Giant-Cell Ependymoma of the Cervical Spinal Cord With Syringomyelia and Pathological Presentation: A Case Report and Review of the Literature - Cureus

Ependymomas are infrequent neuroepithelial tumors of the cardinal tense strategy (CNS) that make from clusters of ependymal cells that borderline the cardinal canal of the spinal cord and the ventricles of the brain. These tumors relationship for 2-6% of CNS tumors [1] to 60-70% of spinal cord tumors [2]. Ependymomas are divided into the pursuing 3 grades by the World Health Organization (WHO):, people 1 includes myxopapillary and subependymoma; people II includes cellular, papillary, wide cell, and tanycytic; and people III includes others, namely, anaplastic [3].

Giant-cell ependymomas (GCEs), a uncommon benignant archetypal described successful 1996, are often categorized arsenic those measuring much than 4 cm [4]. Giant-cell glioblastoma, pleomorphic xanthoastrocytoma, subependymal giant-cell astrocytoma, and anaplastic oligodendroglioma are among the tumors that mightiness correspond a differential diagnosis for GCE [5].

In this report, we contiguous a lawsuit of ependymoma accompanied by syringomyelia, the presumption of the pathogenesis, WHO’s caller classification, and the attraction and prognosis for spinal ependymoma.

A 43-year-old antheral diligent had cervical spine symptom 2 years agone accompanied by numbness of the fingertips. Recently, the numbness of the fingertips appeared much often and had dispersed to the little extremities affecting the patient’s activity.

Physical introspection revealed the numbness of the left-sided digit and wrist, astir the abdomen, and ankles connected 2 sides, biceps reflex +/+, triceps reflex +/+, brachioradialis +/+, Hoffman -/-, patellar tendon reflex ++/++, Achilles tendon reflex ++/++, Babinski-/-, Barre’s sign, Romberg’s sign, and nary cerebellar symptoms.

A massive, multicystic, and partially enhancing cervical intramedullary tumor reaching from C4 to C7 was identified utilizing T2-weighted MRI with the sagittal plane. Additionally, determination was a syringomyelic cavity that connected the medulla oblongata to T7. On T2-weighted, sagittal images, the syringomyelic cavity and cysts were intelligibly disposable (Figure 1). The C2-T1 laminotomy was performed connected the patient.

After the myelotomy, a hard wide mixed with grey and yellowish colors was noted (Figures 2A, 2B). Macroscopically, its features were accordant with a tumor that was not peculiarly good infiltrating. Ependymoma was identified successful the archetypal fewer tiny insubstantial fragments connected frozen-section analysis. The laminar plateau was relocated and fixed with mini plates aft the arachnoid was closed, and the dura mater was sutured. Early neurological betterment with decreased digit and toed numbness was seen pursuing surgery. He began rehabilitation therapy, and his neurological information steadily improved implicit time. Pathological investigation connected hematoxylin and eosin (H&E)-stained paraffin sections (Figures 2C, 2D) showed a moderately cellular tumor with a biphasic pattern. One signifier had perivascular pseudorosettes (Figure 2D). There were besides a fewer ependymal canals with eosinophilic inclusions and tiny columnar cells lining them (Figure 2E). Giant cells and the cells generating perivascular pseudorosettes some substantially expressed GFAP, S-100 protein, and vimentin successful the cytoplasm connected immunohistochemistry (Figure 2F). The Ki-67 labeling scale was astir 5.7% successful the areas.

Postoperatively, the diligent did not person immoderate issues. T2-weighted sagittal MRI confirmed that the full tumor had been removed and determination was nary recurrences (Figures 3A-3C).

Table 1 presents a reappraisal of the lit with cases of elephantine cervical ependymomas associated with syringomyelia.

Clinical presentation

Spinal ependymomas tin alteration greatly. Inflammatory unwellness oregon spinal metastases person been observed successful up to 60-70% of patients, successful summation to benign mechanical discomfort. According to immoderate studies, 10% of patients acquisition radicular pain. Due to the cardinal operation of these tumors, interaction and symptom perceptions are typically the archetypal to beryllium impacted. Sphincter dysfunction is uncommon, with the objection of cone oregon cauda equina tumors. Although uncommon, tumor bleeding tin sometimes effect successful acute neurological deterioration. Due to the anticipation of neurological damage, spinal cord injuries are hard to treat, which is further worsened if they impact giant tumors. Better functional outcomes are present feasible due to the fact that of the advancement of microsurgical methods and aesculapian exertion [10].

MRI utilizing opposition worldly is utilized to corroborate the diagnosis. In astir 75% of instances, the cervical spine is the country that is astir usually impacted. Our diligent experienced the related lesion known arsenic syringomyelia. Syringomyelia resolved post-surgery successful each antecedently documented instances, including our own. A slow-growing spinal cord tumor, changes successful vascular density, changes successful intramedullary pressure, and degenerative changes with vas partition hyalinosis are immoderate of the pathomechanisms of syringomyelia [11]. It was besides shown successful our lawsuit that consequent compression of lengthy spinal tracts, neurons, and artery walls causes degenerative and ischemia necrotic modifications successful the tumor and glial stem beneath the tumor.

Degenerative cysts and tumor ischemic-related syringomyelia aft ependymoma whitethorn hap arsenic a effect of disruption of the blood-brain barrier, which is supported by microcirculation impairment [11].

WHO classification and pathological examination

The word ependymoma, arsenic utilized by the WHO, refers to a people of tumors that are histologically heterogeneous and see cellular, papillary, clear-cell, and tanycytic subtypes [7]. Two examples of GCE of the filum terminale were initially reported by Zec et al. [4] successful 1996.

The histological grading of ependymomas has been controversial, fixed the ample spectrum of anaplastic ependymoma prevalence (7-89%), showing however hard it is to travel to an statement connected a histological classification strategy [12]. The histopathological criteria for classifying an ependymoma arsenic benign oregon malignant is 1 of the causes of inconsistent outcomes. A diagnosis of GCE of the cervical spinal cord was obtained aft taking into relationship the immunohistochemical and morphological characteristics. The astir accessible and invaluable instrumentality for differential diagnosis is immunohistochemistry. GCE is antagonistic for synaptophysin and Melan A portion affirmative for GFAP, vimentin, S-100, and CD99. The astir important antibody for ruling retired further soft-tissue malignancies is GFAP.

However, a important displacement from the accepted histomorphological categorization to a classification of 10 assorted types based connected anatomical determination and familial characteristics has precocious occurred with the 2021 revision of the WHO classification of CNS malignancies. The wide thought of whether a tumor belongs to the ependymoma household oregon not has altered due to the fact that of the improvement of these caller molecular kinds [13].

The 4 chiseled tumor types that marque up ependymal tumors of the spinal cord are spinal ependymoma (SP-EPN), spinal ependymoma with MYCN amplification (SP-MYCN), myxopapillary ependymoma (MPE), and subependymoma (SE). The spinal determination of SP-EPN and the deficiency of MPE oregon SE morphological characteristics service arsenic diagnostic criteria. The astir predominant determination of SP-EPN aft localization successful the thoracic and lumbar spine is the cervical spine [14]. The neurofibromatosis benignant 2 (NF2) cistron is situated connected chromosome 22q, wherever the bulk of SP-EPN person chromosomal deletions. The tumor predisposition syndrome is brought connected by NF2 gene mutations. Recently, SP-MYCN, a caller and clinically assertive benignant of ependymoma was discovered. It often displays aboriginal metastases, accelerated betterment aft relapse, leptomeningeal dissemination, and inadequate effect to multimodal therapeutic strategies [15]. Spinal localization and the beingness of MYCN amplification service to place SP-MYCN. Only a fewer instances person been recorded frankincense acold [15-18]. SP-MYCN has a precocious recurrence complaint of 75-100% [16,18], and a lawsuit survey of 13 tumors indicated a median progression-free endurance of 17 months [15]. The find of this peculiarly assertive tumor signifier among spinal ependymomas, which are often much benign, highlights the value of cautiously reviewing existent tumor classifications by familial and objective tests.

Treatment

The optimal attraction strategy for cervical ependymomas is full excision accompanied by intraoperative neurophysiological monitoring, often somatosensory evoked potentials. There are respective ways to dainty ependymomas. A cervical laminectomy was utilized to dainty the tumor successful 4 of the cases that were reported, portion hemilaminectomy, hemilaminoplasty, and cervical laminoplasty were each performed successful 1 case. A wide C2-T1 cervical laminectomy was performed successful this instance. As successful our instance, neurophysiological monitoring was employed passim the cognition without immoderate modifications.

A precise level betwixt the tumor and the spinal cord, which is the eventual goal, could not beryllium recovered successful our case; therefore, lone 90% of the tumor was removed. Ependymomas person a level that is well-differentiated from steadfast nervus tissue, which makes implicit resection possible. Cerebrospinal fluid fistulas are the astir often documented complication, with 2 occurrences recorded, 1 of which was linked to meningitis and the different to a surgical tract corruption [19].

GCE has a bully prognosis [4] due to the fact that determination is nary transportation betwixt it and assertive conduct. These histological characteristics are accordant with the objective effect successful the existent case. Increasing neurological betterment has been seen during follow-up, and an MRI scan has not revealed immoderate denotation of tumor recurrence contempt the lack of adjuvant therapy, specified arsenic radiation. According to Ho et al. [20], the lack of immoderate illness has been linked to the debased Ki-67 labeling index. Ki-67 is different prognostic marker for ependymomas.