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      Low-grade oligodendroglioma of the pineal gland: a case report and review of the literature


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          Gliomas are a very rare subtype of pineal region tumours, whereas oligodendrogliomas of the pineal region are exceedingly rare, since there have been only 3 cases of anaplastic oligodedrogliomas reported this far.


          We present a case of a low-grade oligodendroglioma arising in the pineal gland of a 37 year-old woman. The patient presented with diplopia associated with a cystic pineal region mass demonstrated on MRI. Total resection was performed and histological examination showed that the cystic wall consisted of tumour cells with a central nucleus a perinuclear halo and minimal pleomorphism. Immnunohistochemical analysis showed that these cells were diffusely positive for CD57, and negative for GFAP, CD10, CD99, cytokeratins, neurofilaments and synaptophysin. FISH analysis was performed in a small number of neoplastic cells, which were not exhausted after immunohistochemistry and did not reveal deletion of 1p and 19q chromosome arms. However, the diagnosis of a low grade oligodendroglioma of the pineal gland was assigned.


          Although the spectrum of tumours arising in the pineal gland is broad, the reports of oligodendrogliomas confined to this location are exceedingly rare, and to the best of our knowledge there is no report of a low-grade oligodendroglioma. However, they should be added in the long list of tumours arising in the pineal gland.

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          Most cited references 23

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          Pathology of pineal region tumors.

           J Hirato,  Y Nakazato (2001)
          Various histological types of tumors arise in the pineal region. The most common tumors are pineal parenchymal tumors and germ cell tumors. Pineal parenchymal tumors are divided into pineocytoma, pineal parenchymal tumor with intermediate differentiation and pineoblastoma. Pineocytomas are well-differentiated tumors and retain the morphological and immunohistochemical features of pineal parenchymal cells. Lobular architectures and pineocytomatous rosettes are also typical features. In contrast, pineoblastomas are embryonal tumors resembling primitive neuroectodermal tumors (PNET). However, pineoblastomas are distinct from PNET in other sites due to their exhibiting photosensory differentiation including Flexner-Wintersteiner rosettes and fleurettes. Although pineal cysts are tumor-like lesions, and not true neoplasms, they are occasionally difficult to distinguish from pineocytoma and astrocytoma. From the therapeutic aspect, a precise differential diagnosis is critical. The pineal region is the most common site of the brain in which germ cell tumors occur. Germinoma, teratoma, embryonal carcinoma, yolk sac tumor and choriocarcinoma are encountered, and the latter three types of tumors usually constitute elements of mixed germ cell tumors. The morphological and immunohistochemical features of intracranial germ cell tumors are very similar to those of gonadal germ cell tumors, although there are some differences in germinoma. Pineal germinoma may exhibit carcinomatous differentiation. Other types of tumors are occasionally observed, including fibrillary and pilocytic astrocytoma, glioblastoma, ependymoma, melanoma, meningioma and so on. Metastatic pineal tumors are also rare. The most common site of origin for pineal metastasis is the lung.
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            Pineal parenchymal tumors: a correlation of histological features with prognosis in 66 cases.

            The WHO classification of CNS tumors divides pineal parenchymal tumors (PPT) into pineocytoma (PC), pineoblastoma (PB) and mixed pineocytoma-pineoblastoma or PPT with intermediate differentiation. The reported incidence of mixed/intermediate PPT varies and this may reflect the difficulty in classifying tumors of this type. In an attempt to overcome the problem of the classification of PPT with intermediate differentiation, we describe the relationship between histological features and patient survival in a large cooperative series of 66 PPT from 12 neurosurgical centres. All tumors were studied with both light microscopy and immunohistochemically using antibodies against glial markers or neural/neuroendocrine markers. Our series included 11 PC, 39 mixed/intermediate PPT and 16 PB. A number of mitoses greater than 6 and the presence of necrosis were associated with a poorer outcome, while positive immunostaining for neurofilaments was associated with a better survival. We propose a new prognostic grading of 4 grades, grade I for PC, grade II for PPT with fewer than 6 mitoses and positive immunolabelling for neurofilaments, grade III for PPT with either 6 or more than 6 mitoses or fewer than 6 mitoses but without immunostaining for neurofilaments and grade IV for PB.
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              Oligodendroglioma: pathology and molecular biology.

              Recently, important new information has become available concerning the histologic recognition and molecular biology of oligodendrogliomas. This information, in turn, impacts the way neurosurgeons diagnose and treat patients with these tumors. The purpose of this paper is to review the pathology and basic science of oligodendroglioma, highlighting these developments. Information for this review was obtained by a Medline search using the term "oligodendroglioma," and limiting the results to articles dealing with pathology. Chapters from standard textbooks were also used, and bibliographies were checked for additional key articles contributing to the understanding of the pathobiology of this disease. On histologic examination, oligodendrogliomas must be differentiated from tumors including the fibrillary astrocytoma, clear cell ependymoma, central neurocytoma, and dysembryoplastic neuroepithelial tumor (DNT). There is no specific immunocytochemical marker allowing for the recognition of human oligodendroglial tumor cells. A current simplified grading scheme separates these tumors into low grade (WHO grade II) and anaplastic (WHO grade III) oligodendrogliomas. New molecular and genetic markers may aid in grading oligodendrogliomas and identifying patients with a better prognosis or response to chemotherapy. Markers studied include Ki-67, PCNA, EGFr, VEGF, platelet-derived growth factor, p16, p18, p53, bcl-2, COX-1, and chromosomal deletions. The combination of allelic losses on chromosomes 1p and 19q has been statistically associated with a longer recurrence-free survival after chemotherapy. A patient with an oligodendroglioma may at times still present a diagnostic challenge for the neuropathologist. Yet making an accurate diagnosis is essential, since the clinical course and optimal therapeutic approach differs from that of other gliomas. In the near future, molecular characterization of oligodendrogliomas is expected to play an even greater clinical role.

                Author and article information

                Diagn Pathol
                Diagnostic Pathology
                BioMed Central
                17 September 2010
                : 5
                : 59
                [1 ]National and Kapodistrian University of Athens, Department of Pathology, Athens, 11527, Greece
                [2 ]Department of Neurosurgery, Athens Naval Hospital, Athens 11521, Greece
                Copyright ©2010 Levidou et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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