Search

In adults, spinal ependymomas are usually found in intramedullary locations. However, intradural extramedullary spinal ependymomas are rare. Additionally, spinal ependymomas usually show iso to hypointensity on T1-weighted images without hemorrhage. Herein, we present a rare case of a 43-year-old female with a pathologically confirmed intradural extramedullary ependymoma that showed hyperintensity on T1-weighted imaging accompanied by hemorrhage.

Background and Importance: Neurenteric cysts (NCs) account for 0.7 to 1.3% of all spinal tumors and are most commonly present during the first three decades of life. Only two cases have been reported in individuals in their 7th and 8th decades of life. This study described a rare case of an NC in a septuagenarian (70 – 79 years of age) who presented with atypical features of the disease. Case Presentation: A 77-year-old female patient presented with acute onset paraparesis. Magnetic resonance imaging revealed a 13.6 × 14.1 × 15.4 mm intradural extramedullary cystic lesion, which was anteriorly located at the C7-D1 level. It was T1 hypointense, T2 hyperintense, and showed faint contrast enhancement at the junction between the cyst and the spinal cord. At surgery, a cyst with a greyish-white thin wall was noted in the spinal canal, displacing the spinal cord posteriorly. The cyst wall was partially excised, and a small portion adhering to the spinal cord was left behind. The histopathology report was consistent with an NC. At the 2-week follow-up, the patient’s lower limb strength had improved. Conclusion: Although rare, NCs can present in the seventh decade of life. Magnetic resonance imaging may reveal unusual findings, like T1 hypointensity and faint contrast enhancement of the cyst wall. In elderly patients, a portion of the cyst wall may be left behind if it adheres to the spinal cord to avoid the risk of neurological deterioration.

Introduction: Leptomeningeal melanocytomas are rare tumours originating from neural crest derived melanocytes. They are usually solitary and presentation with multifocal meningeal melanocytoma is very rare and indicative of potentially more aggressive behaviour. This case report and scoping review sought to evaluate the presentation, and key radiological features that can help differentiate multifocal meningeal melanocytoma from other differentials and provide a discussion of the key management and prognostic points once these tumours are diagnosed. Case presentation: A 26 year old male presented with neck pain radiating to both shoulders and subjective weakness in left shoulder movement. MRI demonstrated a large enhancing C2–C3 intradural-extramedullary lesion with further lesions at the T7/T8 level, left cerebellopontine angle and midline suprachiasmatic region. Whilst the imaging appearances were initially thought be indicative of a phacomatosis such as NF2-related schwannomatosis, surgical excision of the cervical tumour confirmed a melanocytic tumour of leptomeningeal origin, consistent with multifocal meningeal melanocytoma. Patient made a good post-operative recovery and remains under half yearly radiological surveillance, with repeat MRI 6 months after surgery demonstrating subtle growth of the untreated intracranial and spinal lesions. Literature review and conclusions: This is the first description, to our knowledge, of a multifocal meningeal melanocytoma associated with both cerebellopontine angle and suprasellar lesions. This case and included scoping review highlight the need to consider this rare diagnosis whenever multifocal craniospinal lesions are encountered, and the need to consider aggressive management through surgical resection and adjuvant craniospinal radiotherapy once these tumours are diagnosed.

Spinal intradural-extramedullary ependymomas (IEEs) most commonly affect the lumbosacral spine. Because of their neural axis dissemination and adherence to neural structures, managing IEEs is still a controversial neurosurgical challenge. The study aimed to investigate the potential prognostic factors that influence long-term surgical outcomes by evaluating consecutively operated patients with IEEs. During the study period, medical records of all diagnosed patients with spinal tumors were reviewed retrospectively. This study included all patients with consecutive IEEs who underwent surgical intervention in 3 neurosurgical institutions in different periods (February 2004 to December 2020). In 3 neurosurgical institutions, 64 (28 women, 36 men) patients were operated upon for IEE. The mean age of the patients at diagnosis was 38.9 years. The mean preoperative symptom duration was 17.8 months. Radicular pain was the most common symptom, observed in 53 patients. Gross total resection was applied to 48 patients. 54 patients had good clinical outcomes at their last follow-up after 106.9 months on average. Myxopapillary ependymoma was the most common histopathological type (n = 43). The progression was observed in 5 patients. The presence of preoperative neurological deficits, IEEs extending >2 spinal levels, bone-eroded IEEs, surgical complication, capsule violation, and incomplete resection were associated with poor prognosis. Several clinical and surgical factors can affect the functional outcomes of the surgical treatment of IEEs. Some radiological features can make neurosurgeons aware of the prognosis of IEEs. In such cases, the neurosurgeons should plan to remove tumors without violating their integrities (capsules) to obtain satisfactory functional outcomes. [ABSTRACT FROM AUTHOR]

Spinal ependymomas are generally located in the intramedullary compartment in adults. Intradural extramedullary spinal ependymomas are extremely rare. Spinal ependymomas show various contrast enhancements on MRI. In this study, we report a rare case of a 52-year-old female who had a pathologically confirmed intradural extramedullary ependymoma that showed no enhancement on MRI.

Intramedullary ependymoma represent one of the two most common intramedullary spine tumors. While occurrence of spinal ependymoma extramedullary is very rare, especially the tanycytic subtype. We present this case of cervical extramedullary tanycytic ependymoma. Male patient 52 years old, presented with neck pain and severe left brachialgia. MRI of the cervical spine revealed intradural extramedullary hyperintense lesion opposite C5-6. Patient operated with total excision of lesion, and histological examination reported as ependymoma.

Background: The most common location of malignant tumors of the peripheral nerve sheath in the spinal cord is the intradural-extramedullary region, and is rare in the spinal nerve roots in the lumbar region. They mainly affect large female dogs over 6 years of age. Imaging tests assist in the presumptive diagnosis, but confirmation requires histopathological and immunohistochemical examination. The prognosis is guarded. Diagnostic imaging, anatomopathological and immunohistochemical findings of a malignant tumor of the intradural-extramedullary peripheral nerve sheath with medullary infiltration in the lumbar region in a young dog are reported. Case: A body of a 6-year-old Poodle dog was donated for necropsy and diagnostic clarification. In the history, there was a suspicion of lumbar intramedullary neoplasia, detected by computed tomography (CT), with a 4 years progressive chronic evolution. Additionally, the dog had hidden spina bifida (L7 to S3), as detected by radiography and CT. On post mortem radiographic examination (X-ray), there was an enlargement of the vertebral canal (T10 to S2), intense osteolysis (L1 to S2), spinous processes (L5 to L7), and ankylosis (L3 to L7). Necropsy revealed ankylosis (L3 to L7) and intradural-extramedullary mass (9.5 × 2.6 × 2.3 cm) (L2 to L6). No metastases were identified. On microscopy, there was neoplastic proliferation of cells with intense pleomorphism, arranged in bundles interlaced in palisades and sometimes solid mantles. The mitotic index was high, ranging from 10 to 12 mitoses per field. There was also necrosis, hemorrhage, edema, and focal axonal demyelination of the adjacent white matter in the spinal cord. Masson Trichrome staining highlighted an intense diffuse conjunctive stroma. There was a suspicion of a malignant tumor of the peripheral nerve sheath and an immunohistochemical panel was performed for confirmation. There was strong and diffuse positivity for vimentin and S-100 and partial positivity for neuron-specific enolase (NSE), negative for anti-factor VIII, glial fibrillary acidic protein (GFAP), a-actin for smooth muscle, cytokeratin, neurofilament, and desmin. Thus, the diagnosis of malignant neoplasm of the peripheral nerve sheath was confirmed. Discussion: Peripheral nerve sheath tumors are classified as benign or malignant. In dogs, they are frequent in elderly, females, and large breeds. In this case report, the animal was young, female, and small breed. The location of the spinal nerve roots is uncommon, and is more commonly found in the brachial plexus. In the animal reported, the tumor was observed as lumbar swelling. Clinical signs vary with the affected region, however, neurogenic claudication and muscle atrophy are more frequent, as observed in this report. Imaging examinations such as X-rays and CT assist in the presumptive diagnosis. In this case report, spina bifida was identified on radiography, and CT suggested the presence of intramedullary neoplasia and allowed to monitor tumor growth. Post mortem X-ray imaging revealed intense osteolysis and ankylosis, which were confirmed at necropsy, which also elucidated its intradural-extramedullary location with infiltration into the spinal cord. The confirmation of the neoplasm was made by histopathological and immunohistochemical examination; the latter should be made a panel, not restricted to the use of antibodies S-100 and vimentin only. The prognosis of malignant peripheral nerve sheath tumors (MPNST) of the spinal cord is poor, and although there are palliative methods, there is no curative treatment, as complications can interfere with the quality of life of the animal. MPNST should be included in the differential diagnosis of spinal disorders, even in young dogs and small breeds. CT helps in early diagnosis to make decisions aimed at the animal's well-being. [ABSTRACT FROM AUTHOR]

Spinal schwannomas are benign intradural extramedullary tumors arising from spinal nerve root sheath. They are usually solid or heterogeneously solid. Totally cystic schwannomas are rare entities. Herein, we report a 60-year-old male presenting with backache radiating along the chest wall and weakness of both lower limbs. He had spastic paraparesis. Magnetic resonance imaging revealed a cystic mass in the thoracic region. At operation, the cystic mass was seen to be attached to D4 dorsal rootlets. It was excised in toto and histopathology confirmed it to be a schwannoma. The relevant literature is reviewed. [ABSTRACT FROM AUTHOR]