Kayo Suzuki

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.

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.

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.

BCL6 corepressor-cyclin B3 (BCOR‑CCNB3) fusion sarcoma was classified as an emerging subgroup of undifferentiated small round cell sarcoma in 2020. The incidence of BCOR‑CCNB3 fusion sarcoma is reportedly 1.5‑14% among undifferentiated unclassified sarcomas, representing a rare entity among primary malignant bone tumors. The present study reports a case of BCOR‑CCNB3 fusion sarcoma in the proximal tibia of a boy. A 12‑year‑old boy presented with a 6‑month history of knee pain and a slowly growing mass in the anteromedial aspect of the left proximal tibia. Plain radiography and computed tomography of the knee demonstrated a lytic lesion with cortical destruction of the proximal tibia. Magnetic resonance imaging showed the bone tumor expanding into soft tissue with almost homogeneous hypointensity on T1‑weighted imaging and slightly hyperintensity on T2‑weighted imaging. On histopathological evaluation, the tumor comprised a proliferation of small, round to ovoid‑shaped mesenchymal cells without osteoid formation. Histopathologically, BCOR‑CCNB3 sarcoma of bone was finally diagnosed based on immunohistochemical staining and additional molecular analyses. The patient underwent bone tumor resection followed by pre‑ and post‑operative chemotherapy according to a Ewing sarcoma protocol. The patient showed no evidence of local recurrence or distant metastasis at 12 months after completion of adjuvant chemotherapy. We present herein an additional case of BCOR‑CCNB3 sarcoma of the proximal tibia, and review the relevant literature on BCOR‑CCNB3 sarcoma of bone.

Background During cancer treatment, maintenance and improvement of quality of life (QOL) are important, as is prolongation of overall survival. As the disease progresses, patients may experience a decline in QOL due to physical or mental changes. However, few studies have evaluated QOL longitudinally in advanced soft tissue sarcoma (STS) patients according to the antitumor agent. The purpose of this study was to compare health-related QOL (HRQOL) in patients with advanced STS receiving the combination regimen of doxorubicin and ifosfamide (AI) or three novel antitumor agents (pazopanib, trabectedin, eribulin). Methods Twelve patients diagnosed with advanced STS who started chemotherapy between 2010 and 2017 at our hospital were enrolled. HRQOL was assessed using the European Organization on Research and Treatment of Cancer Quality-of-Life Core Questionnaire-30 (EORTC QLQ-C30) after three cycles of chemotherapy before assessing the effect of treatment. Global health status, a functional scale, and a symptomatic scale were compared for the AI regimen and the three novel antitumor agents. Results The mean global health status score of the patients treated with the AI regimen was significantly different from that of those treated with one of the three novel antitumor agents (37.9 and 56.8, respectively). No significant differences were found in the five functional scores. The nausea and vomiting score was significantly different between the AI regimen and eribulin. The constipation score was significantly higher with the AI regimen, and the diarrhea score was significantly higher with pazopanib. Conclusions Compared to the AI regimen, patients can continue treatment with one of the three innovative antitumor agents while maintaining QOL, even with progressive disease. In particular, of the three innovative antitumor agents compared to the AI regimen, patients treated with pazopanib were able to maintain social activities during treatment. In the second-line and subsequent treatments, we could not clearly show a different effect on maintenance of a better QOL among the three innovative drugs, so additional work in the future is needed. If we clarify which of the three drugs most affects QOL, recommendations can be made regarding treatment selection for the second-line and subsequent treatments.

Extraskeletal osteosarcoma (ESOS) is a rare soft tissue sarcoma accounting for 1-2% of all soft tissue sarcomas. ESOS originating in the superficial (cutaneous-subcutaneous) tissue is extremely rare, and only 17 cases with subcutaneous ESOS have been reported in detail to date. The aim of the present study was to report an additional case of subcutaneous ESOS of the lower leg and review previous reports of subcutaneous ESOS, focusing on the clinical characteristics, including the MIB-1 labeling index, treatment methods and outcomes. A 79-year-old healthy man presented with a 3-year history of a painful, slowly growing mass in his right lower leg that measured ~5 cm in greatest dimension. Excisional biopsy was performed, and ESOS was diagnosed based on the histopathological findings. A wide resection was performed when local recurrence developed. Six months after the wide resection, lung metastasis was detected. Considering the patient's age, stereotactic radiotherapy was performed without chemotherapy. The patient showed no evidence of local recurrence or new distant metastases for 2 years after the second surgery. We herein present this case of subcutaneous ESOS and review the previous 17 reported cases of subcutaneous ESOS. The 5-year survival rate of patients with subcutaneous ESOS was 78.6%, which was better compared with that of ESOS cases arising in deep soft tissue. Therefore, patients with subcutaneous ESOS may have a better prognosis compared with those with deep-seated ESOS, although the mean MIB-1 labeling index of subcutaneous ESOS was 24%.

Primary cauda equina lymphoma (CEL) is a rare malignant tumor among various neoplasms that affects the cauda equina nerve roots. The present case report described the case of a 65‑year‑old man who presented with cauda equina syndrome with progressive motor palsy in the legs and gait disturbance over the last 5 months. Magnetic resonance (MR) images showed enlargement of the cauda equina occupying the dural sac from the L1‑S1 level with isointensity to the spinal cord signal on both T1‑ and T2‑weighted imaging. Enhancement of the cauda equina was seen on contrast MR images. On F‑18 2‑fluoro‑2‑deoxy‑glucose positron emission tomography examination, diffuse accumulation of 2‑fluoro‑2‑deoxy‑glucose was observed in the cauda equina with a maximum standardized uptake value of 4.9. Based on elevation of soluble interleukin 2 receptor in cerebrospinal fluid and a biopsy of the enlarging cauda equina, a diagnosis of CEL of the diffuse large B‑cell type was made. The present case report provided a detailed case discussion and a review of the available literature on this rare entity, focusing on clinical characteristics and imaging of primary CEL.

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. 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. 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. 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.

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. 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. 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. 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.

Osteosarcoma, the most common primary bone malignancy, has an extremely poor prognosis and a high rate of local recurrence and distal metastases. Because osteosarcomas of the head and neck region are rare, accounting for less than 10% of all osteosarcoma cases, limited information is available about their treatment and prognosis. Because of the high rate of distal metastases associated with extragnathic osteosarcoma, surgery combined with chemotherapy is currently considered essential in its treatment. However, the role of chemotherapy has not been well elucidated in the treatment of head and neck osteosarcoma because of the rarity of this condition. In this report, we present the case of a 58-year-old Japanese woman with osteosarcoma of the mandible that was treated with radical surgery combined with neoadjuvant and adjuvant chemotherapy. Because the tumor showed rapid growth during neoadjuvant chemotherapy, neoadjuvant chemotherapy was suspended and surgical resection was performed, followed by adjuvant chemotherapy. No evidence of local recurrence and distal metastasis was found 14 months after initial treatment. Local control is considered a principal prognostic factor for head and neck osteosarcoma. Wide surgical excision should be considered a primary goal even during neoadjuvant chemotherapy, especially in cases that respond poorly to neoadjuvant chemotherapy.