|
1
|
Hendem E, Korkmaz M, Araz M, Eryılmaz MK, Çağlayan D, Ayçiçek ST, Kaya B, Artaç M. Extensive metastatic ependymoma with long-term progression-free survival with capecitabine plus temozolomide combination chemotherapy: A case report. J Cancer Res Ther 2024; 20:1625-1627. [PMID: 39412934 DOI: 10.4103/jcrt.jcrt_1664_22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2022] [Accepted: 01/02/2023] [Indexed: 10/18/2024]
Abstract
ABSTRACT We wanted to present a rare case of metastatic grade 2 spinal ependymoma with an atypical course at the time of diagnosis. Temozolomide plus capecitabine chemotherapy was started in May 2018 on a 30-year-old female patient with sacral ependymoma who had extensive lung metastases at the time of diagnosis. The patient remained in remission for approximately 29 months, and the current chemotherapy was continued until it progressed in November 2020. According to this case report, a combination of temozolomide and capecitabine may be the best treatment option for ependymoma patients.
Collapse
Affiliation(s)
- Engin Hendem
- Department of Medical Oncology, Necmettin Erbakan University School of Medicine, Konya, Turkey
| | - Mustafa Korkmaz
- Department of Medical Oncology, Necmettin Erbakan University School of Medicine, Konya, Turkey
| | - Murat Araz
- Department of Medical Oncology, Necmettin Erbakan University School of Medicine, Konya, Turkey
| | - Melek K Eryılmaz
- Department of Medical Oncology, Necmettin Erbakan University School of Medicine, Konya, Turkey
| | - Dilek Çağlayan
- Department of Medical Oncology, Necmettin Erbakan University School of Medicine, Konya, Turkey
| | - Seda T Ayçiçek
- Department of Pathology, Necmettin Erbakan University School of Medicine, Konya, Turkey
| | - Buğra Kaya
- Department of Nuclear Medicine, Necmettin Erbakan University School of Medicine, Konya, Turkey
| | - Mehmet Artaç
- Department of Medical Oncology, Necmettin Erbakan University School of Medicine, Konya, Turkey
| |
Collapse
|
|
2
|
Luan XZ, Wang HR, Xiang W, Li SJ, He H, Chen LG, Wang JM, Zhou J. Extracranial multiorgan metastasis from primary glioblastoma: A case report. World J Clin Cases 2021; 9:10300-10307. [PMID: 34904103 PMCID: PMC8638031 DOI: 10.12998/wjcc.v9.i33.10300] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/08/2021] [Revised: 08/31/2021] [Accepted: 09/16/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Glioblastoma has a high degree of malignancy and poor prognosis. It is common to have in situ recurrence and intracranial metastasis, while extracranial metastasis is rare, and extracranial multiorgan metastasis is extremely rare. We report a case of glioblastoma with extracranial multiorgan metastasis, which will strengthen clinicians’ attention to the extracranial metastasis of glioblastoma and its treatment.
CASE SUMMARY A male patient visited our hospital for treatment of dizziness and headache. Magnetic resonance imaging of the brain revealed a space-occupying lesion in the right temporoparietal occipital region. Chest computed tomography and abdominal ultrasound were normal, and no space-occupying lesions were observed in other organs of the body. The patient underwent surgery and diagnosed with glioblastoma. Postoperative concurrent radiotherapy and chemotherapy were completed. During the follow-up, the tumor was found to have metastasized to the scalp and neck, and a second tumor resection was performed. Postoperative follow-up revealed extracranial metastases to multiple extracranial organs including skull, scalp, ribs, spine, liver and lung. His family members refused further treatment, and requested only symptomatic treatment such as pain relief, and the patient died of systemic multiple organ failure. Survival time from diagnosis to death was 13 mo and from extracranial metastasis to death was 6 mo.
CONCLUSION Glioblastoma extracranial metastasis is extremely rare, clinicians should always pay attention to its existence. The mechanism of glioblastoma extracranial metastasis is still unclear, and genetic and molecular studies are required.
Collapse
Affiliation(s)
- Xing-Zhao Luan
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Sichuan Clinical Research Center for Neurosurgery, Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Hao-Run Wang
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Sichuan Clinical Research Center for Neurosurgery, Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Academician (Expert) Workstation of Sichuan Province, Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Wei Xiang
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Sichuan Clinical Research Center for Neurosurgery, Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Academician (Expert) Workstation of Sichuan Province, Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Shen-Jie Li
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Sichuan Clinical Research Center for Neurosurgery, Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Academician (Expert) Workstation of Sichuan Province, Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Haiping He
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Sichuan Clinical Research Center for Neurosurgery, Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Academician (Expert) Workstation of Sichuan Province, Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Li-Gang Chen
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Sichuan Clinical Research Center for Neurosurgery, Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Academician (Expert) Workstation of Sichuan Province, Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Jian-Mei Wang
- Department of Pathology, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Jie Zhou
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Sichuan Clinical Research Center for Neurosurgery, Southwest Medical University, Luzhou 646000, Sichuan Province, China
- Academician (Expert) Workstation of Sichuan Province, Southwest Medical University, Luzhou 646000, Sichuan Province, China
| |
Collapse
|
|
3
|
Kanno H, Kanetsuna Y, Shinonaga M. Anaplastic myxopapillary ependymoma: A case report and review of literature. World J Clin Oncol 2021; 12:1072-1082. [PMID: 34909401 PMCID: PMC8641005 DOI: 10.5306/wjco.v12.i11.1072] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2021] [Revised: 05/17/2021] [Accepted: 10/11/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Myxopapillary ependymoma (MPE) is a pathological grade I tumor that arises in the filum terminale. MPE with anaplastic features is extremely rare, and only 5 cases have shown malignancy at the time of recurrence.
CASE SUMMARY The patient (a 46-year-old woman) had undergone a MPE operation 30 years ago. After subtotal resection of the tumor located in L4-S1, it had a solid component that extended to the adjacent subcutaneous region. Histologically, the tumor consisted of a typical MPE with anaplastic features. The anaplastic areas of the tumor showed hypercellularity, a rapid mitotic rate, vascular proliferation, and connective tissue proliferation. Pleomorphic cells and atypical mitotic figures were occasionally observed. The MIB-1 index in this area was 12.3%. The immunohistochemical study showed immunoreactivity for vimentin, glial fibrillary acidic protein and S100. The morphological pattern and immunohistochemical profile were consistent with anaplastic MPE. The patient tolerated surgery well without new neurological deficits. She underwent local irradiation for the residual tumor and rehabilitation.
CONCLUSION Although extremely rare, anaplastic MPE occurs in both pediatric and adult patients, similar to other ependymomas. At a minimum, close monitoring is recommended, given concerns about aggressive biological potential. In the future, further study is needed to determine the WHO classification criteria and genetic indicators of tumor progression. The possibility of malignant transformation of MPE should be taken into account, and patients with MPE should be treated with care and follow-up.
Collapse
Affiliation(s)
- Hiroshi Kanno
- Department of Neurosurgery, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| | - Yukiko Kanetsuna
- Department of Pathology, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| | - Masamichi Shinonaga
- Department of Neurosurgery, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| |
Collapse
|
|
4
|
Abdallah A. Spinal Seeding Metastasis of Myxopapillary Ependymoma: Report of Three Pediatric Patients and a Brief Literature Review. Pediatr Neurosurg 2020; 55:127-140. [PMID: 32777780 DOI: 10.1159/000509061] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/04/2019] [Accepted: 06/02/2020] [Indexed: 11/19/2022]
Abstract
OBJECTIVE Myxopapillary ependymomas (MPEs) in children are rarely reported low-grade glial tumors; however, MPEs sometimes possess malignant characteristics such as spinal seeding/drop metastasis (SSM). We aimed to present 3 pediatric MPE cases that experienced SSM at 2 neurosurgical centers. MATERIALS AND METHODS We retrospectively reviewed the medical records of 38 primary spinal MPE cases who underwent surgery at 2 neurosurgical centers spanning 16 years, from 2004 to 2019. All pediatric cases (patient age <18 years) who were diagnosed with MPE and re-presented with SSM were selected as the core sample for this study. Relevant literature was briefly reviewed. RESULTS Three pediatric MPE cases (2 females and 1 male) experienced SSM. The mean age at first presentation was 12.0 ± 1.0 years. The mean preoperative course was 2.9 ± 1.2 months. The predominant location was the lumbar spine in 2 tumors (both originated from terminal filum [TF]). Two tumors were located intradural intramedullary. Gross-total resection was achieved in 2 patients. No patient had neurofibromatosis type 2. No adjuvant treatment was given after the first surgery. The mean period between the first diagnosis and diagnosis of SSM was 44.0 ± 31.5 months. The location of SSM in all patients was the sacral spine (1 patient experienced distant metastasis in her brain besides her sacral metastasis). The mean follow-up was 68.3 ± 53.7 months. CONCLUSIONS We found a statistically significant relationship between SSM in pediatric MPEs and the intramedullary location, TF origin, and number of affected segments. Close clinical and radiological follow-up is essential for pediatric MPE patients.
Collapse
Affiliation(s)
- Anas Abdallah
- Department of Neurosurgery, Bezmialem Vakif University, Istanbul, Turkey,
| |
Collapse
|
|
5
|
Batich KA, Riedel RF, Kirkpatrick JP, Tong BC, Eward WC, Tan CL, Pittman PD, McLendon RE, Peters KB. Recurrent Extradural Myxopapillary Ependymoma With Oligometastatic Spread. Front Oncol 2019; 9:1322. [PMID: 31850213 PMCID: PMC6892774 DOI: 10.3389/fonc.2019.01322] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/08/2019] [Accepted: 11/12/2019] [Indexed: 12/14/2022] Open
Abstract
Myxopapillary ependymomas are a slow-growing, grade I type glial tumor in the lumbosacral region. More rarely, they can present as extradural, subcutaneous sacrococcygeal, or perisacral masses, and it is under these circumstances that they are more likely to spread. Here, we report the presentation of a sacrococcygeal mass in patient that was initially resected confirming extradural myxopapillary ependymoma. At initial resection, multiple small pulmonary nodules were detected. This mass recurred 2 years later at the resection site with an interval increase in the previously imaged pulmonary nodules. Resection of both the post-sacral mass and largest lung metastasis confirmed recurrent myxopapillary ependymoma with oligometastatic spread. Because these tumors are rare, with extradural presentation being even more infrequent, to this date there are no definitive therapeutic guidelines for initial treatment and continued surveillance. For myxopapillary ependymoma, current standard of care is first-line maximal surgical resection with or without postoperative radiotherapy depending on the extent of disease and extent of resection. However, there remains insufficient evidence on the role of radiotherapy to oligometastatic foci in providing any further survival benefit or extending time to recurrence. Thus, prospective studies assessing the role of upfront treatment of oligometastases with local resection and adjuvant radiotherapy are needed for improved understanding of extradural myxopapillary ependymoma.
Collapse
Affiliation(s)
- Kristen A Batich
- Department of Medicine, Duke University Health System, Durham, NC, United States.,Division of Medical Oncology, Department of Medicine, Duke University Health System, Durham, NC, United States.,The Preston Robert Tisch Brain Tumor Center, Duke University Health System, Durham, NC, United States
| | - Richard F Riedel
- Division of Medical Oncology, Department of Medicine, Duke University Health System, Durham, NC, United States.,Duke Cancer Institute, Duke University Health System, Durham, NC, United States
| | - John P Kirkpatrick
- The Preston Robert Tisch Brain Tumor Center, Duke University Health System, Durham, NC, United States.,Duke Cancer Institute, Duke University Health System, Durham, NC, United States.,Department of Radiation Oncology, Duke University Health System, Durham, NC, United States.,Department of Neurosurgery, Duke University Health System, Durham, NC, United States
| | - Betty C Tong
- Duke Cancer Institute, Duke University Health System, Durham, NC, United States.,Division of Cardiovascular and Thoracic Surgery, Department of Surgery, Duke University Health System, Durham, NC, United States
| | - William C Eward
- Duke Cancer Institute, Duke University Health System, Durham, NC, United States.,Department of Orthopaedic Surgery, Duke University Health System, Durham, NC, United States
| | - Char Loo Tan
- Department of Pathology, Duke University Health System, Durham, NC, United States.,Department of Pathology, National University Health System, Singapore, Singapore
| | - Patricia D Pittman
- Department of Pathology, Duke University Health System, Durham, NC, United States
| | - Roger E McLendon
- The Preston Robert Tisch Brain Tumor Center, Duke University Health System, Durham, NC, United States.,Duke Cancer Institute, Duke University Health System, Durham, NC, United States.,Department of Pathology, Duke University Health System, Durham, NC, United States
| | - Katherine B Peters
- The Preston Robert Tisch Brain Tumor Center, Duke University Health System, Durham, NC, United States.,Duke Cancer Institute, Duke University Health System, Durham, NC, United States.,Department of Neurosurgery, Duke University Health System, Durham, NC, United States
| |
Collapse
|
|
6
|
Abstract
Background The aim of this study was to assess the clinical and radiological outcomes of surgical treatment for primary spinal ependymoma in children. Material/Methods Medical records of 46 primary spinal ependymoma patients who underwent surgery in BRSHH hospital during a 12-year period from 2004 to 2015 were retrospectively reviewed. All pediatric patients (patient age <18 years) were selected as the core sample used for this study. Results This series included 1 female and 2 male patients between the ages of 9 and 17 years with mean age 13.3±3.9 years. The mean preoperative course was 9.1±10.5 months. The most common location was the lumbar spinal cord (n=2). The most common presenting symptoms was lower-limb weakness and numbness. Two tumors were located intradural-intramedullary and 1 was located intradural-extramedullary. Gross-total resection (GTR) was achieved in 2 patients, and a near-total resection was performed in 1 patient. No adjuvant treatment was received. The mean follow-up duration was 51.3±37.6 (17–98) months. No complications were recorded. Functional assessment of all patients by the latest follow-up evaluation showed good progress even though the patient is not fully recovered. At 6.3 years after the first operation, 1 patient presented with drop-seeding metastasis. No patients had neurofibromatosis type 2. Conclusions Laminoplasty and intraoperative neurophysiological monitorization are essential in surgical treatment of pediatric spinal ependymomas. GTR and recovery in pediatric spinal ependymoma are more likely than in adults. Despite the GTR, the risk of drop metastasis remains. Therefore, close clinical and radiological follow-up is recommended.
Collapse
Affiliation(s)
- Özden Erhan Sofuoğlu
- Department of Neurosurgery, Health Science University, Bakirköy Research and Training Hospital for Neurology Neurosurgery and Psychiatry, Istanbul, Turkey
| | - Anas Abdallah
- Department of Neurosurgery, Bezmialem Vakif University, Istanbul, Turkey
| |
Collapse
|
|
7
|
Huynh TR, Lu C, Drazin D, Lekovic G. Myxopapillary ependymoma with anaplastic features: A case report with review of the literature. Surg Neurol Int 2018; 9:191. [PMID: 30294495 PMCID: PMC6169347 DOI: 10.4103/sni.sni_422_17] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2017] [Accepted: 06/04/2018] [Indexed: 12/17/2022] Open
Abstract
Background: Myxopapillary ependymoma (MPE) with anaplastic features is extremely rare, with only three case reports in the literature. Case Description: We report the case of a MPE with anaplastic features in a 24-year-old female who presented with a dominant lumbar mass along with intracranial and sacral metastases. Upon gross total resection of the dominant tumor located at L2-L3, it appeared to arise from the filum terminale, and had a solid component in addition to soft or necrotic areas. Histologically, the tumor was composed of the two classic components of MPE: (1) low-grade ependymal cells surrounding blood vessels, producing the papillary appearance and (2) perivascular myxoid material between blood vessels and ependymal cells, creating the myxopapillary appearance. The high-grade anaplastic component showed hypercellularity, brisk mitotic rate, and vascular proliferation, with frequent pleomorphic cells and atypical mitotic figures. It was positive for vimentin and glial fibrillary acidic protein (GFAP); negative for epithelial membrane antigen (EMA), CAM5.2, creatine kinase 7 (CK7), CK20; and the MIB-1 index (Ki-67) was 8–38%. Ten months after initial resection, follow-up magnetic resonance imaging revealed new lesions in (1) the hypothalamus, (2) the left pons, and (3) the left medial temporal lobe, which were treated with radiosurgery. Eight months later (18 months from initial surgery), the patient underwent thoracic laminectomy for a large leptomeningeal metastasis at T6 and T8. Conclusion: The present case of MPE with anaplastic features is the fourth case on record in the medical literature.
Collapse
Affiliation(s)
- Tridu R Huynh
- Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California, USA
| | - Conrad Lu
- Department of Pathology, St. Vincent Medical Center, Los Angeles, California, USA
| | - Doniel Drazin
- Swedish Neuroscience Institute, Swedish Medical Center, Seattle, Washington, USA
| | | |
Collapse
|
|
8
|
Diaz-Aguilar D, Terterov S, Tucker AM, Sedighim S, Scharnweber R, Wang S, Merna C, Rahman S. Simultaneous cerebrospinal fluid and hematologic metastases in a high-grade ependymoma. Surg Neurol Int 2018; 9:93. [PMID: 29770253 PMCID: PMC5938895 DOI: 10.4103/sni.sni_475_17] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2017] [Accepted: 02/16/2018] [Indexed: 01/09/2023] Open
Abstract
Background Ependymomas are relatively uncommon tumors that constitute about 7% of all primary intracranial neoplasms. Among these, high-grade ependymomas are locally aggressive and recur most commonly at the primary site following resection. Ependymomas are also known to be the one glial neoplasm that tends to frequently metastasize inside and outside the central nervous system (CNS) that complicates workup and management. Metastasis due to surgical manipulation is common and neurosurgeons should be well-versed in the most effective methods to remove these tumors in order to avoid such metastases. Case Description Here, we report a case of a 28-year-old female who initially presented with a parenchymal World Health Organization (WHO) grade III anaplastic ependymoma of the occipital lobe without metastasis. After multiple resections, the patient showed no evidence of disease recurrence for 2 years. During follow-up, new metastasis to the frontal lobe as well as to the lung were discovered 2 years after the initial surgery, without recurrence at the tumor's primary site. Conclusions While uncommon, this case demonstrates the possibility for ependymomas to metastasize via cerebrospinal fluid to other locations within the CNS and hematologically to extraneural locations without recurring locally.
Collapse
Affiliation(s)
- Daniel Diaz-Aguilar
- Department of Neurosurgery, University of California, David Geffen School of Medicine, California, USA
| | - Sergei Terterov
- Department of Neurosurgery, University of California, David Geffen School of Medicine, California, USA
| | - Alexander M Tucker
- Department of Neurosurgery, University of California, David Geffen School of Medicine, California, USA
| | - Shaina Sedighim
- Department of Neurosurgery, University of California, David Geffen School of Medicine, California, USA.,Miller School of Medicine, University of Miami, Miami, Florida, USA
| | - Rudi Scharnweber
- Department of Neurosurgery, University of California, David Geffen School of Medicine, California, USA
| | - Stephanie Wang
- Department of Neurosurgery, University of California, David Geffen School of Medicine, California, USA
| | - Catherine Merna
- Department of Neurosurgery, University of California, David Geffen School of Medicine, California, USA
| | - Shayan Rahman
- Department of Neurosurgery, University of California, David Geffen School of Medicine, California, USA
| |
Collapse
|
|
9
|
Trivedi D, Xiong Z. Anaplastic myxopapillary ependymoma in an infant: Case report and literature review. Intractable Rare Dis Res 2017; 6:128-131. [PMID: 28580214 PMCID: PMC5451745 DOI: 10.5582/irdr.2017.01015] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2017] [Revised: 04/17/2017] [Accepted: 04/21/2017] [Indexed: 11/05/2022] Open
Abstract
A 7-month-old boy presented with gastrointestinal disturbance, mild neurologic deficit of the left lower extremity and levo-scoliosis of the thoracic spine. Magnetic resonance imaging demonstrated a large intramedullary lesion involving the thoracic spine, from level T1 to T11. Histologic analysis showed a glial tumor with fibrillary processes arranged in radial pattern around mucoid fibrovascular cores with a high proliferative index (focally up to 80%) and prominent vascular endothelial hyperplasia. These findings were consistent with an anaplastic myxopapillary ependymoma. Subtotal resection was performed via a T3-T10 laminoplasty. A ventricular shunt was placed, and the patient subsequently received chemoradiation therapy. To date, this is the second case of a myxopapillary ependymoma with high-grade anaplastic features and the first case in an infant reported in the literature.
Collapse
Affiliation(s)
- Darshan Trivedi
- Department of Pathology and Laboratory Medicine, Tulane University School of Medicine, New Orleans, LA, USA
| | - Zhenggang Xiong
- Department of Pathology and Laboratory Medicine, Tulane University School of Medicine, New Orleans, LA, USA
| |
Collapse
|
|
10
|
Primary Seeding of Myxopapillary Ependymoma: Different Disease in Adult Population? Case Report and Review of Literature. World Neurosurg 2016; 99:812.e21-812.e26. [PMID: 28040529 DOI: 10.1016/j.wneu.2016.12.022] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2016] [Revised: 12/07/2016] [Accepted: 12/08/2016] [Indexed: 02/08/2023]
Abstract
Myxopapillary ependymoma (MPE) is a slow-growing tumor, occurring most often in adults. It originates from the filum terminale in the area of the conus medullaris and cauda equina and is considered a benign lesion. Despite this classification, however, recurrence after both partial and gross total resection is well known. In the pediatric population, primary MPE seeding and generally more aggressive clinical course is well documented and treated through gross total resection, if possible, followed by irradiation. In adults, however, primary MPE seeding is rarely seen. There are few prior reports describing primary metastases into multiple spinal locations in an adult before resection of an MPE. The reason for this difference among pediatric and adult MPE remains unclear. We present the case of a 32-year-old man with primary seeding of an MPE into multiple lumbosacral areas. The patient underwent gross total resection of the lesions and had an uneventful postoperative course. Primary seeding could be a sign of aggressive behavior in this tumor. Complete craniospinal magnetic resonance imaging studies should be done before and after surgery in patients who present with a multifocal primary MPE. Furthermore, patients with a history of primary tumor seeding of MPE should be thoroughly evaluated radiologically. Unlike in pediatric populations, the need for postoperative irradiation in adults is less clear and further studies-particularly genetic ones-are warranted.
Collapse
|
|
11
|
Güzin K, Bozdağ H, Aydın A, Şahin S, Özkanlı Ş. Uterine Cervix Metastasis of Myxopapillary Ependymoma Originated from the Spinal Cord. Balkan Med J 2016; 33:235-8. [PMID: 27403397 DOI: 10.5152/balkanmedj.2015.151101] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2015] [Accepted: 05/12/2015] [Indexed: 11/22/2022] Open
Abstract
BACKGROUND Myxopapillary ependymomas are well differentiated low-grade tumors which have been documented to local or distant metastasis. In the literature, this is a unique case of myxopapillary ependymoma with metastasis to the uterine cervix. Here, we present a rare case of extra neural metastasis of spinal ependymoma that developed over a long period. CASE REPORT A 34-year-old woman was referred to our hospital for pelvic mass. A mass (110×100 mm) localized between the sacrococcygeal region and the uterus was detected by magnetic resonance imaging. In 2004, she had been operated upon for myxopapillary ependymoma seated in the sacrococcygeal region for the first time. She underwent tumor resection eight times due to the recurrence of spinal tumor in the same region in nine years. Under the diagnosis of uterine neoplasm, we carried out radical hysterectomy, omentectomy and pelvic lymphadenectomy as the surgical procedure. The pathological findings were reported as myxopapillary ependymoma. Immunohistochemically, the myxopapillary ependymal cells showed strong positivity for glial fibrillary acidic protein, whereas they were negative for low molecular weight cytokeratin. The Ki-67 labeling index was about 2-3%. The patient had an uneventful postoperative period. She has remained free of symptoms in the year since surgery. CONCLUSION Extra-spinal myxopapillary ependymoma is very rare, but it must be considered in the differential diagnosis of pelvic mass lesions.
Collapse
Affiliation(s)
- Kadir Güzin
- Department of Obstetrics and Gynecology, İstanbul Medeniyet University Göztepe Training and Research Hospital, İstanbul, Turkey
| | - Halenur Bozdağ
- Department of Obstetrics and Gynecology, İstanbul Medeniyet University Göztepe Training and Research Hospital, İstanbul, Turkey
| | - Abdullah Aydın
- Department of Pathology, İstanbul Medeniyet University Faculty of Medicine, İstanbul, Turkey
| | - Sadık Şahin
- Departmenf of Obstetrics and Gynecology, Zeynep Kamil Training and Research Hospital, İstanbul, Turkey
| | - Şeyma Özkanlı
- Department of Pathology, İstanbul Medeniyet University Faculty of Medicine, İstanbul, Turkey
| |
Collapse
|
|
12
|
Abstract
Myxopapillary ependymoma (MPE) is a rare and distinctive tumor which occurs in the sacrococcygeal area of young adults and children, often intradural in location. Histopathologic features have been well-described in the literature whereas cytological findings have been sporadically reported by various authors mainly as case reports. We report the features of a primary sacrococcygeal MPE on aspirate cytology in a 45-year-old female. Cytology smears displayed a papillary pattern with the presence of fibrovascular cores, rimmed by cuboidal to columnar cells sending fibrillary cytoplasmic processes forming pseudorosettes along with the presence of hyaline globules, and myxoid material. Intranuclear inclusions, nuclear grooves, cytologic atypia or mitotic activity was not evident, in this case. MPEs need to be differentiated from the other tumors occurring in this location which may also show myxoid material and papillary fronds. Hence, the recognition of the characteristic cytologic features plays an important role in establishing a preoperative diagnosis.
Collapse
Affiliation(s)
- Monisha Choudhury
- Department of Pathology, Lady Hardinge Medical College, New Delhi, India
| | - Archna Rautela
- Department of Pathology, Lady Hardinge Medical College, New Delhi, India
| | - Manupriya Nain
- Department of Pathology, Lady Hardinge Medical College, New Delhi, India
| |
Collapse
|
|
13
|
Histologic grade and extent of resection are associated with survival in pediatric spinal cord ependymomas. Childs Nerv Syst 2013; 29:2057-64. [PMID: 23677177 DOI: 10.1007/s00381-013-2149-x] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/15/2013] [Accepted: 05/03/2013] [Indexed: 10/26/2022]
Abstract
PURPOSE Prognostic factors affecting outcomes in pediatric spinal cord ependymomas are limited. We sought to investigate potential associations between extent of resection and histologic grade on progression-free survival (PFS) and overall survival (OS). METHODS A comprehensive literature search was performed to identify pediatric patients who underwent surgical resection for spinal cord ependymomas. Only manuscripts with clearly defined age, tumor grade, extent of resection, and clinical follow-up were included. RESULTS A total of 80 patients were identified with a histologic distribution as follows: 36 % myxopapillary (grade I), 54 % classical (grade II), and 10 % anaplastic (grade III). There was no association between tumor grade and PFS. The only factor associated with improved PFS was gross total resection (GTR), which remained significant in a multivariate model (hazard ratio (HR) = 0.248, p = 0.022). Moreover, older age (HR = 0.818, p = 0.026), GTR (HR = 0.042, p = 0.013), and anaplastic grade (HR = 19.847, p = 0.008) demonstrated a significant association with OS in a multivariate model. CONCLUSIONS Among pediatric patients with spinal cord ependymomas, PFS did not differ across histologic grades but was prolonged among patients who underwent GTR. Age, extent of resection, and tumor grade were all significantly associated with survival.
Collapse
|
|
14
|
Iqbal MS, Lewis J. An overview of the management of adult ependymomas with emphasis on relapsed disease. Clin Oncol (R Coll Radiol) 2013; 25:726-33. [PMID: 23972764 DOI: 10.1016/j.clon.2013.07.009] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/22/2012] [Revised: 04/08/2013] [Accepted: 06/04/2013] [Indexed: 11/16/2022]
Abstract
Ependymomas are rare neoplasms of the central nervous system. Disease-free survival after relapse is poor and approaches to treatment in recurrent disease often palliative. This overview summarises the management of primary disease for which broad consensus exists. We also extensively review treatment options in relapsed disease for which approaches to treatment are varied due to the paucity of literature evidence. Incorporated in this overview is a survey of UK neuro-oncology units to form a snapshot of current UK practise with respect to preferred systemic therapy regimens for patients with recurrent ependymoma. The outcome reflects a preference for mainly oral-based regimens. Universal guidance is lacking in the management of non-operable irradiated recurrent ependymoma and there are worthy therapeutic avenues for further investigation, in particular the role of radical re-irradiation and also the potential of bevacizumab in advanced disease. It is hoped that advances can be achieved by multicentre collaboration in future studies to overcome the difficulties posed by achieving meaningful data in such a rare tumour with extensive natural history.
Collapse
|
|
15
|
Fujimori T, Iwasaki M, Nagamoto Y, Kashii M, Sakaura H, Yoshikawa H. Extraneural metastasis of ependymoma in the cauda equina. Global Spine J 2013; 3:33-40. [PMID: 24436849 PMCID: PMC3854601 DOI: 10.1055/s-0032-1329888] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/02/2012] [Accepted: 05/07/2012] [Indexed: 10/28/2022] Open
Abstract
We report a rare case of extraneural metastasis of spinal ependymoma that developed over a very long period. A 28-year-old man underwent surgery for ependymoma in the cauda equina. After he experienced three local recurrences and had undergone five operations, a lung metastasis occurred 46 years after the first operation. Although the tumor did not show the typical morphological malignancy, the maximum standard uptake value obtained by positron-emission tomography and the antigen Ki-67 labeling index demonstrated the tumor's active proliferative ability. One year after the last operation, the patient died of respiratory failure due to progressing lung metastases. The malignant behavior of spinal ependymomas is demonstrated in a case with long-term follow-up.
Collapse
Affiliation(s)
- Takahito Fujimori
- Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan,Address for correspondence Takahito Fujimori, M.D. Department of Orthopedic Surgery, Osaka University Graduate School of Medicine2-2 Yamadaoka, Suita, Osaka 565-0871Japan
| | - Motoki Iwasaki
- Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
| | - Yukitaka Nagamoto
- Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
| | - Masafumi Kashii
- Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
| | - Hironobu Sakaura
- Department of Orthopedic Surgery, Kansai Rosai Hospital, Hyogo, Japan
| | - Hideki Yoshikawa
- Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
| |
Collapse
|
|
16
|
Pérez-Bovet J, Rimbau-Muñoz J, Martín-Ferrer S. Anaplastic ependymoma with holocordal and intracranial meningeal carcinomatosis and holospinal bone metastases. Neurosurgery 2013; 72:E497-503; discussion E503-4. [PMID: 23422903 DOI: 10.1227/neu.0b013e31827d102e] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
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.
Collapse
Affiliation(s)
- Jordi Pérez-Bovet
- Neurosurgery Department, University Hospital Dr. Josep Trueta, Girona (Girona), Spain.
| | | | | |
Collapse
|
|
17
|
Gepp RDA, Couto JMC, Silva MDD, Quiroga MRS. Mortality is higher in patients with leptomeningeal metastasis in spinal cord tumors. ARQUIVOS DE NEURO-PSIQUIATRIA 2013; 71:40-5. [PMID: 23288021 DOI: 10.1590/s0004-282x2012005000019] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/03/2011] [Accepted: 07/30/2012] [Indexed: 11/22/2022]
Abstract
UNLABELLED Spinal cord tumors are a rare neoplasm of the central nervous system (CNS). The occurrence of metastases is related to poor prognosis. The authors analyzed one series of metastasis cases and their associated mortality. METHODS Clinical characteristics were studied in six patients with intramedullary tumors with metastases in a series of 71 surgical cases. RESULTS Five patients had ependymomas of which two were WHO grade III. The patient with astrocytoma had a grade II histopathological classification. Two patients required shunts for hydrocephalus. The survival curve showed a higher mortality than the general group of patients with no metastases in the CNS (p<0.0001). CONCLUSION Mortality is elevated in patients with metastasis and greater than in patients with only primary lesions. The ependymomas, regardless of their degree of anaplasia, are more likely to cause metastasis than spinal cord astrocytomas.
Collapse
|
|
18
|
Chakraborti S, Govindan A, Alapatt JP, Radhakrishnan M, Santosh V. Primary myxopapillary ependymoma of the fourth ventricle with cartilaginous metaplasia: a case report and review of the literature. Brain Tumor Pathol 2011; 29:25-30. [PMID: 21837503 DOI: 10.1007/s10014-011-0059-8] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2011] [Accepted: 07/10/2011] [Indexed: 02/08/2023]
Abstract
Myxopapillary ependymoma (MPE), which is a benign histological subtype of ependymoma, is found predominantly in the cauda equina region. It occurs rarely in the brain and mostly as a metastatic deposit from a spinal lesion. The occurrence of primary intracranial MPE is exceptional, with only 11 cases reported to date. We report an additional case of intracranial MPE, which is the third reported case in the fourth ventricle. The tumor manifested in a 50-year-old lady, who presented with features of raised intracranial pressure. A gross total resection of the tumor was achieved. Histologically, the tumor had characteristic features of MPE with focal metaplastic cartilaginous deposit. On further evaluation, there was no evidence of a primary tumor in the spinal cord. Intracranial MPE needs further evaluation by craniospinal MRI to exclude an unrecognized primary in the spinal region, which could warrant surgical attention.
Collapse
Affiliation(s)
- Shrijeet Chakraborti
- Department of Neuropathology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore, Karnataka, 560 029, India.
| | | | | | | | | |
Collapse
|
|
19
|
Harieaswer S, Sinha R, Elabassy M. Ovarian ependymoma: Appearance on magnetic resonance imaging. J OBSTET GYNAECOL 2009; 26:710-3. [PMID: 17071458 DOI: 10.1080/01443610600940372] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
Affiliation(s)
- S Harieaswer
- Department of Radiology, Leicester Royal Infirmary, Leicester, UK
| | | | | |
Collapse
|
|
20
|
Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurg Rev 2009; 32:321-34; discussion 334. [PMID: 19221818 DOI: 10.1007/s10143-009-0190-8] [Citation(s) in RCA: 55] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2007] [Revised: 09/30/2008] [Accepted: 01/18/2009] [Indexed: 12/12/2022]
Abstract
Myxopapillary ependymomas, a specific tumor variant of spinal cord ependymomas, occur most commonly in the lumbosacral region. During the study period, 1,013 patients underwent surgery for spinal cord tumors. Fifty-two of the patients had a myxopapillary ependymoma. Forty-eight of these patients underwent surgery at our institutions. There were four patients who came for consultations only. Fourteen pediatric patients were diagnosed with myxopapillary ependymoma. The overall average age at which a patient was diagnosed was 31.8 years. The average age a child was diagnosed was 12.6 years. The adult mean age was 38.7 years. The clinical presentation was of a slow, indolent course, with average symptom duration of 20.8 months. Overall, the pediatric patients had a much more aggressive clinical course with a much higher rate of local recurrence and dissemination of the tumor within the neural axis (64% versus 32%). The median time to disease recurrence was 88 months for the entire group. The overall survival after 11.5 years of follow-up was 94%. The optimal management of patients harboring myxopapillary ependymomas remains somewhat controversial. Excellent outcomes may be obtained, however, with the use of aggressive surgical techniques. No clear benefit for adjunctive chemotherapy, and radiation therapy was demonstrated.
Collapse
|
|
21
|
Bagley CA, Kothbauer KF, Wilson S, Bookland MJ, Epstein FJ, Jallo GI. Resection of myxopapillary ependymomas in children. J Neurosurg 2007; 106:261-7. [PMID: 17465358 DOI: 10.3171/ped.2007.106.4.261] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
OBJECT Currently, the optimal treatment of children harboring myxopapillary ependymomas of the spinal cord remains somewhat debatable. The authors present a retrospective study in which they evaluated the records of patients in whom resection of these lesions had been performed. METHODS Fourteen pediatric patients who had undergone resection of a spinal cord tumor between September 1982 and July 2004 were identified from the database as having histologically classified myxopapillary ependymomas. There were 10 boys and four girls ranging in age from 7 to 18 years (mean age 12.6 years); 71% of the patients were boys. The clinical presentation of the tumor's course was slow and indolent, and the patients had a mean symptom duration of 19.6 months. Twelve patients, who underwent a total of 16 operations, were available for long-term follow-up review. Thirteen gross-total resections and three subtotal resections were performed. There were no deaths due to surgery. Postoperatively, patients initially remained at their preoperative level of function or improved. Patients who had undergone previous surgery and radiotherapy were treated more conservatively than patients who were undergoing surgery for the first time. Four children experienced significant complications following treatment. CONCLUSIONS As the authors demonstrate in this study, excellent outcomes may be obtained with the use of aggressive surgical techniques with the goal being that of gross-total resection. Despite the best of resections, however, the risk of recurrence remains. Therefore, periodic neuroimaging surveillance of the neuraxis and close clinical follow up are warranted throughout the patient's life. The role for adjunctive chemo- and radiotherapy remains to be defined in the management of myxopapillary ependymomas.
Collapse
Affiliation(s)
- Carlos A Bagley
- Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21287-7713, USA
| | | | | | | | | | | |
Collapse
|
|
22
|
Plans G, Brell M, Cabiol J, Villà S, Torres A, Acebes JJ. Intracranial retrograde dissemination in filum terminale myxopapillary ependymomas. Acta Neurochir (Wien) 2006; 148:343-6; discussion 346. [PMID: 16362177 DOI: 10.1007/s00701-005-0693-1] [Citation(s) in RCA: 41] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2005] [Accepted: 10/18/2005] [Indexed: 12/26/2022]
Abstract
Myxopapillary ependymomas (ME) are considered benign tumours (WHO grade I) of the central nervous system with long term survival rates and a tendency to local recurrence. However an aggressive course has occasionally been described, leading to CSF dissemination and even systemic metastases. We describe the case of a 23-year-old man diagnosed with intracranial subarachnoid dissemination of a filum terminale ME three years after the initial diagnosis. We have performed a careful review of the literature on CSF dissemination in ME and finally propose treatment of these cases.
Collapse
Affiliation(s)
- G Plans
- Department of Neurosurgery, University of Barcelona, Hospital Universitari de Bellvitge, Spain.
| | | | | | | | | | | |
Collapse
|
|
23
|
Tzerakis N, Georgakoulias N, Kontogeorgos G, Mitsos A, Jenkins A, Orphanidis G. Intraparenchymal myxopapillary ependymoma: case report. Neurosurgery 2004; 55:981. [PMID: 15934181 DOI: 10.1227/01.neu.0000137278.84588.06] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
OBJECTIVE AND IMPORTANCE Myxopapillary ependymoma is a histological variant of ependymoma found in the cauda equina region. The most characteristic histological feature of myxopapillary tumors is the abundance of intercellular and perivascular mucin and the arborizing vasculature, which tends to form papillae. Primary intracerebral myxopapillary ependymomas are extremely rare; only three cases have been reported in the previous literature. CLINICAL PRESENTATION A 68-year-old man presented with disorientation and dizziness caused by a cystic left frontal intraparenchymal lesion. This proved to be a myxopapillary ependymoma. Similarities to previously reported cases are discussed, as are the findings on magnetic resonance imaging. There is also a literature review of the histological findings, natural history, and outcome of surgically treated myxopapillary ependymoma. INTERVENTION The lesion was totally removed. After surgery, the patient was neurologically intact and had an uneventful recovery. CONCLUSION This is the fourth reported case of histologically proven primary myxopapillary intracranial ependymoma.
Collapse
Affiliation(s)
- Nikolaos Tzerakis
- Department of Neurosurgery, G. Gennimatas General Hospital, Athens, Greece.
| | | | | | | | | | | |
Collapse
|
|
24
|
Awaya H, Kaneko M, Amatya VJ, Takeshima Y, Oka S, Inai K. Myxopapillary ependymoma with anaplastic features. Pathol Int 2004; 53:700-3. [PMID: 14516321 DOI: 10.1046/j.1440-1827.2003.01546.x] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
A case of myxopapillary ependymoma with anaplastic features in 15-year-old boy is reported. The tumor was located in the intradural space extending to the 12th thoracic to 2nd lumbar vertebral level. It was excised with the accompanying spinal arch of the T12 to L2 vertebra. At operation, the tumor was not attached to the surrounding soft and bony tissues. The tumor, measuring 49 x 19 x 15 mm, was brownish-yellow in color and involved the conus medullaris and filum terminale. Histologically, the tumor was composed of biphasic features of a hypercellular papillary growth area and a hypocellular myxoid area. In the papillary growth area, ependymal rosettes and perivascular pseudorosettes were observed. These findings were consistent with those of a myxopapillary ependymoma, although multiple foci of punctate necrosis within the tumor and proliferation of endothelial cells showing glomeruloid structures were observed. Many mitotic figures were also observed. In addition, the Ki-67 labeling index of tumor cells was 10.1%. These findings are unusual for myxopapillary ependymoma, and therefore, it appeared that the diagnosis of myxopapillary ependymoma with anaplastic features was appropriate.
Collapse
Affiliation(s)
- Hirokazu Awaya
- Department of Pathology, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan.
| | | | | | | | | | | |
Collapse
|
|
25
|
Cappabianca S, Barberi A, Grassi R, Lieto E, Fulciniti F, Galizia G. Myxopapillary ependymoma of the ischioanal fossa. Br J Radiol 2003; 76:659-661. [PMID: 14500283 DOI: 10.1259/bjr/12708512] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Ependymomas outside the confines of the cranium and spinal cord are rare. Direct extension into the soft tissues of the sacrococcygeal area may occur from a primary ependymoma of the spinal cord, cauda equina or filum terminale. Alternatively they may occur as a primary pre-sacral, pelvic and abdominal tumour, or as a primary tumour of the skin and subcutaneous tissue of the sacrococcygeal area without any demonstrable connection with the spinal cord. The Authors report a case of myxopapillary ependymoma of the ischioanal fossa, demonstrated by MRI. To our knowledge, our case is the first lesion reported at this site.
Collapse
Affiliation(s)
- S Cappabianca
- Sezione Scientifica di Diagnostica per immagini, Radioterapia e Medicina Nucleare, Italy
| | | | | | | | | | | |
Collapse
|
|
26
|
Kulesza P, Tihan T, Ali SZ. Myxopapillary ependymoma: cytomorphologic characteristics and differential diagnosis. Diagn Cytopathol 2002; 26:247-50. [PMID: 11933271 DOI: 10.1002/dc.10086] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
Abstract
Myxopapillary ependymoma (ME) is a rare tumor with a predilection for sacrococcygeal area of adults. We present the cytomorphology of one such case in a 21-yr-old man, diagnosed by fine-needle aspiration (FNA). The tumor disclosed biphasic morphology with nests and aggregates of epithelioid malignant cells as well as branching cords of myxohyaline material. Most noticeable was the presence of distinct hyaline globules surrounded by neoplastic cells. The differential diagnosis of ME includes chordoma, adenoid cystic carcinoma, mucinous adenocarcinoma, and germ cell tumor. An accurate preoperative diagnosis of ME on FNA can be achieved based on its distinctive cytomorphologic features.
Collapse
Affiliation(s)
- Peter Kulesza
- The John K. Frost Cytopathology Laboratory, Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA
| | | | | |
Collapse
|
|
27
|
Abstract
This paper reports a case of the rare entity of an extraneural metastasizing ependymoma of the spinal cord. The tumor which arose in the conus medullaris and in the cauda equina was first diagnosed in 1956 when a thoracolumbar myeloresection was performed. At autopsy, 40 years after the primary diagnosis, a massive local tumor recurrence with extraneural metastases in the lungs, the pleura, the liver, and the thoracal and abdominal lymph nodes were found. Immunohistochemical stains of the extraneural metastases showed a strong cytoplasmatic expression of glial fibrillary acidic protein (GFAP). Neither the primary tumor nor its metastases showed any of the conventional morphological criteria of malignancy. Reviewing the literature we discuss the possible mechanism of extraneural tumor spread and the incidence of metastases with regard to the tumor type.
Collapse
Affiliation(s)
- M Graf
- University of Heidelberg, Institute of Pathology, Heidelberg, Germany.
| | | | | |
Collapse
|
|
28
|
Piquer J, Riesgo P, Navarro J, Cabanes J, Orozco M, Alvarez Garijo J. Metástasis cerebrales del ependimoma medular: A propósito de tres casos. Neurocirugia (Astur) 1996. [DOI: 10.1016/s1130-1473(96)70746-7] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
|
|
29
|
Abstract
Ependymomas arising from peripheral or spinal nerves are extremely rare. Other ectopic ependymomas are found mostly in the sacrococcygeal region, possibly in association with developmental anomalies. The case of an ependymoma arising from a cranial nerve in a 40-year-old man is described.
Collapse
Affiliation(s)
- N S Little
- Department of Neurosurgery, Royal North Shore Hospital, St. Leonards, New South Wales, Australia
| | | | | |
Collapse
|
|
30
|
Bardales RH, Porter MC, Sawyer JR, Mrak RE, Stanley MW. Metastatic myxopapillary ependymoma: report of a case with fine-needle aspiration findings. Diagn Cytopathol 1994; 10:47-53. [PMID: 8005043 DOI: 10.1002/dc.2840100113] [Citation(s) in RCA: 23] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/28/2023]
Abstract
A case of recurrent and metastasizing myxopapillary ependymoma of the sacral region in a 35-yr-old man is reported. Fifteen years after the original diagnosis, he presented with an abdominal mass, subcutaneous nodules in the previous surgical excision line, and bilateral inguinal lymph node enlargement. FNA cytology of the inguinal lymph nodes showed a poorly cohesive, highly cellular smear pattern exhibiting papillary formations and rosette-like structures composed of slim columnar cells having cytoplasmic processes without evidence of atypia. Histologic and ultrastructural findings confirmed the diagnosis. Chromosomal analysis was also done.
Collapse
Affiliation(s)
- R H Bardales
- Department of Pathology, University of Arkansas for Medical Sciences, Little Rock 72205
| | | | | | | | | |
Collapse
|
|
31
|
Abstract
BACKGROUND Although myxopapillary ependymomas are generally benign with a tendency for slow growth and local recurrence, they are capable of spread within the nervous system and of extraneural metastasis. Histologic features have not been helpful in determining which patients are at risk for recurrence or dissemination, making management decisions difficult. METHODS A retrospective review was conducted of 14 cases of myxopapillary ependymoma. The nucleolar organizing region (NOR) staining method was used to determine if this technique was useful in the management of these tumors. RESULTS Five patients had total resections of encapsulated lesions, four had total resections of adherent tumors, and four had subtotal resections. Twelve received postoperative radiation therapy. With a mean follow-up of 80 months, 12 patients are well and disease-free. Two patients have had recurrences after surgery and irradiation, leading to death in one and disability in the other. The mean number of NOR per cell in eight specimens ranged from 0.4-1.64. The patient who died with intracranial spread had the highest number of NOR per cell. CONCLUSIONS Based on these data and a review of the literature, it is recommended that radiation be delayed until recurrence in tumors that have been totally resected. Local radiation therapy may be indicated in subtotally resected tumors. NOR staining shows promise in predicting the likelihood of spread of tumor. Patients with myxopapillary ependymomas should be followed indefinitely because of the potential for late recurrence, even after aggressive therapy.
Collapse
Affiliation(s)
- D A Ross
- Section of Neurological Surgery, University of Michigan Medical Center, Ann Arbor 48102
| | | | | | | |
Collapse
|
|
32
|
Ponge T, Robert R, Ponge A, de Kersaint-Gilly A, Couverchel L, Cottin S. [Ependymoma and degenerative lumbar pathology]. Rev Med Interne 1988; 9:221-3. [PMID: 2971266 DOI: 10.1016/s0248-8663(88)80126-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
|
|
33
|
Abstract
The study involved 77 myxopapillary ependymomas of the spinal cord encountered during a 60-year period (1924-1983). This variant of ependymoma was, with few exceptions, limited to the lumbosacral region, particularly the filum terminale. The male:female ratio was 1.7:1, and the mean age at diagnosis was 36.4 years (range, 6-82); at presentation, 15 (19%) of the patients were in the first two decades of life. The duration of symptoms ranged from 1 month to 30 years; the most frequent complaint was low-back pain, and eight patients had undergone prior "disc surgery." Generally, myelographic block was disclosed. Preoperative cerebrospinal fluid protein levels averaged 2462 mg/dl. Myxopapillary ependymomas are slow-growing tumors that show no significant tendency to histologic dedifferentiation. Despite some variation in cytologic features and the presence of atypia and modest mitotic activity in most cases, the gross characteristics of the tumors appear to be of greater prognostic significance than the histologic features. Tumors that were encapsulated (25%) and amenable to intact, total surgical removal had a recurrence rate of 10%, whereas those that were removed either piecemeal (34%) or subtotally (41%) had recurrence rates of 19%. Overall survival, however, was more closely related to residual disease; total removal of tumor, whether intact (encapsulated) or piecemeal, resulted in longer survival (19 years) than did subtotal resection (14 years). Patients who died (6.5%) did so after a prolonged course marked by multiple recurrences. Radiotherapy may be of particular benefit to patients whose tumors are not amenable to intact total removal.
Collapse
|
|
34
|
Abstract
From a series of 66 cases of central nervous system ependymomas, three cases of well differentiated ependymoma of the cauda equina are described. These tumors recurred and spread to produce intracranial deposits. No extraneural lesions were found.
Collapse
|
|
35
|
Morris DM, Steinert HR, Wiernik PH. Ineffectiveness of chemotherapy in patients with metastatic ependymoma of the cauda equina. J Surg Oncol 1983; 22:33-6. [PMID: 6823114 DOI: 10.1002/jso.2930220109] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/22/2023]
Abstract
Six cases of intraspinal ependymoma metastasizing outside the central nervous system have been reported. This report documents a seventh case. This patient received multiple courses of combination and single-agent chemotherapy without evidence of tumor regression. All seven patients had four things in common: early onset of disease, numerous operations, massive local recurrence at the time distant metastases were noted, and a long time period from diagnosis to death.
Collapse
|
|
36
|
Yu YL, Crockard HA, Smith JJ, Harries BJ. Extraspinal ependymoma at the cervicothoracic junction. SURGICAL NEUROLOGY 1982; 17:160-2. [PMID: 7079934 DOI: 10.1016/0090-3019(82)90266-x] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/23/2023]
|
|
37
|
Abstract
Spinal ependymomas may rarely arise from heterotopic ependymal cell clusters and thus occur in an extraspinal location. Presentation of three cases and a review of the literature reveal that these tumors have characteristic radiographic and clinical features. They occur mainly in patients in the third decade of life, and present either in the soft tissue posterior to the sacrum or in the pelvis. In the case of posterior tumors, the patient exhibits a mass which is usually mistaken for a pilonidal cyst. Patients whose tumor is pelvic in location present with sphincter disturbances or dysfunction of the sacral nerve roots. Conventional and computerized tomographic studies will reveal erosion of the sacrum. Myelography will demonstrate an extradural mass indenting the thecal sac from below. The protein in the cerebrospinal fluid will be normal. A combined posterior and anterior approach with the goal of complete tumor removal is the procedure of choice. If this is not feasible, then radiation therapy should be employed. Because of the increased incidence of systemic metastases, the average postoperative survival is approximately 10 years.
Collapse
|
|
38
|
Vagaiwala MR, Robinson JS, Galicich JH, Gralla RJ, Helson L, Beattie EJ. Metastasizing extradural ependymoma of the sacrococcygeal region: case report and review of literature. Cancer 1979; 44:326-33. [PMID: 378364 DOI: 10.1002/1097-0142(197907)44:1<326::aid-cncr2820440156>3.0.co;2-4] [Citation(s) in RCA: 48] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
A case is discussed in which the patient presented with a primary extradural sacrococcygeal ependymoma and synchronous pulmonary metastasis. The clinical course has been characterized by recurrent pulmonary metastases. Management has consisted of repeated surgical resections of the pulmonary metastases and the tumor at the primary site; and the use of a wide spectrum of chemotherapeutic agents. Transplantation of this tumor into nude mice initially resulted in rapid growth but there was spontaneous regression in the second transplants. A general discussion of the management of such lesions is presented, and the literature pertaining to this tumor is discussed.
Collapse
|
|
39
|
Liwnicz BH, Rubinstein LJ. The pathways of extraneural spread in metastasizing gliomas: a report of three cases and critical review of the literature. Hum Pathol 1979; 10:453-67. [PMID: 381159 DOI: 10.1016/s0046-8177(79)80051-9] [Citation(s) in RCA: 151] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
|