Malignant Granular Cell Tumor of the Skull Base

Objective and Importance: Malignant granular cell tumors (MGCTs) are extremely rare, high-grade sarcomas of Schwann cell origin. They often metastasize and are associated with short survival. We describe a patient with a large MGCT arising from the suboccipital nerve that eroded the posterior skull base, invaded the perifocal neck muscles, demonstrated perineural extension, and metastasized to regional lymph nodes. Clinical Presentation: A 60-year-old woman with several years' history of neck pain noticed a right-sided suboccipital swelling 4 months prior to seeking medical attention. Magnetic resonance imaging (MRI) showed a 5-cm bone-eroding suboccipital tumor and a second tumor, anterocaudal to this, 4 cm in diameter. Intervention: The patient underwent surgery. A 4-cm multinodular tumor was removed, freeing it from the internal jugular vein. A 5-cm suboccipital tumor infiltrated the trapezius, semispinalis capitis, and longissimus capitis muscles. The major and minor rectus capitis muscles were completely engulfed by tumor and their attachments to the occipital bone completely eroded. The oblique capitis muscle was infiltrated at its attachment to the C1 transverse process. These muscles were resected with a free margin to remove all tumor tissue. We then removed tumor encasing the right vertebral artery, the medial mastoid process up to the transverse sinus and anteriorly to the stylomastoid foramen, and lastly, the posterior third of the occipital condyle, achieving a gross total removal and no visible residual on postoperative contrast-enhanced MRI. Conclusion: This case represents the first report of resected primary MGCT involving the posterior fossa and arising from the suboccipital nerve.     

Schwannoma of the cervical spinal cord with cervical angina: a case report

A case of schwannoma of the cervical spinal cord presenting with cervical angina is reported. A 49-year-old man was admitted to our hospital with severe chest pain, cold sweats, and unconsciousness. Extensive cardiac examination showed no abnormal findings. Neurological deficits were muscular weakness and atrophy of the left arm, bilateral hypersthesia of the arms, and hyporeflexia of the left biceps. MRI revealed a tumor in the left side of the spinal canal between C4 and C5. The diagnosis was neurinoma of the left nerve root in C5. The tumor was completely removed surgically by laminectomy. Surgery confirmed that the tumor had originated from the left posterior root of C5 and that, histologically, it was schwannoma. The severe chest pain immediately disappeared after removal of the tumor with only dull post-operative chest pain remaining. We hypothesized that the severe chest pain was protopathic pain caused by compression of the anterior C5 root by the tumor and/or disturbance of the inhibitory pain mechanisms of the sympathetic nerve located in the posterior horn of the spinal cord. It must be kept in mind that cervical angina caused by spinal schwannoma is one of the differential diagnoses of chest pain.     

Brown-Séquard-plus syndrome due to stab injury: a case report

STUDY DESIGN: Case report. OBJECTIVE: To describe the mechanism of injury in this case and its clinical features. Magnetic resonance (MR) images of hemorrhage in spinal cord injury due to stab wound are discussed. METHODS: We describe the case of a 21-year-old woman who was stabbed in the right side of her neck and developed left-sided Brown-Séquard syndrome plus loss of bilateral proprioceptive sensation. Neither plain radiographs nor computed tomography of the cervical spine demonstrated any foreign bodies or fractures of the cervical spine. T2-weighted cervical MR images confirm spinal cord hemiresection at C5-C6. RESULTS: MR imaging was performed serially at 4 days, 4 weeks, and 8 weeks after trauma. The signal pattern of the spinal cord at the site of injury varied iso, iso, and low on T1-weighted consecutive images. Meanwhile, high signal intensity on T2-weighted images was consistent during the 8 weeks after incidence of trauma. A T2-weighted sagittal image showed a tiny spot of low intensity in the high signal band at the site of penetration, demonstrating hemosiderin formation in the spinal cord. The patient was treated conservatively and, recovered from Frankel grade C to grade D. CONCLUSION: Spinal cord injuries (SCI) following stab wounds are rare. MR imaging is definitely useful for recording and monitoring the pathology of SCI.     

An unusual and spectacular case of spindle cell lipoma of the posterior neck invading the spinal cervical canal and posterior cranial fossa

The authors describe the first case of spindle cell lipoma of the posterior neck invading the upper cervical spinal canal and the posterior cranial fossa. Spindle cell lipoma is an extremely rare variant of benign lipoma. It usually occurs as a solitary subcutaneous well-circumscribed lesion in the posterior neck or shoulders of adult men. Local aggressiveness is unusual. This 61-year-old man presented with an increased left cerebellar syndrome and headaches. He also had a posterior neck tumefaction, which had been known about for a long time. Computed tomography and MR imaging studies revealed a voluminous mass extending to the upper cervical canal and posterior cranial fossa and eroding the neighboring bones. The lesion was well delimited, and contrast enhancement was intense and heterogeneous. The tumor, which had initially developed under the muscles of the posterior neck, was totally resected. Histological assessment revealed numerous fat cells with spindle cells secreting collagen. The large size of the tumor and the submuscular location, bone erosion, and compression of the CNS were unusual in this rare subtype of benign adipose tumor. Its presentation could simulate a sarcoma.     

Discogenic pain in acute nonspecific low-back pain

Acute nonspecific low-back pain is characterized by the sudden onset and severe unendurable low-back pain without radicular pain or neurological deficit in the lower extremities. The study was carried out using 55 patients who visited our hospital for acute nonspecific low-back pain, who exhibited degeneration on T2-weighted MR images, and underwent intradiscal injection of local anesthetics,steroid and contrast medium. Intervertebral disc sites with an obvious enhanced region in the posterior annulus of the disc on enhanced T1-weghted MR images was selected for intradiscal injection. When no enhaced region was detected, the most severely degenerated disc on T2-weighted MR images was selected. Acute nonspecific low-back pain with an improvement rate of 70% or higher 5min after injection was judged to be discogenic. The clinical characteristics and pathogenesis of discogenic acute nonspecific low-back pain were investigated. Forty of the 55 patients (73%) had discogenic acute nonspecific low-back pain. As for the characteristics of patients, the mean age was 37 years, and onset occurred upon casual daily movements in 18 patients (45%). Nineteen patients (48%) had bilateral low-back pain, and 29 patients (73%) had no tenderness in the paravertebral muscles. On plain X-ray radiograms, degeneration of the disc was normal or mild in 36 patients(91%). On the discograms, a radial tear extending to the posterior annulus was noted in all patients, but epidural leakage was seen only in six patients (15%). The degree of disc degeneration on T2-weighted MR images (Gibsons classification) was grade 3 in 30 patients (75%). Gadolinium-DTPA enhanced T1-weighted MR images showed an obvious enhanced region in the posterior annulus of the intervertebral disc in 19 patients (48%). As for the clinical characteristics of discogenic acute nonspecific low-back pain, the relatively young adult patients had no tenderness in the paravertebral muscles, and showed moderately degererated intervertebral discs. The pathogenesis of discogenic acute nonspecific low-back pain is mostly considered to be a re-rupture in an asymptomatic ruputured region in the posterior annulus, repaired by granulation tissue, in a moderately degenerated intervertebral disc with a radial tear.     

An expanding cervical synovial cyst causing acute cervical radiculopathy

Synovial cysts of the cervical spine occur infrequently in the spinal canal and are most often associated with degenerative facet joints. Despite the prevalence of degenerative spinal disease, symptomatic synovial cysts are extremely uncommon. We report a rare case that showed an exacerbation of a cervical radiculopathy due to an acute expansion of the synovial cyst. Magnetic resonance (MR) images originally revealed a small cystic extradural lesion when the patient presented with neck pain and slightly numbness in the right hand. The patient's complaints subsequently subsided after administration of pain killers. However, 2 weeks after this, the patient experienced a spontaneous, sudden, severe radiating pain into the right arm without any accompanying cervical injury. MR images showed that the cyst had become markedly increased in size in the intervening 4 weeks and compressed the spinal cord laterally. Because the arm pain was so severe and neurologic examinations revealed the paralysis of the C8 nerve root, the synovial cyst was excised surgically and a good clinical outcome achieved. Thus, even if symptoms are mild and the size of the synovial cyst is small, acute expansion of the cyst might be rarely observed and careful management, including surgical consideration, is needed.     

A child with C3 anterior dislocation-fracture accompanying Horner’s syndrome

Objective and importance

Injury of the cervical vertebrae in children is very rare, and few cases of dislocation-fracture in the cervical vertebrae below C3 have been reported. We experienced a child case who had a very rare C3/4 flexion-distraction type spinal injury accompanying C5 level spinal cord injury and Horner’s syndrome caused by sympathetic trunk palsy.

Clinical presentation

The patient was a 5-year-old boy. The boy was thrown head first from the backseat of a car in a traffic accident, pinning the neck between the broken window and guardrail. The patient, with an 8 cm long injury to the left upper clavicle region, was transported to a medical emergency center, and referred to our hospital after initial treatment. At the first examination, torticollis with neck pain and limitation of left palpebral opening were observed. The strength of bilateral muscles in the upper limbs was grade 1/5 muscle weakness in the deltoid muscles, 3/5 in the biceps, and 4/5 in others. The injury was diagnosed as a C3 anterior dislocation-fracture by plain radiography and computed tomography.

Intervention

The dislocation was reduced by glisson traction, but the operation of posterior cervical fixation (C3/4 spinous process wiring) was needed because it was difficult to maintain the reduced position using a fixation device.

Conclusion

The postoperative course was good, and bony union was obtained. The strength of muscles in the upper limbs improved to 3/5 in the deltoid muscles, 4/5 in the biceps, and 5/5 in others, and Horner’s syndrome also improved.     

保留颈半棘肌的颈椎后路双开门椎管扩大成形术的疗效研究

目的探讨保留颈半棘肌的颈椎后路双开门椎管扩大成形术的临床疗效.方法回顾性分析2001年2月至2009年7月行后路双开门颈椎椎管扩大成形术的189例患者的临床资料,根据术中是否切断C2棘突半棘肌将患者分为颈半棘肌切断组(C3~C7椎板成形术,102例)和颈半棘肌未切断组(C3椎板切除、C4~C7椎板成形术,87例).采用日本骨科协会(JOA)评分法进行神经功能评价;AS程度采用视觉模拟评分法(VAS)进行评价.结果两组手术时间、颈椎曲度、颈椎活动度、颈托固定时间比较,差异有统计学意义(P<0.05).两组术后6个月JOA评分均较术前有明显升高(P<0.05),但两组间比较,差异无统计学意义(P>0.05).术后轴性症状(AS)总发生率为30.2%(57/189),其中颈半棘肌切断组37例(19.6%)、颈半棘肌未切断组20例(10.6%),两组比较,差异有统计学意义(P<0.05).两组末次随访VAS均较术前明显下降(P<0.05);两组间比较,差异有统计学意义(P<0.05).结论颈椎后路双开门椎管扩大成形术可有效改善神经功能,但保留颈半棘肌的手术方式缩短了手术时间,保持了颈椎曲度和活动度,有助于减少术后AS的发生.     

Acute neck pain caused by calcium pyrophosphate dehydrate deposition in the transverse ligament of the atlas: a case report

We report a case of pseudogout manifested by severe posterior neck pain. Pseudogout of the neck, also known as the crowned dens syndrome, causes acute neck pain characterized by calcium pyrophosphate dehydrate deposition around the odontoid process. Crowned dens syndrome is typified clinically by severe cervical pain and stiffness, often in conjunction with raised inflammatory markers. A 71-year-old man presented with severe neck pain. On admission, elevation of serum CRP level was confirmed. Magnetic resonance images showed no responsible abnormalities except for degenerating change of the spine. The patient was diagnosed as having pseudogout caused by calcium pyrophosphate dehydrate deposition based on cervical computed tomographic imaging, which showed linear calcification in the transverse ligament of the axis. After administration of non-steroidal anti-inflammatory drugs, the fever and neck pain disappeared and the CRP level returned to within the normal range. Pseudogout of the cervical spine should be considered as a differential diagnosis when we examine patients with acute neck pain. Cervical spinal computed tomographic scan is a more sensitive and useful examination method to diagnose this disease rather than magnetic resonance images.     

Solitary fibrous tumor in the cervical spine with destructive vertebral involvement: a case report and review of the literature

Introduction  Recently, solitary fibrous tumors occurring in spine-related lesions have been reported. However, the destruction of vertebral bodies by this type of tumor has not been reported. Materials and methods  A 71-year-old female presented with pain from a mass on the right side of her neck. Plain radiographs of the cervical spine showed collapse of the C5 vertebral body and dislocation of the C4 vertebral body. The MRI image showed a large mass surrounding C4 and C5, which had low signal intensity in the T1W image and high signal intensity in the T2W image. At first, resection of the tumor and spinal fusion was performed by anterior approach. Results  Histology revealed a solitary fibrous tumor with proliferating spindle cells. Immunohistochemistry showed positive stains for vimentin and CD34. One year postoperatively, a local recurrence manifested extensive destruction of the C4 and C5 vertebral bodies. Then, palliative surgery with posterior cervical instrumentation and radiation therapy were performed. Because the destruction proceeded and the rods were broken 2 years after, she underwent additional occipito-cervical instrumentation. Conclusions  This is the first report of a solitary fibrous tumor that involves the destruction of the spinal structure. An extensive destruction of the vertebral body by the solitary fibrous tumor needs to be aware in treating this tumor with spinal involvement.     

Intraoperative MR imaging in a case of a cervical spinal cord lesion

The aim of this article is to describe the feasibility of performing intraoperative MR imaging in patients with spinal cord lesions and the potential value of this technique. The authors report a case involving a 28-year-old man who presented with chronic cervical pain and pain along the ulnar side of the forearms during neck flexion. Findings on clinical examination were normal, but MR imaging revealed a multicystic cervical spinal cord lesion. Surgery was undertaken to open the cysts, evacuate old blood, and search for pathological tissue. Intraoperative MR imaging showed that the caudal cyst was not opened, and surgery was therefore continued. The caudal cyst was fenestrated and a suspected small cavernous malformation was removed. Electrophysiological monitoring was performed both before and after the intraoperative MR imaging. The use of intraoperative MR imaging changed the strategy of the procedure and helped the surgeon to safely enter all the cysts in the cervical cord.     

Atrophy of the nuchal muscle and change in cervical curvature after expansive open-door laminoplasty

We analyzed computed tomography (CT) images and plain X-ray films of 53 patients who had undergone expansive open-door laminoplasty, in a 3-year study. The relationship between the postoperative changes in the nuchal muscles and those in the cervical curvature was investigated. On postoperative CT images, the cross-sectional area of all nuchal muscles was reduced to approximately 80% of its preoperative size. This atrophic change was especially intense in the multifidus muscle and the semispinalis cervicis muscle. Postoperative cross-sectional area of the deep nuchal muscles was reduced approximately 30% from its preoperative size. No significant correlation was found between the all cross-sectional area of the nuchal muscles and the cervical curvature. However, a weak correlation was found between the deep nuchal muscles area and the curve index (correlation coefficient 0.29).     

Intramuscular myxoma of the cervical paraspinal muscle

Myxoma is a neoplasm of mesenchymal origin composed of undifferentiated stellate cells in a myxoid stroma. This tumor can develop in a variety of locations. Myxomas that arise from skeletal muscles are called intramuscular myxomas. They usually occur in large skeletal muscles. Only ten cases of these benign tumors involving the neck muscles were reported in literature. Of them, only three were located at the paraspinal muscles. A 64-year-old woman presented with occiptal and neck pain over 5 years noted an expansive painful lesion located at posterior cervical region with progressive volume increase in the last 12 months. Image exams revealed a large mass located in the left posterior region of the neck in contact with the C2, C3 and C4 laminae with no invasion of the vertebrea. Tumor total removal was performed through normal muscle margins and the vertebral periosteum was scraped. The tumor was encapsuleted, lobulated with a gray-white appearance. The histological examination yielded the diagnosis of intramuscular myxoma. Follow-up at 1 year showed complete resolution of preoperative symptoms and no evidence of local recurrence. In conclusion, although rare, intramuscular myxoma should be included in differential diagnosis of cervical paraspinal tumors. We reported the fourth case of intramuscular myxoma in the paraspinal musculature of the neck. Despite its benign characteristics, local recurrence was reported after subtotal resection. Tumor total removal should be the goal of surgery.     

重度青少年特发性颈椎后凸畸形的手术治疗

目的 探讨重度青少年特发性颈椎后凸畸形的临床特征和手术策略.方法 回顾性分析2003年7月至2007年1月收治的12例重度青少年特发性颈椎后凸畸形患者的临床资料,术前后凸Cobb角55°～73°(平均61°).先以在过伸侧位X线片上测量的椎体后缘切线夹角为依据,决定颈椎后部的椎板及小关节等的截骨角度及范围,行颈后路截骨及前路松解术.术后行颅骨牵引使松解后颈椎后凸达到最大可能的矫正,7～10 d后二期行颈前路矫形、植骨内固定术.分别于术后第3天、3及6个月、1及2年摄颈椎正侧位X线片、MRI,观察矫形效果,植骨融合情况及内固定位置并对比手术前后临床症状的变化.结果 患者畸形外观明显改善,颈部疼痛症状全部消失,神经功能明显恢复.术后MRI显示:颈椎生理曲度重建,后凸畸形区域脑脊液线清晰,脊髓未见压迫.术后第3天X线片显示:后凸Cobb角为-12.3°～11.2°(平均-2.0°).除1例AISA神经功能评分为D级外其他11例AISA神经功能评分均为E级.结论 重度青少年特发性颈椎后凸畸形有其特有的临床特征,对该类患者进行全面评估,分期手术及手术间期持续牵引是较为理想的外科治疗方法.     

Cervical laminoplasty

Cervical laminoplasty is a decompressive surgery that offers preservation of cervical mobility and avoids the complications inherent to arthrodesis. Initially described in Japanese literature for treatment of myelopathy secondary to ossification of the posterior longitudinal ligament, its indications and benefits have expanded as the technique and post-operative care have been improved.¹ Basic principles include preservation of the dorsal elements, maintenance of cervical segment motion, and decompression of the cervical spinal canal by releasing but not resecting the laminae. The primary goal thus becomes to maintain range of motion, provide safe and reliable neurologic recovery or preservation, and avoid the development of postlaminectomy kyphosis. Current trends include preserving the intrinsic spinal musculature and using specifically-designed hardware to ensure durability of the decompression. Ongoing research continues to address the more common complications such as C5 palsy, loss of lordosis, and the persistence of axial neck pain.     

A case of cervical kyphosis after a minor trauma

We have experienced a case of 10-years-old boy who had cervical kyphosis after a minor trauma. On April 4th in 2004, he felt “cervical click” when leaning on the sofa with his neck over-flexed to watch TV at his home. Just after this episode, he had neck pain. He came to our hospital for fixed flexed neck position and severe neck pain on April 8th. His neck was fixed in remarkably flexed position without rotation. In cervical X-ray photograph, the C2–C7 kyphotic angle was 44°. The atlanto-axial rotatory fixation (AARF) did not exist on cervical CT although there was slight asymmetry of atlanto-dental intervals in open mouth view. MRI of his cervical spine showed no abnormal findings about spinal cord and spinal canal although signal intensity of disc from C2 to C6 was low in T2WI. He took rest on the bed all day and received Glisson’s traction of 2 kg for 4 days after admission. After 3 days, his neck pain disappeared and cervical posture became normal. AARF is known as a cervical deformity, which is caused after trivial force. Our cervical kyphosis is much similar to AARF in the clinical pictures.     

Spontaneous resorption in recurrent intradural lumbar disc herniation. Case report

The authors report a case of spontaneous resorption of intradural disc material in a patient with recurrent intradural lumbar disc herniation and review magnetic resonance (MR) imaging and histopathological findings. Intradural lumbar disc herniation is rare, and most patients with this condition require surgical intervention due to severe leg pain and vesicorectal disturbance. In the present case, however, the recurrent intradural herniated mass had completely disappeared by 9 months after onset. Histological examination of intradural herniated disc tissue demonstrated infiltrated macrophages and angiogenesis within the herniated tissue, and Gd-enhanced MR images showed rim enhancement not only at the initial presentation, but also at recurrence. The authors conclude that when rim enhancement is present on Gd-enhanced MR images, there is a possibility of spontaneous resorption even though the herniated mass may be located within the intradural space. Moreover, when radiculopathy is controllable and cauda equina syndrome is absent, conservative therapy can be selected.     

Operative treatment of bilateral retrocollis

Summary A surgical method for bilateral retrocollis (BR) is presented: Selective resection of specific posterior cervical muscles for the treatment of bilateral retrocollis is described. The operative procedure includes partial resection of the upper part of the trapezius, part of the splenius muscles of both sides, part of the semispinalis capitis and semispinalis cervicis muscles of both sides. 15 cases were treated surgically during 1980–1989. Postoperative follow up (3–10 years) showed that the abnormal movements were abolished completely or for the greater part in 13 patients (87%) while preserving the normal posture and mobility.     

Anatomy of the spinal nerves and dermatomes

There are 31 pairs of spinal nerves: eight cervical, 12 thoracic, five lumbar, five sacral and one coccygeal. They form by fusion of a posterior sensory spinal root (bearing its posterior root ganglion) with an anterior motor root. These join at each intervertebral foramen. Typically, the nerve then divides into a posterior and an anterior primary ramus. The former supplies the vertebral muscles and dorsal skin. The anterior primary ramus in the thoracic region bears a white ramus communicans to the sympathetic ganglion. Each spinal nerve receives a grey ramus from the sympathetic chain. The nerves T2–T12 supply the skin and muscles of the trunk sequentially. The other nerves are arranged into the cervical, brachial, lumbar and sacral plexuses. The cervical plexus supplies the skin and anterior muscles of the neck and forms the phrenic nerve (C3–C5), while the brachial plexus supplies the skin and muscles of the upper limb, and the lumbar and sacral plexuses supply the skin of the lower limb and perineum and the muscles of the posterior abdominal wall, pelvis, perineum and lower limb. The segmental nerves are arranged to supply the skin (dermatomes), while the segmental supply to the limb muscles, the myotomes, is more complex.     

Osteoporotic fracture of the dens revealed by cervical manipulation

Osteoporotic vertebral fractures selectively affect the thoracolumbar junction, usually sparing the cervical spine. A 65-year-old woman with documented osteoporotic fractures and chronic alcohol abuse presented with neck pain and occipital neuralgia that started after she suddenly flexed then extended her neck. Following several sessions of cervical manipulation, her pain became more severe, and she was admitted. Imaging studies showed multiple fractures in the dens, C6 and C7. These apparently spontaneous fractures suggested a bone tumor, for which investigations were negative. Osteoporosis was the only identifiable cause. The spinal manipulations probably worsened the lesions which were performed by a chiropractor who is not a physician and did not obtain cervical spine radiographs before treating the patient. Osteoporosis contraindicates spinal manipulation at any level, including the cervical spine.