Intradural Mass Research Articles

Esthesioneuroblastoma metastatic to the thoracic intradural and extradural space

Background context Ethesioneuroblastoma (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. Purpose To report a rare case of recurrent ENB metastatic to the thoracic intradural and extradural space. Study design/setting Case report with a review of the literature. Methods A 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. Results The 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. Conclusions Metastasis 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.

Pilocytic astrocytoma of the spinal cord in an adult

Case presentationA 42-year-old man presented with a 9-month history ofprogressive numbness and paresthesias involving theupper limbs. His symptoms started at the fourth and fifthdigits of both arms, and ascended to involve the elbows.Within several weeks, the lower limbs became involvedwith the numbness and pain starting at the toes andprogressing up to mid thorax. He had no bowel or bladderinvolvement. His examination showed no stigmata ofsystemic disease. He had minimal weakness at hip flex-ion, upper motor neuron signs in all limbs, decreasedsensation in C8 distribution bilaterally, and a sensorylevel at T4 sparing proprioception. Magnetic resonanceimaging (MRI) of the spinal cord 6 and 9 months(September 2006, December 2006) after symptom onsetrevealed T2 prolongation and cord edema (Fig. 1a, c)with an 8 mm enhancing intramedullary lesion at the C5-6 (Fig. 1b, d). The possibility of demyelinating diseasewas considered. Thoracic and brain MRI where unre-markable. Further workup included cerebrospinal fluid(CSF) which revealed leukocytosis of 13 with 88% lym-phocytic predominance, protein of 310 mg/dl, normalglucose and two oligoclonal bands not present in a cor-responding serum sample. The patient continued todevelop progressive neurological deficits that did notrespond to treatment with 5 days of 1 g intravenousMethylprednisolone. Repeated CSF exam showed persis-tence of protein elevation (289 mg/dl), normalization ofleukocytosis and absence of oligoclonal bands. Bothexaminations showed normal cytology. Anti nuclearantibodies (ANA), Lyme serology, angiotensin convertingenzyme (ACE), HTLV-1 serology, B12 level, anti-neutr-ophilic cytoplasmic antibodies (ANCA) were all normal.CT scans of the chest, abdomen and pelvis excludedoccult malignancy and lymphadenopathy. Magnetic reso-nance angiography (MRA) and spinal angiogram showednormal cerebral and spinal vasculature. Repeated spineMRI (February 2007, July 2007) showed mildly increas-ing edema at the site of cervical spinal cord lesion, butstable lesion size and unchanged enhancement pattern.Differential diagnosis included a neoplastic process due tothe persistent duration of enhancement, the elevated pro-tein in the CSF, and absence of clinical or radiographicimprovement after treatment with intravenous steroids.As the etiology of the lesion was not clear preopera-tively, i.e. intramedullary neoplasm or ischemia fromstenosis, the patient underwent C4-T1 decompression,posterior spinal fusion, posterior laminectomy, and intra-dural subtotal mass resection. Intraoperatively the surfaceof the cord and the vasculature looked normal. The cord

An Audit Of Single CNS Masses In Patients With Known Systemic Malignancy

The objective of this audit was to determine the nature of symptomatic single CNS space‐occupying masses in patients referred to neurosurgery who had a prior diagnosis of systemic malignancy outside the CNS. The records and histological slides including immunohistochemistry of 78 patients aged between 22 and 80 with a variety of systemic malignancies and a confirmed tissue diagnosis of an intracranial or intradural spinal mass were reviewed. The study period was 8 years (1999–2006). Results: In 20 of the 78 patients the CNS mass was a new primary tumour of the CNS, (16 gliomas, 4 meningiomas). In 57 patients, the CNS mass was a metastasis from the known systemic malignancy, and one patient had a metastasis from a new undiagnosed primary systemic cancer. In all 14 patients with lung carcinoma, the solitary brain mass was a metastasis from lung. In 20 patients with breast carcinoma, 5 were new primary CNS tumours, 15 were metastases from their primary breast tumour. In 12 patients with melanoma 8 were metastases from melanoma, 4 were new gliomas. We conclude that in this study group, almost 26% of patients referred to our neurosurgery department had a new primary CNS tumour, although the proportion is very different for specific cancer types.

Spinal Cord Malformation Containing a Papillary Neoplasm

A 36‐year‐old man had lower back pain for over one year. MRI revealed an intradural mass with a cystic component, inferior to the spinal cord at L2‐L3, with no bony abnormality of the spine. At surgery, the mass was entirely intradural, inferior to the spinal cord and intimately attached to nerve roots of the cauda equina. On histological reconstruction, it had dorsal columns with a midline sulcus, anterior horns containing anterior horn cells, dorsal roots, and a central canal, resembling a separate spinal cord, with occasional Rosenthal fibers. External to the CNS tissue were adipose cells, smooth muscle bundles and pacinian corpuscles. A cystic region contained a papillary, epithelial‐like neoplasm that was immunopositive for vimentin, EMA and cytokeratin and negative for GFAP, CEA, PLAP and AFP. Because of the resemblance of the solid tissue to the spinal cord, it was considered that this might be a variant of Type II split cord malformation (SCM) of Pang et al (Neurosurgery 31:451, 1992), with mesenchymal and renal cell components. SCM Type II's (diplomyelia) are generally lateral to each other, with two cords within the same dural compartment, unlike this case, in which the second cord was caudal to the first. Alternatively, this lesion might be a mature teratoma containing within it a papillary component. Post‐operatively the patient has no motor deficits.

Surgical consideration of the intraspinal component in extradural dumbbell tumors

Background The dumbbell tumor is considered a distinct group of tumors of the spinal nerve sheath owing to its intriguing clinical and radiological characteristics. More than half of dumbbell tumors are completely restricted to the extradural space, although preoperative MRI in some cases suggests the presence of intradural/extradural tumors. The aim of this retrospective investigation is to elucidate the clinical features and to suggest the surgical strategy to reduce the operating time and prevent additional complications in extradural type. Methods The medical records and radiological studies of 7 consecutive cases (4 male, 3 female, mean age 40.4 years) who underwent removal of the intraspinal component of their extradural dumbbell tumors between January 1996 and December 2005 were analyzed. Diagnosis and preoperative evaluation were performed with MRI and myelogram/CTs. Results The relationship of the tumor mass to dural sac in preoperative MRIs was so indistinct as to suggest an intradural mass in 5 cases. We found that extradural portion was covered with an attenuated dura and that the dural ring was invaginated into the intradural portion in these cases. Therefore, the author began with the epidural removal of the intraspinal and extraspinal component, followed by carefully extracting the intradural-like mass. Conclusions In conclusion, we suggest that invagination of the dural ring is the anatomical feature that may be confused with an intradural/extradural tumor during operations for extradural dumbbell tumors. Initial epidural mass removal, extraction of intradural/extradural tumors subsequent to dural opening, and affirmation of presence of intradural tumor remnant could save time during dumbbell tumor surgery.

Spinal Intradural Extramedullary Mature Cystic Teratoma in an Adult

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 (87x29x20 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.

Intradural Microsurgery and Extradural Gamma Knife Surgery for Hypoglossal Schwannoma: Case Report and Review of the Literature

There have been only 26 cases of hypoglossal schwannomas reported to originate intradurally and extend extradurally into the hypoglossal canal. This 31-year-old mother of two children presented with a 5-day history of progressive headache, nausea, vomiting and vertigo. Her neurological exam was significant for nystagmus and left tongue deviation with marked atrophy. An initial head CT revealed extensive left hypoglossal canal erosion with 4th ventricle compression. T1-weighted MR images with contrast revealed a 4x3 cm left cerebellopontine angle non-homogeneously enhancing mass with an intracranial cystic component and prominent extension into the eroded hypoglossal canal. A left lateral suboccipital craniotomy was performed for subtotal microsurgical resection of the intradural posterior fossa mass. A schwannoma was diagnosed after resection and gamma knife surgery (GKS) was performed three months later for the extradural residual tumor without further deficits. This is a rare report of a hypoglossal schwannoma in a young patient who was treated with a multimodality approach in order to minimize risks. A review of the literature and discussion of the respective benefits of microsurgery versus GKS and long-term follow-up issues are presented.

AJR Teaching File: Lumbar Radiculopathy and Intraspinal Mass

Radiologic Description A sagittal T1-weighted MR image (Fig. 1A) reveals a welldefined isoto hyperintense intradural mass at the level of the L4–L5 vertebrae with several surrounding nerves adhering to the margin. A fat-saturated T2-weighted fast spin-echo image (Fig. 1B) shows a nearly uniform hyperintense mass in the lumbosacral spine. A contrast-enhanced fat-saturated T1weighted image (Fig. 1C) depicts marked heterogeneous enhancement of the lesion. Flow voids are viewed on axial T2weighted images (Fig. 1D) through the intrathecal mass.

Unilateral partial hemilaminectomy in the removal of a large spinal ependymoma

Background context Although the hemilaminectomy technique is known to neurosurgeons performing spinal surgery, laminectomy traditionally has been used during spinal canal surgery for extirpation of spinal cord tumors. Purpose Although the technique of unilateral partial hemilaminectomy is familiar in its various permutations to surgeons, its application in the spinal tumor surgery has been rarely reported. The aim of this study was to review the literature about the management of spinal cord ependymomas and to discuss the major controversies in treatment. Study design Case report. Patient sample A 52-year-old man. Methods The 52-year-old man complained of backache and leg pain bilaterally, dominant on the left side. Spinal magnetic resonance images revealed an intradural mass at the T12–L2 level. A left unilateral hemilaminectomy of the T12–L1 and L2 was performed with the help of high-speed air drills under microscopic magnification and a midline incision was made on the dura. The tumor was totally removed. Results By using microsurgical techniques and with the help of high-speed drills, a unilateral approach to the intramedullary tumors proved itself to be a safe and easy method in this case. It protected the posterior supporting elements and also permitted the surgeon to manipulate the intradural contralateral side easily. The only difficulty during the operation was the suturing of the dural sac. Conclusions This case report emphasizes the need to consider the hemilaminectomy technique in spinal tumor surgery.

THE MANAGEMENT OF PATIENTS WITH INTRADURAL POST-TRAUMATIC MASS LESIONS

Controversy exists about the indications and timing for surgery in head injured patients with an intradural mass lesion. The aim of this study was to survey contemporary approaches to the treatment of head injured patients with an intradural lesion, placing a particular focus on the utilization of decompressive craniectomy.A prospective international survey was conducted over a 3-month period in 67 centers from 24 countries on the neurosurgical management of head injured patients with an intradural mass lesion and/or radiological signs of raised intracranial pressure. Information was obtained about demographic, clinical, and radiological features; surgical management, and mortality at discharge.Over the period of the study, data were collected about 729 patients consecutively admitted to one of the participating centers. The survey included 397 patients with a severe head injury (Glasgow Coma Scale [GCS] 3-8), 155 with a moderate head injury (GCS 9-12) and 143 patients with a mild head injury (GCS 13-15). An operation was performed on 502 patients (69%). Emergency surgery (<24 h) was most frequently performed for patients with an extracerebral mass lesions (subdural hematomas) whereas delayed surgery was most frequently performed for an intracerebral hematoma or contusion. Decompressive craniectomy was performed in a substantial number of patients, either during an emergency procedure (n = 134, 33%) or a delayed procedure (n = 47, 31%). The decompressive procedure was nearly always combined with evacuation of a mass lesion. The size of the decompression was however considered too small in 25% of cases.The results provide a contemporary picture of neurosurgical surgical approaches to the management of head injured patients with an intradural mass lesion and/or signs of raised intracranial pressure in some Neurosurgical Units across the world. The relative benefits of early versus delayed surgery in patients with intraparenchymal lesions and on the indications, technique and benefits of decompressive craniectomy could be topics for future head injury research.

A true “triphasic” pattern: thoracolumbar spinal tumor in a young dog

An 8-month-old male Bernese Mountain Dog was referred with a history of hindlimb weakness that progressed to paresis on the right side. An intradural mass was detected in the spinal canal at the level of the 2nd and 3rd lumbar vertebrae. During surgical removal, 2 small fragments of the mass were prepared for cytologic examination by the squash technique. Cytologic examination revealed 3 different cell types: mesenchymal (stromal) cells, epithelial cells, and small undifferentiated hyperchromatic cells. On the basis of location and the triphasic cytologic pattern, a diagnosis of spinal nephroblastoma (thoracolumbar spinal tumor of young dogs) was made; histologic examination of the mass confirmed the cytologic diagnosis. To our knowledge, this is the first report of a triphasic pattern in a cytologic sample; recognizing this pattern is an important aid in reaching a definitive cytologic diagnosis.

Spontaneous resorption in recurrent intradural lumbar disc herniation

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.

Spinal dural arteriovenous fistula in a case with lipomyelodysplasia

Coexistence of a spinal dural arteriovenous fistula within a dysraphic spinal lesion is a very rare situation. We report a 40-year-old man who presented with low back pain and progressive paraparesis. MR images showed an intradural high signal intensity mass at the L2-L3 level containing irregular signal void structures. Spinal angiography revealed extradural arteriovenous fistula with three connections, drained by a tortuous perimedullary vein.

MRI of primary spinal atypical teratoid/rhabdoid tumor: a case report and literature review

Primary spinal atypical teratoid/rhabdoid tumor (AT/RT) is extremely rare. A 9-month-old boy presented with tetraparesis. Subsequent MRI showed an intradural extramedullary bulky mass extending from C4 to T6. Subtotal removal of the tumor was performed and spinal AT/RT was proven histologically. Despite chemotherapy and radiation therapy, leptomeningeal dissemination was found on follow-up MRI. The patient died 20 months after his initial symptoms. We describe the MRI findings of this case and review the literature describing this rare disease.

Intraoperative Ultrasonography of the Brain and Spine

Intraoperative ultrasonography can play a particularly useful role in facilitating surgery or guiding biopsies in the brain and spinal canal. Given the limited access that is frequently afforded to the operating surgeon, coupled with lesions that may be located in deep or seemingly inaccessible locations, sonography performed through tiny burr holes is useful for confirming location of lesions, guiding biopsy, and confirming extent of resection. In the spine, sonography is most helpful for confirming the precise location of intradural or extradural masses and guiding biopsies. In this review, we describe the technical requirements necessary for scanning the brain and spinal cord and suggest scanning techniques. The spectrum of pathological entities are illustrated along with potential pitfalls that the inexperienced operator is likely to encounter.

Spinal metastasis of occult lung carcinoma causing cauda equina syndrome

Cauda equina syndrome (CES) may be caused by tumor, herniated disc, trauma and spinal infections. However, CES due to occult lung cancer has not been reported in the literature. A 50-year-old man presented with a subacute CES caused by an intradural metastasis of an adenocarcinoma of the lung to the lumbosacral cauda fibers. His lumbosacral magnetic resonance imaging (MRI), showed a well-demarcated, intradural extramedullary mass lesion resembling a neurinoma at the L4/5 level. The patient underwent an L4–L5 laminectomy. The operative findings were also suggestive of neurinoma with involvement of three nerve roots, and a well-demarcated tumor without infiltration into the subarachnoid space. Although the findings of the operation were suggestive of neurinoma, final pathological diagnosis revealed metastatic carcinoma. Immunohistochemistry revealed clear cell adenocarcinoma metastasis. Chest X-ray and high resolution contrasted pulmonary computed tomography were normal. Positron emission tomography (PET) showed a lung mass, at the left apex. The patient was treated with chemotherapy and post-operative spinal radiotherapy was also performed. The CES resolved after the operation and the patient was followed up for 2 years with no recurrence. MRI of intradural cauda equina metastasis may be similar to that of intradural nerve sheath tumor. Surgery and postoperative radiotherapy may be effective for the treatment of CES due to lung carcinoma. Definitive diagnosis is by histopathological examination with immunohistochemistry. If the primary cancer cannot be detected by coventional radiological techniques, PET may be helpful.

Medulloepithelioma: Two unusual locations

Reported herein are two cases of medulloepithelioma (ME) occurring in very unusual locations. Case 1 involved a 44-year-old man who presented with leg pain and urinary incontinence. Spine magnetic resonance imaging (MRI) showed an enhancing epidural and intradural lumbosacral mass. He underwent L3-S2 laminectomy with partial tumor resection. Approximately 2 months following surgery, he developed leptomeningeal spread of the tumor, and died 2 months later. Case 2 involved a 22-month-old girl who presented with painless left proptosis. MRI demonstrated diffuse, fusiform, and contrast-enhancing enlargement of the left optic nerve. Following biopsy, she was treated with chemotherapy. Histologically, the tumors in both cases consisted of primitive pseudostratified neuroepithelial cells, arranged in papillary, tubular, or trabecular configurations. A PAS-stained external limiting membrane (ELM) of the neuroepithelia was present. In case 1 there were scattered cartilaginous nodules within the tumor, in keeping with the diagnosis of a teratoid ME. Immunohistochemically, the tumor cells were positive for synaptophysin, nestin, microtubule-associated protein-2, CD56, CD10, glial fibrillary acidic protein (focal), and S-100 protein (focal). The main differential diagnoses include an immature teratoma and an optic nerve glioma in cases 1 and 2, respectively. Histological demonstration of the ELM by PAS stain is a simple but useful technique.

Posttraumatic intratumoural haemorrhage: an unusual presentation of spinal ependymoma

Ependymomas presenting with intratumoural and/or subarachnoid haemorrhages are seen rarely. These haemorrhages are mostly due to anticoagulation, epidural analgesia or pregnancy. A 62-year-old male farmer with cauda equina syndrome after a work-related trauma is presented. He was admitted to our hospital with paraparesis, faecal incontinence and sensory loss below the level of the lumbar-2 dermatome. Magnetic resonance imaging of the spine displayed an intradural mass lesion at the level of the first lumbar vertebrae. The lesion was excised totally via dorsal midline approach. Histopathologic examination revealed grade-3 ependymoma with intratumoural haemorrhage. The patient's symptoms were relieved completely on postoperative day 7. The patient was given information about periodical examination for recurrence and discharged on the third postoperative week. Asymptomatic spinal lesions should be considered for operation whenever detected because of unpredicted complications.

Primary peripheral primitive neuroectodermal tumors of the spinal cord: report of two cases and review of the literature

Primary intraspinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare tumors with only seven reported cases in the literature. The histopathologic diagnosis of this tumor is complex and has led to a variety of treatment approaches. The distinction between central and peripheral type primary spinal cord PNETs has not always been made in the literature, leading to a paucity of data in this disease. We present here two young patients with primary intraspinal pPNET, their treatment and outcome. The first patient, a 27 year old male, presented with an intradural mass extending from L2 through L5, after multiple relapses, he is currently alive with disease after 72 months, the longest survival yet reported. The second patient, a 16 year old female, presented with an intradural mass at the cauda equina from L2 through L5, and is currently alive with responsive disease at 5 months after initial diagnosis. Here, we discuss the clinical course, the pathology and treatment for this disease and review the literature.

True Intraspinal Neurenteric Cyst in the Lumbosacral Region-Case Report-

A 56-year-old man presented with a very rare true neurenteric cyst in the conus medullaris without evidence of vertebral or visceral anomaly manifesting as a 6-month history of mild low back and bilateral inguinal pain. No motor weakness was found in the bilateral lower extremities. He had also suffered dysesthesia in the bilateral feet for several weeks before admission. Lumbar spine magnetic resonance (MR) imaging demonstrated a cystic intradural extramedullary mass at the L1-2 levels without enhancement after gadolinium injection. MR imaging, computed tomography, and radiography detected no vertebral anomaly. Lumbar laminectomy at the L1-2 levels was performed and the lesion was incompletely removed. Histological examination showed the cystic wall lined with ciliated columnar epithelium. Neurenteric cyst should be considered in the diagnosis of isolated cystic mass lesion at the lumbosacral region even in the absence of vertebral or visceral abnormality.