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- W1552309013 abstract "A patient with a 12-year history of occipital ependymoma was found to have late pleuropulmonary metastases without recurrence of the primary tumor. The pleural metastases were diagnosed by histologic, ultrastructural features and finally by glial fibrillary acidic protein (GFAP) labeling positive reaction. This case is unique because of the long interval between occurrence of the initial tumor and the metastases, and because of the apparent quiescence of the cerebral lesion when the pleuropulmonary metastases were discovered. A patient with a 12-year history of occipital ependymoma was found to have late pleuropulmonary metastases without recurrence of the primary tumor. The pleural metastases were diagnosed by histologic, ultrastructural features and finally by glial fibrillary acidic protein (GFAP) labeling positive reaction. This case is unique because of the long interval between occurrence of the initial tumor and the metastases, and because of the apparent quiescence of the cerebral lesion when the pleuropulmonary metastases were discovered. We report herein a case of late pleuropulmonary metastases of an occipital ependymoma in a young woman. A 27 year-old woman was admitted in February 1985 for evaluation of a right pleural effusion. Twelve years before, she had undergone surgery for a left occipital ependymoma (grade 4). Six subsequent intracranial recurrences were treated by surgery and radiotherapy. Central nervous system (CNS) computed tomography (CT) scan was normal in January 1985. The patient was in no acute distress but had right pleural effusion and sequelae of right hemiparesis. A chest film disclosed right pleuritis and a regular, scalloped thickness of parietal pleura (Fig 1). Pleural fluid contained 4.0 g/dl protein, 240 cells/ml with 90 percent lymphocytes; cultures for Mycobacterium tuberculosis and for pyogens were sterile. After three unsuccessful pleural needle biopsies, a pleurectomy revealed a nodular pleura with right hemidiaphragmatic involvement and several pulmonary nodules. Light microscopic examination disclosed a tubular pattern and perivascular pseudorosettes of spreading polygonal tumoral cells. An intracytoplasmic ciliary pattern and several zonae adherens were found by electron microscopy. A positive reaction to antibody raised against GFAP was obtained by immunocytochemistry, using a labeled biotin-avidin technique, and confirmed the ependymomal origin of the pleural metastasis. A similar pattern was demonstrated on sections of the initial tumor specimen. In spite of chemotherapy (Adriamycin-Lomustine), pleuropulmonary metastases worsened but remained isolated, as demonstrated by CT scan (Fig 2). One year later, pituitary, encephalic, and brain-stem metastases developed. The patient died 18 months after diagnosis of the pleural effusion and 14 years after initial diagnosis of ependymoma. An autopsy was not performed. Ependymoma is a rare tumor of the CNS and is usually complicated by local recurrence or CNS metastases.1Pierre-Kahn A Hirsh JE Renier D Sainte-Rose C. Roux FX Les ependymomes intracraniens de l'enfant: pronostic et perspectives thérapeutiques..Arch Fr Pediatr. 1983; 40: 5-9Google Scholar However, 33 cases of extraneural metastases of ependymoma have been described since 1952.2Wolff M Santiago H Duby MM. Delayed distant metastasis from a subcutaneous sacrococcygeal ependymoma..Cancer. 1972; 30: 1046-1067Crossref PubMed Scopus (82) Google Scholar,3Duflher PK Cohen ME. Extraneural metastases in childhood brain tumors..Ann Neurol. 1981; 10: 261-265Crossref Scopus (51) Google Scholar Twenty-four cases were thoracic metastases involving pleura (n = 8), lung parenchyma (n = 23) and/or mediastinal lymphnodes (n = 15). The initial site of the ependymoma was encephalic (n = 13) or sacrococcygeal (n = 11). Our case is unique because the interval between the initial diagnosis of occipital ependymoma and the pleuro-pulmonary metastases is 12 years; in the other reported cases of metastatic encephalic ependymoma, the longest interval was eight years.2Wolff M Santiago H Duby MM. Delayed distant metastasis from a subcutaneous sacrococcygeal ependymoma..Cancer. 1972; 30: 1046-1067Crossref PubMed Scopus (82) Google Scholar However, in cauda equina primary tumors, the interval was longer probably because of their low malignancy. In all reported cases, there was coexistence of CNS recurrence and systemic metastases. Our patient had no clinical or CT scan evidence of CNS recurrence when the pleural effusion was discovered; however, CNS recurrence was found by a repeat CT scan 12 months later. Moreover, the metastasis was discovered during the life of our patient and not at autopsy, as in 22 of 24 previously reported cases. We emphasize here the immunohistoche-mical GFAP positivity to confirm pleural metastases while histologic and ultrastructural data were inconclusive.4Gullota F Schindler F Schutzler R Wseks-Seifert A. GFAP in brain tumor diagnosis: possibilities and limitations..Path Res Pract. 1985; 180: 54-60Crossref Scopus (27) Google Scholar The frequency of extracranial metastases of CNS primary tumors is estimated at 1 or 2 percent in large series. It increases to 25 percent when autopsies are performed.5Pasquier B Pasquier D N'Gollet A Panh M Couderc P Le potentiel métastatique des tumeurs primitives du Systeme ner-veux central..Rev Neurol. 1979; 135: 269-278Google Scholar Systemic metastases are found with highly malignant CNS tumors such as astrocytoma grade 4, glioblastoma, undifferentiated ependymoma, or medulloblastoma. The route of extraneural metastases of these tumors is not known. In medulloblastoma, bone and/or visceral metastases have been described. One of the authors (M.S.) has seen four such cases; one of them was discovered at the onset of radiation therapy, and the other three were found 50, 39, and 38 months after irradiation.6Haie C Schlienger M Constans JP Meder JF Reynaud A Ghenim C. Results of radiotherapy of medulloblastoma in adults..Int J Radiat Oncol Biol Phys. 1985; 11: 2051-2056Abstract Full Text PDF PubMed Scopus (41) Google Scholar Usually, the survival of patients with highly malignant brain tumors is short. Rare long-time survivors could be more exposed to systemic metastases for a number of reasons, including the treatment." @default.
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- W1552309013 title "Late Pleuropulmonary Metastases of a Cerebral Ependymoma" @default.
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