How Should Syringomyelia be Defined and Diagnosed?

OBJECTIVE: Syringomyelia is considered as a fluid-filled cavitation inside the spinal cord. However, there is no agreement whether a dilated central canal should be included under this heading or how glioependymal cysts, myelomalacias, or cystic tumors should be distinguished from syringomyelia. This article provides a definition of syringomyelia and guidelines for its diagnosis. METHODS: Between 1991 and 2015, of 3206 patients with spinal cord pathologies 2276 demonstrated cystic features. All patients underwent magnetic resonance imaging. Syringomyelia was differentiated from cystic intramedullary tumors, glioependymal cysts, myelomalacias, and dilatations of the central canal by clinical and radiologic criteria. RESULTS: A total of 1535 patients were diagnosed with syringomyelia, 635 with dilatations of the central canal, 52 with glioependymal cysts, 52 with mylomalacias, and 2 with cystic intramedullary spinal cord tumors. Additional neuroradiologic studies revealed the causes of syringomyelia. As a result 604 patients showed pathologies at the craniocervical junction leading to disturbances of cerebrospinal fluid (CSF) flow. The commonest was a Chiari I malformation in 543 patients. Nine hundred thirty-one patients presented with pathologies in the spinal canal. The commonest causes were spinal arachnopathies, leading to CSF flow obstructions in 533 patients, intramedullary tumors in 152 patients, and tethered cord syndromes in 69 patients. CONCLUSIONS: The diagnosis of syringomyelia should be reserved for patients with a fluid-filled cavity in the spinal cord related to either a disturbance of CSF flow, spinal cord tethering, or an intramedullary tumor. For patients in whom such a relation cannot be established, the diagnosis of syringomyelia should be withheld.

Leptomeningeal Metastases.

OPINION STATEMENT: Treatment options for leptomeningeal metastases are expanding with greater tolerability and efficacy than in the past. Improved knowledge of molecular subtypes of some cancers can guide in choosing more effective therapeutic options; however, physicians should be mindful that these molecular types can be different in the central nervous system compared to the rest of the body. This is particularly true in breast and lung cancer, in which some patients now can live for many months or even years after diagnosis of leptomeningeal metastases. Options for intrathecal therapies are expanding, but physicians should be mindful that this is a passive delivery system that relies on normal CSF flow, so therapies will not penetrate bulky or parenchymal disease sites, especially in the presence of abnormal CSF flow. When chemotherapeutic options are lacking or unsuccessful, focal radiosurgery which can provide symptomatic relief and proton craniospinal radiation remain effective options. Hopefully more formal studies will be conducted in the future to verify which treatments are indeed most effective for particular types of cancer.

Primary spinal intradural extramedullary lymphoma: A novel management strategy.

Primary spinal intradural extramedullary lymphoma remains a very rare entity in spinal oncology. In this case report, we present the first treatment of a PSIEL diagnosed by cytopathologic analysis alone followed by urgent radio- and chemotherapy in the literature. At 18-month follow-up, our patient was ambulatory with near total imaging resolution of the lesion. In conclusion, surgical excision or biopsy may not be necessary when suspicion for PSIEL exists, and may delay prompt medical and radiation treatment due to necessity for wound healing. Further research into the management of extramedullary lymphoma treatment strategies is warranted.

Detection of tumor-derived DNA in cerebrospinal fluid of patients with primary tumors of the brain and spinal cord.

Cell-free DNA shed by cancer cells has been shown to be a rich source of putative tumor-specific biomarkers. Because cell-free DNA from brain and spinal cord tumors cannot usually be detected in the blood, we studied whether the cerebrospinal fluid (CSF) that bathes the CNS is enriched for tumor DNA, here termed CSF-tDNA. We analyzed 35 primary CNS malignancies and found at least one mutation in each tumor using targeted or genome-wide sequencing. Using these patient-specific mutations as biomarkers, we identified detectable levels of CSF-tDNA in 74% [95% confidence interval (95% CI) = 57-88%] of cases. All medulloblastomas, ependymomas, and high-grade gliomas that abutted a CSF space were detectable (100% of 21 cases; 95% CI = 88-100%), whereas no CSF-tDNA was detected in patients whose tumors were not directly adjacent to a CSF reservoir (P < 0.0001, Fisher's exact test). These results suggest that CSF-tDNA could be useful for the management of patients with primary tumors of the brain or spinal cord.

Cytologic diagnosis of spinal cord ependymoma in cerebrospinal fluid.

Ependymoma cells are known to rarely exfoliate into cerebrospinal fluid (CSF). However, the frequency of CSF involvement in patients with ependymoma is unclear, and to the author's knowledge the cytomorphologic features of tumour cells have not been well described to date. In this study, the CSF findings in a patient with ependymoma and the cytopathological features of this tumor are reported. The patient presented at the University Hospital of Heraklion, Crete, suffering from a chest to back pain. Computed tomography, scanning and magnetic resonance imaging (MRI) were performed and a mass of 3x2 cm in the thoracic aspect of the spinal cord was found. A sample of cerebrospinal fluid (CSF) was sent for cytologic examination and a diagnosis of ependymoma was made. A biopsy was performed and histology confirmed the cytologic diagnosis of ependymoma grade II (WHO). Exfoliated cells from ependymomas of spinal cord are rarely recognizable in CSF samples. Except in patients with myxopapillary tumours and anaplastic tumours, cytomorphologic features of ependymoma have been described only in case reports of intraoperative imprinting or fine needle aspiration biopsies (FNABs) and not in CSF cytology.

Primary intramedullary spinal cord lymphoma.

OBJECTIVE: Among the rare causes of myelopathies is primary intramedullary spinal cord lymphoma (PISCL). As PISCL is often underrecognized, delaying appropriate treatment, we sought to describe its presentation, imaging characteristics, and outcomes. METHODS: Mayo Clinic medical records, lymphoma database, and autopsies from 1996 to 2009 were searched. Inclusion criteria were clinical myelopathic presentation, intramedullary spinal cord abnormalities, and pathologically confirmed CNS lymphoma. Exclusion criteria were extramedullary lymphoma, secondary intramedullary lymphoma, or other myelopathic etiology. Clinical features, diagnostic methods, neuroimaging, treatment, and outcomes were assessed. RESULTS: The 14 patients' median age at presentation was 62.5 years (range 41-82 years) and 10 were men (71%). Two had lymphoma risk factors (HIV infection 1; chronic immunosuppression postorgan transplant 1). Most had initial presumptive diagnoses of CNS demyelinating disease and definitive diagnosis of lymphoma was delayed a median of 8 months (range 1-22 months). CNS lymphoma was pathologically confirmed by biopsy (brain 6; spinal cord 4), CSF cytology (3), and autopsy (1). Most patients had multifocal, persistently enhancing lesions on spinal MRI and 8 had involvement of conus medullaris, cauda equina, or both. IV methotrexate was the initial treatment in 9 of 12 (75%) but lymphoma recurred in the majority. Half of the patients were wheelchair-dependent at 10 months and 2-year survival was 36%. CONCLUSIONS: PISCL mimics other causes of myelopathy. Spinal MRI demonstrating multifocal lesions, persistent gadolinium enhancement, and conus medullaris or cauda equina involvement is characteristic. Pathologic confirmation often requires CNS biopsy. Despite chemotherapy, morbidity and mortality is high.

Late spinal metastases from an isolated pineal region germinoma mimicking a schwannoma.

A 20-year-old male with a prior history of germinoma presented 8 years after the initial diagnosis with progressive lower back pain. The preoperative diagnosis was schwannoma based on the appearances of a tumor in the lumbosacral region on MRI; however, histologically, a germinoma "drop" metastasis was seen. This report emphasizes the need for long-term follow-up in patients with germinoma. In addition, this patient is unusual in that the preoperative assessment favored schwannoma.

[A case of intramedullary cervical metastasis from cerebellar anaplastic oligodendroglioma without typical MR appearance for CSF dissemination].

We report a case of intramedullary cervical metastasis from cerebellar anaplastic oligodendroglioma without any MR appearance for CSF dissemination or recurrence in the primary tumor site. The case was a 73-year-old female who underwent tumor resection for cerebellar anaplastic oligodendroglioma following irradiation and TMZ chemotherapy. One year and a half later, a small nodular lesion developed at the temporal lobe. While treatment with TMZ was restarted during the course, another intramedullary cervical lesion produced additionally without any CSF dissemination or recurrence at the primary site. The histological examination obtained in spinal surgery revealed evidence of similar features consistent with a previous cerebellar tumor with anaplastic oligodendroglioma. Although CSF dissemination by malignant glioma with leptomeningeal enhancement is relatively well recognized at their terminal stage, cases with intramedullary cervical metastasis without any leptomeningeal enhancement have only been rarely described. We discussed the mechanisms of intramedullary cervical metastasis from intracranial malignant glioma.

Pediatric tumors of the spine.

Spinal cord neoplasms are rare lesions in children. Most of the spinal cord neoplasms are astrocytomas. Frequently the clinical symptoms are non-specific. Back pain is often the leading symptom. Diagnosis is often delayed. Magnetic resonance imaging is the imaging modality of choice. Preoperative brain and spine imaging covering the entire spine is mandatory to exclude cerebrospinal fluid seeding.

Spinal drop metastases from a papillary meningioma: a case report and review of the literature: utility of CSF sampling.

In this paper, we report a rare case of a 29-year-old boy who presented with papillary meningioma originating from the posterior fossa meninges. After a long, disease-free period, however, spinal drop metastases occurred 32 months after resection of the primary tumor. The primary and metastatic lesions had a similar histological appearance, meaning that multiple spinal metastatic lesions occurred through CSF route even after a gross total resection of the tumor. Tumor seeding during surgery is the evident reason for spinal metastasis, although we strictly adhered to the standard precautions for operations for malignant tumors such as obstruction of the cisterna magna with cotton paddies, and changing surgical gloves and instruments during the operation. In this report, we briefly discuss an exceedingly rare variant of meningioma, the papillary variant, and suggest a new approach, a CSF sampling, in the management of both malignant and benign meningiomas. CSF sampling allows for the early detection of metastasis and of tumor cells before metastasis has occurred, thus allowing treatment to begin as soon as possible. This early detection and management is possibly associated with longer survival. Furthermore, we discussed that meningiomas are tumors that are not as benign as initially thought.

Multidisciplinary management of spinal lipoma.

Spinal lipomas (congenital lipomyelomeningoceles) can cause significant aesthetic deformity of the lower spine in addition to recognised neurological problems. Surgical management consists of untethering the cord in symptomatic cases, and debulking of the lipomatous component to improve the spinal contour. However, management remains controversial. Between May 1998 and November 2002, 23 cases of lumbosacral spinal lipoma were operated on by neurosurgeons at our hospital. Of these, eight (34%) patients were subsequently referred to plastic surgery for aesthetic revision, which was performed by a combined team of plastic surgeons and neurosurgeons. In addition, three new patients had their primary surgery as a planned procedure performed by the same combined team. The purpose of this paper is to present the method and results of combined plastic surgery and neurosurgery technique in these 11 cases of spinal lipoma. Follow up ranged between 12 months and 4 years (mean 20 months). One patient had wound break down and three patients developed seroma. Two patients had cerebrospinal fluid (CSF) leakage which required re-exploration and prolonged hospitalisation. The aesthetic results were very satisfactory in 10 out of the 11 patients. The frequency of secondary referral for aesthetic revision, together with the incidence of CSF leak, underlines the importance of managing spinal lipomas in a multidisciplinary team involving neurosurgeons and plastic surgeons. This series demonstrates that aesthetic results are encouraging but decreasing the CSF leakage rate remains a challenge. Further follow up will determine the role of early combined surgery in the management of spinal lipoma.

[Visual acuity loss, the initial sympton of a spinal cord neoplasm]. / Disminución de agudeza visual, primer síntoma de un tumor medular.

CASE REPORT: A 46-year-old woman, who presented with right visual acuity loss, was found to have papilledema, and subsequently shown to have ventricular dilatation in a cerebral Magnetic Resonance Imaging (MRI) assessment. Elevated protein levels were found in the cerebrospinal fluid. Spinal MRI revealed the presence of a spinal cord neoplasm. After surgical removal of the tumor, which turned out to be a neurilemmoma, the patient's visual acuity was restored. DISCUSSION: The ocular presentation and the relationship between intracranial hypertension and spinal tumors are discussed. Likewise, the importance of considering the various causes of papilledema is emphasized.

[MRI monitoring of cerebral spinal fluid metastasis in rabbit model].

OBJECTIVE: To establish a rabbit model of cerebral spinal flow metastasis, to analyze the growth rate of tumor, and to investigate the value of MRI in monitoring the biology of tumor compared with pathology. METHODS: Twenty-four New Zealand white rabbits were inoculated with suspension of VX(2) tumor cells in the subarachnoid space via the foramen magnum (experimental group), and 6 rabbits were inoculated with normal saline (control group). MRI examination, including non-enhanced T(1)WI, T(2)WI, and FLAIR sequences and then T(1)WI, FLAIR after dynamic contrast enhanced with Gd-DTPA were done 7 approximately 22 days after inoculation with a 3-day interval. The rabbits were killed after the last MRI scan with their spinal cords, spinal meninges, and tumor taken out to undergo microscopy. RESULTS: (1) MRI plain scan showed that in the experimental group 2 nodi in the medulla and 1 nodes in the cervical spinal cord were found with low signal on T(1)WI and high signal on T(2)WI; and FLAIR imaging showed local lesions with medial signal in 6 rabbits (25%). And no abnormal signs were seen in the control group. (2) MRI enhancement showed that in the experimental group the images of 15 rabbit models were enhanced markedly with irregular thickening of meninges or nodules at the subarachnoid space on T(1)WI, positive signs were confirmed on FLAIR sequence in 16 of the 24 rabbits, and positive signs were noted on DCE-MRI scanning in 18 of the 24 rabbits (75%). In the control group 5 of the 6 rabbits were negative in images. Microscopy showed thickened of meninges and spinal meninges in 20 of the 24 rabbits of the experimental group and spinal cord metastasis in 22 rabbits. No pathological changes were seen in the control group. Statistics showed a CSF metastasis rate of 91.67%. There were significant difference between the plain scan and T(1)WI with enhancement (P < 0.01) and between FLAIR scan and FLAIR enhancement scans. There was a significant difference between T(1)WI and FLAIR enhancement and pathological findings (P < 0.05). There was no significant difference between DCE-MRI method and pathological results (P > 0.05). CONCLUSION: Gd-DTPA enhanced MRI scan sequences has a high sensitivity and specificity and can be used in monitoring the growth of CSF metastasis. There is a disparity between the MRI signs and pathological findings. It is a key that to improve the spatial resolution of machine and to investigate the best method for detecting early metastasis.