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Journal of Vitreoretinal Diseases 2023To report a case of painless posterior scleritis presenting as a choroidal nodule in a patient with history of a tumor being treated with pembrolizumab. A case and its...
To report a case of painless posterior scleritis presenting as a choroidal nodule in a patient with history of a tumor being treated with pembrolizumab. A case and its findings were analyzed, and a relevant literature review was performed. : A 20-year-old woman with a history of ependymoma presented with painless blurred vision in the right eye after being started on pembrolizumab for a tumor recurrence. Fundoscopy showed a solitary amelanotic choroidal lesion with surrounding subretinal fluid in the affected eye. Ultrasonography showed moderate internal reflectivity and fluid in Tenon capsule consistent with nodular posterior scleritis. After a course of systemic steroids and discontinuation of the pembrolizumab, the choroidal lesion completely resolved. : Clinicians should be aware of posterior scleritis as an ocular complication of this class of medications.
PubMed: 37927317
DOI: 10.1177/24741264231163396 -
Annals of Medicine and Surgery (2012) Nov 2023Ependymomas are central nervous system tumors arising from the ependymal lining of the ventricle and spinal cord. Supratentorial extra-axial ependymomas are very rare,...
INTRODUCTION AND IMPORTANCE
Ependymomas are central nervous system tumors arising from the ependymal lining of the ventricle and spinal cord. Supratentorial extra-axial ependymomas are very rare, most commonly affecting the pediatric population and rarely in adults.
CASE PRESENTATION
The authors report a case of a 71-year-old female with a headache and blurred vision. An MRI scan revealed a lesion at the parafalcine region of the occipital lobe. A parieto-occipital craniotomy was performed. When the dura was opened during the operation, the extra-axially located, well-circumscribed, dirty yellow-white tumor dissected from the surrounding tissue was excised entirely by microdissection. Histopathological examination revealed supratentorial extra-axial anaplastic ependymoma. The patient received postoperative radiation therapy (54 Gray over 30 fractions). No recurrence of the tumor was observed during the 4-year follow-up.
DISCUSSION
Supratentorial ependymomas at the extra-axial region are uncommon; extra-axial anaplastic ependymoma and meningiomas have similar radiological findings, such as a dural tail, subarachnoid plane, and diffuse enhancement after contrast injection. This close similarity might cause misdiagnoses. Total surgical resection was followed by adjuvant radiotherapy and close follow-up in the gold standard of the treatment.
CONCLUSION
The authors report a rare case of anaplastic ependymomas located at the extra-axial region. Anaplastic tumors are prone to recurrence despite total resection and radiation therapy; hence, a close follow-up is warranted.
PubMed: 37915672
DOI: 10.1097/MS9.0000000000000536 -
World Journal of Clinical Cases Oct 2023Malignant proliferating trichilemmal tumor (MPTT) is an infrequent malignant neoplasm originating from cutaneous appendages, with only a handful of documented cases....
BACKGROUND
Malignant proliferating trichilemmal tumor (MPTT) is an infrequent malignant neoplasm originating from cutaneous appendages, with only a handful of documented cases. This report delineates a unique instance of MPTT situated in the neck, accompanied by lymph node metastasis. A comprehensive exposition of its clinical trajectory and imaging manifestation is presented, aiming to enhance comprehension and management of this atypical ailment.
CASE SUMMARY
Patient concerns: A 79-year-old male presented with a longstanding right neck mass persisting for over six decades, exhibiting recent enlargement over the past year. Diagnoses: Enhanced magnetic resonance imaging of the neck unveiled an elliptical mass on the right neck side, characterized by an ill-defined border and a heterogeneous signal pattern. The mass exhibited subdued signal intensity on T1-weighted imaging (T1WI) and a heterogeneous high signal on T2-weighted imaging (T2WI), interspersed with a lengthy T1 and T2 cystic signal motif. Close anatomical association with the submandibular gland joint was noted, and intravenous gadolinium diethylene triamine pentaacetic acid administration facilitated conspicuous enhancement. Substantial enhancement of the solid segment prompted an initial preoperative diagnosis of malignant nerve sheath tumor. However, post-surgery histopathological and immunohistochemical analysis conclusively confirmed the diagnosis as malignant hyperplastic external hair root sheath tumor. Intervention: Complete excision of the tumor was successfully executed. Outcomes: The patient experienced a favorable postoperative recovery.
CONCLUSION
Malignant proliferative trichilemmal tumor external hair root sheath tumor is a cystic-solid lesion, appearing as low signal on T1WI images or high signal on T2WI with enhancement of the solid component. Suspicions of malignancy are heightened when the tumor border is indistinct, tissue planes are breached, or when linear or patchy high signals are observed in the subcutaneous tissue on T1 liver acquisition with volume acceleration enhanced images along with intermediate signal on T2WI and restricted diffusion on diffusion-weighted imaging images. Strong consideration for malignancy should arise if there are signs of compromised adjacent tissue relationships or direct invasion evident on imaging. We have incorporated the above-mentioned content into the entire manuscript.
PubMed: 37901030
DOI: 10.12998/wjcc.v11.i28.6949 -
Medicina (Kaunas, Lithuania) Sep 2023Intramedullary spinal cord tumors (IMSCT) are rare entities. A location in the upper cervical spine as a highly eloquent region carries the risk of postoperative...
Intramedullary spinal cord tumors (IMSCT) are rare entities. A location in the upper cervical spine as a highly eloquent region carries the risk of postoperative neurological deficits, such as tetraparesis or respiratory dysfunction. Evidence for respiratory dysfunction is scarce. This study aimed to describe these highly eloquent tumors' early and late postoperative clinical course. This is a single-center retrospective cohort study. We included 35 patients with IMSCT at levels of the craniocervical junction to C4 who underwent surgical treatment between 2008 and 2022. The authors analyzed the patients' preoperative status, tumor- and surgery-specific characteristics, and follow-up functional status. The study cohort included twenty-two patients with grade II ependymoma (62.9%), two low-grade astrocytomas (5.7%), two glioblastomas (5.7%), six hemangioblastomas (17.1%), two metastases (5.7%), and one patient with partially intramedullary schwannoma (2.9%). Gross total resection was achieved in 76% of patients. Early dorsal column-related symptoms (gait ataxia and sensory loss) and motor deterioration occurred in 64% and 44% of patients. At a follow-up of 3.27 ± 3.83 years, 43% and 33% of patients still exhibited postoperative sensory and motor deterioration, respectively. The median McCormick Scale grade was 2 in the preoperative and late postoperative periods, respectively. Only three patients (8.6%) developed respiratory dysfunction, of whom, two patients, both with malignant IMSCT, required prolonged invasive ventilation. More than 60% of the patients with IMSCT in the upper cervical cord developed new neurological deficits in the immediate postoperative period, and more than 40% are permanent. However, these deficits are not disabling in most cases since most patients maintain functional independence as observed by unchanged low McCormick scores. The rate of respiratory insufficiency is relatively low and seems to be influenced by the rapid neurological deterioration in high-grade tumors.
Topics: Humans; Treatment Outcome; Neurosurgical Procedures; Retrospective Studies; Spinal Cord Neoplasms; Cervical Vertebrae; Respiratory Insufficiency
PubMed: 37893472
DOI: 10.3390/medicina59101754 -
Journal of the Korean Society of... Sep 2023Distinguishing intradural extramedullary (IDEM) spinal ependymoma from myxopapillary ependymoma is challenging due to the location of IDEM spinal ependymoma. This study...
PURPOSE
Distinguishing intradural extramedullary (IDEM) spinal ependymoma from myxopapillary ependymoma is challenging due to the location of IDEM spinal ependymoma. This study aimed to investigate the utility of clinical and MR imaging features for differentiating between IDEM spinal and myxopapillary ependymomas.
MATERIALS AND METHODS
We compared tumor size, longitudinal/axial location, enhancement degree/pattern, tumor margin, signal intensity (SI) of the tumor on T2-weighted images and T1-weighted image (T1WI), increased cerebrospinal fluid (CSF) SI caudal to the tumor on T1WI, and CSF dissemination of pathologically confirmed 12 IDEM spinal and 10 myxopapillary ependymomas. Furthermore, classification and regression tree (CART) was performed to identify the clinical and MR features for differentiating between IDEM spinal and myxopapillary ependymomas.
RESULTS
Patients with IDEM spinal ependymomas were older than those with myxopapillary ependymomas (48 years vs. 29.5 years, < 0.05). A high SI of the tumor on T1W1 was more frequently observed in IDEM spinal ependymomas than in myxopapillary ependymomas ( = 0.02). Conversely, myxopapillary ependymomas show CSF dissemination. Increased CSF SI caudal to the tumor on T1WI was observed more frequently in myxopapillary ependymomas than in IDEM spinal ependymomas ( < 0.05). Dissemination to the CSF space and increased CSF SI caudal to the tumor on T1WI were the most important variables in CART analysis.
CONCLUSION
Clinical and radiological variables may help differentiate between IDEM spinal and myxopapillary ependymomas.
PubMed: 37869110
DOI: 10.3348/jksr.2022.0122 -
BMJ Case Reports Oct 2023Posterior fossa ependymomas (PFEs) are designated histologically as low-grade neoplasms. Despite being characterised as benign, cases of metastasis have been reported...
Posterior fossa ependymomas (PFEs) are designated histologically as low-grade neoplasms. Despite being characterised as benign, cases of metastasis have been reported only a few times with the patients concurrently diagnosed with the primary tumour. Interval drop metastasis or spontaneous second distal tumours are extremely rare and, in most cases, are diagnosed within a few months of primary tumour resection. Here, we report a patient with a grade 2 paediatric PFE exhibiting a 20-year interval to a second sacral ependymoma. The patient was initially diagnosed with a PFE at the age of 10 years and underwent tumour resection and postoperative radiotherapy. In their late 20s, the patient presented with basilar artery occlusion complicated by life-threatening epistaxis. Post-thrombolysis, the patient presented with a large sacral grade 1 myxopapillary ependymoma with cauda equina syndrome-like symptoms. Here, we present a rare case of two ependymomas with a 20-year interval in the same patient with compounding comorbidities.
Topics: Humans; Child; Ependymoma; Magnetic Resonance Imaging; Neoplasm Recurrence, Local; Spinal Cord Neoplasms
PubMed: 37857539
DOI: 10.1136/bcr-2023-256611 -
Neurosurgical Focus: Video Oct 2023Although resection is the gold standard treatment for spinal ependymoma, permanent neurological deterioration has been reported postoperatively in 20%-27% of patients....
The precise midline myelotomy through anatomical posterior median septum by dissecting dorsal column in microsurgical resection of ependymoma (2-dimensional operative video).
Although resection is the gold standard treatment for spinal ependymoma, permanent neurological deterioration has been reported postoperatively in 20%-27% of patients. Despite thorough dissection of the tumor from its surroundings, conventional longitudinally directed midline myelotomy can lead to injury to the dorsal column, possibly due to deformation of the posterior median septum as the tumor grows. To address this issue, the authors have been performing precise midline myelotomy through the anatomical posterior median septum by directly dissecting the dorsal column. This video presents the principles and application of this technique.
PubMed: 37854658
DOI: 10.3171/2023.7.FOCVID2317 -
Neurosurgical Focus: Video Oct 2023Spinal cord ependymomas comprise 25% of all intramedullary tumors and are typically treated with resection. A man in his mid-60s presented with imbalance and sensory...
Spinal cord ependymomas comprise 25% of all intramedullary tumors and are typically treated with resection. A man in his mid-60s presented with imbalance and sensory deficits in both lower extremities, and a spinal thoracic intramedullary ependymoma spanning the levels T2 and T3 was diagnosed. After a laminectomy was performed, the tumor was microsurgically resected, and the patient demonstrated no neurological deficits on postoperative examination. Subsequent MRI showed complete resection of the tumor. This video showcases a thoracic intramedullary ependymoma resected using careful microdissection into the median raphe as a safe entry zone to preserve neurological function.
PubMed: 37854657
DOI: 10.3171/2023.6.FOCVID2386 -
Neurosurgical Focus: Video Oct 2023Because the spinal cord contains a rich concentration of longitudinal and transversal fibers in a very small area, intramedullary surgery could result in a high...
Because the spinal cord contains a rich concentration of longitudinal and transversal fibers in a very small area, intramedullary surgery could result in a high likelihood of morbidity. In this video, the authors demonstrate the microsurgical technique and surgical skills used to perform excision of an intramedullary ependyma. The authors also present tools (electrophysiology and neuroimaging) that are useful for surgical decision-making and planning, and thus are used intraoperatively, that allow safer and more effective resection of an intramedullary tumor.
PubMed: 37854656
DOI: 10.3171/2023.7.FOCVID2368 -
Neurosurgical Focus: Video Oct 2023In this video, the authors present the resection of a large thoracolumbar intradural ependymoma in a 33-year-old female. The patient underwent T9-L3 laminectomies,...
In this video, the authors present the resection of a large thoracolumbar intradural ependymoma in a 33-year-old female. The patient underwent T9-L3 laminectomies, intradural tumor resection, and posterior instrumented fixation and fusion. The surgical procedure aimed to relieve the mass effect, obtain a diagnosis, prevent further neurological decline, and achieve a potential curative resection. The pathology confirmed a myxopapillary ependymoma, a rare tumor with a preference for the conus medullaris, cauda equina, or filum terminale. The video provides insights into the case, surgical steps, clinical outcomes, and background information on myxopapillary ependymomas and treatment options.
PubMed: 37854653
DOI: 10.3171/2023.7.FOCVID2378