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Malik S, Samanta SS, Kolte S. Posterior Fossa Ependymoma in a Child with Extensive Chondro-Osseous Metaplasia Occurring at Cerebellopontine Angle and Masquerading as a Vestibular Schwannoma: An Exceptionally Rare Clinicopathological Manifestation with Review of Literature. J Neurol Surg Rep 2024; 85:e132-e137. [PMID: 39211009 PMCID: PMC11361780 DOI: 10.1055/a-2372-6701] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2024] [Accepted: 06/17/2024] [Indexed: 09/04/2024] Open
Abstract
Ependymoma occurring at the cerebellopontine (CP) angle is an extremely uncommon sight and poses diagnostic and management dilemmas to neurosurgeons, radiologists, and neuropathologists alike. Moreover, the presence of extensive chondro-osseous metaplastic elements in ependymomas is an exceptionally infrequent histopathological manifestation. However, due to the seldom-seen nature of this histomorphological feature, there is no definite consensus regarding its etiopathogenesis and clinical consequences, and there is an extreme scarcity of literature elucidating its clinicopathological spectrum and prognostic significance. Herein, we illustrate an intriguing clinical tale of a 7-year-old male child with posterior fossa ependymoma, central nervous system (CNS) World Health Organization (WHO) grade 3, arising at the right CP angle and masquerading as a vestibular schwannoma, which in itself is a rare presentation, and additionally, exhibiting extensive chondro-osseous metaplasia, which is a very uncommon histomorphological observation. To the best of the authors' knowledge and after a comprehensive literature search, the coexistence of these two rare observations has merely been described once in international literature. This case sheds light on and highlights the importance of keeping ependymoma as a possible differential while coming across CP angle space-occupying lesions. They should be diligently distinguished from schwannomas and other masqueraders that typically occur at this site, as they have diverse management and follow-up protocols, with varying prognostic outcomes for the patients. Moreover, this case also unravels and details the clinicopathological characteristics of a scarcely described feature of chondro-osseous metaplasia in ependymomas.
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Affiliation(s)
- Shaivy Malik
- Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
| | | | - Sachin Kolte
- Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
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Dadario NB, Pruitt R, Silverstein JW, Zlochower A, Teckie S, Harshan M, D'Amico RS. Sarcomatous Change of Cerebellopontine Angle Ependymoma Following Radiosurgery: A Case Report. Cureus 2022; 14:e20864. [PMID: 35145771 PMCID: PMC8803299 DOI: 10.7759/cureus.20864] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/01/2022] [Indexed: 11/11/2022] Open
Abstract
Sarcomatous change in ependymal tumors is rare and has been poorly described. We report on a cerebellopontine angle lesion that demonstrated rapid progression two years after radiosurgery in a 73-year-old female patient. Histopathological diagnosis at clinical progression showed an ependymoma with sarcomatous change (“ependymosarcoma”) that was believed to be due to radiation. The patient underwent a complex tumor resection without complications using an exhaustive multi-modal neuromonitoring paradigm throughout the dissection and resection of the tumor. Given the limited available data on these rare tumors, we review their presentation, imaging, and histopathology in the context of the previous literature, and also discuss the management of these lesions in the cerebellopontine angle.
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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.
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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
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Chondro-Osseous Metaplasia in Ependymoma: A Rare Histopathological Finding. Case Rep Pathol 2020; 2020:1528698. [PMID: 32455040 PMCID: PMC7232711 DOI: 10.1155/2020/1528698] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2019] [Revised: 04/19/2020] [Accepted: 04/20/2020] [Indexed: 01/15/2023] Open
Abstract
Ependymoma is a circumscribed glioma composed of uniform glial cells with bland nuclei in a fibrillary matrix. It is characterized by the presence of perivascular pseudorosettes. Unusual histopathological findings have rarely been reported in ependymomas, 0.5% of all diagnosed cases. Such unusual and exceedingly rare histological findings include osseous or chondroid metaplasia. To the best of our knowledge, only 15 cases of osseocartilaginous ependymomas have been reported in English literature. We report a 3-year-old boy who presented with ataxia, vomiting, and headache for three months. Radiological imaging revealed a posterior fossa lesion. Histopathological examination of the lesion confirmed a posterior fossa ependymoma with chondro-osseous metaplasia. The present case outlines the clinical presentation, histopathological findings, and outcome of chondro-osseous metaplasia in ependymomas. To date, the etiology of chondro-osseous metaplasia in ependymomas remains uncertain. Further research exploring such phenomenon is of paramount importance to explain how these tumors develop.
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Manole CG, Marinescu BG, Marta D, Nicolescu MI. Areas of Cartilaginous and Osseous Metaplasia After Experimental Myocardial Infarction in Rats. Anat Rec (Hoboken) 2018; 302:947-953. [DOI: 10.1002/ar.24010] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/26/2017] [Revised: 09/03/2018] [Accepted: 09/13/2018] [Indexed: 12/12/2022]
Affiliation(s)
- Catalin Gabriel Manole
- Animal Facility Department, “Victor Babeş” National Institute of Pathology 99‐101 Splaiul Independeţei, 050096 Bucharest Romania
- Histology Department, “Carol Davila” University of Medicine and Pharmacy 8 Eroilor Sanitari Blvd., 050474 Bucharest Romania
| | - Bogdan Gabriel Marinescu
- Animal Facility Department, “Victor Babeş” National Institute of Pathology 99‐101 Splaiul Independeţei, 050096 Bucharest Romania
- Faculty of Veterinary MedicineUniversity of Agronomic Sciences 59 Mărăşti Blvd., 011464 Bucharest Romania
| | - Daciana Marta
- Ultrastructural Pathology Department“Victor Babeş” National Institute of Pathology 99‐101 Splaiul Independenţei, 050096 Bucharest Romania
| | - Mihnea Ioan Nicolescu
- Histology Department, “Carol Davila” University of Medicine and Pharmacy 8 Eroilor Sanitari Blvd., 050474 Bucharest Romania
- Radiobiology Department“Victor Babeş” National Institute of Pathology 99‐101 Splaiul Independenţei, 050096 Bucharest Romania
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Cartilage differentiation in ependymoma: histogenetic considerations on a new case. Childs Nerv Syst 2014; 30:1301-5. [PMID: 24448879 DOI: 10.1007/s00381-014-2355-1] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/27/2013] [Accepted: 01/02/2014] [Indexed: 12/13/2022]
Abstract
The presence of cartilage in gliomas is a very unusual finding and has been mainly reported in ependymomas and in astrocytomas. A derivation of cartilage from neuroepithelial cells through a neuroepithelial-mesenchymal transition or directly from blood vessel-associated multipotent stromal elements has been proposed. We herein describe a further case of ependymoma with the presence of cartilage in a child affected by a tumor in the posterior fossa. In this case, only the last recurrence, characterized by focal areas of anaplasia, contained a nodule of cartilage. The immunohistochemical expression of fibronectin, tenascin-C, and CD44 was investigated, and the possible role of these molecules in the process of cartilage formation is discussed. Moreover, the literature on the subject is reviewed.
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Boukas A, Joshi A, Jenkins A, Holliman D. Extensive cartilaginous metaplasia of recurrent posterior fossa ependymoma: case report and review of the literature. Pediatr Neurosurg 2013; 49:93-8. [PMID: 24401698 DOI: 10.1159/000356931] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/07/2013] [Accepted: 10/29/2013] [Indexed: 11/19/2022]
Abstract
Cartilaginous metaplasia in ependymomas is extremely rare and only few cases have been reported in the literature. We describe a case of a 5-year-old patient with a 5th recurrence of 4th ventricle ependymoma. He was previously treated with 4 resections, chemotherapy and radiotherapy. Histopathology revealed well-differentiated chondroid tissue occupying almost the entire lesion. Near total resection was achieved for the 5th time, but the patient died 3 months later achieving a total survival of 48 months, the 3rd longest reported in literature. Multiple resections of tumour recurrence provided a new insight in this very rare tumour, as it gave us the opportunity to observe the progression of tumour aggressiveness from grade II to grade III and finally to chondroid metaplasia. Cartilaginous metaplasia in posterior fossa ependymomas is a very atypical and challenging tumour with poor overall prognosis.
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Affiliation(s)
- Alexandros Boukas
- Department of Neurosurgery, Regional Neurosciences Centre, Royal Victoria Infirmary, Newcastle Upon Tyne, UK
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Wang X, Zhang S, Ye Y, Chen Y, Liu X. Ependymoma with cartilaginous metaplasia might have more aggressive behavior: a case report and literature review. Brain Tumor Pathol 2012; 29:172-6. [PMID: 22228122 DOI: 10.1007/s10014-011-0079-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2011] [Accepted: 12/18/2011] [Indexed: 10/14/2022]
Abstract
Ependymoma with cartilaginous metaplasia with or without bone formation is exceedingly rare. Only eight cases have been reported in the literature. We report a case of ependymoma with cartilaginous and osseous metaplasia in a 5-year-old boy. Microscopically, the tumor was composed of neoplastic ependymal tissue and mature cartilage and bone. Immunohistochemically, glial fibrillary acidic protein and epithelial membrane antigen were positive for ependymoma cells but negative for cartilage and bone. Recurrence occurred after 15-month follow-up. The patient deteriorated rapidly and died after 1 month. Reviewing 8 reported cases and our latest case, we found that 3 cases of ependymoma with cartilaginous metaplasia were treated with radiotherapy. Six cases had recurrence from 6 months to 8 years and 2 cases died on the day of operation. These findings suggest that ependymoma with cartilaginous metaplasia might have more aggressive clinical behavior.
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Affiliation(s)
- Xingfu Wang
- Department of Pathology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.
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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.
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Affiliation(s)
- Shrijeet Chakraborti
- Department of Neuropathology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore, Karnataka, 560 029, India.
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Rare histological variants in ependymomas: histopathological analysis of 13 cases. Virchows Arch 2011; 459:423-9. [PMID: 21792667 DOI: 10.1007/s00428-011-1126-6] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2011] [Revised: 04/19/2011] [Accepted: 04/20/2011] [Indexed: 10/18/2022]
Abstract
Ependymomas are rare brain tumors representing about 3% of all intra-cerebral and spinal neoplasms. The WHO classification recognizes various rare histological ependymoma variants (i.e., lipidized ependymoma, giant cell ependymoma, etc.). However, a detailed analysis of a larger series of such cases is still lacking. We analyzed 13 case of ependymoma presenting unusual histological features. The data analysis of our series and its comparison to the cases published in literature did not reveal any close association between these features and the clinical parameters (such as age or localization). Moreover, some of these features can be found combined in individual tumors, suggesting that these variants may represent a spectrum of differentiation rather than true specific entities. However, awareness on these rare histological patterns in ependymomas is necessary in the differential diagnosis with other primary or secondary brain tumors.
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Vajtai I, von Gunten M, Fung C, Brekenfeld C, Kappeler A, Reinert MM. Oncocytic ependymoma: A new morphological variant of high-grade ependymal neoplasm composed of mitochondrion-rich epithelioid cells. Pathol Res Pract 2011; 207:49-54. [DOI: 10.1016/j.prp.2010.05.006] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2010] [Revised: 05/07/2010] [Accepted: 05/19/2010] [Indexed: 01/24/2023]
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13
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Ependymal tumors with sarcomatous change ("ependymosarcoma"): a clinicopathologic and molecular cytogenetic study. Am J Surg Pathol 2008; 32:699-709. [PMID: 18347506 DOI: 10.1097/pas.0b013e318158234e] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Gliosarcomas are uncommon primary tumors of the central nervous system defined as exhibiting both glial and sarcomatous components. Sarcomatous change occurring in ependymal tumors is rare. We identified 11 such examples. There were 6 female and 5 male patients (median age, 18 y; range, 2 to 63). The tumors were located in the parieto-occipital (n=2), temporal (n=1), parietal (n=1), frontal (n=1), and occipital lobes (n=1), as well as the lateral ventricles (n=2), insula (n=1), cerebellopontine angle (n=1), and fourth ventricle/cerebellopontine angle (n=1). At presentation, the sarcomatous component was noted in 6 (of 10) cases and the ependymal element was grade III in 7 and grade II in 3 tumors, respectively. The sarcomatous component consisted of a reticulin rich, glial fibrillary acidic protein-negative fibrosarcoma (n=5) or pleomorphic spindle cell sarcoma (n=3), and 2 examples with heterologous elements: osseous and cartilaginous (n=1) and osseous only (n=1). The single case involving the fourth ventricle/left cerebellopontine angle consisted of subependymoma and fibrosarcoma components in roughly equal proportions at presentation. Fluorescence in situ hybridization studies performed with probes targeting the NF2 gene and other members of the protein 4.1 gene family demonstrated similar alterations in the ependymal and sarcomatous components in the cases tested, including polysomies/polyploidy (n=3), gains of 1q (n=3), deletions of 22q (n=2) and 6q (n=1), and monosomy 18 (n=1). There was no evidence of MDM2 or CCND1 amplification in any of the cases tested. On follow-up, 5 patients expired 4 months to 18 years after initial resection and 4 to 11 months after development of the sarcomatous component (mean, 7.6 mo); 1 patient is alive at 5 years with recurrent disease, and 1 is alive without recurrence 12 years after initial gross total resection followed by radiation therapy. Although rare, ependymal neoplasms must be included among the gliomas prone to undergo sarcomatous change and we propose the term "ependymosarcoma" for these tumors.
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Central Nervous System Tumors With Ependymal Features: A Broadened Spectrum of Primarily Ependymal Differentiation? J Neuropathol Exp Neurol 2008; 67:177-88. [DOI: 10.1097/nen.0b013e31816543a6] [Citation(s) in RCA: 67] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023] Open
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Rodriguez FJ, Scheithauer BW, Fourney DR, Robinson CA. Ependymoma and intraparenchymal calcifying pseudoneoplasm of the neural axis: incidental collision or unique reactive phenomenon? Acta Neuropathol 2008; 115:363-6. [PMID: 17762936 DOI: 10.1007/s00401-007-0286-6] [Citation(s) in RCA: 40] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2007] [Revised: 08/15/2007] [Accepted: 08/15/2007] [Indexed: 11/28/2022]
Abstract
The so-called "calcifying pseudoneoplasm of the neural axis" is a rare tumefactive lesion presumed to be reactive in nature. To our knowledge, association with a true neoplasm has not been previously reported. We recently encountered the case of a 67-year-old woman who underwent resection of an incidentally discovered cerebellar cystic mass with a distinct, calcified component. Histology demonstrated a partially ossified, lobulated, chondrocalcific lesion surrounded by chronic inflammation, spindle to epithelioid cells, and occasional multinucleated giant cells-all features of calcifying pseudoneoplasm of the neural axis. A low-grade ependymoma associated with marked piloid gliosis was found contiguous to the lesion. The unique combination of an ependymoma with marked reactive gliosis and a calcifying pseudoneoplasm supports the reactive nature of the latter.
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Affiliation(s)
- Fausto J Rodriguez
- Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA.
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