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Background
Chordoid glioma is a rare neoplasm occurring in the third ventricle and, as the name implies, having a chordoid appearance. It is currently considered a glial neoplasm of uncertain histogenesis with distinct clinicopathologic features.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION INCIDENCE Very rare, less than 40 cases reported AGE RANGE-MEDIAN 24-70 years Females slightly favored
PATHOGENESIS CHARACTERIZATION
Arch Pathol Lab Med. 2006 Apr;130(4):460-4. Abstract quote
CONTEXT: Chordoid glioma is a relatively recently described unique glial neoplasm that has been formally codified by the World Health Organization in Pathology and Genetics of Tumours of the Nervous System, in which it is included along with astroblastoma and gliomatosis cerebri under the rubric "Tumors of Uncertain Origin." Many examples of chordoid glioma come to clinical attention only at a relatively large size and occupy a large portion of the third ventricle. Accordingly, the anatomic origin of chordoid glioma has been unclear and debated.
OBJECTIVE: To examine the regional anatomic origin of chordoid glioma.
DATA SOURCES: The clinical, imaging, histologic, immunophenotypic, and ultrastructural data in previously published case series and individual case reports of chordoid glioma were reviewed in conjunction with the study of a new case of chordoid glioma that presented at a relatively small size, thereby facilitating neuroanatomic localization.
CONCLUSIONS: Chordoid glioma exhibits features of specialized ependymal differentiation on ultrastructural examination, and all examples reported in the literature to date have displayed a highly stereotypical suprasellar anatomic localization and an ovoid shape, as seen on neuroimaging studies and gross anatomy. Neuroanatomic, radiologic, and clinical evidence supports an anatomic origin for chordoid glioma from the vicinity of the lamina terminalis.
Chordoid glioma of the third ventricle: immunohistochemical and molecular genetic characterization of a novel tumor entity.Reifenberger G, Weber T, Weber RG, Wolter M, Brandis A, Kuchelmeister K, Pilz P, Reusche E, Lichter P, Wiestler OD.
Institut fur Neuropathologie und Hirntumor-Referenzzentrum der Deutschen Gesellschaft fur Neuropathologie und Neuroanatomie, Rheinische Friedrich-Wilhelms-Universitat, Bonn, Germany.
Brain Pathol 1999 Oct;9(4):617-26 Abstract quote Chordoid glioma of the third ventricle was recently reported as a novel tumor entity of the central nervous system with characteristic clinical and histopathological features (Brat et al., J Neuropathol Exp Neurol 57: 283-290, 1998).
Here, we report on a histopathological, immunohistochemical and molecular genetic analysis of five cases of this rare neoplasm. All tumors were immunohistochemically investigated for the expression of various differentiation antigens, the proliferation marker Ki-67, and a panel of selected proto-oncogene and tumor suppressor gene products. These studies revealed a strong expression of GFAP, vimentin, and CD34. In addition, most tumors contained small fractions of neoplastic cells immunoreactive for epithelial membrane antigen, S-100 protein, or cytokeratins. The percentage of Ki-67 positive cells was generally low (<5%). All tumors showed immunoreactivity for the epidermal growth factor receptor and schwannomin/merlin. There was no nuclear accumulation of the p53, p21 (Waf-1) and Mdm2 proteins.
To examine genomic alterations associated with the development of chordoid gliomas, we screened 4 tumors by comparative genomic hybridization (CGH) analysis. No chromosomal imbalances were detected. More focussed molecular genetic analyses revealed neither aberrations of the TP53 and CDKN2A tumor suppressor genes nor amplification of the EGFR, CDK4, and MDM2 proto-oncogenes.
Our data strongly support the hypothesis that chordoid glioma of the third ventricle constitutes a novel tumor entity characterized by distinct morphological and immunohistochemical features, as well as a lack of chromosomal and genetic alterations commonly found in other types of gliomas or in meningiomas.
HISTOPATHOLOGICAL VARIANTS CHARACTERIZATION GENERAL
- Third ventricular chordoid glioma: clinicopathological study of two cases with evidence for a poor clinical outcome despite low grade histological features.
Kurian KM, Summers DM, Statham PF, Smith C, Bell JE, Ironside JW.
Neuropathology, Department of Pathology, Western General Hospital, Edinburgh, UK.
Neuropathol Appl Neurobiol. 2005 Aug;31(4):354-61. Abstract quote
Chordoid glioma of the third ventricle is a rare glial tumour whose precise histogenesis remains uncertain.
We describe two cases that presented recently to our department and review the background literature. The neoplasm tends to occur in women and its clinical presentation is variable, resulting from acute hydrocephalus or impingement upon local structures. However, the radiological appearance is distinct, with an ovoid shape, hyperdensity and uniform contrast enhancement on computerized tomography and magnetic resonance imaging. Intraoperative smear diagnosis is difficult because of the lack of specific features, although the presence of metachromatic extracellular mucin may be useful.
The characteristic histological appearance is that of cords and clusters of cohesive, oval-to-polygonal epithelioid cells with abundant eosinophilic cytoplasm and a mucinous background. There is often a mixed chronic inflammatory infiltrate with lymphocytes and plasma cells with Russell bodies.
The main differentials for histological diagnosis include chordoid meningiomas, pilocytic astrocytomas and ependymomas. An immunohistochemical panel including antibodies to glial fibrillary acidic protein, CD 34, epithelial membrane antigen, pan cytokeratin, S100 and vimentin can be used to distinguish between these possibilities.
Ultrastructurally the tumour cells have basal lamina and microvilli, reminiscent of ependymomas. The clinical outcome in our cases was poor because of the location of the lesion and its close relation to the hypothalamus. Limited follow-up after surgery with or without radiotherapy suggests that as-full-as-possible resection favours a better outcome, although surgery in this area carries significant operative risks.
Chordoid glioma: report of a case with unusual histologic features, ultrastructural study and review of the literature.
Raizer JJ, Shetty T, Gutin PH, Obbens EA, Holodny AI, Antonescu CR, Rosenblum MK.
Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
J Neurooncol. 2003 May;63(1):39-47. Abstract quote
Chordoid gliomas are an uncommon primary brain tumor with histologic features of a chordoma and immunolabeling for glial fibrillary acid protein.
We report the 32nd case with a review of the literature. The clinical, radiographic and pathologic features of the tumor are presented with new pathologic findings adding support that this lesion may be of ependymal origin.
Treatment and long term outcome are limited but chordoid gliomas appear to be indolent lesions that may be cured with gross total resection.
Chordoid glioma of the third ventricle: a report of two new cases, with further evidence supporting an ependymal differentiation, and review of the literature.Pasquier B, Peoc'h M, Morrison AL, Gay E, Pasquier D, Grand S, Sindou M, Kopp N.
Am J Surg Pathol 2002 Oct;26(10):1330-42 Abstract quote The term chordoid glioma of the third ventricle was first used to describe a rare and slowly growing neoplasm of uncertain histogenesis, with chordoid appearance, occurring preferentially in middle-aged women. Herein we report two additional examples of this novel entity together with a literature review based on the 25 cases previously published.
Our review fully confirms the strikingly stereotyped clinical, neuroradiologic, and pathologic features of this unique tumor. The female/male ratio was 1.7:1, and the age range was 24-70 years (mean 44.9 years). In all 27 cases imaging findings were similar showing a well-defined mass (mean 2.8 cm in largest dimension), ovoid in shape, hyperdense on CT scans, with uniform and intense contrast enhancement, arising in the hypothalamic/suprasellar/third ventricular region.
Histologically, the main consistent characteristics were cords and clusters of epithelioid cells within an abundant mucinous and often vacuolated background. Mitoses were sparse or absent and anaplastic features, endothelial proliferation, and necrosis were not identified. Lymphoplasmacytic infiltrates with Russell bodies were frequent throughout the tumor and its interface with adjacent brain parenchyma. Most of the tumor cells revealed a strong and diffuse expression of vimentin and glial fibrillary acidic protein. Additionally, the vast majority of tumors showed focal coexpression of cytokeratins, CD34, S-100 protein, and epithelial membrane antigen; the MIB-1 labeling indices were uniformly low. Surprisingly for a glioma assigned WHO grade II, the 19 patients with an available but short follow-up (mean 22.5 months; range 6-68 months) experienced a rather poor outcome (three recurrences and seven deaths), probably reflecting the anatomic site of the neoplasm that precludes a complete surgical excision rather than its histologic composition.
Ultrastructural examination of 10 cases demonstrated findings in line with a glial derivation and a putative ependymal origin such as cytoplasmic intermediate filaments, microvilli, intermediate junctions or desmosomes, and focal basal lamina formation. In our case no. 1, and for the first time in this tumor, we observed sparse and abnormal cilia in an aberrant juxtanuclear location, a further argument for considering chordoid glioma as a subtype of ependymoma. However, a better understanding of the biologic behavior and histogenesis of this distinctive clinicopathologic entity needs to be investigated with a larger series.
Nevertheless, taking into account its strikingly consistent anatomic localization, its unique histopathologic and immunohistochemical profile, in conjunction with the most recent and convincing ultrastructural arguments, we suggest that chordoid glioma of the third ventricle could be better classified as chordoid ependymoma of the lamina terminalis area.
VARIANTS CHONDROID
Pediatric chordoid glioma with chondroid metaplasia.Castellano-Sanchez AA, Schemankewitz E, Mazewski C, Brat DJ.
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, 1364 Clifton Road NE, Atlanta, GA 30322, USA.
Pediatr Dev Pathol 2001 Nov-Dec;4(6):564-7 Abstract quote Chordoid gliomas are uncommon primary brain tumors that arise in the region of the third ventricle. Reports of this entity to date have been limited to adults.
We present a case of a chordoid glioma arising in the hypothalamic/third ventricle region of a 12-year-old male who presented with visual symptoms. The neoplasm consisted of cords and clusters of well-differentiated, spindled-to-rounded cells containing abundant eosinophilic cytoplasm within a prominent mucinous matrix. Unlike other chordoid gliomas, this lesion contained islands and sheets showing cartilaginous differentiation intermixed with the glial component. A graded transition between neoplastic glial and chondroid regions was evident, and cells in both regions were strongly immunoreactive for GFAP and S-100.
Cartilaginous metaplasia is infrequent in gliomas, but occurs most often in pediatric neoplasms of the midline such as this chordoid glioma. Thus, chondroid metaplasia represents an unusual histopathologic feature of chordoid glioma-in this case, presenting in a child.
PROGNOSIS AND TREATMENT CHARACTERIZATION
Third ventricular chordoid glioma: a distinct clinicopathologic entity.Brat DJ, Scheithauer BW, Staugaitis SM, Cortez SC, Brecher K, Burger PC.
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
J Neuropathol Exp Neurol 1998 Mar;57(3):283-90 Abstract quote We have encountered a series of 8 third ventricular neoplasms with a distinctive chordoid appearance that appear to represent a clinicopathologic entity.
The tumors occurred in 7 females and 1 male, ranging in age from 31 to 70 years. In all cases, imaging studies showed a large well-circumscribed third ventricular mass; a cystic component was noted in 2. The tumors consisted of cords and clusters of cohesive, oval-to-polygonal epithelioid cells with abundant eosinophilic cytoplasm, relatively uniform round-to-oval nuclei, and inconspicuous nucleoli. Mitotic activity was absent. The stroma consisted of scant, coarse fibrillar processes, as well as prominent, slightly basophilic, extracellular mucin resembling that in chordomas.
Throughout the tumor, and surrounding its well-defined borders, were infiltrates of mature lymphocytes and plasma cells. Russell bodies were prominent in the latter. Adjacent brain tissue showed reactive changes with gliosis and numerous Rosenthal fibers. Immunohistochemically, tumor cells were strongly reactive for GFAP and vimentin, but negative or only weakly staining for EMA. The MIB-1 labeling index was approximately 1%. Ultrastructural examination of 4 cases revealed focal microvilli, scattered "intermediate" junctions, and focal basal lamina formation. Neither desmosomes nor cilia were seen. Total resections were achieved in 2 cases; only subtotal removals were achieved in 6. Subsequent tumor enlargement was noted in 3 of the 6 patients with incomplete resection, and of these, two died at post-operative intervals of 8 months and 3 years. The other patient survives 4 years post-operatively with stable residual disease.
Of the 2 patients with total resection, 1 was lost to follow-up; the other, during a brief follow-up period, did well without evidence of recurrence.
TREATMENT
Third ventricular chordoid glioma: case report and review of the literature.
Nakajima M, Nakasu S, Hatsuda N, Takeichi Y, Watanabe K, Matsuda M.
Department of Neurosurgery, Shiga University of Medical Science, Shiga, Japan
Surg Neurol. 2003 May;59(5):424-8 Abstract quote.
BACKGROUND: Chordoid glioma of the third ventricle is a rare type of brain tumor that was recently characterized as a novel tumor entity. We present a case and review of the literature.
CASE REPORT: A 49-year-old woman presented with progressive headache, memory impairment and urinary incontinence. MRI showed a large well-circumscribed tumor in the third ventricle. The tumor was partially removed via a trans-lamina terminalis approach. The histologic findings indicated chordoid glioma. Residual tumor was treated by stereotactic radiosurgery and showed no regrowth at 2-year follow-up.
CONCLUSIONS: The ideal therapy is total removal of the tumor. However, according to the literature, total removal of the tumor carries a high risk because of its location, and conventional radiation therapy has little effect on the residual tumor. On the other hand, stereotactic radiosurgery appears more promising, and to date, no regrowth has been reported after gamma-knife therapy.Hum Pathol 1999;30:723–26.
J Neuropathol Exp Neurol 1999;57:283–90.
Brain Pathol 1999;9:617–62.
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