bromochloroacetic-acid and Femoral-Neoplasms

Malignant transformation of fibrous dysplasia (FD) is exceedingly rare, occurring in less than 1% of all FD cases, and has been described in both monostotic and polyostotic forms of this entity. We report a case of a large proximal femur mass arising in a 45-year-old man. The biopsy revealed a high-grade pleomorphic malignancy that focally expressed multiple keratins. Based on the presence of keratin immunoreactivity, the morphologic differential diagnosis included metastatic sarcomatoid carcinoma. However, review of the clinical information revealed a history of polyostotic FD, and imaging findings were compatible with malignant transformation of FD. The resected neoplasm was biphasic and composed of areas of conventional FD admixed with a high-grade pleomorphic malignancy. Activating GNAS mutations were identified in both components. To the best of our knowledge, this is the first description of keratin expression in malignant transformation of FD.

Topics: Biomarkers, Tumor; Biopsy; Cell Transformation, Neoplastic; Chromogranins; DNA Mutational Analysis; Femoral Neoplasms; Fibrous Dysplasia, Polyostotic; GTP-Binding Protein alpha Subunits, Gs; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Mutation; Osteotomy

Cytological, biopsy and autopsy findings in a patients suffering from massively metastasizing adrenal angiosarcoma are reported. Histogenetic typing of the tumour initially manifestating itself by osseous and liver metastases was problematic with regard to its partially epithelioid structure and its positivity upon cytokeratin immunostaining. Of relevance for the correct typing was the finding that the tumour cells in addition exhibited positivity for vascular markers. This case confirms literature data according to which cytokeratin expression not infrequently may be encountered in endothelial neoplasms and which by no means should lead to exclude such a tentative diagnosis.

Topics: Adrenal Gland Neoplasms; Adrenal Glands; Adult; Antibodies, Monoclonal; Antigens; Antigens, Differentiation, Myelomonocytic; Biomarkers, Tumor; Cell Adhesion Molecules; Factor VII; Femoral Neoplasms; Femur Head; Hemangiosarcoma; Humans; Keratins; Liver; Liver Neoplasms; Male; Platelet Endothelial Cell Adhesion Molecule-1

Five cases of a previously undescribed variant of epithelioid sarcoma are presented. This variant differs from the usual lesion in its absence of the typical necrobiotic nodular epithelioid pattern. It is instead composed of deceptively bland fibrohistiocytic and myoid cells arranged in a fibroma-like or dermatofibroma-like pattern with an affinity for osseous involvement. The clinical presentation, ultrastructural features, and presence of vimentin and low molecular weight keratin within the tumor cells justifies their designation as an epithelioid sarcoma variant.

Topics: Adolescent; Adult; Bone Neoplasms; Calcaneus; Diagnosis, Differential; Female; Femoral Neoplasms; Fibroma; Fingers; Histiocytoma, Benign Fibrous; Humans; Humerus; Keratins; Male; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Sarcoma; Skin Neoplasms; Soft Tissue Neoplasms; Thigh; Tibia; Toes; Ulna; Vimentin