Cranial nerve and intramedullary spinal malignant peripheral nerve sheath tumor associated with neurofibromatosis-1

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.

Intramedullary Spinal Cord Metastases from Small Cell Lung Cancer: Report of One Case and Review of the Literature

Purpose Spinal cord intramedullary metastasis (ISCM) is a rare malignant tumor of the nervous system. Small cell lung cancer (SCLC) accounts for about 15% of lung cancer, and the incidence of brain metastasis is high, but intramedullary spinal cord metastasis is rare. In recent years, the first-line treatment of patients with ISCM from SCLC has been controversial. For these patients with ISCM, the options are usually depending on the progression of the disease.However,the outcom of treatments are not satisfacted.Thus,We try to indentify the use of immunotherapy in ISCM from SCLC. Methods In our review, we focused on clinical trials of immunotherapy, especially in relation to ISCM in SCLC. Results A patient was diagnosed as ISCM from SCLC. Radiotherapy for ISCM was started on January 11, 2021 Apatinib was taken orally after radiotherapy. The overall survival was almost 8 months, and there was only 1 month after ISCM was diagnosed. Conclusion Finding practical treatment options for SCLC is an important goal. Previous trials have shown that immunotherapy with checkpoint inhibitors may be an effective approach for long-term disease control and a new breakthrough in the treatment of ISCM form SCLC.

Magnetic resonance imaging findings and antemortem cytologic diagnosis of intramedullary spinal cord ependymoma in a dog

CASE DESCRIPTION A 3-year-old 31.1-kg castrated male mixed-breed dog was evaluated because of a 1- to 2-week history of paraparesis, knuckling of the hind feet, and difficulty posturing to urinate or defecate. CLINICAL FINDINGS The dog was paraparetic but weakly ambulatory with a kyphotic posture, a mildly decreased patellar reflex in the right pelvic limb, increased tone in both pelvic limbs, and marked hyperesthesia on paraspinal palpation of the lumbar region. The urinary bladder was enlarged and firm on palpation. Neuroanatomic findings were primarily consistent with localization to the T3-L3 spinal cord segments. Magenetic resonance imaging of the thoracolumbar spinal column revealed a discrete intramedullary spinal cord mass from the cranial aspect of L4 to the middle of L5. The mass was sampled by fine-needle aspiration, and on cytologic evaluation, the suspected diagnosis was an ependymoma. TREATMENT AND OUTCOME Owing to poor prognosis and limited treatment options, the owner elected euthanasia. Postmortem examination of the spinal cord and histologic findings for samples of the mass supported a likely diagnosis of ependymoma. CLINICAL RELEVANCE Ependymoma is a rare neoplasm in dogs but should be considered in young patients with evidence of a tumor in the CNS. Fine-needle aspiration of the spinal cord mass was possible in the dog of this report, and the cytologic findings provided useful diagnostic information.

Distinguishing Intramedullary Spinal Cord Neoplasms from Non-Neoplastic Conditions by Analyzing the Classic Signs on MRI in the Era of AI

: Intramedullary lesions can be challenging to diagnose given the wide range of possible pathologies. Each lesion has unique clinical and imaging features, which are best evaluated on magnetic resonance imaging. Radiological imaging is unique with rich, descriptive patterns and classic signs—which are often metaphorical. In this review, we present a collection of classic MRI signs, ranging from neoplastic to non-neoplastic lesions, within the spinal cord. The differential diagnosis (DD) of intramedullary lesions can be narrowed down by careful analysis of the classic signs and pattern of involvement in the spinal cord. Furthermore, the signs are illustrated memorably with emphasis on the pathophysiology, mimics and pitfalls. Artificial intelligence (AI) algorithms, particularly deep learning, have made remarkable progress in image recognition tasks. The classic signs and related illustrations can enhance a pattern recognition approach in diagnostic radiology. Deep learning can potentially be designed to distinguish neoplastic from non-neoplastic processes by pattern recognition of the classic MRI signs.

Surgery for intramedullary spinal cord ependymomas in the neuromonitoring era: results from a consecutive series of 100 patients

OBJECTIVE The established treatment of intramedullary spinal cord ependymomas (ISCEs) is resection. Surgical series reporting treatment results often lack homogeneity, as these are collected over long time spans and their analysis is plagued by surgical learning curves and inconsistent use of intraoperative neurophysiological monitoring (IONM). The authors report the oncological and functional long-term outcomes in a modern series of 100 consecutive ISCEs that were resected between 2000 and 2015 by a surgically experienced team that consistently utilized IONM. METHODS In this retrospective study, the authors tailored surgical strategy and multimodal IONM, including somatosensory evoked potentials, muscle motor evoked potentials (mMEPs), and D-waves, with the aim of gross-total resection (GTR). Preservation of the D-wave was the primary objective, and preservation of mMEPs was the second functional objective. Functional status was evaluated using the modified McCormick Scale (MMS) preoperatively, postoperatively, and at follow-up. RESULTS Preoperatively, 89 patients were functionally independent (MMS grade I or II). A GTR was achieved in 89 patients, 10 patients had a stable residual, and 1 patient underwent reoperation for tumor progression. At a mean follow-up of 65.4 months, 82 patients were functionally independent, and 11 lost their functional independence after surgery (MMS grades III–V). Muscle MEP loss predicted short-term postoperative worsening (p < 0.0001) only, while the strongest predictors of a good functional long-term outcome were lower preoperative MMS grades (p < 0.0001) and D-wave preservation. D-wave monitorability was 67%; it was higher with lower preoperative MMS grades and predicted a better recovery (p = 0.01). CONCLUSIONS In this large series of ISCEs, a high rate of GTR and long-term favorable functional outcome were achieved. Short- and long-term functional outcomes were best reflected by mMEPs and D-wave monitoring, respectively.

Non-dysraphic Subpial Intramedullary Lipoma of Cervicothoracic Spine

Intramedullary spinal cord tumors are rare and comprise less than one percentage of all central nervous system (CNS) neoplasms. When encountered, most are found in the lumbosacral region with coinciding spinal dysraphism. Here, we discuss a case of isolated non-dysraphic intramedullary lipoma of cervicothoracic spine in an adolescent who was surgically managed with posterior decompression and subtotal resection via laminoplasty. A 21-year-old male adolescent, without a history of spinal dysraphism, presented with a progressive sensory ataxia. This manifestation was attributed to magnetic resonance documented intramedullary lipoma, extending from C7 to T2 spinal level. Patient underwent subtotal surgical resection of the lesion; and histopathology report was confirmed as spinal lipoma. Surgical management of this rare pathology has a wide variety of options depending on clinical presentation. In our case, a subtotal resection and laminoplasty was achieved with no further worsening of neurologic symptoms.

Lentiviral-Induced Spinal Cord Gliomas in Rat Model

Intramedullary spinal cord tumors are a rare and understudied cancer with poor treatment options and prognosis. Our prior study used a combination of PDGF-B, HRAS, and p53 knockdown to induce the development of high-grade glioma in the spinal cords of minipigs. In this study, we evaluate the ability of each vector alone and combinations of vectors to produce high-grade spinal cord gliomas. Eight groups of rats (n = 8/group) underwent thoracolumbar laminectomy and injection of lentiviral vector in the lateral white matter of the spinal cord. Each group received a different combination of lentiviral vectors expressing PDGF-B, a constitutively active HRAS mutant, or shRNA targeting p53, or a control vector. All animals were monitored once per week for clinical deficits for 98 days. Tissues were harvested and analyzed using hematoxylin and eosin (H&E) and immunohistochemical (IHC) staining. Rats injected with PDGF-B+HRAS+sh-p53 (triple cocktail) exhibited statistically significant declines in all behavioral measures (Basso Beattie Bresnahan scoring, Tarlov scoring, weight, and survival rate) over time when compared to the control. Histologically, all groups except the control and those injected with sh-p53 displayed the development of tumors at the injection site, although there were differences in the rate of tumor growth and the histopathological features of the lesions between groups. Examination of immunohistochemistry revealed rats receiving triple cocktail displayed the largest and most significant increase in the Ki67 proliferation index and GFAP positivity than any other group. PDGF-B+HRAS also displayed a significant increase in the Ki67 proliferation index. Rats receiving PDGF-B alone and PDGF-B+ sh-p53 displayed more a significant increase in SOX2-positive staining than in any other group. We found that different vector combinations produced differing high-grade glioma models in rodents. The combination of all three vectors produced a model of high-grade glioma more efficiently and aggressively with respect to behavioral, physiological, and histological characteristics than the rest of the vector combinations. Thus, the present rat model of spinal cord glioma may potentially be used to evaluate therapeutic strategies in the future.