levels were obtained in 162 patients with cervical OPLL. Ossification lesions were categorized as plateau and hill shapes. Accordingly, patients were divided into plateau-shaped (51 males and 33 females; mean age: 67.7 years) and hill-shaped (50 males and 28 females; mean age: 67.2 years) groups. SI joint changes were classified into four types and three subtypes, as previously described. Interactions among ossification shapes, hs-CRP levels, and morphological changes in the SI joint were investigated. The plateau shape was more common in the vertebral segments (59.5%), compared to the hill shape, which was predominant in the intervertebral regions (65.4%). Serum hs-CRP levels in the plateau-shaped group (0.11 ± 0.10 mg/dL) were significantly higher than those in the hill-shaped group (0.07 ± 0.08 mg/dL). SI joint intra-articular fusion was the main finding in the plateau-shaped group and showed significantly higher hs-CRP levels compared to the anterior para-articular bridging, which more frequently occurred in the hill-shaped group. Our findings suggested a possible inflammation mechanism that might contribute to the new bone formation in OPLL, particularly the plateau shape.

Clinical Outcome of Low-Grade Myofibroblastic Sarcoma in Japan: A Multicenter Study from the Japanese Musculoskeletal Oncology Group.

This retrospective multicenter study aimed to analyze the clinical features and prognosis of 24 patients diagnosed with LGMS between 2002 and 2019 in the Japanese sarcoma network. Twenty-two cases were surgically treated and two cases were treated with radical radiotherapy (RT). The pathological margin was R0 in 14 cases, R1 in 7 cases, and R2 in 1 case. The best overall response in the two patients who underwent radical RT was one complete response and one partial response. Local relapse occurred in 20.8% of patients. Local relapse-free survival (LRFS) was 91.3% at 2 years and 75.4% at 5 years. In univariate analysis, tumors of 5 cm or more were significantly more likely to cause local relapse (p < 0.01). In terms of the treatment of relapsed tumors, surgery was performed in two cases and radical RT was performed in three cases. None of the patients experienced a second local relapse. Disease-specific survival was 100% at 5 years. A wide excision aimed at the microscopically R0 margin is considered the standard treatment for LGMS. However, RT may be a viable option in unresectable cases or in cases where surgery is expected to cause significant functional impairment.

SYT-SSX breakpoint peptide vaccines in patients with synovial sarcoma: a study from the Japanese Musculoskeletal Oncology Group.

In the present study, we evaluated the safety and effectiveness of SYT-SSX-derived peptide vaccines in patients with advanced synovial sarcoma. A 9-mer peptide spanning the SYT-SSX fusion region (B peptide) and its HLA-A*2402 anchor substitute (K9I) were synthesized. In Protocols A1 and A2, vaccines with peptide alone were administered subcutaneously six times at 14-day intervals. The B peptide was used in Protocol A1, whereas the K9I peptide was used in Protocol A2. In Protocols B1 and B2, the peptide was mixed with incomplete Freund's adjuvant and then administered subcutaneously six times at 14-day intervals. In addition, interferon-α was injected subcutaneously on the same day and again 3 days after the vaccination. The B peptide and K9I peptide were used in Protocols B1 and B2, respectively. In total, 21 patients (12 men, nine women; mean age 43.6 years) were enrolled in the present study. Each patient had multiple metastatic lesions of the lung. Thirteen patients completed the six-injection vaccination schedule. One patient developed intracerebral hemorrhage after the second vaccination. Delayed-type hypersensitivity skin tests were negative in all patients. Nine patients showed a greater than twofold increase in the frequency of CTLs in tetramer analysis. Recognized disease progression occurred in all but one of the nine patients in Protocols A1 and A2. In contrast, half the 12 patients had stable disease during the vaccination period in Protocols B1 and B2. Of note, one patient showed transient shrinkage of a metastatic lesion. The response of the patients to the B protocols is encouraging and warrants further investigation.

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis.

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. We report a case of IHSP followed up for 20 years in a 46-year-old man. Expansive laminoplasty was performed in 1991, and this case has been previously reported by a co-author. After 17 years, the patient's gait disturbance returned. Physical examination and imaging confirmed IHSP that had developed into syringomyelia at the T2-L1 conus level. This case was diagnosed as adhesive spinal arachnoiditis due to pachymeningitis caused by syringomyelia. T1-T4 laminectomy, a syringo-subarachnoid shunt (S-S shunt), and L2-L3 laminectomy were performed. The patient again developed dysesthesia and gait disturbance 3 years after the second operation. Most reports of IHSP have limited their focus to short-term follow-up after initial treatment with no long-term results. At present, there are only five reports referring to long-term results of greater than 5 years. All but one case needed additional surgery. To the best of our knowledge, this is the first case in which syringomyelia occurred in a patient with IHSP. It is important to note that syringomyelia may be a cause of symptom recrudescence during long-term follow-up in IHSP patients.