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Synovial sarcoma most commonly occurs in the peri-articular regions of the extremities. We report a case of primary pulmonary monophasic synovial sarcoma. This tumor is extremely rare and shows a particular immunohistochemical pattern of great help for the diagnosis. Cytogenetic study confirm the diagnosis by showing the specific t (X; 18) chromosomal translocation, characteristic of synovial sarcoma in all anatomic locations.

To evaluate irradiation effects on choroidal melanomas, histopathologic findings of 18 eyes whose primary treatment was enucleation were compared to 15 eyes enucleated after proton beam irradiation. Irradiated tumors showed more likely necrosis (p = 0.01) had balloon cells (p = 0.01), and inflammatory infiltrate (p = 0.05). In the irradiated group, the prevalence of tumor blood vessel damage was higher (p = 0.0002) and mitotic figures were fewer (p = 0.01). These findings suggest that proton beam irradiation damages tumor cells and alters the tumor's capacity for cellular reproduction. It damages blood vessels leading to tumor necrosis. It induces an inflammatory response of unknown effects. Radiosensitivity of choroidal melanomas cannot be assessed using conventional histologic methods. However, tumor necrosis, mitotic activity and rate of balloon cells can help to establish tumoral sensitivity to irradiation.

Glomus tumors are benign vascular tumors usually located in the fingertip dermis. Extracutaneous glomus tumors are extremely rare. A new case of sino-nasal glomangioma is reported and both clinical and histological features and biological behavior of glomus tumors in this exceptional site are described.

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.

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.

From 1975 to 1983, ten cases of actinomycosis have been reported. The anatomical localizations were cervicofacial (3 cases), thoracic (2 cases), abdominal (4 cases) and aneurysmal (1 case). The interest and limits of sampling and histological staining methods are discussed. In 8 cases out of 10, the surgical biopsy established the diagnosis of actinomycosis. The "actinomycosis granule" is revealed by the standard staining and characterized with the Gram stain. This latter allowed us to confirm the diagnosis of actinomycosis in 6 cases and to rectify it in 3 other cases that were botryomycosis. Culture of tissue gave the diagnosis in one case of actinomycosis non identified by histology.

We report a case of lymphangiomyomatosis (LAM) which occurred in a twenty six year old woman, presenting with protein losing enteropathy and pancreatic fibrosis due to lymphostasis. Despite having no pulmonary lesion, the patient died quickly. The proliferation of smooth muscle involved the mesenteric, peripancreatic lymphatic channels but also the submucosal lymphatic vessels of the duodenum. Only one other case of LAM presenting with protein losing enteropathy has been reported, in which there were no abnormalities in the intestinal tractus or in the pancreas.

The authors report two cases of mucoargyrophilic carcinomas of the breast, occurring in 73 and 81 year old women. Both tumors consist of a central mucoid carcinoma surrounded by solid areas with lobular or endocrinoid features. Most cells showed an abundant eosinophilic granular cytoplasm or a mucinous appearance. The histochemical reactions (Grimelius-Alcian Blue, Grimelius-Mucicarmine) and the ultrastructural study of the peripheral areas disclosed several cell types: poorly differentiated or mucinous cells were present in both cases; but most of the cells were argyrophilic and contained in their hyaloplasm numerous dense core "neurosecretory" granules; such cells revealed many microfilaments arranged in bundles or in whorls. Amphicrine cells were easily demonstrated by histochemical staining and their cytoplasm showed both endocrine granules and mucinous vesicles. These features suggest a possible relationship to other argyrophilic tumors of the breast. The histogenesis of these tumors is still little known.