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
There have been 18 reported cases of primary spinal intradural, extramedullary ependymomas reported in the literature. One of the 18 cases had an extradural component and was benign. Our case is the second spinal intradural, extramedullary ependymoma with an extradural component and the first with its initial presentation as an anaplastic ependymoma.
A 50-year-old male presented with bilateral upper thigh weakness and thoracic numbness. His exam showed the pin-prick level at T5. Magnetic resonance imaging (MRI) of the thoracic spine showed an enhancing lesion at T5–6 with severe compression of the spinal cord with a dumbbell shape extension of the tumor through the right T5–6 neural foramen. The patient had a laminectomy at T4–T6 with the resection of the tumor. Postoperatively, the patient regained full strength in his lower extremities. Intraoperatively, the tumor was found to be intradural, extramedullary with an extradural component. The tumor was found to be an anaplastic ependymoma.
Even though spinal intradural extramedullary ependymomas are very rare, surgeons must be aware that on MRI, they can be mistaken for meningiomas or nerve sheath tumors especially if there is an extradural component. Our case report is the first intradural, extramedullary ependymoma that is anaplastic and has an extradural component. A review of the literature provides little information on the treatment and prognosis for these tumors especially if they are anaplastic. We propose that the treatment, as done in our case, should be complete resection of the tumor with spinal radiotherapy to the tumor level.
Anaplastic ependymoma; extradural; extramedullary; intradural; spinal cord
Intramedullary + extramedullary hemangioblastomas with largely extramedullary growth are rare and often incorrectly assigned as intradural-extramedullary tumors preoperatively. Preoperative evaluation of the precise tumor location is important for total resection of tumor and improving the surgical outcome. The aim of this study was to provide the first identification of the key differences among of preoperative MR images of hemangioblastomas in different locations and to correlate these with pathological findings. The subjects were 26 patients with surgery for spinal hemangioblastoma in our department, including 6 with an intramedullary tumor who were complicated with von Hippel Lindau disease. Intramedullary, intramedullary + extramedullary, and intradural-extramedullary tumors were present in 22, 3 and 1 cases, respectively. Sagittal MR images showed that intramedullary and intramedullary + extramedullary tumors gave intramedullary T2 high intensity areas (HIAs) spreading toward the craniocaudal sides of the tumor, whereas such findings were absent for the intradural-extramedullary tumor. All the tumors showed strong contrast on axial images, with focal enhancement of hemangioblastomas limited to the intramedullary region (focal type); smooth boundary lines between the spinal cord and the extramedullary tumor (smooth type); and a snowman sign for intramedullary + extramedullary tumors, which provided a key characteristic for differentiating intramedullary + extramedullary tumors from those limited to the extramedullary region. In pathological findings, the Ki67 activity was less than 1% for intramedullary and intradural-extramedullary tumors, but 18–25% in all cases with an intramedullary + extramedullary tumor. In conclusion, on preoperative MRI, a change in the intramedullary HIAs spreading the craniocaudal sides of the tumor on sagittal T2 weighted image (T2WI) and a snowman sign on contrast axial T1WI may be important for differentiation among spinal hemangioblastomas in different locations. Pathologically, we found that intramedullary + extramedullary hemangioblastoma has high cell proliferative activity, which may suggest that enlargement of this tumor occurs faster than that of intramedullary hemangioblastoma.
Spinal hemangioblastoma; Exophytic intramedullary hemangioblastoma; Differentiation of localization; MRI findings; Pathological findings
Pituitary tumors are rare, and pituitary carcinomas are rarer still. Prognosis is poor, with less than 50% of patients surviving past 1 year after diagnosis. In this case of spinal metastasis from an adrenocorticotropic hormone-secreting pituitary carcinoma, the intradural extramedullary metastases recurred in the same lumbar area 6 years apart.
Fourteen years prior to presentation in our clinic, a 48-year-old woman was diagnosed with pituitary adenoma which was treated with resection followed by radiation. Eight years later, an intradural extramedullary spinal drop metastasis at L2–L3 was again treated with resection and radiation. Three years later, magnetic resonance imaging (MRI) revealed a mass encasing the right carotid artery, which was treated for 1 year with chemotherapy using temozolomide (Temodar). Three years later, MRI showed intradural extramedullary metastases at the L3–L4 intervertebral disc space and behind the L3 vertebral body; treatment was again resection followed by radiation. Back pain and weakness resolved after surgery and her neurological examination returned to baseline. There was no evidence of recurrence 1 year after surgery.
In this unusual case, this pituitary carcinoma metastasized twice in 6 years to virtually the same intradural extramedullary lumbar region. Surgical resection of these masses aided in relieving neurological symptoms and prolonging life.
Pituitary carcinoma; Metastases; Neoplasm; Spinal cord neoplasms; Intradural-extramedullary; L2–L3; Pituitary adenoma; ACTH-secreting; Disc; Intervertebral; L3–L4
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
Solitary fibrous tumor is rare benign mesenchymal neoplasm. The spinal solitary fibrous tumor is extremely rare. The authors experienced a case of intramedullary solitary fibrous tumor of cervicothoracic spinal cord in a 48-year-old man with right lower extremity sensory disturbance. Spinal MRI showed intradural mass lesion in the level of C7-T1, the margin between the spinal cord and tumor was not clear on MRI. A Left unilateral laminectomy and mass removal was performed. Intra operative finding, the tumor boundary was unclear from spinal cord and it had intramedullary and extramedullary portion. After surgery, patient had good recovery and had uneventful prognosis. Follow up spinal MRI showed no recurrence of tumor.
Solitary fibrous tumor; Intramedulla; Spine; Benign
Capillary hemangiomas are benign tumors found in the skin and soft tissues in younger people. They occur in the central nervous system only rarely, and intradural occurrence is extremely rare. We report here a 60-year-old man presenting with thoracic girdle pain and progressive gait disturbance. Magnetic resonance images of the thoracic spine showed a 12 × 8 × 20 mm, well-defined intradural mass at the T2 level, compressing the spinal cord laterally. Relative to the spinal cord, the mass was hypo- to isointense on T1-weighted images and relatively hyperintense on T2-weighted images, with strong enhancement on contrast-enhanced T1-weighted images. The patient underwent T1-2 hemilaminectomy with resection of the intradural extramedullary tumor, which showed characteristics of a capillary hemangioma on histologic examination. The patient's symptoms improved following the surgery and no clinical or radiological evidence of recurrence was noted at the 2-year follow-up. We present this case with a review of the literature, highlighting features for differential diagnosis.
Intervertebral intradural lumbar disc herniation (ILDH) is a quite rare pathology, and isolated intradural lumbar disc herniation is even more rare. Magnetic resonance imaging (MRI) may not be able to reveal ILDHs, especially if MRI findings show an intact lumbar disc annulus and posterior longitudinal ligament. Here, we present an exceedingly rare case of an isolated IDLH that we initially misidentified as a spinal intradural tumor, in a 54-year-old man hospitalized with a 2-month history of back pain and right sciatica. Neurologic examination revealed a positive straight leg raise test on the right side, but he presented no other sensory, motor, or sphincter disturbances. A gadolinium-enhanced MRI revealed what we believed to be an intradural extramedullary tumor compressing the cauda equina leftward in the thecal sac, at the L2 vertebral level. The patient underwent total L2 laminectomy, and we extirpated the intradural mass under microscopic guidance. Histologic examination of the mass revealed a degenerated nucleus pulposus.
Intradural disc herniation; Spinal intradural tumor; Magnetic resonance imaging
Chordoma is a relatively rare tumor originating from the embryonic remnants of the notochord. This is an aggressive, slow growing and invasive tumor. It occurs mostly at the two ends of neuroaxis which is more frequent in the sacrococcygeal region. Chordoma in vertebral column is very rare. This tumor is extradural in origin and compresses neural tissues and makes the patient symptomatic. This tumor found extremely rare in the spinal region as an intradural tumor.
The present study reports a rare case of intradural chordoma tumor as well as its clinical manifestations and treatment options.
The patient was a 50-year-old female presented with 9 months history of progressively worsening neck pain, cervical spine chordoma resembling neurinoma and right arm numbness. Physical examination showed no weakness in her limbs, but she had upward plantar reflex and mild hyperreflexia. In a magnetic resonance imaging (MRI) scan of the cervical spine there was an ill-defined enhancing mass in the posterior aspect of C2-C3 body caused cord compression more severe in right side as well as foraminal scalloping. The patient underwent surgery and after midline posterior cervical incision and paravertebral muscle stripping a laminectomy was performed from C1 through C4 using a high speed drill. Needle biopsy revealed chordoma on frozen section and all of accessible parts of tumor were excised. The gross and microscopic histopathological appearance was consistent with chordoma.
Chordomas are malignant tumors that arise from remains of embryonic notochord. These ectopic rests of notochord termed “ecchordosis physaliphora “can be found in approximately 2% of autopsies. These are aggressive, slow growing, locally invasive and destructive tumors those occur in the midline of neuroaxis. They generally thought to account for 2% to 4% of all primary bone neoplasms and 1% to 4% malignant bone neoplasms. They are the most frequent primary malignant spinal tumors after plasmacytomas. The incidence has been estimated to be 0.51 cases per million. The most common location is sacrococcygeal region followed by the clivus. These two locations account for approximately 90% of chordomas. Of the tumors that do not arise in the sacrum or clivus, half occur in the cervical region, with the remainder found in the lumbar or thoracic region, in descending order of frequency. Cervical spine chordomas account for 6% of all cases. Distal metastasis most often occurs in young patients, those with sacrococcygeal or vertebral tumors, and those with atypical histological features. These tumors usually spread to contiguous anatomical structures, but they may be found in distant sites (skin, musculoskeletal system, brain, and other internal organs). Seeding of the tumor has also been reported, and the likely mechanism seems to be tumor cell of contamination during the surgical procedures. The usual radiological findings in chordomas of spine are destructive or lytic lesions with occasional sclerotic changes. They tend to lie anterolateral, rather than dorsal towards the cord, and reportedly known to invade the dura. The midline location, destructive nature, soft tissue mass formation and calcification are the radiological hallmarks of chordomas. Computed Tomography (CT) scan is the best imaging modality to delineate areas of osteolytic, osteosclerotic, or mixed areas of bone destruction.Chordoma is usually known as a hypovascular tumor which grows in a lobulated manner. Septal enhancement which reflects a lobulated growth pattern is seen in both CT and MRI and even in gross examination. Other epidural tumors include neurinoma, neurofibroma, meningioma, neuroblastoma, hemangioma, lymphoma and metastases. Their differentiation from chordoma may be difficult due to the same enhancement pattern on CT and MRI.
A dumbbell-shaped chordoma is a rare pathogenic condition. The dumbbell shape is a characteristic finding of neurinomas in spine but in spinal neurinomas extention to transverse foramina has not yet been reported. Although our case mimicked a dumbbell shaped neurogenic tumor, its midline location and destructive pattern were characteristic feature indicating a clue to the diagnosis of chordoma that was already confirmed with histopathology.
This unusual behavior of tumor extension can be explained by the soft and gelatinous nature of the tumor enabling the mass to extend or creep into the existing adjacent anatomical structures.
Cervical Chordoma, Intradural, Computed tomography
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
Primary spinal cord melanoma is a rare central nervous system malignant tumor. Usually it resembles an intradural extramedullary (IDEM) nerve sheath tumor or melanoma. We experienced a patient with upper thoracic primary IDEM spinal cord melanoma who was diagnosed to be with hydrocephalus and without intracranial lesions. Initial symptoms of the patient were related to the hydrocephalus and the primary spinal cord melanoma was diagnosed eight months later. At the first operation, complete resection was impossible and the patient refused additional radiotherapy or chemotherapy. At 22 months after surgery, the patient revisited our institution with recurrent both leg weakness. Leptomeningeal dissemination was present in the whole spinal cord and only partial resection of tumor was performed. The symptoms slightly improved after surgery. Primary spinal cord melanoma is extremely rare but complete resection and additional radiotherapy or chemotherapy can prolong the disease free interval. Hydrocephalus or signs of increased intracranial pressure may be the diagnostic clue of spinal cord malignancy and progression.
Spinal cord tumor; Melanoma; Leptomeningeal dissemination; Hydrocephalus
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
Intradural lumbar disc herniation is a rare complication of disc disease. The reason for the tearing of the dura matter by a herniated disc is not clearly known. Intradural disc herniations usually occur at the disc levels and are often seen at L4–L5 level but have also been reported at other intervertebral disc levels. However, intradural disc herniation at mid-vertebral levels is rare in the literature and mimics an intradural extramedullary spinal tumor lesion in radiological evaluation. Although magnetic resonance imaging (MRI) with gadolinium is useful in the diagnosis of this condition, preoperative correct diagnosis is usually difficult and the definitive diagnosis must be made during surgery. We describe here a 50-year-old female patient who presented with pain in the lower back for 6 months and a sudden exacerbation of the pain that spread to the left leg as well as numbness in both legs for 2 weeks. MRI demonstrated an intradural mass at the level of L5. Laminectomy was performed, and subsequently durotomy was also performed. An intradural disc fragment was found and completely removed. The patient recovered fully in 3 months. Intradural lumbar disc herniation must be considered in the differential diagnosis of mass lesions in the spinal canal.
Intervertebral disc herniation; Intradural disc herniation; Intraspinal tumor; L5
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
Liposarcomas are malignant tumors of the soft tissue, with myxoid liposarcoma being the second most common subtype, tending to occur in the limbs, particularly in the thighs. Myxoid liposarcomas have an intermediate prognosis between well-differentiated and pleomorphic tumors. Spinal metastasis is usual but intradural involvement is extremely rare. We present an unusual case of a multicentric myxoid liposarcoma with intradural involvement. A 41-year-old woman complained of tingling sensation on her left arm. Radiological evaluation revealed multiple masses in her cervical spine, abdominal wall, liver, heart and right thigh, all of which were resected. She was histologically diagnosed with small round cell myxoid sarcoma and underwent adjuvant chemotherapy. However, magnetic resonance imaging analysis after 1 year revealed a large metastatic mass with bony invasion at the C6-T1 level. This mass consisted of extradural and intradural components causing severe compression of the spinal cord. She underwent resection via a posterior facetectomy of C6-7 and an anterior C7 corpectomy. However, the patient died of multiple metastases 18 months after the first diagnosis.
Cervical spine; lntradural; Multicentric; Myxoid liposarcoma; Metastasis
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.
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
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
The authors report a rare case of primary spinal ependymomatosis in a young adult man. Multiple primary ependymomatous lesions were seen on magnetic resonance imaging and no anaplasia was identified on the surgical-pathological analysis. The aetio-pathological mechanism and surgical significance of this rare occurrence is discussed.
A 26-year-old man of Polish origin presented with a ten-day history of pain in the left leg and lower back. This was followed by difficulty in urinating and a decrease in sensation in both legs. Examination revealed pyramidal signs and mild weakness in both lower limbs. He had early sphincter involvement requiring catheterization. Magnetic resonance imaging of the brain was normal. However, that of the spinal cord revealed multiple intradural spinal lesions, both intra- and extramedullary, extending from the cervical cord down to the cauda equina roots. T12-L1 laminectomy was performed. Multiple intradural, extra- and intra-medullary tumors were seen. After the operation, the patient deteriorated with a sensory level at T4. Post-operative cranio-spinal radiotherapy was administered but there was no clinical improvement in the lower limbs.
Primary spinal ependymomatosis is a rare phenomenon involving multiple spinal segments in the absence of a primary intracranial tumor. Radical excision is unrealistic in this condition. Biopsy followed by radiotherapy is the preferred method of treatment.
Epidermoid cyst is generally regarded as congenital disease, and commonly related to other congenital spinal anomalies. However, it also develops iatrogenically. We report one rare case of epidermoid cyst that we experineced among intradural extramedullary tumors. A 21-year-old female patient was admitted to the hospital due to low back pain with radiating pain into a right lower extremity that initiated about a month ago. She complained sensory lose and motor weakness (grade 4+) on her right extremity as well as urinary dysfunction and sphincter dysfunction. She had a lumbar puncture three times due to Pneumococcal meningitis when she was 13 years old. The well-circumscribed intradural extramedullary mass of 1.8×1.6×4 cm size was found on the L4-5 in a magnetic resonance image. Gross total tumor removal was garried out after the total laminectomy L5 and partial laminectomy L4. The tumor mass was gray-colored and so fragile that it was easy to be removed. The histological diagnosis confirmed epidermoid cyst without malignancy. After the operation, the patient progressively showed remarkable neurological recovery. In this case, the cause of epidermoid cyst is considered iatrogenic concerning history of several times of lumbar puncture as meningitis.
Epidermoid cyst; Lumbar puncture; Iatrogenic
Ependymoma has been described typically as an intramedullary tumor derived from ependymal cells. Intradural extramedullary presentation is rarely described and almost always as a unique lesion. Myxopapillary ependymoma is a histological variant that distinguishes from the ordinary type of ependymoma because of its generally better prognosis. We present two cases of multicentric extramedullary myxopapillary ependymomas.
Case 1 was a 30-year-old man with progressive paresthesia and paresis in the lower limbs, urinary sphincter disturbances, gait instability, ataxia, and chronic low back pain with multiple intradural extramedullary lesions at C2-C3, D2-D4-D5, and D12-L1. Case 2 was a 32-year-old man, presented with low back pain and mild paresthesia in the right lower limb. Magnetic resonance imaging (MRI) showed multiple intradural extramedullary lesions with homogeneous enhancement after gadolinium injection at C7, D2, D4, D5, D8, D10, D11, L1, L3, L5, S1, and S2. Complete tumor resection of the approached tumors was archived in both cases. Histological studies confirm myxopapillary ependymomas. Patient's neurologic outcome was good and no residual tumor was present at MRI control at 10 years in case 1 and 12 months in case 2.
We report the first two cases of multicentric extramedullary myxopapillary ependymomas, this etiology must be taken into account in the differential diagnosis of intradural extramedullary tumors.
Extramedullary ependymoma; multicentric ependymoma; myxopapillary ependymoma
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
Extraosseous Ewing's sarcoma (EES) involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET) by identification of the chromosomal translocation (11;22)(q24;q12). We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970–2009). Reverse transcriptase polymerase chain reaction (RT-PCR) identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months) or death (4 years). This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.
extraosseous ewing's sarcoma; spinal cord; intradural; meninges.
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