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Journal of Neuro-oncology Feb 2012Pediatric infratentorial ependymomas are difficult to cure. Despite the availability of advanced therapeutic modalities for brain tumors, total surgical resection...
Pediatric infratentorial ependymomas are difficult to cure. Despite the availability of advanced therapeutic modalities for brain tumors, total surgical resection remains the most important prognostic factor. Recently, histological grade emerged as an independent prognostic factor for intracranial ependymoma. We retrospectively reviewed the treatment outcome of 33 pediatric patients with infratentorial ependymoma. Progression-free survival (PFS) and overall survival (OS) rates were calculated and relevant prognostic factors were analyzed. Fourteen patients (42%) were under the age of 3 at diagnosis. Gross total resection was achieved in 16 patients (49%). Anaplastic histology was found in 13 patients (39%). Adjuvant therapies were delayed until progression in 12 patients (36%). Actuarial PFS rates were 64% in the first year and 29% in the fifth year. Actuarial OS rates were 91% in the first year and 71% in the fifth year. On univariate analysis, brainstem invasion (P = 0.047), anaplastic histology (P = 0.004), higher mitotic count (P = 0.001), and higher Ki-67 index (P = 0.004) were significantly related to a shorter PFS. Gross total resection (P = 0.029) and a greater age at diagnosis (P = 0.033) were significantly related to a longer PFS. On multivariate analysis, anaplastic histology alone was significantly related to a shorter PFS (P = 0.023). Gross total resection (P = 0.039) was significantly related to a longer overall survival (OS) on multivariate analysis. Anaplastic histology and gross total resection were the most important clinical factors affecting PFS and OS, respectively. Anaplastic histology, mitotic count, and Ki-67 index can be used as universal and easily available prognostic parameters in infratentorial ependymomas.
Topics: Brain Neoplasms; Child; Child, Preschool; Disease Progression; Electronic Health Records; Ependymoma; Female; Follow-Up Studies; Humans; Infant; Infratentorial Neoplasms; Ki-67 Antigen; Male; Proportional Hazards Models; Retrospective Studies; Survival Analysis
PubMed: 21863401
DOI: 10.1007/s11060-011-0699-x -
Spinal Cord Series and Cases Jan 2021Of the 23 cases of spinal intradural-extramedullary ependymomas which have been reported to date, 11 were diagnosed as anaplastic. Here we present a very rare case of a...
INTRODUCTION
Of the 23 cases of spinal intradural-extramedullary ependymomas which have been reported to date, 11 were diagnosed as anaplastic. Here we present a very rare case of a thoracic intradural-extramedullary (not intramedullary) anaplastic ependymoma in an adult along with a literature review.
CASE PRESENTATION
A 29-year-old man presented with rapidly progressive gait disturbance, a sensory-deficit below the trunk and urination disorders that had begun a few months earlier. Magnetic resonance imaging of his thoracic spine revealed a dorsal-located intradural-extramedullary tumor at T4-5. The rapid deterioration of his symptoms within several months led him to refer to our department for surgery. Within one month the size of tumor increased to involve the T4-6 level, consequently worsening his gait disturbance. He underwent surgery and tumor mass was resected. However, there was leptomeningeal dissemination of the tumor cells on the surface of cord. A near-total resection was therefore achieved. Histopathology revealed the resected specimen had immunoreactivity for EMA/Vimentin/CD56/CD99/S-100/GFAP, with a Ki-67 index of ~35%. These factors led to the diagnosis of anaplastic ependymoma. Seven weeks postoperatively he received adjuvant radiotherapy to the whole brain and the whole spinal cord. He recovered as an independent ambulator without recurrence 1 year postoperatively.
DISCUSSION
Because of their rarity, there are no clear treatment or adjuvant therapy guidelines for spinal anaplastic ependymoma. Adjuvant radiotherapy to the whole brain and spinal cord was necessarily indicated after near-total resection. Although the patient's condition has not recurred 1 year after surgery, careful and serial follow-up is necessary for this individual.
Topics: Adult; Ependymoma; Humans; Magnetic Resonance Imaging; Male; Radiotherapy, Adjuvant; Spinal Cord Neoplasms; Spine
PubMed: 33468988
DOI: 10.1038/s41394-020-00367-1 -
Acta Neuropathologica Feb 2020According to the WHO classification, ependymal tumors are classified as subependymomas, myxopapillary ependymomas, classic ependymomas, anaplastic ependymomas, and...
According to the WHO classification, ependymal tumors are classified as subependymomas, myxopapillary ependymomas, classic ependymomas, anaplastic ependymomas, and RELA-fusion-positive ependymomas (RELA-EPN). Among classic ependymomas, the WHO defines rare histological variants, i.e., the clear cell, papillary, and tanycytic ependymoma. In parallel, global DNA methylation patterns distinguish nine molecular groups, some of which tightly overlap with histopathological subgroups. However, the match of the aforementioned histological variants to DNA methylation classes remains unclear. We analyzed histomorphology, clinical parameters, and global DNA methylation of tumors with the initial histological diagnoses of tanycytic (n = 12), clear cell (n = 14), or papillary ependymoma (n = 19). Forty percent of these tumors did not match to the epigenetic profile of ependymomas, using a previously published DNA methylation-based classifier for brain tumors. Instead, they were classified as low-grade glioma (n = 3), plexus tumor (n = 2), CNS high-grade neuroepithelial tumor with MN1 alteration (n = 2), papillary tumor of the pineal region (n = 2), neurocytoma (n = 1), or did not match to any known brain tumor methylation class (n = 8). Overall, integrated diagnosis had to be changed in 35.6% of cases as compared to the initial diagnosis. Among the tumors molecularly classified as ependymoma (27/45 cases), tanycytic ependymomas were mostly located in the spine (5/7 cases) and matched to spinal or myxopapillary ependymoma. 6/8 clear cell ependymomas were found supratentorially and fell into the methylation class of RELA-EPN. Papillary ependymomas with a positive ependymoma match (12/19 cases) showed either a "papillary" (n = 5), a "trabecular" (n = 1), or a "pseudo-papillary" (n = 6) growth pattern. The papillary growth pattern was strongly associated with the methylation class B of posterior fossa ependymoma (PFB, 5/5 cases) and tumors displayed DNA methylation sites that were significantly different when compared to PFB ependymomas without papillary growth. Tumors with pseudo-papillary histology matched to the methylation class of myxopapillary ependymoma (4/6 cases), whereas the trabecular case was anatomically and molecularly a spinal ependymoma. Our results show that the diagnosis of histological ependymoma variants is challenging and epigenetic profiles may improve diagnostic accuracy of these cases. Whereas clear cell and papillary ependymomas display correlations between localization, histology, and methylation, tanycytic ependymoma does not represent a molecularly distinct subgroup.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Brain Neoplasms; Child; Cohort Studies; DNA Methylation; Ependymoma; Female; Humans; Male; Middle Aged; Neoplasm Grading; Progression-Free Survival; Survival Rate; Young Adult
PubMed: 31679042
DOI: 10.1007/s00401-019-02090-0 -
Neurosurgery Mar 2013Ependymomas are the most frequent intramedullary neoplasms in adult patients. Anaplastic histology, extramedullary location, meningeal dissemination at initial...
BACKGROUND AND IMPORTANCE
Ependymomas are the most frequent intramedullary neoplasms in adult patients. Anaplastic histology, extramedullary location, meningeal dissemination at initial diagnosis, and extraneural metastases are rare findings. We describe a case of extramedullary anaplastic ependymoma that presented with holocordal and intracranial leptomeningeal carcinomatosis and bone metastases in all the vertebral bodies and the sternum. Such an aggressive dissemination at initial diagnosis has not been previously reported.
CLINICAL PRESENTATION
A 36-year-old woman presented with headache, multiple cranial nerve palsies, visual hallucinations, confusion, hemiparesis, hemihipoestesia, episodes of disconnection, and toxic syndrome. Magnetic resonance imaging and positron emission tomography scan revealed leptomeningeal carcinomatosis in the brainstem, the cerebellum, and along the whole spinal cord. Various nodular, intradural extramedullary lesions were present at multiple dorsal and lumbar levels. Metastatic bone disease affected all the vertebral bodies and various extraspinal bones. An intradural and bone biopsy was performed at L4, providing the diagnosis of anaplastic ependymoma (World Health Organization grade III) with focal neuronal differentiation. Despite chemotherapy, the patient's symptoms quickly progressed, and she died 7 weeks after diagnosis.
CONCLUSION
To our knowledge, there are no previous descriptions of ependymomas with this extensive leptomeningeal, spinal, intracranial, and extraneural dissemination at clinical onset. Bone metastases in spinal ependymoma have not been previously reported.
Topics: Adult; Biopsy; Bone Neoplasms; Bone and Bones; Ependymoma; Fatal Outcome; Female; Headache; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Meningeal Carcinomatosis; Spinal Cord Neoplasms
PubMed: 23422903
DOI: 10.1227/NEU.0b013e31827d102e -
Journal of Neurosurgery Jun 1997The authors conducted a retrospective review of the clinical and treatment characteristics and outcomes in 28 pediatric patients with anaplastic ependymoma treated with...
The authors conducted a retrospective review of the clinical and treatment characteristics and outcomes in 28 pediatric patients with anaplastic ependymoma treated with radiation therapy since the advent of computerized tomography (CT) (1978-1994). Twelve patients received craniospinal irradiation followed by a boost to the primary site, two received whole-brain radiation therapy followed by a boost to the primary site, and the remaining 14 were treated with focal radiation therapy. The mean dose to the primary site was 5486 cGy. With a median follow-up period of 86 months for the 14 surviving patients (range 31-201 months), the median disease-free survival, measured from the date of diagnosis to the time of recurrence after radiation therapy, was 40 months. The median disease-free survival measured from the start of radiation therapy was 32 months. The median overall survival rate has not been reached and the actuarial estimates of overall survival rates at 5 and 10 years were 56% and 38%, respectively. According to univariate analysis, the disease-free survival rate was significantly improved (p < 0.01) in patients who underwent a gross-total resection at diagnosis. Overall survival rates were negatively influenced by treatment with craniospinal and whole-brain irradiation. As calculated by multivariate analysis, increasing dosage to the primary site (p < 0.05), infratentorial location (p < 0.01), and gross-total resections (p < 0.02) resulted in the longest disease-free survival times. All 19 patients in whom treatment failed after radiation therapy suffered a recurrence at the primary site. In addition, one of these patients experienced subarachnoid dissemination. Radiation treatment recommendations for patients with ependymoma have been based on the tumor's location, perceived risk for dissemination, and malignant propensity. The significance of anaplastic histological classification is controversial. Differences in the disease-free and overall survival rates have been demonstrated between ependymomas and anaplastic ependymomas treated in the pre-CT era. The results of this study show that there is no benefit from craniospinal irradiation in this group of patients.
Topics: Adolescent; Adult; Brain; Brain Neoplasms; Child; Child, Preschool; Combined Modality Therapy; Ependymoma; Female; Humans; Male; Neoplasm Recurrence, Local; Retrospective Studies; Spinal Cord; Survival Analysis; Treatment Failure
PubMed: 9171172
DOI: 10.3171/jns.1997.86.6.0943 -
Neurology Nov 2009Ependymoma is a rare type of glioma, representing 5% of all CNS malignancies. Radiotherapy (RT) is commonly administered, but there is no standard chemotherapy. At...
BACKGROUND
Ependymoma is a rare type of glioma, representing 5% of all CNS malignancies. Radiotherapy (RT) is commonly administered, but there is no standard chemotherapy. At recurrence, ependymoma is notoriously refractory to therapy and the prognosis is poor. In recurrent glioblastoma, encouraging responses with bevacizumab have been observed.
METHODS
In this Institutional Review Board-approved study, we retrospectively analyzed the records of 8 adult patients treated for recurrent ependymoma and anaplastic ependymoma with bevacizumab containing chemotherapy regimens. We determined radiographic response (Macdonald criteria), median time to progression (TTP), and median overall survival (OS; Kaplan-Meier method).
RESULTS
There were 4 men and 4 women with a median age of 40 years (range, 20-65). Prior treatment included surgery (n = 8), RT (8), temozolomide (5), and carboplatin (4). Bevacizumab (5-15 mg/kg every 2-3 weeks) was administered alone (2) or concurrently with cytotoxic chemotherapy including irinotecan (3), carboplatin (2), or temozolomide (1). Six patients achieved a partial response (75%) and 1 remained stable for over 8 months. Median TTP was 6.4 months (95% confidence interval 1.4-7.4) and median OS was 9.4 months (95% confidence interval 7.0-not reached), with a median follow-up of 5.2 months among 5 surviving patients (63%).
CONCLUSIONS
The radiographic response rate to bevacizumab-containing regimens is high. A prospective study is warranted.
Topics: Adult; Aged; Angiogenesis Inhibitors; Antibodies, Monoclonal; Antibodies, Monoclonal, Humanized; Bevacizumab; Brain Neoplasms; Disease Progression; Drug Therapy, Combination; Ependymoma; Female; Follow-Up Studies; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Neoplasm Recurrence, Local; Retrospective Studies; Time Factors; Treatment Outcome; Young Adult
PubMed: 19917990
DOI: 10.1212/WNL.0b013e3181c1df34 -
Handbook of Clinical Neurology 2016Ependymomas are tumors that typically occur with an age-based site preference, with adults harboring supratentorial and spinal tumors and pediatric tumors being mainly... (Review)
Review
Ependymomas are tumors that typically occur with an age-based site preference, with adults harboring supratentorial and spinal tumors and pediatric tumors being mainly in the posterior fossa. Despite their similar histologic appearance, the prognosis varies significantly by age and tumor location, with a better prognosis in increasing age. The mainstay of treatment remains surgical excision with or without radiation therapy as the tumor biology is poorly understood and chemotherapy is generally considered to be ineffective. More recently, molecular biology data have increased our understanding of the genetic and epigenetic changes that drive these tumors, but still it will take a lot of effort to find effective chemotherapeutic regimens. Currently, we are trying to define a subset of tumors, for which radiation therapy can be avoided.
Topics: Brain Neoplasms; Ependymoma; Humans; Neuroimaging; Pathology, Molecular
PubMed: 26948369
DOI: 10.1016/B978-0-12-802997-8.00025-6 -
Pathology International Dec 2005Ependymomas generally arise in the central nervous system (CNS), although rare primary extraneural ependymomas have been observed. Reported herein for the first time is...
Ependymomas generally arise in the central nervous system (CNS), although rare primary extraneural ependymomas have been observed. Reported herein for the first time is the case of a patient with primary ectopic cervical anaplastic ependymoma. The tumor was found in the right neck root region of a 35-year-old man. No additional tumor was found in the CNS or in other parts of the body. The patient received surgery and post-surgical local radiotherapy. Microscopically, the tumor consisted of round to oval cells with fine chromatin, distinct nucleoli, moderate nuclear atypia and numerous mitoses (>25/10 high-power fields) in a densely cellular growth pattern with characteristic fibrillary cytoplasm and formation of perivascular pseudorosettes. By immunohistochemistry, the tumor cells were positive for glial fibrillary acidic protein, epithelial membrane antigen (EMA), vimentin and S-100 protein. EMA staining showed a membranous as well as a paranuclear pattern of immunoreactivity. Electron microscopic studies revealed that tumor cells form micro rosettes, into which microvilli and cilia projected. The diagnosis was World Health Organization grade III anaplastic ependymoma. There is no evidence of local tumor recurrence or distant metastasis after 30 months follow up. The present case adds yet another unique example to the already diverse spectrum of head and neck neoplasms encountered in surgical pathology.
Topics: Adult; Ependymoma; Glial Fibrillary Acidic Protein; Head and Neck Neoplasms; Humans; Male; Mucin-1; Vimentin
PubMed: 16287493
DOI: 10.1111/j.1440-1827.2005.01906.x -
Histopathology Dec 2014To report an exceptional case of papillary ependymoma occurring in the endometrium.
AIMS
To report an exceptional case of papillary ependymoma occurring in the endometrium.
METHODS AND RESULTS
A clinicopathological study was performed regarding a case of papillary ependymoma occurring in the endometrial cavity of a 61-year-old patient who had presented with a solid-type, stage III anaplastic ependymoma of the ovary, treated with cytoreductive surgery that included total abdominal hysterectomy. The uterus was enlarged and showed a dilated cavity, with broadly implanted papillary excrescences without myometrial invasion that were covered by tall, cylindrical cells. These cells had glial fibrillary acidic protein-expressing cytoplasm that was also positive for cytokeratins 7, 8, 18, and 34β-E12, epithelial membrane antigen, S100 protein, vimentin, and oestrogen and progesterone receptors.
CONCLUSIONS
Pathogenetically, the presence of this uterine ependymoma could represent either an example of multicentricity or a phenomenon of transtubal implantation of the ovarian tumour. Exceptionally, papillary ependymoma can occur in the endometrium, and may prompt differential diagnoses with other papillary endometrial tumours. Pathologists should consider this rare possibility in the differential diagnosis of papillary ovarian and endometrial lesions.
Topics: Endometrial Neoplasms; Ependymoma; Female; Humans; Middle Aged; Neoplasms, Second Primary; Ovarian Neoplasms
PubMed: 24845054
DOI: 10.1111/his.12462 -
Indian Journal of Pathology &... 2010We report an unusual case of a recurrent fourth ventricular anaplastic ependymoma with prominent chondroid metaplasia in a 16-year-old male. On initial presentation, the... (Review)
Review
We report an unusual case of a recurrent fourth ventricular anaplastic ependymoma with prominent chondroid metaplasia in a 16-year-old male. On initial presentation, the patient had a WHO Grade II tumor. However, at recurrence 1 year later, the tumor progressed to WHO Grade III tumor with more cellularity, necrosis and brisk mitotic activity. Chondroid metaplasia was present in both the initial and recurrent tumors.
Topics: Adolescent; Brain Neoplasms; Chondrogenesis; Cranial Fossa, Posterior; Ependymoma; Glial Fibrillary Acidic Protein; Head; Histocytochemistry; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Male; Metaplasia; Mucin-1; Radiography; Recurrence; Severity of Illness Index
PubMed: 21045418
DOI: 10.4103/0377-4929.72092