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Background: Primary spinal glioblastomas are extremely rare neoplasms and account for only 0.2% of glioblastoma cases. Due to the rare incidence of spinal cord glioblastoma in the literature, its natural history/outcome remains undetermined. The present article describes the clinical presentation, radiological/pathological characteristics, and outcome of the primary spinal cord glioblastoma. Case Description: Two young patients initially presented with paresis that rapidly progressed to paraplegia. Nondermatomal sensory deficits were also noted, mainly affecting the lower limbs. Neuroradiological imaging revealed an extensive intramedullary spinal cord lesion, with no evidence of concurrent intracranial space-occupying lesions. Thoracic laminectomy, followed by tumor debulking and/or biopsy, was performed. The histomorphology was suggestive of glioblastoma, the World Health Organization grade 4 (Isocitrate Dehydrogenase-wild type). They were discharged in stable condition and were started on chemoradiotherapy, with clinicoradiological follow-up. One patient passed away after 9 months of initial presentation. The other patient was alive at 6-month follow-up. Conclusion: Primary spinal glioblastoma is a rare and challenging tumor. Patients commonly present with a progressive paresis, resulting in paraplegia, regardless of the surgical resection extent, and received adjuvant chemotherapy. Therefore, primary spinal cord glioblastoma should be considered in patients reporting a rapid lower limb weakness with neuroradiological evidence of extensive, exophytic intramedullary lesion of the spine. A biopsy-proven histopathological diagnosis is of indisputable importance to establish the final diagnosis and plan treatment options. [ABSTRACT FROM AUTHOR]

Background: Cervicomedullary glioblastoma is an extremely rare clinical entity and the principles of its management are not well understood. Case Description: We report two cases of cervicomedullary glioblastoma in young patients aged 12 and 30 years with contrasting clinical presentation and outcomes. The 12-year-old child had rapid onset bulbar symptoms, with frank infiltration of the medulla due to which the patient succumbed within 4 weeks of surgery. The 30-year-old adult had a relatively slow disease onset and progression and made a good neurological recovery without disease progression at 16 months after surgery. To the best of our knowledge, we also report only the second adult patient in the literature with a dorsally exophytic cervicomedullary glioblastoma. Difficulties in diagnosis and management are discussed with a review of the pertinent literature. Conclusion: The overall outcome depends on the rapid progression and severity of preoperative symptoms and the degree of tumor infiltration noted in imaging and during surgery. [ABSTRACT FROM AUTHOR]

Background: Granular cell tumors (GCTs) are uncommon peripheral nerve sheath tumors of Schwann cell origin that may occur throughout the body. However, they rarely occur in the spinal canal. Case Description: A 49-year-old male presented with burning sensation in the left knee. The MRI of the lumbar spine showed an L3-L4 intradural extramedullary tumor. Complete surgical resection was successfully performed and the L3 root burning improved. Histopathologically, the lesion proved to be a benign GCT. Conclusion: Spinal GCTs are rare benign tumors that may be found in an intradural extramedullary location in the spine. The preferred treatment is complete surgical resection as subtotal/partial resection may result in recurrence warranting radiation therapy. [ABSTRACT FROM AUTHOR]

Background: Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon but aggressive neoplasms associated with radiation exposure and neurofibromatosis Type I (NF1). Their incidence is low compared to other nervous system cancers, and intramedullary spinal lesions are exceedingly rare. Only a few case reports have described intramedullary spinal cord MPNST. Case Description: We describe the clinical findings, management, and outcome of a young patient with NF1 who developed aggressive cranial nerve and spinal MPNST tumors. This 35-year-old patient had familial NF1 and a history of optic glioma treated with radiation therapy (RT). She developed a trigeminal MPNST that was resected and treated with RT. Four years later, she developed bilateral lower extremity deficits related to an intramedullary cervical spine tumor, treated surgically, and found to be a second MPNST. Conclusion: To the best of our knowledge, this is the first report of cranial nerve and intramedullary spinal MPNSTs manifesting in a single patient, and only the third report of a confined intramedullary spinal MPNST. This unusual case is discussed in the context of a contemporary literature review. [ABSTRACT FROM AUTHOR]

Background: Histoplasma capsulatum infection is largely seen in endemic regions; it results in symptomatic disease in <5% of those infected and is most often a self-limiting respiratory disease. Disseminated histoplasmosis is considered rare in the immunocompetent host. Central nervous system (CNS) dissemination can result in meningitis, encephalitis, and focal lesions in the brain and spinal cord, stroke, and hydrocephalus. An intramedullary spinal cord lesion as the only manifestation of CNS histoplasmosis has been rarely described. Case Description: We present an atypical case of a 44-year-old man from a nonendemic region, on adalimumab therapy for ulcerative colitis who developed an isolated intramedullary spinal cord lesion in the setting of disseminated histoplasmosis. His course was initially indolent with vague systemic symptoms that led to consideration of several other diagnoses including sarcoidosis and lymphoma. Biopsies of several positron emission tomography positive lymph nodes revealed granulomatous inflammation, but no firm diagnosis was achieved. He was ultimately diagnosed with histoplasmosis after an acute respiratory infection in the setting of anti-tumor necrosis factor therapy. With appropriate antifungal therapy, the spinal cord lesion regressed. The previous systemic biopsies were re-reviewed, and rare fungal elements consistent with H. capsulatum were identified. A presumptive diagnosis of CNS histoplasmosis was made in the absence of direct laboratory confirmation in the setting of rapid and complete resolution on antifungal therapy. Conclusion: Disseminated histoplasmosis should be considered in granulomatous disease, even if the patient resides in a nonendemic region. Furthermore, clinicians should be mindful that CNS histoplasmosis may present in an atypical fashion. [ABSTRACT FROM AUTHOR]

Background: Thoracic intramedullary neurosarcoidosis is an uncommon but serious manifestation of spinal cord disease. Its concomitant occurrence with thoracic disc herniation can mislead the physician into attributing neurologic and radiographic findings in the spinal cord to disc pathology rather than inflammatory disorder. Here, we present such a rare case of concomitant thoracic disc and spinal neurosarcoidosis. Case Description: A 37-year-old male presented with progressive right lower extremity weakness and numbness. Magnetic resonance imaging (MRI) of the thoracic spinal cord revealed a T6-T7 paracentral disc eccentric to the right with T2 signal change extending from T2 to T10 level. This prompted acquiring a contrasted MRI that also depicted intramedullary enhancement around the T6-T7 disc bulge. Computed tomography scan of the chest showed mediastinal lymphadenopathy concerning for sarcoidosis. Lymph node biopsy confirmed the diagnosis of sarcoidosis, and high-dose steroid treatment was initiated. The patient had significant symptomatic improvement with steroids with full neurological recovery and improvement of his symptoms. Conclusion: While stenosis from thoracic disc disease could potentially suggest a mechanical etiology for the patient's symptoms, attention must be paid to the imaging findings as well as the degree and extent of cord signal change and intramedullary contrast enhancement. Appropriate and timely diagnosis is essential to avoid unnecessary invasive procedures. [ABSTRACT FROM AUTHOR]

Background: A spinal intramedullary abscess is a rare clinical entity in which patients classically present with a subacute myelopathy and progressive paraplegia, sensory deficits, and/or bowel and bladder dysfunction. We report the second case of spinal intramedullary abscess caused by Candida albicans to ever be published and the first case of its kind to be surgically managed. Case Description: A 44-year-old female presented with severe lumbar pain associated with paraparesis, incontinence, and paraplegia. She reported multiple hospital admissions and had a history of seizures, having already undergone treatment for neurotuberculosis and fungal infection of the central nervous system unsuccessfully. Nevertheless, no laboratory evidence of immunosuppression was identified on further investigation. Magnetic resonance imaging showed a D10-D11, well-circumscribed, intramedullary mass within the conus, which was hypointense on T1-weighted imaging and hyperintense on T2/STIR weighted. The patient underwent surgery for removal and biopsy of the lesion, which provided the diagnosis of an intramedullary abscess caused by C. albicans, a very rare condition with only one case reported in literature so far. Conclusion: C. albicans intramedullary abscess is a very rare clinical entity, especially in immunocompetent patients. We highlight C. albicans as an important etiology that must be considered in differential diagnosis. Critical evaluation of every case, early diagnosis, timely referral and surgical management of the abscess is essential to improve neurological outcome. [ABSTRACT FROM AUTHOR]

Background: Solitary fibrous tumors (SFTs) are benign tumors derived from mesenchymal tissues that predominantly occur in the pleura. Establishing the diagnosis of these very rare intramedullary spinal lesions, with no clear-cut pathognomonic radiographic characteristics, is particularly challenging. Case Description: Two males, 30 and 41 years of age, presented with progressive cervical myelopathies attributed to a cervical intramedullary exophytic tumor with associated spinal cord edema. One patient showed that the lesion was highly vascularized. Both patients underwent surgical excision of firm, solid, focal, and, particularly in one of them, very vascular/hemorrhagic tumors; at surgery, there was some adherence between the tumors and the cord tissue, but gross-total resections were achieved in both cases, demonstrated on postoperative MR scans. Histological and immunohistochemical findings confirmed the diagnosis of SFT (WHO Grade I). After a 6-month postoperative period, both patients neurologically improved and had no MR evidence of tumor recurrence. Conclusion: Intramedullary cervical exophytic SFTs are extremely rare. Although these solid tumors may present with hemorrhagic features and at surgery demonstrate significant adherence to the pial/cord surface, complete surgical resections are feasible resulting in good outcomes. [ABSTRACT FROM AUTHOR]

Background: Intramedullary spinal schwannomas constitute only 0.3% of primary spine tumors. We could identify only 13 such cases involving the conus that were not associated with neurofibromatosis (NF). Here, we report a 70-year-old male without NF who was found to have a paraparesis due to a schwannoma of the thoracolumbar junction/conus (D11-L2). Case Description: A 70-year-old male presented with an L1-level paraparesis with urinary incontinence. The magnetic resonance showed an intramedullary mass of 85 × 10 mm extending from D11 to L2; it demonstrated significant patchy enhancement. The patient underwent a D12 and L1 laminectomy with gross total excision of the mass that proved to be a schwannoma. Three months postoperatively, he was able to ambulate with support, and regained sphincter function. Conclusion: Intramedullary schwannomas involving the conus/thoracolumbar junction are rare, and can be successfully excised resulting in good outcomes. [ABSTRACT FROM AUTHOR]

Background: Clear cell ependymomas (CCEs) are a rare variant of tumors of the nervous system, the main location is the intracranial compartment. Special differential diagnosis should be done with oligodendrogliomas, neurocytoma, glioneurocytoma, astrocytoma, or metastatic renal cell carcinoma, lesions that somehow share cells with clear cytoplasm. Most of these lesions are benign but differential diagnosis is essential to decide further treatment. Few case reports of intramedullary CCEs have being published and there is no strict consensus on the diagnostic criteria. Case Description: We hereby describe a new case of an intramedullary clear CCE with very few neurological symptoms, surgical treatment is satisfactory, histological and immunohistochemical analysis was confirmatory. After gross total resection and 3-year follow-up no recurrence of the lesion is evident. Conclusion: After this case presentation and review of the limited literature, it is evident that methodical clinical suspicion, radiological imaging combined with histological, and modern immunohistochemical techniques are essential for the diagnosis. Surgical options with gross total resection remain the cornerstone of its treatment. Neurophysiological monitoring is extremely useful to avoid postoperative morbidity. [ABSTRACT FROM AUTHOR]

Background: Many patients with spinal juvenile pilocytic astrocytoma can experience prolonged remission after resection. However, some reports suggest that pregnancy may be associated with progression. Case Description: The authors provide an image report highlighting a case of rapid and aggressive transformation of an intramedullary astrocytoma of the cervical spine in a pregnant patient. Over the course of 1 year, the lesion progressed from a juvenile pilocytic astrocytoma to an anaplastic astrocytoma. Genetic testing revealed mutations associated with aggressive behavior. Conclusion: The case and associated imaging demonstrate the importance of close neurologic monitoring and counseling regarding risk of progression in pregnant patients with spinal gliomas. [ABSTRACT FROM AUTHOR]

Background: Intramedullary cervical cystic lesions are typically attributed to tumors, infection, or trauma. Here, a patient newly presented with quadriparesis due to a chronic cervical intramedullary hematoma attributed to a cervical epidural steroid injection (CESI) performed 4 years earlier. Case Description: A 38-year-old patient had a CESI in 2014. Resulting in a transient quadriparesis attributed to an inadvertent intramedullary cord injection. Now, at age 42, she presented with a recurrent cervical myelopathy due to an MR-documented C3-C6 intramedullary cystic lesion that at surgery proved to be a chronic liquified hematoma rather than a syrinx. Conclusion: CESI can result in inadvertent intramedullary hemorrhages and spinal cord injuries. Here, a 42-yearold female presented with recurrent myelopathy due to a chronic intramedullary C3-C6 cervical hematoma attributed a prior intramedullary CESI injection performed 4 years previously. [ABSTRACT FROM AUTHOR]

Background: Intramedullary spinal cord metastases represent 4-8.5% of the central nervous system metastases and affect only 0.1-0.4% of all patients. Those originating from renal cell carcinoma (RCC) are extremely rare. Of the eight patients described in the literature with metastatic RCC and intramedullary cord lesion, only five were found in the cervical spine. Here, the authors add a 6th case involving an RCC intramedullary metastasis at the C1-C2 level. Case Description: A 78-year-old male patient presented with intermittent cervicalgia of 5 months duration accompanied by few weeks of a progressive severe right hemiparesis, up to hemiplegia. The magnetic resonance imaging (MRI) examination revealed an intramedullary expansive lesion measuring 10 mm×15 mm at the C1-C2 level; it readily enhanced with contrast. A total body computed tomography (CT) scan documented an 85 mm mass involving the right kidney, extending to the ipsilateral adrenal gland, and posteriorly infiltrating the ipsilateral psoas muscle. The subsequent CT-guided fine-needle biopsy confirmed the diagnosis of an RCC (Stage IV). The patient next underwent total surgical total removal of the C1-C2 intramedullary mass, following which he exhibited a slight motor improvement, with the right hemiparesis (2/5). He died after 14 months due to global RCC tumor progression. Conclusion: The present case highlights that a patient without a prior known diagnosis of RCC may present with an intramedullary C1-C2 metastasis. In such cases, global staging is critical to determine whether primary lesion resection versus excision of metastases (e.g., in this case, the C1-C2 intramedullary tumor) are warranted. [ABSTRACT FROM AUTHOR]

Background: Intramedullary primitive neuroectodermal tumors (PNETs) are tumors found rarely in the cervical region, with only five such cases described in the literature. The available literature contains only one report regarding cervicomedullary junction PNET. Case Description: The authors present a case report of a 45-year-old male patient who had undergone urgent hospitalization owing to progressive tetraparesis and subtle impairment of respiratory function. He underwent magnetic resonance imaging, which showed an extensive enhancing cervical intramedullary tumor extending from C5 to the bulbar region. Since he developed severe impairment of respiratory function, he required tracheostomy. He then underwent microsurgery 2 days after his admission, and a partial tumor resection was performed. The pathological diagnosis of PNET of the cervicomedullary junction (CMJ) was made. He had slight worsening of strength after surgery with subsequent deterioration over the next 3 weeks. The tumor displayed aggressive growth; thus, radiotherapy was indicated. Unfortunately, he developed severe febrile neutropenia and died after 2 weeks of radiotherapy. Given the rarity of the condition, we wish to review the epidemiology, pathophysiology, and treatment options of his population. Conclusion: Intramedullary PNETs of the cervical spine and CMJ are exceedingly rare in adults; treatment of such patients remains a challenge, despite the modern neurosurgical armamentarium that is available. [ABSTRACT FROM AUTHOR]

Background: Too many patients, with or without significant cervical disease, unnecessarily undergo cervical epidural steroid injections (CESIs). These include interlaminar (ICESI) and transforaminal ESI (TF-CESI) injections that are not Food and Drug Administration (FDA) approved, have no documented long-term efficacy, and carry severe risks and complications. Methods: Here we reviewed recent reports of morbidity and mortality attributed to the various types of CESIS. Major complications included; epidural hematomas, infection (abscess/meningitis), increased neurological deficits due to intramedullary (quadriparesis/quadriplegia), and intravascular injections (e.g., vertebral artery injections leading to cord, brain stem, and cerebellar strokes). The latter injections leading to strokes were typically attributed to the particulate steroid matter (e.g., within the methylprednisolone injection solution) that embolized into the distal arterial branches. Results: Complications of cervical CESI/TF-CESI injections producing epidural hematoma, new neurological deficits (intramedullary injections), or intravascular injections resulting in strokes to the cord, brain stem, and cerebellum are often underreported. Interestingly, several other cases involving adverse events of CESI/TF-CESI may now be found in the medicolegal literature. Conclusions: Cervical epidural injecions (e.g., CESI, ICESI, and TF-CESI) which are not FDA approved, provide no long-term benefit, and are being performed for minimal to no indications. They contribute to significant morbidity and mortality, including; epidural hematomas, infection, inadvertent intramedullary cord injections or cord, brain stem, and cerebellar strokes. Furthermore, these injections are increasingly required by insurance carriers prior to granting permission for definitive surgery, thus significantly delaying in some cases necessary operative intervention, while also subjecting patients at the hands of the insurance companies, to the additional hazards of these procedures. [ABSTRACT FROM AUTHOR]

Background: Intramedullary epidermoid cyst (IEC), typically associated with spinal dysraphism, is rare, with fewer than 80 such cases reported in the literature. Here we present an adult with an IEC without spinal dysraphism. Case Description: A 41-year-old female presented with the gradual onset of a progressive spastic paraparesis attributed to a magnetic resonance imaging (MRI)-documented D8-9 intramedullary lesion. Following microsurgical excision of the IEC, she fully recovered. Conclusion: IECs are rare lesions, typically found in conjunction with spinal dysraphism. Utilizing MRI studies to document the lesion location, gross total microsurgical excision is the procedure of choice. Early excision of these benign lesions maximizes functional recovery. [ABSTRACT FROM AUTHOR]

Background: Intramedullary and subarachnoidal tubercular abscesses are rare forms of spinal tuberculosis as compared with extradural collections secondary to vertebral tuberculosis. Case Description: We herein present a 33‑year‑old, apparently healthy male patient who presented clinically as transverse myelitis, with a lesion at detected at conus cauda, developing fulminant holocord intramedullary tubercular abscess, treated with surgical evacuation and much later with anti‑tubercular drugs. Atypical clinical, serological, imaging findings in addition to lack of knowledge of occurrence of fulminant intramedullary tuberculosis led to the delay in starting anti‑tubercular treatment. Conclusion: Early diagnosis requires a high index of suspicion, search for a primary focus of tubercular infection, investigation with magnetic resonance imaging (MRI) of spinal cord, biopsy, and confirmation with microscopy and culture, even in immunocompetent individuals. Early diagnosis, prompt treatment with surgical evacuation of abscess, and anti‑tubercular drugs can lead to a good neurological recovery. [ABSTRACT FROM AUTHOR]

Background: Neurenteric cysts are benign tumors of the central nervous system (CNS) that represent 0.3% to 0.5% of all spinal cord tumors. They are usually extramedullary and found in the lower cervical and thoracic spine. Only 12.2% of neurenteric cysts are documented to be intramedullary. Case Description: The authors report a case of a 35-year-old female that presented with progressive weakness and loss of coordination in her legs. Magnetic resonance imaging (MRI) showed an intramedullary cystic lesion in the thoracolumbar region and a low-lying conus medullaris suggesting tethered cord. The patient was taken to the operating room for detethering of her spinal cord and resection of the lesion. Pathologic examination of the tissue confirmed the diagnosis of a neurenteric cyst. Conclusion: A search of the literature since the advent of MRI showed 29 published cases of intramedullary neurentic cysts. Of the 24 published cases with a follow-up MRI, the average recurrence rate was 25% with a mean follow up of 51 months. [ABSTRACT FROM AUTHOR]

Background: It has been our impression in recent years that there is a significant delay in diagnosis (DID) of patients in Israel harboring intradural spinal cord tumors (IDSCTs). DID can lead to irreversible deficits and unnecessary suffering. Our goal was to identify the incidence and the specific reasons for DID of IDSCTs in patients operated upon at our institution. Methods: A retrospective record review, with additional telephone survey, of 101 patients operated upon at our institute between the years 1996 and 2009 was conducted. The patients who were not diagnosed locally and those who were diagnosed during routine spinal imaging studies as part of their basic disease check-up were excluded. Accordingly, neurofibromatosis and medical tourist patients were excluded. Results: The clinical presentation of IDSCTs in our study was similar to the descriptions given in previous reports. The average age was 41.9 ± 23.3 years. Most tumors were ependymomas, astrocytomas, and schwannomas. The most common symptoms were motor or sensory disturbance, back pain, walking disturbance, and sphincter control deficit. The median time to diagnosis was 12.0 ± 37.0 months (range 3 days to 20 years). We found DID in 82.2% of the cases. 62.4% of the cases were defined as "unreasonable delay." The most common reasons for DID were "classical symptoms with a wrong diagnosis" and "delayed imaging." Conclusions: Based on the results of this study, the incidence of unreasonable delays in diagnosis of primary IDSCTs in Israel is very high. In order to shorten the time to diagnosis, primary and secondary care physicians need to increase their awareness of symptoms that may be associated with these lesions. We hereby offer feedback for care providers, relevant to the diagnostic workup of these patients. Such a feedback must be delivered by neurosurgeons to the community they are serving. [ABSTRACT FROM AUTHOR]

Background: Intramedullary spinal hemangioblastomas are known to be accompanied by syringomyelia. Case Description: Here, we report a patient who presented with symptoms of a Chiari malformation but was found to have a D4 intramedullary hemangioblastoma with a holocord syrinx. Conclusions: Although rare, neurosurgeons should keep in mind the possibility of an intramedullary hemangioblastoma in patients presenting with symptoms of a Chiari malformation. [ABSTRACT FROM AUTHOR]