Cauda Equina Region Research Articles

SDH-B, INSM1, and GATA3 expression in cauda equina neuroendocrine tumors: report on 21 cases from a North indian tertiary care center

Primary neuroendocrine tumors (NETs) of the central nervous system are rare, primarily seen in the cauda equina region, known as cauda equina NETs. This study was carried out to evaluate the morphological and immunohistochemical characteristics of cauda equina NETs. All cases of histologically proven NETs that originated within the spinal cord from 2010 to 2021 were retrieved from the surgical pathology electronic database. For each case, the clinical presentation, site, radiological features, functional status, and preoperative diagnosis were recorded. Immunohistochemical stains for GFAP, synaptophysin, chromogranin A, cytokeratin 8/18, INSM1, Ki-67, GATA3, and SDH-B were performed for every case using an automated immunostainer. GATA3 immunohistochemistry was repeated manually. A retrospective probe of records revealed 21 cases of NETs having a mean age of 44 years and slight male dominance (M:F ratio: 1.2:1). Cauda equina was the most prevalent site of involvement (19, 90.5%). The most typical presentation was lower backache and weakness of bilateral lower limbs. The histopathological features were similar to NETs seen at other sites. Reactivity for at least one neuroendocrine marker was seen in all cases while GFAP was negative. Cytokeratin 8/18 was expressed in the majority (88.9%) of cases. INSM1 and GATA3 expression was seen in 20 (95.2%) and 3 (14.3%) cases, respectively. All cases retained SDH-B cytoplasmic staining. Higher Ki-67 index (≥3%) was associated with a higher risk of recurrence. Cauda equina NETs rarely express GATA3 and are unlikely to be associated with SDH mutations. Recurrent cases may be negative for synaptophysin, chromogranin, and cytokeratin; thus, INSM1 immunohistochemistry is helpful.

Cauda Equina Compression an Ayurvedic management w.r.s to Gridrasi and Pakwasaya Gata Vata

Cauda equina compression at the level of L4/L5 or L5/S1 level is a surgical emergency with potentially significant consequences, including motor and sensory dysfunction. The causes include disc herniation, spinal stenosis, cancer, trauma, epidural abscess. The common cause is usually a disc herniation which is most commonly due to trauma, age, connective tissue disorders and congenital disorders. Cauda equina syndrome a disorder caused by compression of the nerve roots in the cauda equina region. This can cause permanent loss of bladder and bowel control if not treated within time. The incidence of a herniated disc is about 5 to 20 cases per 1000 adults annually and is most common in people in their third to the fifth decade of life with a male to female ratio 2:1. If decompressive surgery is delayed, there can be catastrophic consequences for the patient in terms of bladder, bowel and sexual function. In the present case study, a 36 year old female patient who was diagnosed as a case of Cauda Equina compression due to an extruded L4-L5 disc refused to undergo surgery and opted for Ayurvedic treatment. She was admitted in the Kayachikitsa IPD for 45 days. She was treated with the principle of Gridrasi along with Pakwasayagata vata. Deepana pachana and Vasti or Sodhana is the main treatment choice. Rooksha and Snigdha swedas were done which also help to reduce the stiffness and pain. After the management her physical condition and quality of life improved.

Molecular characterization of CNS paragangliomas identifies cauda equina paragangliomas as a distinct tumor entity

Paragangliomas/pheochromocytomas are rare neuroendocrine tumors that arise from the adrenal gland or ganglia at various sites throughout the body. They display a remarkable diversity of driver alterations and are associated with germline mutations in up to 40% of the cases. Comprehensive molecular profiling of abdomino-thoracic paragangliomas revealed four molecularly defined and clinically relevant subtypes. Paragangliomas of the cauda equina region are considered to belong to one of the defined molecular subtypes, but a systematic molecular analysis has not yet been performed. In this study, we analyzed genome-wide DNA methylation profiles of 57 cauda equina paragangliomas and show that these tumors are epigenetically distinct from non-spinal paragangliomas and other tumors. In contrast to paragangliomas of other sites, chromosomal imbalances are widely lacking in cauda equina paragangliomas. Furthermore, RNA and DNA exome sequencing revealed that frequent genetic alterations found in non-spinal paragangliomas—including the prognostically relevant SDH mutations—are absent in cauda equina paragangliomas. Histologically, cauda equina paragangliomas show frequently gangliocytic differentiation and strong immunoreactivity to pan-cytokeratin and cytokeratin 18, which is not common in paragangliomas of other sites. None of our cases had a familial paraganglioma syndrome. Tumors rarely recurred (9%) or presented with multiple lesions within the spinal compartment (7%), but did not metastasize outside the CNS. In summary, we show that cauda equina paragangliomas represent a distinct, sporadic tumor entity defined by a unique clinical and morpho-molecular profile.

A case of recurring spinal ependymoma in 37-years old man after surgery and adjuvant therapy

We present the case of a 39-years old man who underwent three surgeries because of spinal ependymoma located in cauda equina region. He presented at the Oncology Institute of Vojvodina for combined adjuvant chemo- and radiotherapy after the second surgery. An MRI examination performed after six cycles of therapy showed no signs of disease. 26 months later, a follow-up MRI showed recurrence of disease in the form of small drop metastasis. Six months later, the patient underwent the third surgery. The patient is currently receiving another cycle of radiotherapy, and is scheduled for additional cycles of chemotherapy. Ependymomas are the most common spinal cord malignancy in adults. The symptoms are nonspecific which often causes a delay in diagnosis. An MRI examination of the spine with contrast admission is the study of choice for detecting spinal cord masses. Surgery is the first-line therapy for ependymomas. Recurrence rate is associated with the extent of surgical resection, with en bloc and gross-total resection being associated with lower rates of disease recurrence. In children under 3 years, adjuvant chemotherapy is advocated, while older children and adults undergo adjuvant radiotherapy or combined chemo- and radiotherapy in cases of subtotal resection or tumor recurrence.

Electrical stimulation and denervated muscles after spinal cord injury.

Spinal cord injury (SCI) population with injury below T10 or injury to the cauda equina region is characterized by denervated muscles, extensive muscle atrophy, infiltration of intramuscular fat and formation of fibrous tissue. These morphological changes may put individuals with SCI at higher risk for developing other diseases such as various cardiovascular diseases, diabetes, obesity and osteoporosis. Currently, there is no available rehabilitation intervention to rescue the muscles or restore muscle size in SCI individuals with lower motor neuron denervation. We, hereby, performed a review of the available evidence that supports the use of electrical stimulation in restoration of denervated muscle following SCI. Long pulse width stimulation (LPWS) technique is an upcoming method of stimulating denervated muscles. Our primary objective is to explore the best stimulation paradigms (stimulation parameters, stimulation technique and stimulation wave) to achieve restoration of the denervated muscle. Stimulation parameters, such as the pulse duration, need to be 100–1000 times longer than in innervated muscles to achieve desirable excitability and contraction. The use of electrical stimulation in animal and human models induces muscle hypertrophy. Findings in animal models indicate that electrical stimulation, with a combination of exercise and pharmacological interventions, have proven to be effective in improving various aspects like relative muscle weight, muscle cross sectional area, number of myelinated regenerated fibers, and restoring some level of muscle function. Human studies have shown similar outcomes, identifying the use of LPWS as an effective strategy in increasing muscle cross sectional area, the size of muscle fibers, and improving muscle function. Therefore, displaying promise is an effective future stimulation intervention. In summary, LPWS is a novel stimulation technique for denervated muscles in humans with SCI. Successful studies on LPWS of denervated muscles will help in translating this stimulation technique to the clinical level as a rehabilitation intervention after SCI.

Quadrantectomy for resection of spinal ependymomas with a new classification of unilateral approaches regarding bone drilling and the use of a new tool: The Balak ball-tipped water jet dissector

Abstract Objective The “unilateral approach” is widespread in surgery for spinal degenerative pathologies. However, it is seldom used for resection of spinal intradural lesions. We present two cases where the “quadrantectomy approach”, a sub-category of the unilateral approach, was used for resection of spinal ependymoma and propose a new classification system of the unilateral approach regarding bone drilling and describe the use of the Balak ball-tipped water jet dissector in this procedure. Patients and methods Two patients who underwent surgical treatment of spinal intradural tumor are presented. The tumors were resected using microscopic dissection techniques and neuro-monitoring. An instrument specifically designed for the dissection of the cauda equina nerve roots was used. The surgical technique is presented with stepwise photographs. Results Contrast enhanced magnetic resonance imaging scans obtained on the second day after surgery showed that the tumors in both cases had been totally removed and the nerve roots had regained their natural shape. Histopathological diagnosis was a myxopapillary ependymoma in both cases. There were no postoperative neurological deficits and both patients recovered completely. At two-year and one-year follow-up respectively after surgery, the patients were perfectly well. Conclusion A quadrantectomy approach, which preserves the facet joints and midline structures, should be regarded as a safe subtype of unilateral approaches to spinal intradural lesions. This new terminology will be helpful in proper comparison of the results of various surgical approaches in terms of postoperative spinal instability. The ball-tipped water jet dissector is a beneficial tool for surgery in the cauda equina region.

Ancient Schwannoma of the Cauda Equina: Our Experience and Review of the Literature.

Ancient schwannomas (AS) are exceedingly rare variant of common schwannomas (CS). Only two cases involving the cauda equina region have been previously reported in literature. AS are typically associated with a higher histological degree of degenerative changes (Antoni B areas). It is of peculiar importance, according to our opinion, to outline that, because of their extremely slow growth (which explains the increase of the degenerative changes in respect to the CS) and their typical soft consistency in respect to their standard counterparts, AS usually imply an even better prognosis.

The outcomes of treatment of cauda equina ependymomas in adults

Ependymoma is a rare tumor that accounts for about 4% of all central nervous system tumors. Ependymomas typically have intramedullary localization; however, sometimes the tumor is located outside of the spinal cord and affects the cauda equina nerve roots. To study the outcomes of treatment in patients diagnosed with extramedullary ependymoma. Fifty patients (23 males and 27 females) aged 38.7 years (range: 18-76 years) with ependymoma of the cauda equina region were operated on at the 10th Department of the N.N. Burdenko Neurosurgical Institute between January 2009 and December 2013. Thirty-six patients were newly diagnosed with tumors. Fourteen patients were admitted to the N.N. Burdenko Neurosurgical Institute with recurrent or continued tumor growth. The patients were subdivided into two groups according to this criterion. The outcomes of treatment were evaluated using the Frankel, the Karnofsky, and the VAS scales. The criteria proposed by Kawabata et al. were used to assess the long-term outcomes of surgical treatment. Tumor growth was monitored by contrast-enhanced MRI. Tumors were divided into two subtypes: the encapsulated vs. infiltrative forms. Ependymomas were resected subtotally in 5 patients; continued growth of ependymoma was observed in 3 patients. Positive results were obtained for both groups according to the evaluation performed using the scales. According to the criteria of Kawabata et al., the patients were distributed in a following way: in group 1 patients, the good outcome (class 1) was observed in 26 (72%); the fair outcome (class 2), in 8 (22.5%) patients; while the results were equivocal in 2 (5.5%) patients. A number of patients received radiotherapy as a component of combination treatment. Tumor growth stabilization was achieved. Microsurgical intervention is obligatory, since it has a positive effect on the outcomes of surgical treatment of extramedullary intradural tumors, including ependymomas of the cauda equina region. Treatment effectiveness decreases for the infiltrative subtype of tumor growth. Radiation therapy should be used if continued tumor growth is detected or degree of tumor resection was subtotal.

Detrusor overactivity in patients with cauda equina syndrome.

Retrospective cross-sectional study. To delineate the neurogenic bladder type in patients with cauda equina syndrome (CES) and to suggest, in light of the clinical, radiological, and electrophysiological findings, a possible cause of bladder dysfunction. Many patients with CES experience bladder dysfunction, although the type of neurogenic bladder is quite variable in the clinical setting. Bladder dysfunction in patients with CES is usually areflexic or acontractile detrusor. However, detrusor overactivity (DOA) also reported the cases that cannot be explained by pure root injuries in the cauda equina region. Patients with CES with neurogenic bladder were studied, all of whom (n = 61; mean age ± SD, 48.0 ± 15.9 yr) underwent urodynamic analysis, magnetic resonance imaging (MRI), and electrophysiology. According to the urodynamic findings, the neurogenic bladder was classified into 2 types: DOA and detrusor underactivity or acontractility. The highest level of injury (HLI) or level of injury was determined and analyzed on the basis of the clinical-urodynamic and electrophysiological findings, respectively. Twenty patients with CES (32.8%) showed DOA; in most of them (85.0%, 17/20 patients), the HLI on electrophysiological assessment was L2 or above. Forty-one patients with CES showed detrusor underactivity or acontractility; and most of the patients with CES whose HLI was L3 or below showed detrusor underactivity or acontractility (91.2%, 31/34 patients). None of the HLI or level of injury from the clinical or magnetic resonance imaging findings correlated with neurogenic bladder type. We also found that urodynamic findings including maximal detrusor pressure and bladder capacity was partially correlated with the HLI on electrophysiological assessment (r² = 0.244, P < 0.001 and r² = 0.330; P < 0.001, respectively). DOA was seen most often in patients with CES whose HLI was L2 or above, and might be associated with combined conus medullaris lesion. Electrophysiology might be the most useful assessment tool for prediction of neurogenic bladder type in patients with CES.

Metastatic Lymphoma of the Cauda Equina: Case Report

Introduction Central nervous system (CNS) lymphoma is a very rare form of extranodal non-Hodgkin lymphoma. Lymphomas in the cauda equina region are even rarer. Immunocompromised patients are at high risk for its development. Because of the uncommonness of the disease and unspecific clinical signs and symptoms, the diagnosis and management are aggravated. We present clinical and histopatological findings of a rare metastatic cauda equina lymphoma. Materials and Methods A 57-year-old lady presented with 1 month of severe sciatica, bladder and bowel dysfunction, and motor and sensory disturbances in the inner parts of both thighs (mild motor weakness with limited range of left dorsal flexion, light touch, and pin prick sensations were diminished in the L1-S3 dermatomes on the left side with saddle anesthesia and affected sphincter control). The working diagnosis was a herniated lumbar disc at L5-S1 level, compressing the conus medullaris or cauda equina. On the contrary, MRI of the lumbar spine revealed a tumorous lesion at the level of L5 to S2 on the left side of the spinal cord with compression of the neural structures. No clear differentiation of extradural or intradural location of the lesion was possible. The mass showed marked and homogenous enhancement after intravenous application of gadolinium. No disc pathology was found. The intradural formation was radiologically classified as intradural metastasis. Results A classical laminectomy was performed from first to third sacral vertebra. A soft, bluish violet tumorous mass, lying predominantly extradurally, enchasing the spinal roots of L5 to S2 levels on the left side was found. The dura was infiltrated with the tumor, which was enchasing also the spinal nerve roots. The mass was removed as much as possible, in order not to additionally damage the nervous tissue. After the operation, the nervous status showed improvement of the left leg pain without improvement of neurological deficits. The frozen section showed leukemic infiltration or possibly primary cell B-type lymphoma, as was later confirmed during the definitive histological examination. A lesion was later found also retroperitoneally enchasing the left kidney. The disease was then classified as disseminated extranodal lymphoma, stage 4. After oncological treatment, resolution of the lesions and clinical improvement was documented. Conclusion The CNS is rarely affected by non-Hodgkin lymphoma, except in the late phase of widely disseminated disease. Especially the immunocompromised patients are at risk for its development. Therefore, the extradural non-Hodgkin lymphoma should be considered in the differential diagnosis of spinal cord and root compression. The treatment is tissue biopsy for diagnosis to avoid the nervous damage, followed by chemotherapy and irradiation. Glucocorticoid treatment may temporarily improve the symptoms but on the contrary may obscure the diagnosis. Disclosure of Interest None declared References Knopp EA, Chynn KY, Hughes J. Primary lymphoma of the cauda equina: myelographic, CT myelographic, and MR appearance. AJNR Am J Neuroradiol 1994;15(6):1187–1189 Shen WC, Lee SK, Ho YJ, Lee KR. Myelography, CT and MRI in leukaemic infiltration of the lumbar theca. Neuroradiology 1993;35(7):516–517 Mandawat A, Alraies MC, Miller K, Ondrejka S, Smith S. Cauda equina lymphoma: a case report including postmortem examination. Clin Adv Hematol Oncol 2011;9(5):414–418

Lumbar intradural paragangliomas: report of two cases

Paraganglioma (PG) is a rare tumor of the dispersed neuroendocrine system. PG derives from the embryonic sympathetic and parasympathetic nervous system. Paraganglioma is a neoplasm of the dispersed neuroendocrine system that affects a variety of anatomic sites, mainly the head and neck. It occurs commonly in the carotid body, glomus jugulare, mediastinum and retroperitoneum. Primary spinal paragangliomas are quite rare tumors. They are commonly located in the cauda equina region, and manifest their own clinical and radiological features. In this study, two cases of lumbar intradural paragangliomas are presented. Both of the tumors were totally resected with surgery and there was no evidence of recurrence or metastases during follow-up period. It was concluded that paragangliomas should be taken into consideration during the preoperative workup studies of lumbar intradural tumors.

Clinical Reasoning: A 40-year-old man with CIDP-like illness resistant to treatment

A 40-year-old man developed tingling and numbness in the feet 2 years ago. Three months later, he noticed difficulty standing on his toes. Outside evaluation showed a small immunoglobulin G (IgG) lambda paraprotein, elevated CSF protein of 335 mg/dL (<40 mg/dL), and nerve root thickening with mild gadolinium enhancement in the cauda equina region on lumbar spine MRI. He was presumed to have Guillain-Barre syndrome (GBS) and was treated with IV immunoglobulin (IVIg).

Tanycytic ependymoma of the filum terminale associated with multiple endocrine neoplasia type 1: first reported case

Background contextEpendymoma associated with multiple endocrine neoplasia type 1 (MEN-1) is an extremely rare clinical entity. To the best of our knowledge, only five cases of ependymoma associated with MEN-1 have been previously described. Furthermore, there has been no case of tanycytic ependymoma of the filum terminale associated with MEN-1. PurposeThe present case report illustrates a 53-year-old man with tanycytic ependymoma of the filum terminale associated with MEN-1. We review the literature on ependymoma with MEN-1 and tanycytic ependymoma of the cauda equina region and also discuss the risk of recurrence. Study designA case report. MethodsThe patient presented with complaints of nocturnal pain in the lower back, accompanied by numbness around the anus and intermittent claudication for approximately 1 year. Magnetic resonance imaging (MRI) identified an intradural-enhancing, large mass lesion at the level from Th12 to L2 vertebrae, with a cranial cystic lesion. ResultsOpen-door laminoplasty of the Th12, L1, and L2 and en bloc tumor resection with thickened filum terminale were performed. Histopathologic examination of the tumor specimens showed tanycytic ependymoma (World Health Organization Classification Grade II). At the time of the 2-year and 8-month follow-up examination, MRI did not show tumor recurrence. ConclusionsThis is the first reported case of this clinical entity. A careful follow-up of patients with this unusual tumor is strongly recommended.

Paragangliomes de la queue de cheval : à propos de six cas et revue de la littérature

Background and purposeParagangliomas of the cauda equina are rare tumors. The standard treatment is surgical resection. Our study aims to compare our clinical, radiological, prognostic data to the literature and to offer management and follow-up recommendations. MethodsIn this retrospective study, six patients with paraganglioma of the cauda equina region were treated. Symptoms included radicular nerve pain and low back pain with occasional sphincter dysfunction and motor deficit. MRI showed well-circumscribed lesions with homogeneous enhancement following gadolinium injection. Treatment involved complete surgical resection of the tumor under electrophysiological control. In addition to the characteristics of the tumor, we assessed operating results as well as postoperative morbidity and follow-up. ResultsAll patients had complete removal of the tumor, which required in most cases the resection of the carrying root. The intervention allowed a regression of the initial symptoms, with possible postoperative regressive sphincter disorders. Clinical and radiological follow-up (19 months on average), showed no tumor recurrence. ConclusionsThe reference treatment of these tumors is complete surgical resection, usually requiring the sacrifice of the carrying nerve root. Intra-operative nerve roots stimulation is recommended to reduce the risk of motor deficit linked to this radical treatment. A long-term clinical and radiological follow-up is recommended.

Intraspinal Epidermoid Tumor of the Cauda Equina Region

Retrospective case series. To investigate the clinical features and surgical outcomes of patients with intraspinal epidermoid tumor of the cauda equina region. Intraspinal epidermoid tumor of the cauda equina region is very rare, and the majority of the existing literature of this condition comprises case reports with a few case series. The clinical features and surgical outcomes of 7 patients (2 males, 5 females; age range, 4-66 y) with intraspinal epidermoid tumor of the cauda equina region were retrospectively studied, and a literature review was performed. All patients complained of neurologic symptoms and underwent microscope-assisted surgery. The mean duration of postoperative follow-up was 126 months (range, 52-209 mo). Antecedent lumbar puncture had been performed on 3 patients, and 4 cases thought to be of congenital origin without a past history of lumbar puncture had no associated anomalies, such as spina bifida or dermal sinus. Postoperatively, complications occurred in 3 patients concerning cauda equina symptom. Two patients (29%) had tumor recurrence, diagnosed 1 and 13 years after surgery, respectively. Immediately after additional surgery for tumor recurrence, both patients had severe paresis of the hemilateral foot. Complete removal without tear of the tumor was difficult in our case series, because the capsule of the tumor was thin and often adhered to the cauda equina nerve roots or dura mater. However, total resection of the capsule is important, because patients with epidermoid tumor are at risk for recurrence. On the other hand, aggressive resection of the capsule adhering to the neural elements can cause a high rate of neurological complications postoperatively, especially after surgery for tumor recurrence.

Paraganglioma of cauda equina

Spinal paragangliomas are rare benign tumors. Preoperative diagnosis is difficult, as they share common clinical and radiological features with other lesions at this site. We report an unusual case of paraganglioma in the cauda equina region in a 60-year-female. Ventricular ectopics were noted intraoperatively during surgical removal. Characteristic histology led to definitive diagnosis.

Primary myxopapillary ependymoma of the fourth ventricle with cartilaginous metaplasia: a case report and review of the literature

Myxopapillary ependymoma (MPE), which is a benign histological subtype of ependymoma, is found predominantly in the cauda equina region. It occurs rarely in the brain and mostly as a metastatic deposit from a spinal lesion. The occurrence of primary intracranial MPE is exceptional, with only 11 cases reported to date. We report an additional case of intracranial MPE, which is the third reported case in the fourth ventricle. The tumor manifested in a 50-year-old lady, who presented with features of raised intracranial pressure. A gross total resection of the tumor was achieved. Histologically, the tumor had characteristic features of MPE with focal metaplastic cartilaginous deposit. On further evaluation, there was no evidence of a primary tumor in the spinal cord. Intracranial MPE needs further evaluation by craniospinal MRI to exclude an unrecognized primary in the spinal region, which could warrant surgical attention.

A Randomized, Placebo-Controlled Trial of Repetitive Spinal Magnetic Stimulation in Lumbosacral Spondylotic Pain

Lumbar spondylosis is a degenerative disorder of the spine, whereby pain is a prominent feature that poses therapeutic challenges even after surgical intervention. There are no randomized, placebo-controlled studies utilizing repetitive spinal magnetic stimulation (SMS) in pain associated with lumbar spondylosis. In this study, we utilize SMS technique for patients with this condition in a pilot clinical trial. We randomized 20 patients into SMS treatment or placebo arms. All patients must have clinical and radiological evidence of lumbar spondylosis. Patients should present with pain in the lumbar region, localized or radiating down the lower limbs in a radicular distribution. SMS was delivered with a Medtronic R30 repetitive magnetic stimulator (Medtronic Corporation, Skovlunde, Denmark) connected to a C-B60 figure of eight coil capable of delivering a maximum output of 2 Tesla per pulse. The coil measured 90 mm in each wing and was centered over the surface landmark corresponding to the cauda equina region. The coil was placed flat over the back with the handle pointing cranially. Each patient on active treatment received 200 trains of five pulses delivered at 10 Hz, at an interval of 5 seconds between each train. "Sham" SMS was delivered with the coil angled vertically and one of the wing edges in contact with the stimulation point. All patients tolerated the procedure well and no side effects of SMS were reported. In the treatment arm, SMS had resulted in significant pain reduction immediately and at Day 4 after treatment (P < 0.05). In the placebo arm, however, no significant pain reduction was seen immediately and at Day 4 after SMS. SMS in the treatment arm had resulted in mean pain reduction of 62.3% postprocedure and 17.4% at Day 4. The placebo arm only achieved pain reduction of 6.1% postprocedure and 4.5% at Day 4. This is the first study to show that a single session of SMS resulted in significant improvement of pain associated with lumbar spondylosis in a randomized, double-blind, placebo-controlled setting. The novel findings support the potential of this technique for future studies pertaining to neuropathic pain.

Primary intraosseous paraganglioma of the sacrum

Primary intraosseous sacral paraganglioma is a rare case of location for spinal paragangliomas, which generally manifest as intradural extramedullary tumors of the cauda equina region. The diagnosis can be elusive considering the rarity of sacral paragangliomas. The clinical importance of recognizing this relatively benign tumor cannot be overemphasized as the outcome, extent of surgery and management differs significantly from other common bony sacral tumors which are generally malignant. We report a rare case of sacral paraganglioma that was diagnosed only after histopathological examination, along with a relevant review of the literature.

Paraganglioma of the cauda equina region: A report of three cases

Background:Cauda equina paragangliomas (CEP) are rare tumors. Low back pain and sciatica are the main presenting symptoms. Magnetic resonance imaging (MRI) is the study of choice and treatment consists of total excision when feasible. Definitive diagnosis can only be made after immunohistochemical investigation. CEP is classified as grade I WHO and after total removal the prognosis is excellent. Nonetheless, after subtotal removal, tumor recurrence can occur.Case Description:We present 3 cases of CEP, preoperatively diagnosed as an intradural mass on MRI and suspected to be ependymoma. All 3 patients presented with low back pain and variable sciatic pain. Total resection of the tumor was performed after which all patients fully recovered. There is no recurrence after 13, 11, and 5 years, respectively.Conclusion:CEP is a rare tumor. We diagnosed 3 paragangliomas out of a series of 105 intradural extramedullary tumors in adults (1994–2005). No recurrence was seen after total resection. In retrospect, both the intraoperative appearance and the MR image were not completely typical for schwannoma or ependymoma, but final diagnosis can only be made histologically.