Magnetic resonance image findings of primary intradural Ewing sarcoma of the cauda equina: case report and review of the literature

Background contextInvolvement of the cauda equina in Ewing sarcoma (ES) is extremely rare, and only few cases are reported in literature. However, ES of cauda equina shares some neuroradiological features with other neoplasms that can involve the intradural space. Therefore, differential diagnosis with other tumors of cauda equina should be considered by neuroradiologists and neurosurgeons to provide appropriate treatment.PurposeTo present a rare case of intradural extramedullary primary ES.Study designCase report.MethodsWe report a case of a 44-year-old woman presenting with the rapid onset of cauda equina syndrome. Radiological analysis showed multiple intradural masses, extending from L1 to S3 level. After radical surgery, lesions were histologically defined as ES. We present a literature review, analyzing magnetic resonance image (MRI) features of primary intradural ES of the cauda equina.ResultsFour cases of primitive ES arising from the cauda equina have been reported in the literature.ConclusionsBecause of the low number of reported cases, it is not possible to describe pathognomonic MRI findings for intradural ES of the cauda equina. However, few tumors show similar MRI features. Therefore, despite its rarity, intradural ES should be taken into account in the differential diagnosis of spinal tumors involving cauda equina.     

Presentation of cauda equina syndrome due to an intradural extramedullary abscess: a case report

Background contextCauda equina syndrome is caused by compression or injury to the nerve roots distal to the level of the spinal cord. This syndrome presents as low back pain, motor and sensory deficits in the lower extremities, and bladder as well as bowel dysfunction. Although various etiologies of cauda equina syndrome have been reported, a less common cause is infection.PurposeTo report a case of cauda equina syndrome caused by infection of an intradural extramedullary abscess with Staphylococcus aureus.Study design/settingCase report and review of the literature.MethodsThe literature regarding the infectious causes of cauda equina syndrome was reviewed and a case of cauda equina syndrome caused by infection of an intradural extramedullary abscess with Staphylococcus aureus was reported.ResultsA 37-year-old woman, with history of intravenous drug abuse, hepatitis C, and hepatitis B, presented with low back pain lasting 2 months, lower extremity pain, left greater than right with increasing weakness and difficulty ambulating, and urinary and fecal incontinence. Her presentation was consistent with cauda equina syndrome. The patient underwent a T12–L2 laminectomy, and intradural exploration revealed an abscess. Methicillin-resistant Staphylococcus aureus was found on wound culture.ConclusionsCauda equina syndrome, presenting as a result of spinal infection, such as the case reported here, is extremely rare but clinically important. Surgical intervention is generally the recommended therapeutic modality.     

Acute onset of paraganglioma of filum terminale: A case report and surgical treatment

IntroductionParagangliomas of filum terminale are rare benign tumors, arising from the adrenal medulla or extra-adrenal paraganglia. These lesions usually present with chronic back pain and radiculopathy and only two cases of acute neurological deficit have been reported in literature.Presentation of caseA case with an acute paraplegia and cauda equina syndrome due to an hemorrhagic paraganglioma of the filum terminale is described. Magnetic resonance imaging showed an intradural tumor extending from L1 to L2 compressing the cauda equina, with an intralesional and intradural bleed. An emergent laminectomy with total removal of the tumor was performed allowing a post-operative partial sensory recovery. Histopathological examination diagnosed paraganglioma.DiscussionParagangliomas are solid, slow growing tumors arising from specialized neural crest cells, mostly occurring in the head and neck and rarely in cauda equina or filum terminale. MRI is gold standard radiological for diagnosis and follow-up of these lesions. They have no pathognomonic radiological and clinical features and are frequently misdiagnosed as other spinal lesions. No significant correlation was observed between the duration of symptoms and tumor dimension. Acute presentation is unusual and emergent surgical treatment is fondamental. The outcome is very good after complete excision and radiotherapical treatment is recommended after an incomplete resection. Conclusion: Early radiological assessment and timely surgery are mandatory to avoid progressive neurological deficits in case of acute clinical manifestation of paraganglioma of filum terminale.     

Primary extraosseous intradural spinal Ewing’s sarcoma: report of two cases

Two cases of primary extraosseous intradural spinal Ewing’s sarcoma are reported with a review of the current literature. This rare neoplasm shares features with cerebral primitive neuroectodermal tumors, complicating a correct diagnosis. Gross total resection seems to be the main treatment, although adjuvant therapies could improve the prognosis. In case 1, a 56-year-old man presented with cauda equina syndrome. MRI showed an intradural tumor from L4 to S2. An emergency laminectomy was performed with gross total resection of a hemorrhagic tumor, followed by adjuvant treatment. In the second case, a 25-year-old female developed leg and lumbar pain. MRI study identified a homogeneously enhancing intradural mass at the L2-L3 level. A laminoplasty was performed, followed by tumor resection; no adjuvant treatment was administered afterwards. Immunohistochemical workup confirmed the diagnosis of Ewing’s sarcoma in both cases.     

Primary liposarcoma of the lumbar spine

BackgroundWe describe a rare case of the cauda equina syndrome caused by a primary pleomorphic liposarcoma of the lumbar spine.Case reportA 35-year-old man presented a 2-month history of back pain and slowly progressive weakness of lower limbs without sphincter dysfunction. It had a laminectomy for a supposed disc herniation three years previously. A diagnosis of recurrence hernia or fibrosis was discussed.ResultsMagnetic Resonance Imaging (MRI) and CT scan of the lumbar vertebral column shows diffuse involvement of L4/L5 vertebral bodies, and an intradural lesion surrounding the nerve roots of cauda equina. The tumor occupied almost the whole width of the spinal canal. The diagnosis of plasmocytoma or metastasis was advocated and the patient was managed by an extended posterior approach in emergency. Histopathological findings revealed a pleomorphic liposarcoma. The patient received a course of radiotherapy and unfortunately he died at 3 months follow-up.ConclusionIn spite of his unusual location in the spine, pleomorphic liposarcoma should be considered in the differential diagnosis of primary spinal tumors.     

Primary cauda equina epidermoid cyst. A case report

A primary epidermoid cyst of the cauda equina was diagnosed in an eleven-year-old boy. The patient had a previous two months history of lumbar pain with unilateral L 4 radicular pain. The diagnosis of an epidermoid cyst of the cauda equina, suspected by MRI, was confirmed by histology following surgery. Wide tumor resection, as complete as possible, was performed with minimum trauma. Presence of an epidermoid cyst in the filum terminale is a rare finding, few cases have been reported in the literature. However, surgical treatment and favorable post-operative course are similar to that observed in intraspinal localizations. MRI is helpful for the preoperative and differential diagnosis and for post-operative follow-up.     

Esthesioneuroblastoma metastatic to the thoracic intradural and extradural space

Background contextEthesioneuroblastoma (ENB) is a rare tumor of the olfactory epithelium that has been shown to metastasize mostly to the cervical lymphatics, with only infrequent spread to other locations. We report a rare case of ENB characterized by recurrence and distant metastasis to the T7–T8 intradural and extradural space.PurposeTo report a rare case of recurrent ENB metastatic to the thoracic intradural and extradural space.Study design/settingCase report with a review of the literature.MethodsA 64-year-old man with recurrent ENB presented with chronic pain in the neck, shoulder, and back. His neurologic exam was normal. Computed tomography of the chest showed no pulmonary metastasis and a high-attenuation spinal canal mass at T8 was noted on magnetic resonance imaging. A laminectomy at T7–T8 was performed for resection of a large epidural mass. A tumor was seen penetrating through the dura, and a midline durotomy was performed for resection of a large intradural mass. Frozen section and permanent stains were consistent with metastatic ENB.ResultsThe postoperative period was uneventful, and included pain management and physical therapy, followed by chemotherapy and radiation. The patient remains free of spinal recurrence 2 years after surgery.ConclusionsMetastasis of ENB to the spinal column is rare, and of those instances, 80% are localized to the cauda equina. Recurrent ENB metastatic to the thoracic intradural and extradural space is extremely rare, and was successfully treated with surgical resection.     

Metastatic hemangiopericytoma to the cauda equina: a case report.

BACKGROUND CONTEXT: Hemangiopericytoma is an aggressive tumor associated with high recurrence and metastasis. Metastases are usually delayed, long after diagnosis of the primary lesion. Metastatic hemangiopericytoma to the spinal cord is especially rare. PURPOSE: To report a rare clinical presentation of a metastatic intradural, intramedullary hemangiopericytoma to the cauda equina from a cerebellar hemangiopericytoma. STUDY DESIGN: Case report with a review of the literature. METHODS: Clinical history, physical findings, and magnetic resonance imaging studies of a patient with a metastatic intradural, intramedullary hemangiopericytoma to the cauda equina are reported. RESULTS: A case report is presented of a female with an intradural, intramedullary lesion at the L4-S1 level, presenting initially with progressive pain and motor weakness affecting the left lower extremity. She had a history of a cerebellar hemangiopericytoma, which had been treated with total resection and radiotherapy 4 years earlier. This patient developed urinary urgency and frequency. Pathological analysis revealed a hemangiopericytoma, which had a similar character to a cerebellar lesion. After radiotherapy, the tumor was mostly diminished and her symptoms totally resolved. CONCLUSIONS: Hemangiopericytomas have a strong tendency to both local recurrence and metastasis. Common metastatic sites are the skeletal system, lung, liver, and abdominal cavity. To the authors' knowledge, there have been no reports of spinal intradural, intramedullary metastasis of hemangiopericytoma.     

Large cell neuroendocrine carcinoma of the lung metastatic to the cauda equina

Background ContextLarge cell neuroendocrine carcinoma of the lung is an aggressive tumor with unique histopathological features. It is not known to metastasize to the spine.PurposeTo report a metastatic case of this rare tumor to the cauda equina.Study DesignCase report.MethodsRetrospective case review and review of the literature.ResultsThe authors report a rare case of a large cell neuroendocrine lung metastasis to the lumbar spine, causing right foot drop. Magnetic resonance imaging revealed a heterogeneously enhancing intradural extramedullary mass at L2/L3 level compressing the surrounding nerve roots. During surgery, the identified nerve roots were encased by the tumor, and the dissection was tedious. Postoperatively, the patient reported significantly improved back pain and he had severe foot weakness. The functional outcome was poor because the patient lost entirely his foot function; however, his back pain improved significantly after surgery.ConclusionsThis is the first published study in which the authors described a metastasis of a rather uncommon lung cancer to the cauda equina. When a lesion of the cauda equina presents with a rapid progressive neurological deficit, leptomeningeal metastasis should be in the differential diagnosis.     

Paraganglioma of the Lumbar Spine: A case report and literature review

IntroductionLumbar paragangliomas are rare, vascular, neuroendocrine tumors. They are notoriously difficult to diagnose radiologically and can prove challenging to manage intraoperatively, if capable of catecholamine secretion.Case reportWe report the case of a 45-year-old man, who presented with a lumbar spinal paraganglioma. The patient described a 2-year history of worsening lower back pain and sciatica. Neurological examination was normal. MRI revealed a lesion at L3, with prominent vessels, compressing the cauda equina. Gross total resection (GTR) of the tumor was performed. The patient recovered well, with relief of pain and no neurological deficit.DiscussionA literature search of lumbar paraganglioma cases, from January 1970 to April 2018 was carried out. Results of this review highlighted the importance of inclusion of paraganglioma as a differential diagnosis in lumbar spinal tumor and also the requirement for preoperative investigations to determine any potential secretory activity.ConclusionsLumbar paraganglioma behavior is most commonly benign and rates of recurrence are low after GTR. However, long-term postoperative follow-up is crucial, due to findings of late metastatic recurrence.     

Clear cell meningioma of the cauda equina in an adult: case report and literature review

In the pediatric population, clear cell meningiomas are more frequently intracranial than intraspinal in location. Tumors recur in up to 40% of cases within 15 postoperative months and are often managed with repeated resection with or without radiation therapy. The management strategy for adults with clear cell meningiomas involving the lumbar spinal canal (cauda equina) is less clearly defined. A 41-year-old woman presented with mild, right greater than left, lower extremity paresis. An enhanced magnetic resonance (MR) scan revealed a homogeneously enhancing intradural lesion filling the spinal canal at the L3-L4 level. Preoperative noncontrast MR studies of the brain and cervical and thoracic spine were negative. An L2-L5 laminectomy was performed for gross total excision of the intradural lesion, which was adherent to one nerve root of the cauda equina. Frozen-section diagnosis confirmed clear cell tumor. Differential diagnoses included meningioma versus renal cell carcinoma. Negative postoperative chest, abdominal, and pelvic computed tomography studies ruled out tumor of renal cell origin. Enhanced MR studies of the neuraxis proved negative. Consultations with multiple oncologists and radiation therapists recommended neither radiation nor chemotherapy following this initial surgery. She remains disease-free 1 year postoperatively. The high recurrence rate for clear cell meningiomas in children requires repeated tumor resection with or without secondary radiation therapy. Following gross total resection of lumbar tumors in adults, reserving radiation therapy for secondary recurrences provides optimal management.     

Intradural disc mimicking: a spinal tumor lesion

STUDY DESIGN: A case report of intradural disc hernia mimicking an intradural extramedullary spinal tumor lesion in radiological evaluation. OBJECTIVE: To describe a lumbar intradural disc herniation with atypical radiological appearance and point out the role of contrast magnetic resonance imaging (MRI) of the lumbar spine. SETTING: Turkey. CASE REPORT: A 58-year-old man with suspected lumbar intradural mass and neurological involvement received L5 total laminectomy. L5 total laminectomy was performed, and on inspection dura was swollen and immobile. A longitudinal incision was made in the dura and an intradural-free disc fragment was removed. The patient's postoperative period was uneventful and he had full recovery in 3 months. CONCLUSIONS: Lumbar intradural disc rupture must be considered in the differential diagnosis of mass lesions causing nerve root or cauda equina syndromes. Contrast-enhanced MRI scans are useful to differentiate a herniated disc from a disc space infection or tumor. This case demonstrates the role and the importance of contrast MRI in the diagnosis of intradural disc herniation.     

Cavernous Angiomas of the Cauda Equina: Clinical Characteristics and Surgical Outcomes

Cavernous angioma (CA) is a rare hamartomatous vascular lesion, consisting of abnormal, dilated, and packed sinusoidal vascular channels without interposed nervous tissue. CAs of the cauda equina are exceedingly rare and have been previously reported in the literature as case reports. The aim of this study was to discuss the clinical presentation and the outcomes of microsurgery for these rare lesions. We retrospectively reviewed the records of 10 patients who underwent microsurgery for CAs of the cauda equina. All patients had performed pre- and postoperative magnetic resonance imaging (MRI). CAs of the cauda equina generally exhibited mixed intensity on T₁- and T₂-weighted images. Contrast-enhanced T₁-weighted images showed heterogeneous enhancement. The hemosiderin ring which surrounded the cauda equina CA was rare. Gross total resection was achieved in all cases. All patients were followed up, with a mean duration of 41.1 months. Long-term neurological function was improved in nine patients and remained stable in one patient. No recurrence was observed on MRI. CAs should be considered in the differential diagnosis of cauda equina tumors. Because of the excessive vascularity of CAs, en bloc resection is recommended. For symptomatic patients, early surgery should be performed before neurological deficits deteriorate.     

伴有马尾神经松弛影像学改变的腰椎椎管狭窄症患者手术疗效

目的探讨伴马尾神经松弛影像学改变的腰椎椎管狭窄症患者的临床特征及手术效果。方法收集2016年9月—2017年9月接受手术治疗的16例影像学上存在马尾神经松弛改变的腰椎椎管狭窄症患者的临床及影像学资料。16例患者腰椎MRI均可见狭窄节段上方马尾神经迂曲成团,均行常规腰椎后路椎板减压椎间植骨融合内固定术治疗,其中3例患者因术中硬膜撕裂行硬膜内探查。记录所有患者术前与术后3个月的日本骨科学会(JOA)评分评估手术疗效。结果所有患者术后腰痛及下肢放射痛等症状均缓解,无并发症发生。3例患者硬膜内探查可见马尾神经迂曲成团,无粘连及占位。16例患者术后3个月JOA评分为9～14(12.56±0.75)分,较术前4～9(7.44±0.73)分明显改善,差异有统计学意义(P 0.05)。其中6例患者术后3个月复查腰椎MRI,显示马尾神经迂曲成团现象消失。结论马尾神经松弛是腰椎椎管狭窄症发展进程的一部分,椎管内马尾神经迂曲成团是导致其影像学改变的原因。治疗腰椎原发病可以获得较好的治疗效果,不需要松解马尾神经,也不必担心马尾神经松弛现象。     

脊髓内胆脂瘤的诊断和手术治疗

目的提高脊髓内胆脂瘤的诊断水平及选择治疗方法。方法总结１９７８～１９９５年收治的经手术和病理证实的１０例脊髓内胆脂瘤的经验。结果患者临床表现无特异性。本组ＣＴ检查８例，显示肿瘤呈现均匀低密度影像，边界清楚，强化扫描多无增强。ＭＲ检查４例，肿瘤呈现信号强度变化不定的特点，边界清楚。１０例患者均行手术治疗，肿瘤全切除１例，次全切除９例。８例术后得以随访，无复发。结论肿瘤全切是治疗本病的最好选择。     

Ganglioneuroblastoma of the cauda equina

A 39-year-old lady presented with low back pain and neurogenic claudication. Magnetic resonance imagining revealed an intradural neoplasm in the cauda equina region. The patient underwent lumbar laminectomy and total excision of the neoplasm. Biopsy showed it to be a ganglioneuroblastoma, which is rare in the spinal canal and so far does not appear to have been reported in the region of the cauda equina. Its management is discussed.     

Thoracolumbar intradural extramedullary bronchiogenic cyst

Summary Intradural extramedullary bronchiogenic cysts are rare findings. All five reported cases were located cervically or upper thoracically. To our knowledge, we describe the first case of an intraspinal bronchiogenic cyst in a thoracolumbar location.We present the case of a 41-year-old patient with a known spina bifida occulta who suffered from a continuous, sharp, and therapy-refractory pain in the left leg. Magnetic resonance imaging of the thoracic and lumbar vertebra revealed an intradural extramedullar mass at T12 to L1 level. After laminectomy T-12 through L-1/L-2 and longitudinal opening of the dura mater, the cystic mass was shown to be attached to the conus medullaris and the cauda equina, and therefore could be removed only partially. Histopathological examination revealed the diagnosis of bronchiogenic cyst. We therefore conclude that intradural extramedullary bronchiogenic cysts may appear also at thoracolumbar levels. Surgical resection can be achieved with good outcome.     

Spinal intradural extramedullary cavernous angioma: case report and review of the literature

STUDY DESIGN: Case report and review of the literature. OBJECTIVE: To report an unusual case of a spinal intradural extramedullary cavernous angioma (CA), located at the cauda equina, and to compare it with the previously published 22 surgically treated cases in the literature. SETTING: Ankara, Turkey. METHOD: A 67-year-old man presented with a 4-month history of back pain and sciatica and a 4-week history of progressive lower extremity paresthesia, difficulty walking, and loss of bladder and bowel sphincter control. Neurological examination revealed bilateral hypoesthesia below the T12 dermatome with spastic paraparesis. Magnetic resonance imaging (MRI) of the cauda equina revealed a heterogeneous enhancing intradural extramedullary mass obliterating the spinal canal and expanding the spinal cord. T12 laminectomy and total tumor removal were performed without additional neurological deficit. Pathological examination confirmed the diagnosis of a CA. RESULT: The patient's neurological status improved during postoperative recovery. He was ambulatory without assistance and regained full sphincter control on the eighth postoperative week. CONCLUSION: CAs of the spinal cord are extremely rare lesions. Typically, they present with low back pain and sciatica, neurological deficits, or as a subarachnoid hemorrhage. These lesions have characteristic features on MRI and should be considered in the differential diagnosis of intradural spinal lesions. Following a thorough literature review of reported cases, the authors propose that for patients presenting with severe preoperative neurological signs, immediate microsurgical tumor excision or decompression increases the chance of neurological improvement.     

Primary Spinal Intradural Extramedullary Hydatid Cyst in a Child

Abstract

Background/Objective: Spinal hydatid cyst is a serious form of hydatid disease affecting less than 1% of the total cases of hydatid disease. We present a case of pathologically confirmed primary intradural spinal cyst hydatid in an otherwise healthy patient who showed no other evidence of systemic hydatid cyst disease.

Case Report: An 8-year-old boy presented with back pain, left leg pain, and difficulty in walking. The patient had no other signs of systemic hydatid cyst disease. An intradural extramedullary cystic lesion was identified with magnetic resonance imaging and was shown to be a hydatid cyst by histopathologic examination after the surgical removal.

Conclusion: Although extremely rare, primary intradural extramedullary hydatid cyst pathology might be the cause of leg pain and gait disturbance in children living in endemic areas.     

Lumbar intradural hydatid cyst. Case report

Hydatid disease is a serious public health problem in developing countries. Cerebrospinal localizations account for 1% of all cases of hydatid disease. The spino-radicular form is exceptional. We report a case of a 25-year-old women with cauda equina. Thoraco-lumbar MRI has showed an intradural process extending from L3 to L5, a low-intensity signal on T1 weighted images and a high-intensity signal on T2 weighted images, which were not enhanced after gadolinium injection. There was no vertebral involvement. After L3 L4 and L5 laminotomy and opening of the dura-matter, multiple vesicles were found scattered among the roots, with no adhesion to meninges or nervous structures. Total extirpation was easy. The outcome was marked by recovery of the motor deficit and sphincter disorders. This localization is serious but its prognosis is excellent if the diagnosis is made early enough to prevent cyst rupture and to allow total resection.