A Rare Case of Intramedullary Lipoma of Brainstem to Thoracic Cord––What to Do?
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ABSTRACT:
SUBMITTER: Bishnoi I
PROVIDER: S-EPMC7519731 | biostudies-literature | 2020 Jan
REPOSITORIES: biostudies-literature
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Project description:Introduction Spinal intradural lipoma is a rare condition, accounting for < 1% of all spinal cord tumors. Spinal cord lipomas are frequently associated with dysraphism and occur in the thoracic spine. Another common finding is that spinal cord lipomas tend to present in the pediatric population. Isolated nondysraphic cervical lipomas are a rare entity. We discuss a case of nondysraphic cervical lipoma with an exophytic component. Case A 31 year-old woman presented with bilateral numbness in her hands and a burning and aching sensation in her arms for ∼ 6 months. The patient did not have any weakness or myelopathic signs. Magnetic resonance imaging T1 showed a T1 hyperintense, T2 hypointense, non-contrast-enhancing mass on the dorsal aspect of the spinal cord with significant compression. The patient underwent a dorsal cervical laminectomy with subtotal resection of an isolated cervical lipoma with an exophytic component. The pathology confirmed the diagnosis of a lipoma. Conclusions Surgical management of this rare pathology has a wide variety of options. Depending on the neurologic deficits, observation to gross total resection may be reasonable options. In our case, a subtotal resection was achieved with no further worsening of neurologic symptoms.
| S-EPMC4520978 | biostudies-other
Project description:43 year-old female presented with anterior hip pain not responding to conservative treatments. Plain radiograph showed radio-lucent area with sclerotic margin in the femoral neck region. On MRI scan the diagnosis of intraosseous lipoma was confirmed. Due to persistent pain and risk of fracture, decision of operative treatment was made. She underwent curettage of the lesion under image-intensifier and reinforcement of femoral neck with non-vascularised fibular grafting. Post-operative x-rays showed incorporation of the fibular graft with good clinical and functional outcome. Intraosseous lipoma in the femoral neck is a rare presentation and in cases with persistent symptoms or risk of impending fracture, operative treatment gives a good functional outcome.
| S-EPMC6823791 | biostudies-literature
Project description:An 85-year-old male referred to the Gastroenterology (GI) clinic with three-month history of failure to thrive and three-week history of nausea, vomiting, and melanotic stools. Ulcerative mass obstructing gastric outlet was found on endoscopy and on follow-up CT abdomen a homogeneous submucosal mass in the gastric antrum was identified. Radiological diagnosis of giant gastric lipoma was established and patient was evaluated for surgery and, however, was rendered unfit for surgery due to his comorbid conditions. Patient was taken for endoscopic resection of the mass. On endoscopy, only partial resection was achieved due to the size of the mass, but endoloops were deployed at the stalk at the end of the procedure in hope of limiting blood supply to the lesion. On six-week follow-up endoscopy, patient's mass had completely disappeared with limited scar tissue at the site.
| S-EPMC6393926 | biostudies-literature
Project description:The internal thoracic artery (ITA) arises from the subclavian artery and terminates as the musculophrenic and inferior epigastric arteries. During routine cadaveric dissection, an aberrant left ITA was discovered. A medial and a lateral branch of the ITA branched directly off the subclavian artery as opposed to bifurcating at the 6th or 7th intercostal space. To our knowledge, this is the first reported case of this particular ITA variation arising from the third part of the subclavian artery. Additionally, such a variant might also be considered a high bifurcation of the ITA. Our report examines this variation and its potential implications for coronary artery bypass grafts where the ITA is commonly used.
| S-EPMC7527130 | biostudies-literature
Project description:Idiopathic spinal cord herniation (ISCH) is an uncommon condition located predominantly in the thoracic spine and often associated with a remote history of a major traumatic injury. ISCH has an incompletely described presentation and unknown etiology. There is no consensus on the treatment algorithm and surgical technique, and there are few data on clinical outcomes.In this case series and technical report, we describe the atypical myelopathy presentation, remote history of traumatic injury, radiographic progression, treatment, and outcomes of 5 patients treated at Washington University for symptomatic ISCH. A video showing surgical repair is presented. In contrast to classic compressive myelopathy symptomatology, ISCH patients presented with an atypical myelopathy, characterized by asymmetric motor and sensory deficits and early-onset urinary incontinence. Clinical deterioration correlated with progressive spinal cord displacement and herniation observed on yearly spinal imaging in a patient imaged serially because of multiple sclerosis. Finally, compared with compressive myelopathy in the thoracic spine, surgical treatment of ISCH led to rapid improvement despite a long duration of symptoms.Symptomatic ISCH presents with atypical myelopathy and slow temporal progression and can be successfully managed with surgical repair.
| S-EPMC4134727 | biostudies-other
Project description:Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms that have limited treatment options and are associated with high rates of morbidity and mortality. To better understand the genetic basis of these tumors we performed whole exome sequencing on 45 tumors and matched germline DNA, including twenty-nine spinal cord ependymomas and sixteen astrocytomas. Though recurrent somatic mutations in IMSCTs were rare, we identified NF2 mutations in 15.7% of tumors (ependymoma, N = 7; astrocytoma, N = 1), RP1 mutations in 5.9% of tumors (ependymoma, N = 3), and ESX1 mutations in 5.9% of tumors (ependymoma, N = 3). We further identified copy number amplifications in CTU1 in 25% of myxopapillary ependymomas. Given the paucity of somatic driver mutations, we further performed whole-genome sequencing of 12 tumors (ependymoma, N = 9; astrocytoma, N = 3). Overall, we observed that IMSCTs with intracranial histologic counterparts (e.g. glioblastoma) did not harbor the canonical mutations associated with their intracranial counterparts. Our findings suggest that the origin of IMSCTs may be distinct from tumors arising within other compartments of the central nervous system and provides the framework to begin more biologically based therapeutic strategies.
| S-EPMC6904446 | biostudies-literature
Project description:Intramedullary spinal cord abscess (ISCA) is a rare clinical pathology of the central nervous system that usually accompanies other underlying comorbidities. Traditionally it has been associated with significant mortality and neurological morbidities because it is often difficult to diagnose promptly, owing to its nonspecific clinical and neuroimaging features. The mortality rate and the outcome of these infections have been improved by the introduction into clinical practice of antibiotics, advanced neuroimaging modalities, and immediate surgery. We report the case of a 65-year-old male patient who presented with a progressive spastic gait and lumbar pain, predominantly in the left leg. An MRI image revealed an expansile intramedullary cystic mass in the thoracic spinal cord, which was initially diagnosed as a spinal tumor. He underwent laminectomy and myelotomy, and eventually the pus was drained from the abscess. The follow-up MRI showed improvement, but the patient's paraplegia persisted. In light of his persistent hypoesthesia and paraplegic gait with developing neuropathic pain, he was readmitted, and an MRI of his lumbar spine revealed multilevel degenerative disease and tethered spinal cord syndrome with compression of the medulla at the L2-L3 level. The patient underwent central flavectomy with bilateral foraminotomy at the L2-L3 level, and the medulla was decompressed. Postoperatively, his neurological symptoms were significantly improved, and he was discharged from hospital on the third day after admission. In support of our case, we systematically reviewed the recent literature and analyzed cases published between 1949 and May 2022, including clinical features, mechanisms of infection, predisposing factors, radiological investigations, microbial etiologies, therapies and their duration, follow-ups, and outcomes. Initial clinical presentation can be misleading, and the diagnosis can be challenging, because this condition is rare and coexists with other spinal diseases. Hence, a high index of suspicion for making an accurate diagnosis and timely intervention is required to preclude mortality and unfavorable outcomes. Our case is a clear example thereof. Long-term follow-up is also essential to monitor for abscess recurrences.
| S-EPMC9457049 | biostudies-literature
Project description:BackgroundIntramedullary spinal cord lesions prove to be a diagnostic challenge due to their non-specific clinical and radiological presentation. There is a preference for empiric medical therapy, given the inherent risks of surgical intervention to the spine. These factors can lead to delay in diagnosis. Primary central nervous system lymphoma is a rare cause and presents with atypical features in the immunosuppressed patient, including a lack of response to steroid therapy.Case descriptionWe present a 64-year-old male patient with underlying sarcoidosis who reported progressive neuropathy with imaging showing a spinal cord lesion. Based on the above, multiple courses of empiric therapy were employed, including systemic steroids, chemotherapy and immunotherapy. Despite this, there was further clinical deterioration and interim imaging showed disease progression. The decision was made to perform open biopsy of the spinal cord lesion to aid diagnosis. Histological analysis diagnosed Epstein-Barr virus (EBV)-positive high grade large B-cell lymphoma. The patient received rituximab and methotrexate with radiological response but no clinical benefit. He continued to suffer treatment-related complications including encephalopathy and recurrent infections which eventually lead to death.ConclusionsPrimary central nervous system lymphoma is an aggressive disease and failure to respond to empiric treatment should prompt clinician's to consider biopsy for definitive diagnosis. A lack of response to steroids does not exclude lymphoma.
| S-EPMC9929664 | biostudies-literature
Project description:Background: Split cord malformations (SCMs) are uncommon congenital anomalies. They can be divided into Type 1 (bony septum and two separate dural sheaths) and Type 2 (fibrous septum and a single dural sheath).[1,2,4] Rarely,SCM can be associated with orthopedic anomalies (mostly seen in older children), such as scoliosis, followed by kyphosis, and/or both.[1-3] Conversely, patients with congenital scoliosis have 40% risk of congenital underlying neural deformities, with 16.3% attributed to SCM.[1,4] Those with thoracic or lumbosacral SCM may have congenital tethering lesions, predominantly consisting of intradural lipomas.[3,4] Surgery is optimally performed in two stages: first, removal of the spinal septum and untethering of the cord, and second, correction of the kyphosis, scoliosis, and/or both.[1-5]Case Description: In this video, a 44-year-old female initially presented after having progressively developed kyphoscoliosis since childhood. Three months before presentation, she had developed increased left lower extremity pain with hypoesthesia followed 1 month later by the onset of the left lower extremity monoplegia (0/5). The computed tomography and magnetic resonance imaging studies revealed a SCM at the L3 level with bony and membranous septum, accompanied by an intradural lipoma, and tethered cord. She underwent a two- stage procedure; first, removal of the bony and membranous septum, resection of the intradural lipoma, and untethering of the cord; second, she had correction of the kyphoscoliosis. Postoperatively, although the pain and sensory deficits improved, the left lower extremity monoplegia remained.Conclusion: SCM is a rare cause of spinal deformity. If left untreated, the associated neurological deficits may progress. Treatment should include a two-staged approach; first, the bony and membranous septum should be removed followed by lipoma resection and untethering the cord with adequate cord decompression, while second, a fusion may be performed to address attendant kyphoscoliosis.Keywords: Intradural lipoma, Kyphoscoliosis, Split cord malformation, Tethered cord
| S-EPMC7332706 | biostudies-literature
Project description:Study Design Case report. Objective Malignant mesothelioma (MM) is an uncommon tumor of the pleural epithelium with a predilection for local spread into adjacent tissues. The sarcomatoid type accounts for ∼10% of MM cases and is associated with poorer survival than the epithelioid, desmoplastic, and biphasic types. MM commonly presents with involvement of the vertebral body or epidural space. However, intradural spinal extension of MM is extremely rare. Only eight cases of intradural spinal extension have been reported. We report this rare case and discuss the clinical manifestations of intradural spinal extension of MM with literature review. Methods This report describes the case of a 62-year-old man with Brown-Séquard syndrome and radiculopathy of the left C5 nerve root detected during treatment for pleural sarcomatoid MM. Magnetic resonance imaging (MRI) showed an intramedullary lesion at the C3 level and a small nodule at the left C5 nerve root with cervical canal stenosis. Results The patient underwent surgery, and intramedullary metastasis of sarcomatoid MM was diagnosed. Subsequently, radiotherapy was administered, resulting in temporary improvement of the patient's condition. Thereafter, his condition gradually deteriorated, and follow-up MRI showed a more extensive residual C3 intramedullary lesion. Thus, a second surgery was performed after chemotherapy, but the patient died 5 months after the initial diagnosis. Conclusion We present this rare case, and emphasize intramedullary spinal cord metastasis of MM as differential diagnosis in primary cord lesion.
| S-EPMC4078123 | biostudies-other