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Epithelioid hemangioendothelioma (HEE) is an unusual malignant neoplasm of vasculo-endothelial origin, arising in soft tissues, lung and liver. One case of hepatic HEE is reported in a 32 year-old man wit an isolated multinodular hepatomegaly. The diagnosis was made by the histologic examination of biopsy specimens. At the time of diagnosis, there was evidence of pulmonary metastasis. However, an orthotopic liver transplantation was performed. The tumor cells were immunoreactive with factor VIII-related antigen, BNH9 and vimentin. There was no expression of cytokeratin and epithelial membrane antigen. The patient is alive and well 24 months after. The thoracic X-ray are unchanged. This case is compared with the 84 others cases of the literature. Fourteen experienced a favorable outcome after transplantation. Extra-hepatic dissemination doesn't contra-indicate the graft. But hilar vascular involvement may be correlated with worse prognosis.

Two mediastinal lymphangiomas (KLM) are described, in 36 and 41 year-old women. They presented as an incidental finding on chest radiograph. The mediastinal mass was anterior or posterior, compressing the adjacent vital structures, and cystic on computed tomography. The complete surgical removal was possible, but complicated by the size (12 x 7 x 4 cm and 10 x 7 x 5 cm), the hypervascularization and the infiltrative tendency of the lesions. The patients are well 36 and 30 months after removal. We compare these cases with 109 previously reported. They account for 1 p. 100 of the whole lymphangiomas and for 0.5 to 4.5% of all mediastinal tumours. The congenital or infantile cervico-mediastinal lymphangiomas are more frequent (10%). The general signs, symptoms, pathological findings, treatment and pathogenetic theories are discussed.

The phenotypic characteristics of 7 subependymal giant cell astrocytomas (GSECG) (6 of these being associated with tuberous sclerosis) are studied using morphological and immunohistochemical methods with antiserums against vimentine, glial fibrillary acid protein (GFA), S100 protein, and neurofilaments. The glycoproteic secretion of the tumor cells was also analyzed after exposure to Concanavalin A (CON A) by a direct fluorescent method. Our results suggest that some GSECG originate from specialized ependyma (circum-ventricular organs). They have the same location (foramen of Monro), present some common ultrastructural features (cytoplasm with lumen containing cilia), are positive with certain immunohistochemical markers (staining with S100 protein in 4 cases, with vimentin in 3 cases) and show a strong glycoproteic secretion (positive with CON A). Therefore, some GSECG might be considered hamartomas of specialized ependyma, with a reduced evolutivity potential.

We report a new case of aggressive juvenile fibromatosis (A.J.F.) in a 20-month-old girl. The lesion affected the inferior border of the left mandible and the adjacent soft tissues. The child presented with a painless mass, which had grown over a period of 2 months. Radiographs and computed tomographic scan showed a multilacunar bone defect with subcutaneous and gingival involvement. A biopsy was performed, followed by a partial hemimandibulectomy and a costal graft. The surgical specimen measured 4 X 3 X 3 cm. The patient did well 1 year after surgery. 16 cases of A.J.F. have been reported; with ours, 14 are mandibular. A.J.F. is a locally aggressive lesion, which doesn't metastasize. It occurs chiefly in childhood and adolescence from 1 1/2 to 18 years (median 6.5 year-old). Duration of symptoms prior to presentation varies from a few weeks to months. Clinically, it is a firm nodule. Radiographs are non-specific, but 9 cases have poorly defined destruction of the mandibular inferior border. Following a block resection of the tumor (13 cases), there is no recurrence. In this review, we discuss the clinico-pathologic diagnosis of this impressive tumor which is compared with other mandibular fibrous tumors in children.

6 thyroid gland and 3 cervical lymph node clear-cells carcinomas are investigated by means of indirect immunofluorescence detection of thyroglobulin. Immunofluorescence was positive only in 1 case, which was a primitive thyroid carcinoma. In 7 other cases, its negativity pointed at the metastatic nature of the lesion, from a clear cell carcinoma of kidney (in 4 cases, nephrectomy was very remote, and in 3 cases, the renal lesion was discovered after our examination of the thyroid tumour). One observation showed negative staining reaction, and was a propagation of a parathyroid carcinoma to the thyroid gland. It is concluded that thyroglobulin can be used as an immunohistochemical marker to establish whether a clear cell cervical tumour originates from thyroid gland, or is a metastatic neoplasm.

A case of intra medullary ganglioglioma is reported in a 25 year old woman, who had presented an acute poliomyelitis when she was 2 years old. Since 7 years, new neurological signs occurred with spastic paraplegia, sensitive and sphincter disturbances. Cervical and lumbar myelography showed an intra medullary tumor, extending from C2-C3 to T7-T8. Macroscopically, the lesion was well circumscribed, except on the cervico-dorsal junction where it engulfed the anterior spinal artery. Histologically, the tumor was a ganglioglioma, grade I. Two years after surgery, the patient remains paraplegic. This case is compared with the 13 other cases of the literature.

A case of lumbar epiduro-vertebral actinomycosis is related, in a 45 years old man, who was immuno depressed by an evolutive pulmonary sarcoidosis. 72 similar cases are reported in the literature. 50 of them are autopsied++. Clinically our patient looked like a Pott disease. The infection began probably in the kidney. The smear's diagnosis was actinomycosis, confirmed by the routine examination. An atypical mycobacteria, Mycobacterium xenopi was bacteriologically isolated twice in the pus. It was considered as the pathogen agent for 14 months. Actinomycosis was never identified bacteriologically. The patient responded to surgery and penicillin therapy. In view of the pathologic constatations and the therapeutic response, the final diagnostic was epiduro-vertebral actinomycosis. Mycobacterium xenopi acted probably as an "accompaning " germ in this case.