A 67-year-old woman presenting with a 9-month history of polymyositis, suddenly worsened her clinical state and died. An autopsy was performed, finding a tumor measuring 8 x 6 x 1 cm, located in the left pulmonary hilum. The thyroid gland, of normal volume, was firm. The histological analysis of the tumor, the thyroid gland and several skeletal muscles led to the following diagnoses: thymic carcinoma, Hashimoto's thyroiditis and polymyositis. The aim of the discussion is this thymic carcinoma, the originality of which are firstly its ectopic location, secondly its association with immune diseases usually described during the course of thymomas rather than thymic carcinomas.

Click to increase image sizeClick to decrease image sizeKey Words: large multilobated cell lymphomaT-cell lymphomaB-cell lymphomaPinkus lymphomaelectron microscopy

Seven cases of papillary meningioma are reported. The patients, 3 females and 4 males, were aged between 21 and 69 years. Five tumors were supratentorial, 1 was located in the left temporal bone, and 1 in the thoracic spinal canal. Five patients had local recurrences and died within 1.4 to 9 years of the original operation. In Case 2, one small pulmonary metastatic nodule was found at autopsy. Microscopically, these meningiomas showed foci of necrosis, numerous mitotic figures and local invasiveness. Psammoma bodies were occasional or absent. Forty-six papillary meningiomas have been identified in the literature. Certain histologic features (necrosis, high mitotic index, rich peripapillary reticulin network) and evolutive events (high rate of local recurrence, development of distant metastases) suggest that this aggressive variant of meningioma could form a histologic link between syncytial, fibroblastic, and hemangiopericytic meningiomas.

A 55 year-old woman was admitted to hospital in January 1981 with transient expressive dysphasia. Past personal history was unremarkable except for a six-month history of renal colic and thrombophlebitis in the veins of the right leg. Computed tomographic scan of the head and carotid angiogram revealed a left calcified temporoparietal tumor. Because of pulmonary embolism it was decided to refute a cerebral biopsy. The patient also declined radiotherapy. In May 1983, a thorough workup revealed an incomplete fracture of the first lumbar vertebra and a diffuse demineralization of the rachis and pelvis. Four weeks later she developed temporal epilepsy and pulmonary embolism. A whole brain irradiation (60 Gy) was performed in August 1983. The patient's condition remained clinically stable until December 1984 when she was readmitted to hospital with a severe weight loss, diffuse osseous pain and pancytopenia. A bone marrow biopsy from the iliac crest showed a diffuse tumor involvement. Peroxidase-antiperoxidase staining using monoclonal antiserum to glial fibrillary acidic protein was strongly positive in numerous tumors cells. The pathological diagnosis was bone marrow metastasis by glioma. She died in March 1985, 4 years and 3 months after the first admission to hospital. Autopsy was not performed. A literature search reveals only 9 cases of extraneural spreading of astrocytomas and glioblastomas in the absence of previous craniotomy with post-mortem examination. The authors also comment on the clinical, pathological and histogenic aspects of extraneural metastasis of gliomas.

Two cases of pleomorphic xanthoastrocytoma (P X A) of young subjects (Kepes et al., 1979) are reported. Case 1 arose in 15-year-old boy admitted to the hospital with the complaint of severe headaches associated with nausea and vomiting of 1 month's duration. Computed tomographic scans showed a large well-defined low density area in the left temporo-parietal region of which an anterior portion was enhanced by contrast medium. Craniotomy revealed a large superficial and cystic tumor with a mural nodule. Histological and immunohistochemical features were those of a P X A confirmed by an electron microscopic study. No radiotherapy was given. The patient made a complete recovery, and 32 months later was asymptomatic. Case 2, a 17-year-old boy was admitted to the hospital in 1977. He presented with seizures that started 18 months prior to surgery. Carotid and humeral angiograms and air studies indicated the presence of a right, internal temporal mass with herniation. The craniotomy revealed a firm superficial tumor with an infratentorial, extraparenchymal extension. The histological diagnosis was giant cell glioblastoma or gliosarcoma. The patient received post-operative radiation of 5.500 rads and chemotherapy (CCNU and VM 26). He died on the 7th post-operative month. In this 2nd case, the diagnosis of P X A was made retrospectively based upon histological and immunohistochemical observations similar to case 1. We are aware of 24 P X A in the literature. In their clinical and histological features these neoplasms resemble closely each other. P X A are superficial, supratentorial astrocytomas occurring in youngs subjects (ages 3 to 32). Their typical microscopic structure include a marked cellular pleomorphism with bizarre giant cells, some mitotic figures and no necrosis. Many cells contain lipid and hyalin droplets in their cytoplasm. Characteristically, the tumoral stroma contain a very rich reticulin fiber network. Immunoperoxidase technique reveal glial fibrillary acidic protein in the tumor cells. Electron microscopic studies demonstrate abundant intracytoplasmic glial filaments. Individual cells or group of cells are surrounded by a prominent basal lamina. Some hemidesmosomes or primitive attachments are seen at the margins of the tumor cells. The biological behaviour of PXA with or without radiotherapy is relatively favorable. Long survival times (up to 25 years) are reported but in 5 cases, P X A follow a less favorable course with malignant transformation and death. Morphologic and immunohistochemical studies support the subpial astrocytic origin of P X A.

A case of cranial fasciitis in the skull of a male child is described. At the age of a few weeks was noted a "lump" beneath the scalp of the right parietal region. Physical examination was otherwise normal. Radiographs showed erosion of the skull deep to the palpable mass. At operation, a firm, 2,5 X 2 X 2 cm nodule was found, which had destroyed the underlying bone and invaded the dura matter. This nodule both extra and intracranial was excised en bloc with the surrounding bone. Histological features were those of a fibroblastic proliferation closely resembling nodular fasciitis. The child is well and symptom-free a year after treatment. The authors also include diagnostic, prognostic and histogenetic comments about cranial fasciitis of childhood, new entity described by Lauer and Enzinger in 1980.

A 48-year-old female developed a slowly progressive paraparesis due to a posterior epidural mass extended on several thoracic segments. Clinical and myelographic appearance was strongly in favour of metastasis, the most common tumors in the spinal epidural space. A laminectomy was carried out and disclosed an encapsulated epidural tumor which was loosely adherent to the dura matter. Histological features were those of an angiolipoma. A brief review of clinical and pathological characteristics of spinal lipomas is performed. Extradural spinal angiolipomas are unusual benign neoplasms. The results of surgical treatment in this location are mostly excellent.

The presence of glial fibrillary acidic protein (GFA) was tested in biopsy or autopsy specimens of 207 human central nervous system tumors. Samples were fixed, paraffin-embedded and GFA was detected using the peroxidase-antiperoxidase technique. The second part of this study dealt mainly with medulloblastomas and hemangioblastomas. Of the 17 cerebellar medulloblastomas, 15 contained less than 5% GFA positive cells. These were classified according to the Mannoji et al., 1981, criteria. Ten medulloblastomas contained type 1, 2 and 3 cells; 1 was composed of only type 1 cells, and 4 were composed of only type 3 cells. Of the 8 cerebellar or medullary hemangioblastomas, 3 contained only a few GFA positive cells which were, in two cases stroma cells and in another, astrocytes. GFA positive cells were found in 7 oligoastrocytomas, 3 gangliogliomas and 1 hamartoma of the hypothalamus. Similarly, a GFA positive astrocytic differentiation was found in a pineocytoma. In a case of tuberous sclerosis no GFA was found in the giant cells of cortical tubers. Nor was GFA observed in one intracerebral tumor of tuberous sclerosis. GFA was not found in 5 meningiomas, 4 oligodendrogliomas, 4 pituitary adenomas, 3 neurinomas of the VIII cranial nerve, 1 primary cerebellar malignant lymphoma, 1 familial lymphohistiocytosis with cerebellar involvement and 4 brain metastases. The authors also include diagnostic, histogenetic and nosological comments -about neuroglial tumors. The findings in the two parts of this study fail to establish a correlation between GFA positivity and histological grade of astrocytomas and glioblastomas.

The presence of glial fibrillary acidic protein (GFA) was tested in biopsy or autopsy specimens of 207 human central tumors. Samples were fixed, paraffin-embedded and GFA was detected using the peroxidase-antiperoxidase technic. The first part of this study dealt mainly with glioblastomas (36 cases) and astrocytomas (87 cases). Tumors were classified in three categories according to their histological grade: 24 astrocytomas grade I and II, 63 astrocytomas grade III and IV and 36 glioblastomas (grade IV). For each of these tumors GFA positive cells were counted in 5 different fields using objectives of 2,5, 10 and 25. The results were as follows: less than 50% GFA positive cells were found in 22 out of 24 low grade astrocytomas, 49 out of 63 high grade astrocytomas and 32 out of 36 glioblastomas. Conversely, over 50% GFA positivity was found in 2 benign astrocytomas, 14 malignant astrocytomas and 4 glioblastomas. In 3 high grade tumors (2 astrocytomas, 1 glioblastoma) GFA positivity was found to be over 75%. Of the 17 ependymomas, 14 were GFA positive; however, no correlation could be established between the degree of GFA positivity and histological grade. The three papillomas of the choroid plexus of the lateral ventricles found in children were GFA negative. In adults, a GFA positive focus of ependymal differentiation was found in a papilloma of the choroid plexus in the posterior cerebral fossa.