Biomedical subjects
Publications and source records attributed to D Pasquier.
A case of parameningeal cervical rhabdomyosarcoma with severe bone destruction is reported in a 3 month-old infant; symptoms were present at birth. The treatment consisted of exclusive intensive chemotherapy. The outcome was favourable with complete tumor regression and vertebral bone reconstruction. The child was on complete remission without sequellae two years later.
An attempt to create experimental endometriosis in the rabbit by the usual technique was tried on eight animals. It was disappointing. A new technique which is easier to carry out implanting endometrium was suggested. The results were evaluated in 57 animals. The technique proved to be easier and more reliable. The technique cannot be used unless the animal has been through puberty.
A 39-year-old female was admitted to the hospital because of a sudden meningeal syndrome followed by diplopia, cervical, dorsal and sciatic nerve pains, and right peripheral facial palsy. Cerebrospinal fluid obtained by lumbar puncture showed a protein level at 23 g/l. Myelography and magnetic resonance imaging (MRI) were in favor of a lumbar arachnoiditis. A meningeal biopsy revealed a tumour infiltration with foci of cells that were stained with anti-glial fibrillary acidic protein antibody. Cerebral MRI was performed to search for a central nervous system (CNS) primary tumour, and disclosed a pineal mass. Five months after the onset of the disease, the patient worsened her clinical state and died. Necropsy confirmed the presence of a pineocytoma with astrocytic differentiation and diffuse leptomeningeal spread. This exceptional occurrence leads us to discuss about primary tumours of the CNS with leptomeningeal spread.
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A 36-year-old female presented with vertigo and worsening of a right hearing loss with tinnitus. Clinical and radiologic investigations revealed a tumor of the right internal acoustic meatus, first diagnosed as a neuroma. Histologic study of surgical samples led to a final diagnosis of lipoma. Cranial nerve lipomas are rare and usually located in the cerebellopontine angle. Lipomas confined to the internal acoustic meatus are much rarer. The review of the literature however, shows that their histologic characteristics and their behavior are identical to those of cerebellopontine angle lipomas, and that a minimal surgical resection is therefore advised.
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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.
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.
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Anatomical and clinical findings in 9 cases of primary brain tumors with metastases within (7 cases) or outside of (2 cases) the nervous system are reported. A review of the published literature revealed 248 cases of histologically-confirmed central nervous system tumors with metastases outside of the nervous system. These secondary sites are seen more frequently in adult males. Their frequency, in descending order, was gliomas (39,5 p. 100), meningeal tumors and sarcomas (28,2 p. 100), neuronal tumors (14,1 p. 100), lymphoma-microgliomas (12,5 p. 100), pineal body tumors (4 p. 100), and melanomas (1,6 p. 100). Ten cases out of 248 developed through a shunt. Nineteen cases were discovered without any surgical operation. There are still many unknown factors concerning their pathogenesis. It appears, however, than in some series of patients the prolongation of the survival-rate and the late effects of surgical operation are positive factors for metastatic dissemination.
Extraneural metastases of central nervous system tumours are very rare (248 of histologically proven examples in the literature). They are mainly in adult males and in the following descending order of frequency: gliomas (39.5%), meningeal tumours and sarcomas (28.2%), neuronal tumours (14.1%), lymphomas-microgliomas (12.5%), pineal tumours (4%) and melanomas (1.6%); 10 cases out of 248 developed through a shunt. 19 were observed without any surgical operation. There are still many uncertainties about their pathogenesis. It seems however that in some tumours the increased survival time and the repetition of the craniotomy favorises the metastasis.
A case of primary intracranial melanoma in a 65-year-old female is described. A complete postmortem examination revealed multiple metastases in the liver, and eliminated the possibility of an extraneural primary melanoma. A review of the literature confirmed that such cases are highly exceptional, and that an exhaustive autopsy including particularly the eyes is indispensable to substantiate the diagnosis. When these conditions are fulfilled, there is no reason not to consider the eventuality of visceral deposits from a primary melanoma of the central nervous system.