Atypical teratoid/rhabdoid tumor of the spine is a rare pediatric neoplasm with poor prognosis. We report a case of an atypical teratoid/rhabdoid tumor of the cervical spine in a 2-months-old infant. The patient presented with rapidly progressing tetraparesis and respiratory failure. Magnetic resonance imaging of the spinal cord revealed an intradural, extramedullary mass occupying the spinal canal on the right at the level of C1-C5. Tumor cells were immunohistochemically positive for epithelial membrane antigen, vimentin, cytokeratins, S-100 protein, and CD57/Leu-7 antigen. Despite chemotherapy, the infant presented with progressive neurological deterioration and died 6 months after initial diagnosis. We review the literature on spinal malignant rhabdoid tumor and discuss the pathology, treatment, and outcome of these rare neoplasms.
atypical teratoid tumor; rhabdoid tumor; hydrocephalous; chromosome 22q; spine; chemotherapy
A 61-year-old woman with a very rare case of totally ossified large thoracic spinal metaplastic meningioma, showing progressing myelopathy is presented. Computed tomographic images showed a large totally ossfied intradural round mass occupying the spinal canal on T9-10 level. Magnetic resonance imaging revealed a large T9-10 intradural extramedullary mass that was hypointense to spinal cord on T1- and T2-weighted sequences, partial enhancement was apparent after Gadolinium administration. The spinal cord was severely compressed and displaced toward the right at the level of T9-10. Surgical removal of the tumor was successfully accomplished via the posterior midline approach and the histological diagnosis verified an ossified metaplastic meningioma. The clinical neurological symptoms of patient were improved postoperatively. In this article we discuss the surgical and pathological aspects of rare case of spinal totally ossified metaplastic meningioma.
Ossified spinal tumor; Metaplastic meningioma; Grossly total resection
The authors present the case of an inflammatory myofibroblastic tumor that involves the cervical spinal cord meninges, presenting in a manner mimicking en plaque meningioma, which has never been previously reported. During the first surgical procedure, which did not involve exploration of the intradural space, inflammatory epidural tissue was found. We performed a second operation to remove the tumor that was finally intradural, dural-based and very tough. Imaging studies, surgical findings, and histopathological examinations were used to support the diagnosis. Intradural extramedullary inflammatory myofibroblastic tumor is a rare entity that has only been described nine times in the literature. Surgery remains the treatment of choice. Although histologically benign, spinal inflammatory myofibroblastic tumor can be aggressive and requires a large resection and long-term follow-up of the entire central nervous system with magnetic resonance imaging.
Inflammatory myofibroblastic tumor; Cervical spine; Intradural mass; Inflammatory pseudotumor; Plasma cell granuloma
To report a case of thoracic myelopathy secondary to intradural extramedullary bronchogenic cyst.
A 20-year-old man presented to the emergency department with increasing back pain and lower-extremity weakness. Magnetic resonance imaging demonstrated a cystic lesion at the T4 level with mass effect on the spinal cord.
The lesion was resected, and histopathologic evaluation showed a cyst lined by respiratory-type epithelium consistent with a bronchogenic cyst.
Intradural extramedullary bronchogenic cysts of the thoracic spine have been reported previously but are extremely rare. The treatment of choice is surgical resection.
Bronchogenic cyst; Myelopathy, compressive; Neoplasm, extramedullary intradural spinal cord; Paraparesis
Although primary spinal cord tumors (PSCTs) comprise a minority of primary central nervous system tumors, they often impose a great deal of morbidity on their victims. Few epidemiologic studies have addressed PSCTs in Iran.
Materials and Methods:
We analyzed the demographic/clinical features of all primary intraspinal tumors (with a specific focus on primary intradural spinal cord tumors) identified between 1992 and 2004 in three of the major related hospitals in Isfahan, Iran. We also tracked the malignant cases until 2012.
102 patients with primary intraspinal tumors were found; 82 tumors were Intradural (36 intramedullary and 46 extramedullary) and 20 extradural. The principal intradural histological subtypes were nerve sheath tumor (33%), ependymoma (22%), astrocytoma (16%), and meningioma (15%). 20 (19%) of the tumors were malignant. Local pain (43%) and motor disabilities (36%) were the most common first-presenting symptoms in the patients. Male-to-female ratio was significant only in ependymoma (male:female ratio = 3.6, P < 0.05). The mean age in meningioma (57 years, standard error [SE]: 15.7) was significantly higher than other types (one-way ANOVA, P < 0.05).
Our results reflect analogous frequency of distribution for PSCTs compared with most of the previous counterpart studies worldwide. The only notable exception was the comparatively fewer frequency of spinal cord meningioma in our study.
Epidemiology; spinal cord neoplasms; survival rate
We report two patients with ventral schwannoma in the thoracolumbar region manifesting as low back pain with or without paraparesis. In both patients magnetic resonance imaging (MRI) revealed a heterogeneously-enhanced intradural extramedullary mass in the thoracolumbar region. The tumors were successfully removed via the posterior approach. Their histology was consistent with schwannoma. Postoperative MRI showed no evidence of a tumor in either patient. Spinal schwannomas are common benign intradural extramedullary spinal neoplasms; most arise from the dorsal- and very few from the anterior roots. A literature review revealed that ventral schwannomas, including giant tumors as in the one from case 2 in our study, affect mainly the cervical region, and most are surgically addressed via the posterior approach. Careful handling of the spinal cord is mandatory for satisfactory surgical results.
Spinal schwannoma; Intradural lesion; Ventral schwannoma; Giant
Giant intradural extramedullary schwannomas of the thoracic spine are not common. Schwannomas, that is, tumors derived from neoplastic Schwann cells, and neurofibromas represent the most common intradural extramedullary spinal lesions. We report the case of a patient with a giant thoracic schwannoma presenting unusually with acute abdominal pain and with delayed neurological impairment.
A 26-year-old Hispanic man with no previous medical problems presented with acute periumbilical pain. After extensive work-up including an exploratory laparotomy for appendectomy, magnetic resonance imaging scans of the lumbar and thoracic spine revealed a giant intradural extramedullary thoracic schwannoma within the spinal canal posterior to the T9, T10, and T11 vertebral bodies. Magnetic resonance imaging signal prolongation was noted in the spinal cord both rostral and caudal to the schwannoma. The patient underwent an urgent laminectomy from T8 to L1. After sacrificing the T10 root, the tumor was removed en bloc. Postoperatively, the patient improved significantly gaining antigravity strength in both lower extremities.
The T10 dermatome is represented by the umbilical region. This referred pain may represent a mechanism by which a giant thoracic schwannoma may present as acute abdominal pain. Acute, intense abdominal pain with delayed neurologic deficit is a rare presentation of a thoracic schwannoma but should be considered as a possible cause of abdominal pain presenting without clear etiology. Although these lesions may be delayed in their diagnosis, early diagnosis and treatment may lead to an improved clinical outcome.
Primary central nervous system (CNS) melanoma is a rare condition that accounts for only 1% of all melanomas. A 34-year-old Korean female presented with a two-month history of progressive weakness in both legs. Spinal magnetic resonance image (MRI) revealed a spinal cord tumor at the level of T4, which was hyperintense on T1-weighted imaging and hypointense on T2-weighted imaging. The intradural and extramedullary tumor was completely resected and diagnosed as melanoma. There were no metastatic lesions. At three years after surgery, the patient is still alive, with no evidence of tumor recurrence. We present the details of this case along with a comprehensive review of spinal cord melanoma.
Melanoma; Spinal neoplasm; Surgical treatment; Prognosis
Clinical, imaging, and histological features of 8 canine spinal meningiomas, including a cervical cystic meningioma with imaging and intraoperative features of an arachnoid cyst, are described. All meningiomas were histologically classified and graded following the international World Health Organization human classification for tumors. Six meningiomas were located in the cervical spinal cord. Myelography showed intradural/ extramedullary lesions in 3/4 cases. Magnetic resonance imaging revealed hyperintense intradural/extramedullary masses on pre-contrast T1-weighted and T2-weighted images with homogeneous contrast enhancement in 7/8 cases. One dog had a cerebrospinal fluid-filled subarachnoid cavity dorsal to the cervical spinal cord. A spinal arachnoid cyst was diagnosed on imaging, but the histopathological study of the resected tissue revealed a grade I meningothelial cystic meningioma. There were no differences in outcome associated with tumor grade and surgical treatment (6/8). Cystic meningioma should be considered in the differential diagnosis of intraspinal cystic lesions, and biopsy is necessary for definitive diagnosis.
Although very rare, a few cases of intradural extramedullary (IDEM) spinal tumor migration have been reported since Tomimatsu first reported a mobile schwannoma of the cervical cord in 1974. Schwannoma is a neurogenic tumor which originates from nerve sheath that it is relatively well-marginated tumor with little attachment or adhesion to surrounding tissue. Mobility of tumor in spinal canal sometimes can result in negative exploration at the expected area. We found three interesting cases in which different tumor locations observed in repeated magnetic resonance image (MRI) findings. All tumors were intradural and extramedullary schwannoma. We reviewed the literature about moving tumor in the spine through PUBMED search.
Intradural extramedullary; Schwannoma; Mobility
To present the profiles of spinal cord tumors that can be removed through a unilateral hemilaminectomy and to demonstrate its usefulness for benign spinal cord tumors that significantly occupy the spinal canal.
From June 2004 to October 2010, 25 spinal cord tumors were approached with unilateral hemilaminectomy. We calculated the cross-sectional occupying ratio (CSOR) of tumor to spinal canal before and after the operations.
The locations of the tumors were intradural extramedullary in 20 cases, extradural in 2, and intramedullary in 3. The levels of the tumors were lumbar in 12, thoracic 9, and cervical 4. In all cases, the tumor was removed grossly and totally without damaging spinal cord or roots. The mean height and width of the lesions we195re 17.64 mm (3-47.5) and 12.62 mm (4-32.7), respectively. The mean CSOR was 69.40% (range, 27.8-96.9%). Postoperative neurological status showed improvement in all patients except one whose neurologic deficit remained unchanged. Postoperative spinal stability was preserved during the follow-up period (mean, 21.5 months) in all cases. Tumor recurrence did not develop during the follow-up period.
Unilateral hemilaminectomy combined with microsurgical technique provides sufficient space for the removal of diverse spinal cord tumors. The basic profiles of the spinal cord tumors which can be removed through the unilateral hemilaminectomy demonstrate its role for the surgery of the benign spinal cord tumors in various sizes.
Laminectomy; Microsurgery; Spinal cord neoplasms; Unilateral hemilaminectomy; Spinal ligaments
Neurocysticercosis (NCC) is one of the most common parasitic diseases affecting the central nervous system. Typically spinal NCC involvement has a concomitant cranial involvement. Spinal involvement by NCC, either intramedullary or extramedullary is very uncommon. The authors report a case of D12-L1 intradural extramedullary lesion in a 38-year-old female patient who presented with complaints of back pain and weakness of lower limbs. She underwent laminectomy and excision of the lesion. Histopathology revealed extramedullary cysticercal abscess. Post-operatively she was treated with albendazole. She had a successful recovery post-operatively and at 8 months follow up had no neurological deficits. This current case presents a very rare case of cysticercal abscess of dorsolumbar spine, without any evidence of cranial involvement. This report is to reemphasize the importance of including NCC as a differential diagnosis in intradural extramedullary lesion at the conus level in endemic areas like India.
Conuslesion; intradural extramedullary lesion; neurocysticercal abscess; spine; surgical excision
Intradural extramedullary spinal ependymomas are extremely rare. Herein, we describe a lesion-type spinal ependymoma that followed a malignant course, and discuss its clinical presentation, etiopathogenesis, and treatment. We present a patient who was diagnosed with an intradural extramedullary spinal tumor at T4-T6. The patient underwent gross total resection of the tumor without damage to the spinal cord. Histological examination, classified the lesion as a World Health Organization (WHO)-grade 2 ependymoma. One and a half years later, magnetic resonance imaging detected a recurring tumor at T4-T5. The tumor was removed and classified as a WHO-grade 3 anaplastic ependymoma. The patient was started on a course of regional spinal cord radiotherapy. The patient achieved tumoral control and clinical stabilization after the recurrence. We must consider the differential diagnosis of intradural extramedullary spinal tumors. The best treatment for this lesion is gross total resection and adjunctive radiotherapy is necessary in cases of malignant-change.
Ependymoma; Intradural extramedullary spinal cord neoplasms; Spinal cord tumor
Ependymomas are the most common intramedullary tumors in adults and are the most common in mid-adult years. The presence of synchronous ependymomas in different sites of the spine is not common and it is even more infrequent to find hemorrhage from a spinal ependymoma as a cause of neurological deterioration.
A 32-year-old man presented with back pain and progressive paraparesia. Magnetic resonance (MR) showed two intradural extramedullary lesions on spinal canal with signs of acute hemorrhage. The patient underwent emergent surgical decompression and resection. Pathology revealed myxopapillary ependymomas.
To our knowledge, we report the first case of a patient with acute neurological deterioration as a consequence of synchronous bleeding of two spinal ependymomas located at different levels in the spinal cord. This study illustrates the importance of recognizing the rare, but known occurrence of acute neurological deterioration after spontaneous hemorrhage in spinal ependymomas.
Deterioration; ependymoma; hemorrhage; spine; tumor bleeding
Spinal cord tumors are a relatively rare diagnosis, accounting for 1% to 10% of all pediatric central nervous system tumors. Understanding the etiology and clinical outcomes of these tumors is therefore very important. This study presents detailed information regarding clinical presentation, histological findings, outcomes, functional assessment, and management of a series of patients with this diagnosis.
Retrospective, descriptive study.
Thirty-five children with a final diagnosis of spinal cord tumor or mass, excluding dysraphism.
Neurodevelopmental tumors (dermoid tumors, epidermoid tumors, and teratomas) were the most common tumor type (31%), followed by astrocytomas (29%) and neuroblastomas (14%). Other types included schwannomas, meningiomas, giant cell tumors, extradural cystic masses, leukemic-related masses, and masses related to neurofibromatosis. Mean age at diagnosis was 6.6 years (SD = 5.5 y) and did not vary significantly by tumor type except for children with neuroblastoma (mean = 0.4 y, SD = 0.5 y). More boys (57%) were identified in the series than girls (43%); however, there was no association between tumor type and sex. Presenting complaints of pain were noted in 57% and were localized to the back, neck, or extremities. Extremity weakness was reported as an initial presenting symptom in 46%. Three children had scoliosis as a presenting issue and 14 had gait abnormalities. Regardless of treatment modality, mobility was retained in 83% of children with or without gait aids. Neurogenic bowel and/or bladder were present in 23% of the population.
This study corroborates other studies indicating that intramedullary tumors are the predominant form of pediatric spinal cord tumor. This population, however, presented with an unusually large number of developmental tumors, contrary to several published studies. The disparity may be the result of this institution acting as a regional referral center, thus increasing the number of this type of patient. The population is too small to make any other conjecture. The predominance of astrocytomas and neuroblastomas among those patients with poor outcomes and the prevalence of developmental tumors suggest the need for broader investigation. Although, in general, spinal cord tumors are relatively rare, this preliminary study supports the need to further evaluate associations between tumor type, presenting symptoms, treatment, and functional outcome in children with spinal cord tumors.
Myelopathy; neoplastic; Spinal cord tumor; extramedullary; developmental; extradural; Child; Outcome
Conventional laminectomy is the most popular technique for the complete removal of intradural spinal tumors. In particular, the central portion intramedullary tumor and large intradural extramedullary tumor often require a total laminectomy for the midline myelotomy, sufficient decompression, and adequate visualization. However, this technique has the disadvantages of a wide incision, extensive periosteal muscle dissection, and bony structural injury. Recently, split-spinous laminectomy and tubular retractor systems were found to decrease postoperative muscle injuries, skin incision size and discomfort. The combined technique of split-spinous laminectomy, using a quadrant tube retractor system allows for an excellent exposure of the tumor with minimal trauma of the surrounding tissue. We propose that this technique offers possible advantages over the traditional open tumor removal of the intradural spinal cord tumors, which covers one or two cervical levels and requires a total laminectomy.
Cervical cord tumor; Split-spinous laminectomy; Quadrant tube retractor
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.
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.
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.
Myelopathy, infectious; Hydatid disease, spinal, intradural, extramedullary; Echinococcus granulosus; Child
Spinal cord tumors in pediatric patients are rare, representing less than 1% of all central nervous system tumors. Two cases of pediatric primary intramedullary spinal cord glioblastoma at ages 14 and 8 years are reported. Both patients presented with rapid onset paraparesis and quadraparesis. Magnetic resonance imaging in both showed heterogeneously enhancing solitary mass lesions localized to lower cervical and upper thoracic spinal cord parenchyma. Histopathologic diagnosis was glioblastoma. Case #1 had a small cell component (primitive neuroectodermal tumor-like areas), higher Ki67, and p53 labeling indices, and a relatively stable karyotype with only minimal single copy losses involving regions: Chr8;pter-30480019, Chr16;pter-29754532, Chr16;56160245–88668979, and Chr19;32848902-qter on retrospective comparative genomic hybridization using formalin-fixed, paraffin-embedded samples. Case #2 had relatively bland histomorphology and negligible p53 immunoreactivity. Both underwent multimodal therapy including gross total resection, postoperative radiation and chemotherapy. However, there was no significant improvement in neurological deficits, and overall survival in both cases was 14 months.This report highlights the broad histological spectrum and poor overall survival despite multi modality therapy. The finding of relatively unique genotypic abnormalities resembling pediatric embryonal tumors in one case may highlight the value of genome-wide profiling in development of effective therapy. The differences in management with intracranial and low-grade spinal cord gliomas and current management issues are discussed.
glioblastoma; spinal cord; intramedullary; pediatric
Ectopic adrenocortical neoplasms arising in the nervous system are very rare. We encountered an intradural, extramedullary case of an adrenocortical neoplasm of indeterminate malignant potential affecting a spinal nerve root in the distal lumbar region of a 5-month-old girl. The lesion recurred 6 months after the initial gross total resection. The tumor in both resections had increased mitotic activity (5/10 high power fields) and MIB-1 labeling indices of 23% and 33% at initial resection and recurrence, respectively. Both tumors demonstrated gains of chromosomes 5 and 12 by interphase cytogenetics, whereas insulin growth factor 2 was identified in the recurrent tumor by immunohistochemistry. This report demonstrates that ectopic adrenocortical tumors in the nervous system may exhibit clinicopathologic and cytogenetic features suggestive of adrenocortical carcinoma.
nervous system; spinal cord; adrenocortical tumor; ectopic adrenal; immunohistochemistry; FISH
Spinal intradural extramedullary teratoma is a rare condition that develops more commonly in children than in adults and may be associated with spinal dysraphism. We report a rare case of adult-onset intradural extramedullary teratoma in the thoracolumbar spinal cord with no evidence of spinal dysraphism and without the history of prior spinal surgery. The patient was a 38-year-old male whose chief complaint was urinary incontinence. X-ray images of the thoracolumbar spine showed the widening of the interpedicular distance and posterior marginal erosion of the vertebral bodies and pedicles at the T11, T12, and L1 level. Magnetic resonance imagings of the lumbar spine showed a lobulated inhomogeneous high signal intradural mass (87×29×20 mm) between T11 and L1 and a high signal fluid collection at the T11 level. Laminectomy of the T11-L1 region was performed, and the mass was subtotally excised. The resected tumor was histopathologically diagnosed as a mature cystic teratoma. The patient's symptom of urinary incontinence was improved following the surgery.
Spinal cord neoplasm; Mature teratoma
Neurenteric cysts are benign tumors of the central nervous system (CNS) that represent 0.3% to 0.5% of all spinal cord tumors. They are usually extramedullary and found in the lower cervical and thoracic spine. Only 12.2% of neurenteric cysts are documented to be intramedullary.
The authors report a case of a 35-year-old female that presented with progressive weakness and loss of coordination in her legs. Magnetic resonance imaging (MRI) showed an intramedullary cystic lesion in the thoracolumbar region and a low-lying conus medullaris suggesting tethered cord. The patient was taken to the operating room for detethering of her spinal cord and resection of the lesion. Pathologic examination of the tissue confirmed the diagnosis of a neurenteric cyst.
A search of the literature since the advent of MRI showed 29 published cases of intramedullary neurentic cysts. Of the 24 published cases with a follow-up MRI, the average recurrence rate was 25% with a mean follow up of 51 months.
Intramedullary; neurenteric cyst; tethered spinal cord
Capillary hemangiomas are benign tumors and tumor like conditions commonly involving skin and mucus membrane of head and neck region. They are extremely rare in the spinal cord. We report a 35-year-old male presenting with gradual progressive paraparesis over a period of 4 months. Magnetic resonance imaging showed a hypo- to isointense intradural mass at the level of D12 vertebral body on T1-weighted images and homogenous enhancement on gadolinium contrast. Complete surgical resection revealed intradural extramedullary tumor, which on histopathologic examination showed characteristics of capillary hemangioma. At 1.5 years followup patient was asymptomatic.
Capillary hemangioma; intradural extramedullary tumor; lower thoracic spine
Total laminectomy for the removal of intradural-extramedullary spinal cord tumors has been used widely, but postoperative complications often develop, such as kyphosis, spinal instability, and persistent back pain. In this study, we evaluated seven patients with intradural-extramedullary spinal cord tumors with respect to the value of unilateral limited laminectomy. Our cases included six schwannomas, and one meningioma. The cervical region was involved in four cases, the thoracolumbar region in two cases, and the lumbar region in one case. The rationale for choosing a unilateral approach is to preserve musculoligamentous attachments and posterior bony elements as much as possible. The patients were mobilized on the third postoperative day and preoperative neurological symptoms were recovered within a few weeks. We did not observe any complication relating to unilateral limited laminectomy and at follow-up evaluation (at 3 and 12 months postoperatively), none of the patients showed spinal deformity or spinal instability. We think that the unilateral limited laminectomy is a safe and efficient technique for the treatment of intradural-extramedullary spinal cord tumors. We suggest that this technique is one of the best treatments for these tumors.
Laminectomy; Intradural extramedullary spinal cord neoplams
Meningiomas of the spine are the most common benign intradural extramedullary lesions and account for 25%–46% of all spinal cord tumors in adults. The goal of treatment is complete surgical resection while preserving spinal stability. Usually, these lesions occur in the thoracic region and in middle-aged women. Clinical presentation is usually nonspecific and the symptoms could precede the diagnosis by several months to years, especially in older people, in whom associated age-related diseases can mask the tumor for a long time. We report a series of 30 patients, aged 70 years or more, harboring intradural extramedullary spinal meningiomas. No subjects had major contraindications to surgery. A minimally invasive approach ( hemilaminectomy and preservation of the outer dural layer) was used to remove the tumor, while preserving spinal stability and improving the watertight dural closure. We retrospectively compared the outcomes in these patients with those in a control group subjected to laminectomy or laminotomy with different dural management. In our experience, the minimally invasive approach allows the same chances of complete tumor removal, while providing a better postoperative course than in a control group.
spinal meningioma; elderly; hemilaminectomy; outer dural layer; inner dural layer; minimally invasive surgery
Thoracic intramedullary schwannomas are rare spinal cord tumors. Most of these tumors have been reported as a single lesion in the spinal cord. The authors report the first case of intramedullary schwannoma accompanying by extramedullary beads-like daughter masses of the thoracic spine. A 68-year-old male presented with walking disturbance and decreased sensation below T10 dermatome. Imaging workup revealed an intramedullary mass at T6 and T7. T6 and T7 laminectomy and mass removal were performed. Intraoperatively, extramedullary beads-like daughter masses along the nerve roots adjacent to intramedullary mass were identified. Total removal of intramedullary lesion and partial resection of extramedullary masses were done. Histological analysis confirmed the diagnosis of schwannoma. The patient could ambulate independently at postoperative 1 month without any neurological sequelae. The authors experienced a surgical case of intramedullary schwannoma accompanying by extramedullary beads-like same pathologies in the thoracic spine.
Neurilemmoma; Intramedullary lesion; Spinal cord neoplasm