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World Neurosurgery Feb 2022Ependymomas are rare central nervous system tumors. The current treatment strategy is gross total tumor removal. Whether adjuvant therapy will be beneficial is... (Review)
Review
OBJECTIVE
Ependymomas are rare central nervous system tumors. The current treatment strategy is gross total tumor removal. Whether adjuvant therapy will be beneficial is controversial. We retrospectively analyzed 3 cases of World Health Organization (WHO) grade III posterior fossa anaplastic ependymomas treated with different treatment modalities. We aimed to identify possible treatment options for infratentorial WHO grade III anaplastic ependymoma in adults.
METHODS
We performed a retrospective analysis of 3 patients diagnosed with infratentorial anaplastic ependymomas in our institution from 2016 to 2020. The demographic data were documented. This case series of 3 patients does not meet the Department of Health and Human Services definition of research and does not need Institutional Review Board approval. All patients' informed consents have been obtained.
RESULTS
One patient underwent subtotal tumor resection combined with adjuvant radiotherapy and Gamma Knife radiosurgery while the other 2 patients underwent gross total tumor removal combined with Gamma Knife radiosurgery or adjuvant radiotherapy. Tumors recurred in the first patient 20 months later, while the other 2 patents did not develop recurrence. The modified Rankin scale scores of these patients were 1, 0, and 0. All patients are followed up with regular magnetic resonance imaging at our facility.
CONCLUSIONS
The strategy for treating WHO grade III anaplastic ependymomas is controversial, but gross total tumor resection remains the key element. Adjuvant stereotactic radiosurgery after tumor removal might be considered if radiotherapy is not an option. The role of chemotherapy is unclear, and the use of chemotherapy should be tailored to individual patients.
Topics: Adult; Ependymoma; Humans; Infratentorial Neoplasms; Radiotherapy, Adjuvant; Retrospective Studies
PubMed: 34839047
DOI: 10.1016/j.wneu.2021.11.074 -
Neuro-oncology Mar 2021No standard medical treatment exists for adult patients with recurrent ependymoma, and prospective clinical trials in this population have not succeeded because of its...
BACKGROUND
No standard medical treatment exists for adult patients with recurrent ependymoma, and prospective clinical trials in this population have not succeeded because of its rarity and challenges in accruing patients. The Collaborative Ependymoma Research Network conducted a prospective phase II clinical trial of dose-dense temozolomide (TMZ) and lapatinib, targeting the unmethylated O6-methylguanine-DNA methyltransferase (MGMT) promoter status and increased expression of ErbB2 (human epidermal growth factor receptor 2) and ErbB1 (epidermal growth factor receptor) in ependymomas.
METHODS
Patients age 18 or older with histologically proven and progressive ependymoma or anaplastic ependymoma were eligible and received dose-dense TMZ and daily lapatinib. The primary outcome measure was median progression-free survival (PFS). Landmark 6- and 12-month PFS and objective response were measured. Serial assessments of symptom burden using the MD Anderson Symptom Inventory Brain Tumor (MDASI-BT)/MDASI-Spine Tumor modules were collected.
RESULTS
The 50 patients enrolled had a median age of 43.5 years, median Karnofsky performance status of 90, and a median of 2 prior relapses. Twenty patients had grade III, 16 grade II, and 8 grade I ependymoma. Half had spinal cord tumors; 15 had a supratentorial tumor, 8 infratentorial, and 2 had disseminated disease. Treatment was well tolerated. The median PFS was 7.8 months (95% CI: 5.5,12.2); the 6- and 12-month PFS rates were 55% and 38%, with 2 complete and 6 partial responses. Measures of symptom burden showed reduction in moderate-severe pain and other disease-related symptoms in most patients.
CONCLUSIONS
This treatment, with demonstrated clinical activity with objective responses and prolonged disease control associated with disease-related symptom improvements, is an option as a salvage regimen for adult patients with recurrent ependymoma.
Topics: Adolescent; Adult; Brain Neoplasms; Dacarbazine; Disease-Free Survival; Ependymoma; Humans; Lapatinib; Prospective Studies; Spinal Cord Neoplasms; Temozolomide
PubMed: 33085768
DOI: 10.1093/neuonc/noaa240 -
World Neurosurgery Feb 2022Myxopapillary ependymomas (MPEs) with anaplastic features are rarely reported, with only 21 cases identified to date, and long-term recurrence is rarely presented. A... (Review)
Review
BACKGROUND
Myxopapillary ependymomas (MPEs) with anaplastic features are rarely reported, with only 21 cases identified to date, and long-term recurrence is rarely presented. A case series is presented to expand understanding of this disease by describing 3 unique cases, including 2 that arose from MPE after a prolonged clinical course.
METHODS
A literature review was performed, and 3 cases of MPE with anaplastic features from our institution were included.
RESULTS
Patient 1 was a 13-year-old boy who presented with an avidly enhancing intradural lumbar mass. On gross total resection, the tumor was found to be a solid mass with areas of myxopapillary architecture and MIB-1 (Ki-67) index of 12%. Patient 2 was a woman who initially presented at age 22 with a lumbosacral tumor that was treated with surgery and radiation. A recurrent tumor was resected at age 24. At age 50, the patient presented with a large heterogeneous exophytic mass in the sacrum extending into the presacral space and Ki-67 index of 8%. This was treated with complete resection. Patient 3 was a man who initially presented at age 35 with a lower thoracic, upper lumbar mass at L2 extending into the sacrum. Following resection and radiation, a metastatic focus followed an indolent course until causing pain at the age of 48. Ki-67 index was 16%.
CONCLUSIONS
The presented cases of MPE with anaplastic features make a total of 24 cases on record in the medical literature and demonstrate 2 examples of late recurrence.
Topics: Adolescent; Adult; Ependymoma; Female; Humans; Ki-67 Antigen; Lumbosacral Region; Male; Middle Aged; Sacrum; Spinal Cord Neoplasms; Young Adult
PubMed: 34800728
DOI: 10.1016/j.wneu.2021.11.053 -
Journal of Neuropathology and... Oct 2020Myxopapillary ependymoma (MPE) is a relatively common neoplasm arising primarily in the filum terminale/lumbosacral region of the spinal cord. It is designated as a... (Review)
Review
Myxopapillary ependymoma (MPE) is a relatively common neoplasm arising primarily in the filum terminale/lumbosacral region of the spinal cord. It is designated as a grade I tumor in the most recent WHO Classification of Tumours of the CNS, although aggressive clinical behavior can be observed, especially in cases arising in an extradural location. Anaplastic transformation in MPE is exceedingly rare with <20 examples reported in the English literature, and consensus on diagnostic features and definitive grading remain to be determined. Here, we present 2 cases of recurrent MPE with anaplastic features, both of which had histology consistent with conventional MPE as well as areas with significant atypia, frequent mitotic figures, elevated Ki-67 proliferation indices (>10%-50%), necrosis, and focal vascular proliferation. Targeted next-generation sequencing panels revealed no definitive pathogenic mutations or fusion proteins in either case. Copy number profiling, methylation profiling, and t-Distributed Stochastic Neighbor Embedding were performed to investigate the molecular characteristics of these tumors. To the best of our knowledge, these are the first reported cases of MPE with anaplastic features with methylation profiling data. In addition, we review the literature and discuss common histologic and molecular findings associated with anaplastic features in MPE.
Topics: Aged; Ependymoma; Humans; Male; Spinal Cord Neoplasms; Young Adult
PubMed: 32743660
DOI: 10.1093/jnen/nlaa077 -
The Journal of Obstetrics and... Jul 2019To the best of our knowledge, there are currently no reports on primary uterine ependymoma. We operated on a 38-year-old woman suspected with uterine fibroids; surgical... (Review)
Review
To the best of our knowledge, there are currently no reports on primary uterine ependymoma. We operated on a 38-year-old woman suspected with uterine fibroids; surgical findings led to the diagnosis of primary anaplastic ependymoma of the uterus. Most extraneural ependymomas arising from the ovary and perigenital peritoneum occur in women of reproductive age and express estrogen and progesterone receptors. The etiology of such tumors is unknown, and a treatment strategy has not been established yet. Therefore, it is essential to accumulate cases for identifying mechanisms underlying the pathogenesis and disease progression to facilitate diagnosis and development of an effective treatment.
Topics: Ependymoma; Female; Humans; Uterine Neoplasms; Uterus
PubMed: 31060102
DOI: 10.1111/jog.13984 -
Acta Neurologica Belgica Dec 2022
Topics: Humans; Ependymoma; Magnetic Resonance Imaging; Brain Neoplasms
PubMed: 33423219
DOI: 10.1007/s13760-020-01586-x -
Neuro-oncology Mar 2018Ependymal tumors are rare CNS tumors and may occur at any age, but their proportion among primary brain tumors is highest in children and young adults. Thus, the level... (Review)
Review
Ependymal tumors are rare CNS tumors and may occur at any age, but their proportion among primary brain tumors is highest in children and young adults. Thus, the level of evidence of diagnostic and therapeutic interventions is higher in the pediatric compared with the adult patient population.The diagnosis and disease staging is performed by craniospinal MRI. Tumor classification is achieved by histological and molecular diagnostic assessment of tissue specimens according to the World Health Organization (WHO) classification 2016. Surgery is the crucial initial treatment in both children and adults. In pediatric patients with intracranial ependymomas of WHO grades II or III, surgery is followed by local radiotherapy regardless of residual tumor volume. In adults, radiotherapy is employed in patients with anaplastic ependymoma WHO grade III, and in case of incomplete resection of WHO grade II ependymoma. Chemotherapy alone is reserved for young children <12 months and for adults with recurrent disease when further surgery and irradiation are no longer feasible. A gross total resection is the mainstay of treatment in spinal ependymomas, and radiotherapy is reserved for incompletely resected tumors. Nine subgroups of ependymal tumors across different anatomical compartments (supratentorial, posterior fossa, spinal) and patient ages have been identified with distinct genetic and epigenetic alterations, and with distinct outcomes. These findings may lead to more precise diagnostic and prognostic assessments, molecular subgroup-adapted therapies, and eventually new recommendations pending validation in prospective studies.
Topics: Ependymoma; Humans; Practice Guidelines as Topic; Prognosis
PubMed: 29194500
DOI: 10.1093/neuonc/nox166 -
Neurology India 2021Anaplastic ependymoma in the pineal region is rare. Here, we present a rare case of anaplastic ependymoma of the pineal region on a 42-year-old woman who came to our...
Anaplastic ependymoma in the pineal region is rare. Here, we present a rare case of anaplastic ependymoma of the pineal region on a 42-year-old woman who came to our hospital with headache associated with blurring of vision since one month. MRI brain showed a contrast enhancing mass lesion measuring 30 × 30 × 35 mm in the pineal region with obstructive hydrocephalus. Initially, the ventriculoperitoneal (VP) shunt was done, followed by total tumor excision by the infratentorial supracerebellar approach. Histopathological and immunohistochemistry examinations of the tumor showed the features of anaplastic ependymoma (WHO Grade III). Patient made uneventful recovery and underwent radiotherapy. Only 12 cases of pineal ependymoma have been reported so far, of which only three have been anaplastic ependymoma.
Topics: Adult; Ependymoma; Female; Humans; Hydrocephalus; Magnetic Resonance Imaging; Pineal Gland; Ventriculoperitoneal Shunt
PubMed: 34507442
DOI: 10.4103/0028-3886.325365 -
Neuro-Chirurgie Sep 2021
Topics: Ependymoma; Humans; Spinal Cord Neoplasms
PubMed: 33189738
DOI: 10.1016/j.neuchi.2020.10.007 -
Seminars in Neurology Feb 2018Ependymoma can arise throughout the whole neuraxis. In children, tumors predominantly occur intracranially, whereas the spine is the most prevalent location in adults.... (Review)
Review
Ependymoma can arise throughout the whole neuraxis. In children, tumors predominantly occur intracranially, whereas the spine is the most prevalent location in adults. Significant variance in the grade II versus grade III distinction of ependymomas has led to the acknowledgment that the clinical utility of histopathological classification is limited. Epigenomic profiling efforts have identified molecularly distinct groups of ependymomas that adequately reflect the biological, clinical, and histopathological heterogeneities across anatomical compartments, age groups, and grades. The recent update of the World Health Organization classification of central nervous system tumors has already integrated one of these groups, and molecular classification will be part of future clinical trials to improve risk stratification. Clinical management of this rare disease is challenging, making professional experience and intensified multidisciplinary cooperation pivotal factors for treatment success. Novel research strategies are currently applied for target discovery in ependymomas since for most molecular groups, genetic drivers are unknown.
Topics: Adolescent; Adult; Central Nervous System Neoplasms; Child; Ependymoma; Humans
PubMed: 29548057
DOI: 10.1055/s-0038-1636503