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Ugeskrift For Laeger Nov 2015Subcutaneous myxopapillary ependymoma is a very rare entity, and to our knowledge this is the first published case from Denmark. A previously healthy 32-year-old male...
Subcutaneous myxopapillary ependymoma is a very rare entity, and to our knowledge this is the first published case from Denmark. A previously healthy 32-year-old male presented with subcutaneous swelling and tenderness located at the top of the intergluteal cleft. The circular soft tumour, measuring 1.7 × 1.7 × 1.2 cm, was removed surgically. After histopathological examination with several immunohistochemical and special stainings, the diagnosis surprisingly was subcutaneous myxopapillary ependymoma. The tumour was removed with free margins and no metastases were found on follow-up CT- and MRI scans.
Topics: Adult; Ependymoma; Humans; Male; Sacrococcygeal Region
PubMed: 26651556
DOI: No ID Found -
Current Opinion in Oncology Nov 2017To synthesize, integrate, and comment on recent research developments to our understanding of the molecular basis of ependymoma (EPN), and to place this in context with... (Review)
Review
PURPOSE OF REVIEW
To synthesize, integrate, and comment on recent research developments to our understanding of the molecular basis of ependymoma (EPN), and to place this in context with current treatment and research efforts.
RECENT FINDINGS
Our recent understanding of the histologically defined molecular entity EPN has rapidly advanced through genomic, transcriptomic, and epigenomic profiling studies.
SUMMARY
These advancements lay the groundwork for development of future EPN biomarkers, models, and therapeutics. Our review discusses these discoveries and their impact on our clinical understanding of this disease. Lastly, we offer insight into clinical and research areas requiring further validation, and open questions remaining in the field.
Topics: Animals; Central Nervous System Neoplasms; Ependymoma; Humans
PubMed: 28885433
DOI: 10.1097/CCO.0000000000000411 -
Journal of Neuro-oncology Sep 2011We report a case of anaplastic ependymoma with extracranial metastases in a 22-year-old female. The patient originally presented with headaches and dysarthria....
We report a case of anaplastic ependymoma with extracranial metastases in a 22-year-old female. The patient originally presented with headaches and dysarthria. Neuroimaging revealed a large solid and cystic right fronto-temporal lesion. It was located completely extraventricularly and a glioblastoma was suspected based on the neuroimaging findings. A gross total resection was achieved. Histopathologic examination revealed an anaplastic ependymoma. The patient was treated with radiotherapy. Approximately 1 year after the initial surgery, the patient presented with metastatic disease to the scalp. At 2 years, an intraparotid metastasis was detected. Subsequent neck dissection revealed positive lymph nodes at several levels. It was followed by radiotherapy to the neck. 5 years after the initial surgery, the patient has residual metastatic disease. The case is discussed and the literature on extraventricular ependymal neoplasms is reviewed.
Topics: Brain Neoplasms; Ependymoma; Female; Head and Neck Neoplasms; Humans; Scalp; Young Adult
PubMed: 21222217
DOI: 10.1007/s11060-010-0525-x -
International Journal of Clinical and... 2012Ependymomas constitute the most common type of primary spinal cord tumors, and are subclassified as myxopapillary ependymoma, classic ependymoma, and anaplastic... (Review)
Review
Ependymomas constitute the most common type of primary spinal cord tumors, and are subclassified as myxopapillary ependymoma, classic ependymoma, and anaplastic ependymoma. Ependymomas can be further subclassified based on morphologic phenotype: cellular, papillary, tanycytic, clear cell, pigmented and epithelioid. Giant cell ependymoma (GCE), a rare variant, has recently been described. Reported cases have exhibited a wide anatomic distribution, including spinal cord, cerebrum and cerebellum. We report here three cases of GCE, arising from cerebrum in a 5-year-old girl, spinal cord in a 34-year-old female and cerebellum in an 86-year-old female respectively. Histologically those cases showed prominent pleomorphic giant cells with focal perivascular pseudorosettes in all cases. Tumor cells were immunopositive for GFAP and EMA. Only the first case was qualified for anaplastic ependymoma. No recurrence was noted in these three cases after 57, 46 and 6 months of follow-up respectively. By reviewing the literature, GCEs arising from spinal cord and cerebellum tended to have low-grade morphology while supratentorially located GCEs tended to have anaplastic features. GCEs were preferentially located in extraventricular regions. Anaplastic GCEs in adult population seemed to pursue a more aggressive behavior. Gross total resection should still be the main treatment for GCEs.
Topics: Adult; Aged, 80 and over; Biomarkers, Tumor; Cerebellar Neoplasms; Cerebrum; Child, Preschool; Ependymoma; Female; Giant Cells; Humans; Spinal Cord Neoplasms; Treatment Outcome
PubMed: 22808300
DOI: No ID Found -
Pediatric Blood & Cancer Mar 2016A 3-year-old boy with sickle cell anemia (SCA) presented with progressive daily emesis and was found to have an anaplastic ependymoma. Radiation therapy and chemotherapy...
Anaplastic Ependymoma in a Child With Sickle Cell Anemia: A Case Report Highlighting Treatment Challenges for Young Children With Central Nervous System Tumors and Underlying Vasculopathy.
A 3-year-old boy with sickle cell anemia (SCA) presented with progressive daily emesis and was found to have an anaplastic ependymoma. Radiation therapy and chemotherapy are usually employed after subtotal resections of anaplastic ependymomas, although the benefits from chemotherapy are unclear. To mitigate the risks of adjuvant treatment in this patient at risk for SCA-associated vasculopathy, renal impairment, and other end-organ damage, proton beam irradiation without chemotherapy was chosen. Scheduled packed red blood cell transfusions were instituted to maintain sickle hemoglobin levels less than 30%. This case highlights treatment complexities for malignant brain tumors in patients predisposed to treatment-related adverse effects.
Topics: Anemia, Sickle Cell; Brain Neoplasms; Child, Preschool; Ependymoma; Humans; Magnetic Resonance Imaging; Male
PubMed: 26488903
DOI: 10.1002/pbc.25809 -
Pediatric Blood & Cancer Jan 2016The benefit of postoperative chemotherapy for anaplastic ependymoma remains unknown. We report two pediatric patients with refractory anaplastic ependymoma treated with...
The benefit of postoperative chemotherapy for anaplastic ependymoma remains unknown. We report two pediatric patients with refractory anaplastic ependymoma treated with temozolomide (TMZ). We did not detect O(6) -methylguanine-DNA methyltransferase (MGMT) promoter methylation in tumor samples; however, MGMT protein expression was low. With TMZ treatment, one patient had a 7-month complete remission; the other, stable disease for 15 months. Three other patients did not respond to TMZ; two had high and one low MGMT expression, and two showed no MGMT promoter methylation. These findings suggest that TMZ may be effective for pediatric refractory anaplastic ependymoma with low MGMT protein expression.
Topics: Antineoplastic Agents, Alkylating; Brain Neoplasms; Child, Preschool; DNA Modification Methylases; DNA Repair Enzymes; Dacarbazine; Ependymoma; Female; Humans; Immunohistochemistry; Infant; Male; Temozolomide; Tumor Suppressor Proteins
PubMed: 26305586
DOI: 10.1002/pbc.25696 -
Neurologia Croatica : Glasilo Udruzenja... 1991During 1988 and 1989, 16 cases of ependymomas were diagnosed at the Department of Pathology, University Hospital "Sestre milosrdnice", Zagreb. The specimens were...
During 1988 and 1989, 16 cases of ependymomas were diagnosed at the Department of Pathology, University Hospital "Sestre milosrdnice", Zagreb. The specimens were obtained from the Department of Neurosurgery of the same hospital. Seven tumors out of these 16 were supratentorial, four of them being diagnosed as anaplastic ependymomas. At the time of the first operation, patients with anaplastic ependymoma were aged 2 to 18 years, and there were three man and one woman. The criteria for this diagnosis were cellular pleomorphism and anaplasia, vascular hyperplasia with marked endothelial proliferation, and the most important of all, the presence of numerous mitoses. Necroses, cystic degenerations and bleeding were found in all four tumors. The aim of this study is the survey of morphology, tumor localisation and patient's age at the moment of diagnosis as relevant factors for the prognosis of these tumors. It seems significant to indicate a relatively great number of these tumors in only a two-year period in one hospital, while statistical data reported in the literature have been taken from several institutions in various towns or states for much longer period of even several decades. Hence, this study could be in favour of the idea presented in the recent literature data, that this tumor type is increasing.
Topics: Adolescent; Central Nervous System Neoplasms; Child; Child, Preschool; Ependymoma; Female; Humans; Male
PubMed: 1810398
DOI: No ID Found -
Experimental and Clinical... Sep 2022Ependymomas are the most common intramedullary spinal tumors in adults and constitute around 20% of all spinal tumors in adults. There are 3 subgroups of ependymomas...
Ependymomas are the most common intramedullary spinal tumors in adults and constitute around 20% of all spinal tumors in adults. There are 3 subgroups of ependymomas according to World Health Organization classification: subependymoma or myxopapillary (grade 1), ependymoma (grade 2), and anaplastic (grade 3). Therapy for patients is aimed at safe and total surgical removal and, in selected cases, postoperative radiotherapy. Bleeding from a spinal ependymoma, with subsequent urgent surgery, is extremely rare. Here, we present a case of a renal transplant patient who had a cervical ependymoma. Although a considerable volume of peritumoral blood was observed during surgery, the patient had no neurologic deficits and no signs of deterioration.
Topics: Adult; Ependymoma; Hemorrhage; Humans; Kidney Transplantation; Neurosurgical Procedures; Spinal Cord Neoplasms; Spinal Neoplasms; Treatment Outcome
PubMed: 30251937
DOI: 10.6002/ect.2017.0283 -
European Radiology Mar 2024Posterior fossa ependymoma group A (EPN_PFA) and group B (EPN_PFB) can be distinguished by their DNA methylation and give rise to different prognoses. We compared the...
OBJECTIVES
Posterior fossa ependymoma group A (EPN_PFA) and group B (EPN_PFB) can be distinguished by their DNA methylation and give rise to different prognoses. We compared the MRI characteristics of EPN_PFA and EPN_PFB at presentation.
METHODS
Preoperative imaging of 68 patients with posterior fossa ependymoma from two centers was reviewed by three independent readers, blinded for histomolecular grouping. Location, tumor extension, tumor volume, hydrocephalus, calcifications, tissue component, enhancement or diffusion signal, and histopathological data (cellular density, calcifications, necrosis, mitoses, vascularization, and microvascular proliferation) were compared between the groups. Categorical data were compared between groups using Fisher's exact tests, and quantitative data using Mann-Whitney tests. We performed a Benjamini-Hochberg correction of the p values to account for multiple tests.
RESULTS
Fifty-six patients were categorized as EPN_PFA and 12 as EPN_PFB, with median ages of 2 and 20 years, respectively (p = 0.0008). The median EPN_PFA tumoral volume was larger (57 vs 29 cm, p = 0.003), with more pronounced hydrocephalus (p = 0.002). EPN_PFA showed an exclusive central position within the 4th ventricle in 61% of patients vs 92% for EPN_PFB (p = 0.01). Intratumor calcifications were found in 93% of EPN_PFA vs 40% of EPN_PFB (p = 0.001). Invasion of the posterior fossa foramina was mostly found for EPN_PFA, particularly the foramina of Luschka (p = 0.0008). EPN_PFA showed whole and homogeneous tumor enhancement in 5% vs 75% of EPN_PFB (p = 0.0008). All mainly cystic tumors were EPN_PFB (p = 0.002). The minimal and maximal relative ADC was slightly lower in EPN_PFA (p = 0.02 and p = 0.01, respectively).
CONCLUSION
Morphological characteristics from imaging differ between posterior fossa ependymoma subtypes and may help to distinguish them preoperatively.
CLINICAL RELEVANCE STATEMENT
This study provides a tool to differentiate between group A and group B ependymomas, which will ultimately allow the therapeutic strategy to be adapted in the early stages of patient management.
KEY POINTS
• Posterior fossa ependymoma subtypes often have different imaging characteristics. • Posterior fossa ependymomas group A are commonly median or lateral tissular calcified masses, with incomplete enhancement, affecting young children and responsible for pronounced hydrocephalus and invasion of the posterior fossa foramina. • Posterior fossa ependymomas group B are commonly median non-calcified masses of adolescents and adults, predominantly cystic, and minimally invasive, with total and homogeneous enhancement.
Topics: Child; Adult; Adolescent; Humans; Child, Preschool; Young Adult; Magnetic Resonance Imaging; Prognosis; Ependymoma; Head; Hydrocephalus
PubMed: 37658900
DOI: 10.1007/s00330-023-10182-5 -
The Journal of Veterinary Medical... Sep 2004An 8-year and 6-month-old female Maltese dog showed a stoop with rigidity of her cervix and back. Neurologic examination showed loss of proprioception, and deficiency of... (Comparative Study)
Comparative Study
An 8-year and 6-month-old female Maltese dog showed a stoop with rigidity of her cervix and back. Neurologic examination showed loss of proprioception, and deficiency of pain response. Postmortem examination revealed the neoplastic mass replacing the central area in the cervical spinal cord at the level from 4th to 5th segments. Histologically, the mass was composed of neoplastic ependymal cells. The neoplastic cells showed marked atypism, and occasionally formed ependymal rosettes. Based on the morphologic features, the tumor was diagnosed as anaplastic ependymoma. Immunohistochemistry showed that the neoplastic cells were negative for glial fibrillary acid protein, and slightly positive for vimentin and cytokeratin.
Topics: Animals; Cervical Vertebrae; Dog Diseases; Dogs; Ependymoma; Fatal Outcome; Female; Immunohistochemistry; Spinal Cord Neoplasms
PubMed: 15472485
DOI: 10.1292/jvms.66.1155