[Orbital metastasis of liposarcoma]. / Métastase orbitaire d'un liposarcome.

INTRODUCTION: Liposarcoma, the most common soft tissue sarcoma in adults, rarely involves the orbit. Primary orbital liposarcomas are extremely rare, with less than 30 cases previously reported. Metastatic orbital liposarcomas are also extremely rare, with only a few cases documented in the literature. We report a new case of an orbital metastatic liposarcoma and review the literature. OBSERVATION: A 57-year-old man with a history 5 years before of myxoid liposarcoma on the right calf, completely surgically resected, was admitted for recent proptosis of the left eye. A computed tomographic scan revealed a heterogeneously enhancing, left orbital mass measuring 30x23x20 mm. The mass adhered to the internal medial muscle without extension to the optic nerve. A biopsy was taken, which showed typical aspects of myxoid liposarcoma. The patient underwent an orbital left exenteration after first refusing any proposed treatment. CONCLUSION: Liposarcoma metastatic to the orbit is exceptional. It should be suspected in a patient with exophthalmia caused by a space-occupying lesion and a history of liposarcoma.

[Deep benign fibrous histiocytoma: a case report]. / L'histiocytofibrome bénin profond: à propos d'un cas.

Bening fibrous histiocytoma is one of the most frequent benign tumors. Most tumors are found in the skin, particularly on the limbs. Tumor size is usually small. According to the WHO, a deep location is found for less than 1% of all benign fibrous histiocytomas. Most deep tumors occur in the subcutaneous tissue. Deep locations in other organs have also been reported. We report a case and discuss the pathological and clinical aspects of this rare tumor.

[Soft tissue epithelioid hemangioendothelioma: a case report]. / Hémangio-endothéliome épithélioïde des tissus mous. A propos d'un cas.

We report a case of epithelioid hemangioendothelioma observed in soft tissue in a 35-year-old man who presented a painful mass of the right arm which progressed in size for two years. Imaging revealed a 7-cm poorly limited expansive tumor process located in the medial muscle compartment of the right arm. Surgical resection was performed. Histological and immunohistochemical examination led to the diagnosis of soft tissue epithelioid hemangioendothelioma. The tumor recurred in the right arm and the right axillary fossa with development of multiple nodules in the contralateral thoracic wall and pleural effusion. Adjuvant chemotherapy and radiotherapy were performed, but the patient died at two years.

[Extraskeletal mesenchymal chondrosarcoma: a case report and review of the literature]. / Le chondrosarcome mésenchymateux extra-squelettique. A propos d'une observation et revue de la littérature.

We report a new case of mesenchymal chondrosarcoma in a 25-Year-old woman who presented a tumefaction of the anteromedial aspect of the mid left leg which had developed over two Years. Computed tomography of the lower left limb demonstrated the presence of a soft tIssue tumor limited to the gastrocnemus medial. The 7.5 x 5 cm tumor was centered on a zone of calcification. Histological examination confirmed the diagnosis of malignant mesenchymatous proliferation with two components: a well differentiated cartilaginous component and a totally undifferentiated component. Surgical resection was performed but tumor recurrence was observed in the popliteal fossa as well as a pulmonary metastasis with a unique parenchymal nodule. Amputation of the left lower limb and metastasectomy were performed. At last follow-up, the patient presented permeation nodules on the scalp.

Primary synovial sarcoma of the heart. A clinicopathologic study of one case and review of the literature.

Primary synovial sarcomas of the heart are aggressive and extremely rare tumors. Only thirteen cases have been reported previously. We report the fourteenth case and review the previous ones. Our patient is a 45 year-old man who presented with congestive heart failure, which was related to a right atrial pedunculated tumor. The tumor was resected completely and the patient has remained disease-free after 5 years. The histopathologic examination and immunohistochemistry study confirmed the diagnosis. Thirteen cases are reported in the literature (11 men, 2 women) with a median age of 37.38 years (13 to 53 years). Most of the tumors occur in the right side (in 8 cases) particularly in the right atrium (in 5 cases). Ten patients died, seven within one year of diagnosis. However, in our case, the patient is still alive and free of disease.

[Primary bone leiomyosarcoma. Anatomo-clinical, immunohistochemical, and ultrastructural study]. / Le léiomyosarcome primitif de l'os. Une observation anatomo-clinique, immuno-histochimique et ultrastructurale.

A variety of tissues in bone can be the origin of neoplastic primitive lesions. Consequently, it can be the site of various sarcomas of tissue type, incursing leiomyosarcoma. The leiomyosarcoma of the bone is considered as primitive after exclusion either a bony extension of a soft tissue tumor of vicinity or the presence of a leiomyosarcoma elsewhere. We report a case of a primary leiomyosarcoma arising on the left femur of a 40-year-old woman. The diagnosis was confirmed by immuno-histochemistry and electron microscopic study. A bloc resection followed by a prothesis was performed. The patient underwent a fractioned postoperative radiotherapy with a total dose of 75 Gy. The postoperative course one year later revealed local recurrence and pulmonary metastases. Despite the resection of pulmonary metastases and the amputation of left lower limb, the patient had developed local recurrence on the stump of amputated limb and was on the outside of all therapeutic resource. In this report, we present the clinicopathologic, immuno-histochemical and ultrastructural profiles of these rare primary bone tumors.