Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity

Prolonged Observation ◽

Slow Growing

AbstractParagangliomas are rare, benign and slow-growing neuroendocrine tumors that can arise from the adrenal medulla (85–90%) or from the extra-adrenal paraganglia.In the central nervous system (CNS), they can be found at several sites, but more often at the cauda equina and filum terminale region, where they account for between 2.5 and 3.8% of total tumor cases of that region. There are only 8 cases described in the literature that mention the presence of the gangliocytic variant of this entity at the filum terminale.We present the case of a 41-year-old man with chronic lumbar pain refractory to medical treatment, without any associated neurological deficits. Magnetic resonance imaging (MRI) revealed an intradural, extramedullar oval lesion with regular contours and homogeneous caption of contrast at L1 level.He was submitted to surgical treatment, with complete resection of the lesion. The histological analysis revealed a gangliocytic paraganglioma of the filum terminale. At 5 years of follow-up, he remains asymptomatic and without any signs of relapse.These are lesions with an overall good prognosis with gross total resection. Although the recurrence rate is extremely low, prolonged observation is recommended due to the slow-growing nature of the tumor, being estimated that between 1 and 4% can recur even after gross total removal.

Primary filum terminale ependymoma: a series of 16 cases

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2008000400017 ◽

2008 ◽

Vol 66 (3a) ◽

pp. 529-533 ◽

Cited By ~ 12

Author(s):

Murilo S. Meneses ◽

André Giacomelli Leal ◽

Larissa B. Periotto ◽

Jerônimo Buzetti Milano ◽

Maurício Coelho-Net ◽

...

Keyword(s):

Cauda Equina ◽

Radicular Pain ◽

Neurological Deficits ◽

Lumbar Pain ◽

Filum Terminale ◽

High Incidence ◽

Microsurgical Resection ◽

Slow Growing ◽

Symptoms And Signs

Filum terminale ependymomas are slow growing tumors of the cauda equina with a high incidence in young adults. Although a complete microsurgical resection can lead to a cure, recurrence is not uncommon. Sixteen cases of filum terminale ependymomas treated at the Instituto de Neurologia de Curitiba were analyzed. Eleven patients were females and 5 males, their age ranging from 7 to 84 years. Symptoms and signs included lumbar pain (31.25%), radicular pain (56.25%) and neurological deficits (12.5%). In three cases, patients had previously undergone surgery in other hospitals. All were tested through MRI and were operated on. Two underwent a laminoplasty and 14 a laminectomy. The last 8 patients of this series had neuro-physiological monitoring during surgery. In all patients a total microsurgical resection was achieved. Histologically, 2 cases were cellular ependymomas and 14 cases myxopapillary ependymomas. There was no recurrence during a 2 to 84 month follow-up period.

Multifocal glioblastoma multiform with “encephalitis-like presentation” : a case report

Egyptian Journal of Radiology and Nuclear Medicine ◽

10.1186/s43055-021-00407-2 ◽

2021 ◽

Vol 52 (1) ◽

Author(s):

Issam Sa’adeh ◽

Mohamed Jamal Saadh

Keyword(s):

Vasogenic Edema ◽

Neurological Deficits ◽

Herpes Simplex Virus 1 ◽

Glioblastoma Multiform ◽

Atypical Clinical Presentation ◽

Mri Scans ◽

Simplex Virus ◽

Multifocal Glioblastoma

Abstract Background Glioblastoma multiform is the most common and aggressive type of primary malignant tumor that affects the central nervous system in adults. It clinically presents with seizures, headache, and/or progressive focal neurological deficits. Radiologically, glioblastoma multiform appears as a single distinguishable, large heterogeneous lesion affecting the cerebrum with characteristic central necrosis, marginal enhancement, and surrounding vasogenic edema. This article describes a patient that exhibited an atypical clinical presentation of multifocal glioblastoma multiform with misleading early radiological features that simulated herpetic encephalitis. Results A 66-year-old female that presented with left-sided hemiparesis and left partial motor seizures underwent multi-slice computed tomography (MSCT) and magnetic resonance imaging (MRI) scans. A cerebrospinal fluid (CSF) polymerase chain reaction (PCR) test was also performed to screen for herpes simplex virus 1 (HSV-1). Conclusions The early stages of glioblastoma may manifest as symptoms typical to encephalitis, which can delay diagnosis and treatment. Therefore, early diagnosis and identification of atypical glioblastoma multiform presentations, as reported in this article, are essential.

Embryonal rhabdomyosarcoma with distant spinal cord metastasis: case and MR-imaging

Pediatric Hematology/Oncology and Immunopathology ◽

10.24287/1726-1708-2020-19-4-158-164 ◽

2020 ◽

Vol 19 (4) ◽

pp. 158-164

Author(s):

N. A. Strumila ◽

A. S. Krasnov ◽

M. M. Andrianov ◽

G. V. Teresсhenko

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Treatment Protocol ◽

Soft Tissue Sarcomas ◽

Distant Metastases ◽

Good Prognosis ◽

Embryonal Rhabdomyosarcoma ◽

Magnetic Resonance Imaging Mri

Embryonal rhabdomyosarcoma (eRMS) is one of the most common soft tissue sarcomas in children, accounting for 4.5% of all childhood tumors. Half of the eRMS occuring in the head and neck are parameningeal. About 40% of patients with eRMS can develop distant metastases. In patients with intracranial tumors, metastatic spread can occur along the central nervous system (CNS) meninges. The literature describes only 4 clinical cases of eRMS with distant metastases in the spinal cord and along the meninges. Only in two out of these four cases, CSF cytology was positive (meaning that tumor cells were detected in cerebrospinal fluid). Magnetic resonance imaging (MRI) of the central nervous system with contrast enhancement can be used to detect distant metastases in the CNS and meninges. We present a clinical case of a 4-year old girl with parameningeal eRMS. MRI of the CNS performed as part of a diagnostic check-up revealed nodal metastatic foci along the meninges of the spinal cord. In accordance with the treatment protocol, the patient was diagnosed with stage 4 disease and received intensive polychemotherapy resulting in the disappearance of the nodal lesions in the spinal cord and a good prognosis. The parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.

Giant gangliocytic paraganglioma of the filum terminale

Journal of Neurosurgery ◽

10.3171/jns.1990.73.3.0459 ◽

1990 ◽

Vol 73 (3) ◽

pp. 459-461 ◽

Cited By ~ 17

Author(s):

Michel Djindjian ◽

Patrick Ayache ◽

Pierre Brugières ◽

Denis Malapert ◽

Marielle Baudrimont ◽

...

Keyword(s):

Cauda Equina ◽

Filum Terminale ◽

Pathological Features ◽

Gangliocytic Paraganglioma ◽

Content Type ◽

Considerable Similarity ◽

Large Neurons ◽

Clinical And Pathological Features ◽

Fine Print

✓ The clinical and pathological features of a giant cauda equina paraganglioma arising from the intradural filum terminale is described. Scattered mature large neurons characterized the tumor as a gangliocytic paraganglioma. Histologically, these neoplasms have considerable similarity with ependymoma and the diagnosis can be easily missed unless special techniques are employed.

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

Cauda Equina Paraganglioma

10.1017/s0317167100045133 ◽

1983 ◽

Vol 10 (4) ◽

pp. 266-269 ◽

Cited By ~ 6

Author(s):

Ming-Sound Tsao ◽

Juan Bilbao ◽

Peter Richardson ◽

Morrison Finlayson

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Basement Membrane ◽

Secretory Granules ◽

Cauda Equina ◽

Clinical Syndrome ◽

Filum Terminale ◽

Supporting Cells ◽

Similar Morphology ◽

The Central Nervous System

SUMMARY:Two paragangliomas resected from the cauda equina had similar morphology, both containing cells having dense core secretory granules with no associated sustentacular or supporting cells. The cells were arranged in characteristic lobules that were surrounded by basement membrane and separated by a fibrovascular stroma. This tumor, although rarely found elsewhere in the central nervous system, does occur in the region of the filum terminale, and causes a clinical syndrome typical of tumors in that area.

Myxopapillary Ependymoma of the Filum Terminale and Cauda Equina in Childhood: Report of Seven Cases and Review of the Literature

Neurosurgery ◽

10.1227/00006123-198402000-00015 ◽

1984 ◽

Vol 14 (2) ◽

pp. 204-210 ◽

Cited By ~ 47

Author(s):

Helen S. L. Chan ◽

Laurence E. Becker ◽

Harold J. Hoffman ◽

Robin P. Humphreys ◽

Bruce E. Hendrick ◽

...

Keyword(s):

Tumor Recurrence ◽

Tumor Resection ◽

Cauda Equina ◽

Good Prognosis ◽

Myxopapillary Ependymoma ◽

Filum Terminale ◽

Spinal Ependymoma ◽

Neuroectodermal Tumors ◽

Lost To Follow Up

Abstract Seven of fourteen children with spinal cord ependymoma had myxopapillary tumors of the filum terminale. These tumors made up 15.9% of all primary spinal neuroectodermal tumors in children (44 cases) seen during a 62-year period (1919 to 1981). Their clinical presentation, radiological features, pathological findings, treatment, and outcome are reported. Six of the seven patients were known to be alive at the time of writing. The seventh patient was lost to follow-up after 3 years without tumor recurrence. Of 5 patients whose primary mode of treatment was operation alone, 3 had intraspinal or intracranial recurrences. Despite tumor recurrences, 2 patients were long term survivors after further operation and irradiation, whereas the third patient recently received craniospinal irradiation for intracranial tumor recurrence. The 2 patients who did not have tumor recurrence after operation alone had been followed for 3 and 7 years, respectively. Two children with subtotal tumor resection and spinal irradiation had no recurrences at 1 and 17 years, respectively. Our data suggest that this unusual subtype of spinal ependymoma is not uncommon during childhood and has a good prognosis. All patients with this tumor require prolonged follow-up for tumor recurrence after operation and irradiation.

Pneumorrhachis causing cauda equina syndrome: a case report and literature review

Egyptian Journal of Radiology and Nuclear Medicine ◽

10.1186/s43055-020-00330-y ◽

2020 ◽

Vol 51 (1) ◽

Author(s):

Areez Shafqat ◽

Hamzah M. F. Magableh ◽

Shameel Shafqat ◽

Syed Shafqat Ul Islam

Keyword(s):

Cauda Equina ◽

Neurological Symptoms ◽

Neurological Deficits ◽

Disc Disease ◽

Surgical Interventions ◽

Vacuum Phenomenon ◽

Perineal Region ◽

Radicular Symptoms

Abstract Background Pneumorrhachis (PR) describes the rare presence of intraspinal air, mainly following traumatic or iatrogenic procedures. According to the localization of air in the spinal canal, PR has been classified into internal (subdural) and external (epidural). PR rarely manifests in neurological deficits and usually resolves spontaneously without recurrence, with air being passed directly into the bloodstream. Here, we report a case of external PR occurring spontaneously (without any underlying trauma or surgical interventions) manifesting as neurological deficits. This is an extremely rare finding with only a limited number of cases in the literature. Case presentation We report a case of spontaneous external PR manifesting as neurological symptoms in a 62-year-old male diabetic patient with chronic low backpain who developed numbness in his perineal region mainly on the left side. His medical history was normal, without trauma or surgical intervention. Magnetic resonance imaging (MRI) and computed tomography (CT) in the past 2 years demonstrated degenerative changes in the lumbar spine, including end plates and disc spaces, with intervertebral disc vacuum phenomenon (VP); the CT additionally showed intraspinal air in the epidural space at L5-S1 levels compressing the cauda equina. A diagnosis of spontaneous external PR was made. A follow-up MRI upon exacerbation of neurological deficits showed an increase in air locule size. Our patient was managed conservatively on a nonsteroidal anti-inflammatory agent (NSAID) and was advised for regular follow-ups. No aspiration or surgery has been performed to date. Conclusions Spontaneous external pneumorrhachis manifesting as neurological symptoms is extremely rare. Due to degenerative disc disease producing vacuum phenomenon, we propose that spontaneous PR secondary to intradiscal VP be considered as part of the differential for radicular symptoms, especially with increasing age. The most effective noninvasive investigation for the diagnosis of PR is CT. MRI is less beneficial in the case of PR as gas and calcifications are hard to distinguish, both being of low-intensity signals on all MR sequences.

Progressive multifocal leukoencephalopathy during ixazomib-based chemotherapy

Current Oncology ◽

10.3747/co.25.3674 ◽

2018 ◽

Vol 25 (1) ◽

pp. 99 ◽

Cited By ~ 7

Author(s):

C.P. Sawicki ◽

S.A. Climans ◽

C.C. Hsia ◽

J.A. Fraser

Keyword(s):

Multiple Myeloma ◽

Progressive Multifocal Leukoencephalopathy ◽

Demyelinating Disease ◽

Opportunistic Infections ◽

Case Reports ◽

Neurological Deficits ◽

Resonance Imaging ◽

Latent Form ◽

Magnetic Resonance Imaging Mri

Progressive multifocal leukoencephalopathy (pml) is a rare demyelinating disease of the central nervous system that most often affects immunocompromised individuals. It is caused by the reactivation of the John Cunningham virus (jcv), which is found in latent form in the majority of adults. We describe a 59-year-old man with multiple myeloma who developed severe neurological deficits during treatment with ixazomib-based chemotherapy. A diagnosis of pml was established with gadolinium-enhanced magnetic resonance imaging (mri) and by detection of jcv in the cerebrospinal fluid. Despite cessation of chemotherapy and treatment with mirtazapine, he had an inexorable neurological decline and died two months after presenting to hospital. Multiple myeloma and its treatments can predispose patients to opportunistic infections including pml. Although there have been case reports of pml in patients with multiple myeloma treated with bortezomib (a different proteosome inhibitor), this is, to our knowledge, the first documented case of pml in a patient treated with a regimen that includes ixazomib.

Amiloride Alleviates Neurological Deficits Following Transient Global Ischemia and Engagement of Central IL-6 and TNF-α Signal

Current Molecular Medicine ◽

10.2174/1566524019666190704100444 ◽

2019 ◽

Vol 19 (8) ◽

pp. 597-604

Author(s):

Li Pang ◽

Shouqin Ji ◽

Jihong Xing

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Caspase 3 ◽

Global Ischemia ◽

Neurological Deficits ◽

Global Cerebral Ischemia ◽

Caspase 9 ◽

Tnf Α ◽

Transient Global Ischemia

Background: Central pro-inflammatory cytokine (PIC) signal is involved in neurological deficits after transient global ischemia induced by cardiac arrest (CA). The present study was to examine if blocking acid sensing ion channels (ASICs) using amiloride in the Central Nervous System can alleviate neurological deficits after the induction of CA and further examine the participation of PIC signal in the hippocampus for the effects of amiloride. Methods: CA was induced by asphyxia and then cardiopulmonary resuscitation was performed in rats. Western blot analysis and ELISA were used to determine the protein expression of ASIC subunit ASIC1 in the hippocampus, and the levels of PICs. As noted, it is unlikely that this procedure is clinically used although amiloride and other pharmacological agents were given into the brain in this study. Results: CA increased ASIC1 in the hippocampus of rats in comparison with control animals. This was associated with the increase in IL-1β, IL-6 and TNF-α together with Caspase-3 and Caspase-9. The administration of amiloride into the lateral ventricle attenuated the upregulation of Caspase-3/Caspase-9 and this further alleviated neurological severity score and brain edema. Inhibition of central IL-6 and TNF-α also decreased ASIC1 in the hippocampus of CA rats. Conclusion: Transient global ischemia induced by CA amplifies ASIC1a in the hippocampus likely via PIC signal. Amiloride administered into the Central Nervous System plays a neuroprotective role in the process of global ischemia. Thus, targeting ASICs (i.e., ASIC1a) is suggested for the treatment and improvement of CA-evoked global cerebral ischemia.

Beyond Oncological Hyperthermia: Physically Drivable Magnetic Nanobubbles as Novel Multipurpose Theranostic Carriers in the Central Nervous System

Molecules ◽

10.3390/molecules25092104 ◽

2020 ◽

Vol 25 (9) ◽

pp. 2104 ◽

Cited By ~ 1

Author(s):

Eleonora Ficiarà ◽

Shoeb Anwar Ansari ◽

Monica Argenziano ◽

Luigi Cangemi ◽

Chiara Monge ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Brain Tumors ◽

Ad Hoc ◽

Superparamagnetic Iron Oxide ◽

Conventional Radiotherapy ◽

The Brain

Magnetic Oxygen-Loaded Nanobubbles (MOLNBs), manufactured by adding Superparamagnetic Iron Oxide Nanoparticles (SPIONs) on the surface of polymeric nanobubbles, are investigated as theranostic carriers for delivering oxygen and chemotherapy to brain tumors. Physicochemical and cyto-toxicological properties and in vitro internalization by human brain microvascular endothelial cells as well as the motion of MOLNBs in a static magnetic field were investigated. MOLNBs are safe oxygen-loaded vectors able to overcome the brain membranes and drivable through the Central Nervous System (CNS) to deliver their cargoes to specific sites of interest. In addition, MOLNBs are monitorable either via Magnetic Resonance Imaging (MRI) or Ultrasound (US) sonography. MOLNBs can find application in targeting brain tumors since they can enhance conventional radiotherapy and deliver chemotherapy being driven by ad hoc tailored magnetic fields under MRI and/or US monitoring.